Inflammation of the subcutaneous adipose tissue symptoms. Panniculitis: clinical manifestations, diagnosis and treatment methods

Erythema nodosum, Weber-Christian panniculitis

RCHD (Republican Center for Health Development of the Ministry of Health of the Republic of Kazakhstan)
Version: Clinical protocols MH RK - 2017

Weber-Christian recurrent panniculitis (M35.6), Erythema nodosum (L52)

Rheumatology

general information

Short description

Approved by the joint commission on the quality of medical services
Ministry of Health of the Republic of Kazakhstan
dated November 28, 2017
Protocol #33

Panniculitis (fatty granuloma)- this is a group of heterogeneous inflammatory diseases characterized by damage to the subcutaneous adipose tissue, and often occurring with involvement in the process of the musculoskeletal system and internal organs.

Erythema nodosum - septal panniculitis, occurring predominantly without vasculitis, due to a non-specific immuno-inflammatory process that develops under the influence of various factors (infections, medications, rheumatological and other diseases).

Weber-Christian idiopathic panniculitis(IPVC) is a rare and poorly understood disease that is characterized by recurrent necrotic changes in the subcutaneous adipose tissue (SAT), as well as damage to internal organs.

INTRODUCTION

ICD-10 code(s):

Date of development/revision of the protocol: 2017

Abbreviations used in the protocol:

Mon	-	panniculitis
PVC	-	Weber-Christian panniculitis
GSIP	-	Weber-Christian idiopathic panniculitis
PZhK	-	subcutaneously adipose tissue
SPn	-	septalpanniculitis
LPn	-	lobular panniculitis
UE	-	erythema nodosum
VUE	-	secondary erythema nodosum
AG	-	arterial hypertension
AT	-	antibodies
ANCA	-	autoantibodies to neutrophil cytoplasm
GC	-	glucocorticosteroids
CT	-	CT scan
KFK	-	creatinine phosphokinase
INR	-	international normalized ratio
NSAIDs	-
SW	-	systemic vasculitis
SRP-	-	C-reactive protein
ESR	-	sedimentation rate of erythrocytes
CNS	-	central nervous system
UZDG	-	ultrasound dopplerography
ultrasound	-	ultrasound procedure
FGDS	-	fibrogastroduodenoscopy
ECG	-	electrocardiogram
ECHOCG	-	echocardiography
MRI	-	Magnetic resonance imaging
INN	-

Protocol Users: doctors general practice, therapists, rheumatologists, dermatologists.

Evidence level scale:

BUT	High-quality meta-analysis, systematic review of RCTs, or large RCTs with very low probability (++) bias results that can be generalized to an appropriate population.
AT	High-quality (++) systematic review of cohort or case-control studies or High-quality (++) cohort or case-control studies with very low risk of bias or RCTs with low (+) risk of bias, the results of which can be generalized to the appropriate population .
FROM	Cohort or case-control or controlled trial without randomization with low risk of bias (+). Results that can be generalized to an appropriate population or RCTs with very low or low risk of bias (++ or +) that cannot be directly generalized to an appropriate population.
D	Description of a case series or uncontrolled study or expert opinion.
GPP	Best Clinical Practice.

Classification

Classification :
Mon depending on the etiology and pathomorphological picture.
In accordance with the predominant predominance of inflammatory changes in the connective tissue septa (septa) or fatty lobules, septal (SPN) and lobular Pn (LPn) are distinguished. Both types of PN can occur with or without signs of vasculitis, which is reflected in the clinical picture of the disease.
septal;
· Lobular;
· Undifferentiated.

erythema nodosum are classified depending on the etiological factor, according to the nature of the course of the process and the stage of the node. Forms and variants of the course of the disease are presented in Table 1.

Table 1.Thomas and variants of the course of erythema nodosum:

By the presence of an etiological factor	According to the severity, course and prescription of the inflammatory process	clinical characteristic, flow options.
Primary (idiopathic)- no underlying disease identified secondary- Identified the underlying disease	acute subacute (migratory) Chronic	acute onset and fast development bright red painful confluent nodes on the legs with swelling of the surrounding tissues. Concomitant manifestations: temperature up to 38-39°C, weakness, headache, arthralgia/arthritis The disease is usually preceded by asstreptococcal tonsillitis / pharyngitis viral infections. The nodes disappear without a trace after 3-4 weeks without ulceration. Relapses are rare. Clinical manifestations are similar to the acute course, but with a less pronounced asymmetric inflammatory component. Additionally, single small nodules may appear, including on the opposite leg. There is a peripheral growth of nodes and their resolution in the center. The illness can last up to several months. Persistent recurrent course, usually in middle-aged and elderly women, often against the background of vascular, allergic, inflammatory, infectious or neoplastic diseases. Exacerbation occurs more often in spring and autumn. The nodes are localized on the legs (on the anterior-lateral surface), the size of Walnut with moderate soreness and swelling of the legs / feet. Relapses last for months, some nodes can dissolve, others appear.

Classification of Weber-Christian panniculitis:
plaque form;
Nodal shape;
Infiltrative form;
Mesenteric form.

Plaque form. Plaque panniculitis is manifested by the formation of multiple nodes that grow together quickly enough to form large conglomerates. In severe cases, the conglomerate spreads over the entire area subcutaneous tissue affected area - shoulder, thigh, lower leg. In this case, the seal causes compression of the vascular and nerve bundles, which causes swelling. Over time, due to a violation of the outflow of lymph, lymphostasis may develop.

Nodal form. With nodular panniculitis, nodes with a diameter of 3 to 50 mm are formed. The skin over the nodes acquires a red or burgundy hue. Nodes are not prone to fusion in this variant of the development of the disease.

Infiltrative form. In this variant of the development of panniculite, the resulting conglomerates melt with the formation of fluctuations. Externally, the site of the lesion looks like a phlegmon or abscess. The difference is that when the nodes are opened, no pus is observed. The discharge from the node is a yellowish liquid of an oily consistency. After the node is opened, an ulceration forms in its place, which does not heal for a long time.

mesenteric panniculitis is a relatively rare pathology, which is characterized by chronic non-specific inflammation of the adipose tissue of the intestinal mesentery, omentum, and fatty tissue of the pre- and retroperitoneal regions. The disease is regarded as a systemic variant of idiopathic Weber-Christian panniculitis.

Diagnostics

METHODS, APPROACHES AND DIAGNOSIS PROCEDURES

Diagnostic criteria UE [1,4-8,11,20, 21,27- 29]

Complaints:
dense painful rashes red-pink color, predominantly on the lower extremities;
pain and swelling in the joints.

Anamnesis:
Acute, subacute onset
the presence of a previous infection of the pharynx, intestines;
taking medications (antibiotics, contraceptives);
hereditary predisposition;
pathology of the pancreas and liver;
foreign trips, etc.;
· vaccination;
pregnancy.

Physical examination:
During examination and palpation, the features of physical examination are determined by the stages of maturation, the expanded stage, the stage of resolution of the nodes.
NB!Maturation stage (Ist.) characterized by moderate pink painful induration without clear boundaries, develops during the first 3-7 days of the disease.
Expanded (mature) stage (IIst.) is a painful bright red-purple node with clear boundaries and pasty surrounding tissues, which lasts for 10-12 days of illness.
Permission stage (IIIst.)- Painless subcutaneous or blue-yellow-green color ("bruise" symptom) induration without clear boundaries, lasting from 7 to 14 days.
· Resolution of nodes without ulceration or scarring.

Diagnostic criteria for Weber-Christian panniculitis
Complaints:
fever 38-39°C;
dense painful rashes mainly on the trunk, buttocks, thighs, limbs;
pain and swelling in the joints;
fatigue, malaise;
· headache;
· abdominal pain;
· nausea;
diarrhea.

Anamnesis:
no underlying disease
acute onset.

Physical examination:
Palpable painful subcutaneous nodes on the trunk, buttocks, thighs and extremities;
possible opening of the node with the release of a yellow oily mass (with an infiltrative form);
post-inflammatory atrophy of the pancreas (s-m "saucers");
a tendency to relapse;
sharp pain in the epigastric region on palpation.

Laboratory research:
· UAC- normochromic anemia, thrombocytosis and neutrophilic leukocytosis and an increase in ESR;
· biochemical analysis blood(total protein and protein fractions, aspartate aminotransferase, alanine aminotransferase, amylase, lipase, trypsin, α 1-antitrypsin, cretin phosphokinase, lipid spectrum, CRP, glucose) - an increase in CRP, alpha-2 immunogloublins, amylase, lipase, trypsin;
· OAM- proteinuria, hematuria;
· Serological study(antistreplolysin-O, antibodies to Yersinia, the Herpesviridae family, etc.) - an increase in ASL "O", antibodies to Yersinia, antibodies to HSV.

Instrumental research:
· Plain radiography of the lungs - to detect infiltrates, cavities, granulomatous changes, enlarged mediastinal lymph nodes;
· Ultrasound of the abdominal organs- to detect organic damage to organs gastrointestinal tract, hepatosplenomegaly with mesenteric form;
· ECG- to detect electrophysiological damage to the heart;
· ECHOCG- to detect valvular and muscular lesions of the heart in case of suspected ARF;
· X-ray of affected joints- to detect erosive-destructive lesions of the joints;
· Computed or magnetic resonance imaging abdominal cavity to detect signs of mesenteric panniculitis and lymphadenopathy of mesenteric lymph nodes;
· Nodule biopsy: with PVK - lobular panniculitis without signs of vasculitis. Granulomatous inflammation of the subcutaneous tissue. Pronounced lymphohistiocytic infiltration, a large number of giant multinucleated cells such as a foreign body. In UE, septal panniculitis without vasculitis.

Indications for expert advice:
consultation of a phthisiatrician - for coughing, hemoptysis, weight loss, fever;
consultation of an infectious disease specialist - with diarrhea to exclude yersiniosis, articular syndrome to exclude brucellosis;
consultation of an oncologist - in case of suspected lymphoma;
consultation with a pulmonologist - if sarcoidosis is suspected;
Consultation of a surgeon - if mesenteric panniculitis is suspected.

Diagnostic algorithm:

Differential Diagnosis

Differential diagnosis and rationale for additional studies [1,4-8, 11, 20, 21]:

Diagnosis	Rationale for differential diagnosis	Surveys	Diagnosis Exclusion Criteria
Superficial migratory thrombophlebitis	Numerous linear seals on the lower, less often on the upper limbs. Ulcer formation is not observed.	USDG of vessels lower and upper limbs. Consultation with a vascular surgeon.	The presence of thrombi and occlusions along the vessels
Sarcoidosis	Recurrent nature of UE, shortness of breath, lung damage and mediastinal lymphadenopathy, articular syndrome.	Plain radiography of the respiratory organs; Biopsy of the skin muscle flap; CT scan of the lungs; Functional tests - spirography, body plethysmography
Indurative tuberculosis, or Bazin's erythema	Recurrent nature of UE, shortness of breath, hemoptysis, focal or infiltrative lung damage, fever.	Plain radiography of the respiratory organs. CTG of the lungs. Biopsy of the musculocutaneous flap. Intradermal tuberculin test.	The presence of sarcoid epithelioid cell granulomas without caseous decay in the morphological examination of the skin.
erysipelas	Erythematous asymmetric inflammation of the skin with clear hyperemic boundaries, with a roller along the periphery of the inflammatory focus. The edges of the plot are uneven, reminiscent of the outlines geographical map. Characterized by lymphangitis and regional lymphadenitis, blistering, fever.	Association with streptococcal infection, ASL "O", ASA, AGR. Infectionist consultation.	Asymmetric lesion, bright red rash, clear boundaries, confluent rash, association with streptococcal infection.
Behçet's disease	The presence of aphthous stomatitis, genital ulcers, uveitis, pseudopustular rash, venous and arterial thrombosis.	HLAB51, consultation with an ophthalmologist, gynecologist, dermatologist. FGDS	Recurrent aphthous stomatitis, ulcerative lesions of the mucous membranes of the genital organs, uveitis, ulcerative lesions of the gastrointestinal tract, arterial and venous thrombosis.
Lupus panniculitis	Knotty painful rashes on the face, shoulders (butterfly on the face, arthritis, nephrotic syndrome.	Immunological study(ANA, ENA, Antibodies to ds-DNA, ANCA, RF, cryoglobulins) APL (lupus anticoagulant, AT to cardiolipin) - positive for ANA; daily proteinuria	Positivity for antibodies to ds-DNA, ANA, lupus anticoagulant, antibodies to phospholipids, proteinuria, hematuria, polyserositis.
Crohn's disease	Diarrhea with mucus and blood, aphthous stomatitis, non-destructive arthritis.	Calprotectin, colonoscopy, consultation with a gastroenterologist.	The presence of ulcerative lesions of the gastrointestinal mucosa, increased calprotectin.

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Treatment

Drugs (active substances) used in the treatment

Treatment (ambulatory)

TACTICS OF TREATMENT AT THE OUTPATIENT LEVEL
UE patients are treated primarily on an outpatient basis. . If there is no effect at the outpatient stage within 10 days, in the case of an acute course of UE, it is necessary to refer to inpatient treatment. Effective in this disease complex therapy. The tactics of using drugs is always determined by the form of the disease and the nature of its course. Comprehensive treatment includes both non-drug and drug treatment.
The main method of therapy for UE is the elimination of the provoking factor. Drugs that can induce UE should be discontinued based on an assessment of the risk-benefit ratio and on the basis of consultation with the prescribing physician. Infections and neoplasms that may underlie the development of UE should be treated appropriately.
Drug therapy is usually symptomatic, since in most cases the pathological process resolves spontaneously. Patients should be warned about the possible activation of the process within 2-3 months. Relapses of UE develop in 33-41% of cases, the likelihood of their development increases if the trigger factor of the disease is unknown.
Treatment regimens depend on the stage of diagnosis of the underlying disease and the effectiveness of treatment.
The main drugs in the treatment of UE are antibacterial drugs in the presence of streptococcal infection, antiviral drugs in the presence of a viral load; anti-inflammatory drugs - non-steroidal anti-inflammatory drugs, glucocorticosteroids; angioprotectors, antioxidants.

Non-drug treatment:
· Mode: patients with UE are prescribed half-bed rest.
· Diet: table number 15, with a sufficient content of protein and vitamins, with the elimination of extractives.
· Changes in lifestyle: giving up bad habits, avoiding hypothermia, intercurrent infections, significant mental and physical overstrain.

Medical treatment:
Therapy regimens depend on the stage of diagnosis of the underlying disease and the effectiveness of treatment.

Stages of UE therapy .
At stage I, before examining the patient ( initial appointment sick) it is necessary to take non-steroidal anti-inflammatory drugs (NSAIDs) (to reduce inflammatory changes in the area of \u200b\u200bthe nodes):
Diclofenac sodium 150 mg per day in 2-3 oral doses for 1.5-2 months (LE-D);
or
Meloxicam 15 mg daily IM for 3 days followed by 15 mg daily orally for 2 months (LE-D).
Local therapy on the node area (with analgesic, absorbable, anti-inflammatory purpose):
applications with 33% dimethyl sulfoxide solution 2 times a day for 10-15 days
or
Clobetasol dipropionate 0.05% ointment 2 times a day on lesions for 1 month.

Stage II - the underlying disease is verified (re-admission of the patient)
Stage I treatment continues + depending on the cause of UE:
In VUE associated with β-streptococcal infection of group A of the pharynx (tonsillitis, pharyngitis) with angina or tonsillitis, antibacterial drugs are prescribed: benzathinabenzylpenicillin 2.4 million units intramuscularly once every 3 weeks for 6 months (LE - D) and whether 1000 mg twice daily for 10 days (LE-D).
VUE associated with mycoplasma or chlamydial infection:
Doxycycline 0.1 g 2 times a day for 7 days
or
· Clarithromycin 0.25 g 2 times a day for 7 days.
VUE atmixt- infections: antibacterial drugs are prescribed (see above) and/or virostatics
· aciclovir 200 mg 5 times a day for 7-10 days (LE - D).
Or
Valaciclovir 500 mg twice daily for 7–10 days (LE: D).
With VUE due to allergic exposure:
withdrawal of the offending drug, or chemical agent etc.
· Systemic antihistamines:
-Fexofenadine 180 mg per day orally for 2 weeks (LE-D)
or
-cetirizine 1 mg per day orally for 2 weeks.
VUE in rheumatic diseases, Crohn's disease, etc.:
The underlying disease is being treated.

Stage III- carried out at lack of effect from therapy of stages I and II, torpid course of UE.
In the absence of infections as the cause of UE, it is necessary to repeat the complex of examinations in order to clarify the underlying disease, followed by a consultation with a rheumatologist, pulmonologist, gastroenterologist, etc.
Systemic glucocorticoids (with anti-inflammatory purpose):
Prednisolone 5-15 mg per day orally for 1.5-2 months, then reduce by ¼ tablet once every 7 days to 10 mg per day, then ¼ tablet once every 14 days to 5 mg per day and ¼ tablet once every 21 days until canceled

Treatment of PVC has not been definitively developed and is carried out mainly empirically. Medical therapyPVC depends on the form of the disease
Knotted shape:
· NSAIDs(diclofenac sodium, lornoxicam, nimesulide, etc.) for 2-3 weeks;
glucocorticoids - prednisolone 10-15 mg / day for 3-4 weeks, then switching to a maintenance dose with gradual decline doses by 2.5 mg to maintenance 2.5-5 mg.
With single nodes, good therapeutic effect noted from the introduction of glucocorticoids by the method of chipping lesions without the development of pancreatic atrophy. At the same time, the course doses of HA are significantly lower than with oral administration.
· aminoquinoline drugs(hydroxychloroquine 400-600 mg/day);
· application therapy(creams with clobetasol, hydrocortisone, heparin).

plaque the form:
Glucocorticoidsin the middle therapeutic doses -prednisolone 20-30 mg / day. and the appointment of cytostatic drugs: cyclophosphamide, methotrexate, azathioprine.

medicinal group	International generic name	Mode of application	Level of Evidence
Antibacterial drugs	Benzathinebenzylpenicillin		UD -D
Antibacterial drugs	Amoxicillin + clavulanic acid		UD -D
Antivirals	Acyclovir		UD -D
Non-steroidal anti-inflammatory drugs	Diclofenac sodium or		UD -D
Non-steroidal anti-inflammatory drugs	Meloxicam		UD -D
	Methylprednisolone or	8-16 mg per day orally until the condition stabilizes	UD -D
	Prednisolone	10-20 mg per day orally until the condition stabilizes	UD -D
	Hydrocortisone 5%	Outwardly, 2 times a day until the nodes disappear	UD -D

medicinal group	International non-proprietary name	Mode of application	Level of Evidence
Antihistamines	Fexofenadine or		UD -D
Antihistamines	cetirizine	10 mg. 1 time per day for 10 days	UD -D
Antisecretory drugs	Omeprazole or	40 mg per day. Orally while taking NSAIDs or GCS	UD -D
Antisecretory drugs	Pantoprazole	40 mg. per day against the background of the entire intake of NSAIDs or GCS	UD -D

Surgical intervention: no.

Further management:
All patients are subject to dispensary observation:
in case of acute course of the disease within a year from preventive examination a doctor 2 times a year, with an examination to identify the cause of the disease (in case of unidentified), or monitoring factors identified as the cause of panniculitis with the appointment of antioxidants (vitamin E);
In the case of a chronic course of the disease, long-term observation with a preventive examination by a doctor 2 times a year, with an examination to identify the cause of the disease (in the case of an unknown one), or monitoring factors established as the cause of panniculitis with the appointment of antioxidants (vitamin E), as well as control treatment with correction of complications of drug therapy.

complete involution of knots;
The absence of relapses.

Treatment (hospital)

TACTICS OF TREATMENT AT THE STATIONARY LEVEL: patients who have no effect from outpatient treatment, as well as in the case of subacute and chronic course, in which issues are considered parenteral administration glucocorticosteroids.
Patients with PVK are subject to hospitalization with an infiltrative form, in the case of panniculitis with systemic manifestations (fever, signs of damage to the PVK of the retroperitoneal space).

Patient follow-up card, patient routing:

Non-drug treatment:
· Mode 2;
· Table No. 15, a diet with sufficient protein and vitamins;
In case of malnutrition: hypoallergenic diet, determination of body weight deficiency.

Diet selection:
In case of aspiration: clarify the presence of pneumonia, swallowing disorders.
Choose a position for feeding, reduce salivation (see the section on drug treatment, the introduction of botulinum toxin A into the salivary glands);
in case of gastroesophageal reflux: control pain syndrome, feeding postures, increased muscle tone. Selection of a hypoallergenic diet with dietary fiber, in the presence of esophagitis or gastritis (taking omeprazole) is possible through gastric tube or gastrostomy;
· if the treatment of gastroesophageal reflux is not effective - laparoscopic fundoplasty.

Medical treatment

Infiltrative form: Glucocorticoids at a daily dose of 1-2 mg/kg per day until the condition stabilizes along with the use of cytostatic drugs: azathioprine 1.5 mg/kg, mycophenolate mofetil (in combination with HA) - 2 g/day cyclosporine A ≤5 mg/kg/day up to the complete abolition of glucocorticoids.

List of essential medicines (having 100% probability of use):

medicinal group	International non-proprietary name	Mode of application	Level of Evidence
Antibacterial drugs	Benzathinebenzylpenicillin	2.4 million IU IM once a week for 6 months.	UD -D
Antibacterial drugs	Amoxicillin + clavulanic acid	625 mg 3 times a day for 10 days. Orally.	UD -D
Antivirals	Acyclovir	200 mg 5 times a day for 7-10 days Orally.	UD -D
Non-steroidal anti-inflammatory drugs	Diclofenac sodium or	50 mg. 1-2 times/day for 2 weeks	UD -D
Non-steroidal anti-inflammatory drugs	Meloxicam	15 mg once a day for 2 weeks	UD -D
Systemic glucocorticosteroids	Methylprednisolone or	IV 250-1000 mg once a day. 3 days then transition to oral administration of 8-16 mg per day orally throughout the stay in the hospital	UD -D
Systemic glucocorticosteroids	Prednisolone	30-180 mg 1 time per day, IV, IM 3-5 days, then switch to oral intake 10-20 mg per day orally during the entire stay in the hospital	UD -D
Glucocorticosteroids of local external action	Hydrocortisone 5% or Dexamethasone 0.025% or Clobetasol propionate 0.05% or Betamethasone valerate	Outwardly, 2 times a day during the entire stay in the hospital	UD -D
Immunosuppressive drugs	Cyclophosphamide or	200 mg powder for solution 200-600 gr 1 time for the entire stay in the hospital	UD -D
	Cyclosporin A or	inside 25 mg., 50-100 mg 1-2 times a day throughout the stay in the hospital	UD -D
	Mycophenolatamofetil	2000 mg per day orally, throughout the stay in the hospital	UD -D

List of additional medicines (havingless than 100% probability of application):

medicinal group	International non-proprietary name	Mode of application	Level of Evidence
Antihistamines	Fexofenadinili or	180 mg per day orally for 2 weeks.	UD -D
Antihistamines	cetirizine	10 mg. 1 time per day for 10 days	UD -D
Antisecretory drugs	Omeprazole or	40 mg per day. Orally. For the entire time of taking NSAIDs or GCS	UD -D
Antisecretory drugs	Pantoprazole	40 mg. per day against the background of the entire intake of NSAIDs or	UD -D

Surgical intervention: no.

Further management:
After discharge from the hospital, the patient continues to take glucocorticosteroids and cytotoxic drugs at a dose recommended by the hospital doctor with a gradual decrease in the dose of corticosteroids until clinical and laboratory stabilization of the condition (disappearance of nodes, normalization of acute phase inflammation indicators) followed by a decrease in the dose of glucocorticoids by 2.5 mg of prednisolone every 2 weeks to 10 mg, then slower dose reduction by 1.25 mg of prednisone every 2 to 2 weeks. The entire period of reducing the dose of GCS is accompanied by the intake of a cytostatic drug prescribed in the hospital. Appointment of cytostatics for a long period of 1-2 years. With continued use of corticosteroids and cytostatics, a complete blood count with platelet count, liver tests, and blood creatinine should be performed to monitor the side effects of these drugs.
Patients should visit an outpatient doctor 2 weeks after discharge from the hospital, then monthly - 3 months and once every 3 months for the entire subsequent observation period - while the patient is taking cytostatic therapy. When you stop taking cytostatics, in case of a stable improvement in the patient's condition dispensary observation(see section 3.4).

Treatment effectiveness indicators:
· achievement minimal activity and / or clinical and laboratory remission;
no complications in mesenteric panniculitis;
involution of knots;
The absence of relapses.

Hospitalization

INDICATIONS FOR HOSPITALIZATION WITH INDICATING THE TYPE OF HOSPITALIZATION

Indications for planned hospitalization:
inefficiency of outpatient therapy;
relapses of new rashes;
severe systemic manifestations (fever);
mesenteric lobular panniculitis.

Indications for emergency hospitalization: no.

Information

Sources and literature

Minutes of the meetings of the Joint Commission on the quality of medical services of the Ministry of Health of the Republic of Kazakhstan, 2017
1. 1) Blake T.., Manahan M., Rodins K. Erythema nodosum – a review of an uncommon panniculitis. Dermatol. Onine J. 2014; 20 (4): 22376. 2) Belov B.S., Egorova O.N., Radenska-Lopovok S.G. Erythema nodosum: vasculitis or panniculitis? Modern. Revmat., 2009;(3):45–49. 3) Requena L., Yus E. S. Erythemanodosum. Semin. Cutan. Med. Surg, 2007;26: 114–125. 4) Gilchrist H., Patterson J.W. Erythema nodosum and erythema induratum(nodular vasculitis): diagnosis and management. DermatolTher 2010; 23:320–327. 5) Cribier B., Caille A., Heid E., Grosshans E. Erythemanodosum and associated diseases. A study of 129 cases. IntJDermatol 1998;37:667–672. 6) Schwartz R.A., Nervi S.J. Erythema nodosum: a sign of systemic disease. Am Fam Physician 2007;75:695–700. 7) Belov B.S., Egorova O.N., Karpova Yu.A., Balabanova R.M. Erythema nodosum: modern aspects. Scientific and practical rheumatism. 2010; 4:66–72. 8) Vermel A.E. Erythema nodosum in the clinic of internal diseases. Klin.med., 2004,4,4-9. 9) Belov B.S., Egorova O.N., Karpova Yu.A. Erythema nodosum (clinical lecture). Consilium Medicum: Dermatology. 2010;1: 3–6. 10) Mert A., Ozaras R., Tabak F. et al. Erythema nodosum: an experience of 10 years. Scand J Infect Dis 2004:36:424–427. 11) Mert A., Kumbasar H., Ozaras R. et al. Erythema nodosum: an evaluation of 100 cases. ClinExpRhematol, 2007:25:563–570. 12) Balabanova R.M., Karpova Yu.A. Meloxicam: prospects for use in erythema nodosum. Modern Rheumatology 2010;(1): 41–44. 13) Belov B.S., Egorova O.N., Balabanova R.M. Idiopathic erythema nodosum and pregnancy: a case report. Modern Rheumatology 2009; (2): 56–59. 14) Kosheleva, N.M., Khuzmieva S.I., Alekberova Z.S. Systemic lupus erythematosus and pregnancy. Scientific-practical. Rheumatology, 2006,2:52-59. 15) Belov B.S., Egorova O.N., S.G. Radenska-Lopovok. Panniculitis in the practice of a rheumatologist (lecture). Scientific and practical rheumatology 2013; 51(4): 407-415. 16) Federal clinical guidelines for the management of patients with erythema nodosum. Moscow, 2016 .19 p. 17) Szyszymar B., Gwiezdzinski Z. Treatment of recurrent pwith methotrexate. PrzeglDermatol 974;61:623–7. 18) Mashkunova O.V. "Comparative evaluation of complex pathogenetic therapy of erythema nodosum in rheumatological practice". Collection of scientific papers " Actual problems modern rheumatology”, Issue No. 30, Volgograd, 2013. – P. 63-65. 19) Egorova O.N., Belov B.S., Karpova Yu.A. "Spontaneous panniculitis: modern approaches to treatment." Scientific and practical rheumatology 2012; 54 (5): 110-114 20) Mavrikakis J., Georgiadis T., Fragiadaki K., Sfikakis P. Orbitallobularpanniculitis in Weber-Christian disease: sustained response to anti-TNF treatment and review of the literature. SurvOphthalmol 2010;55(6):584–89. 21) Al-Niaimi F., Clark C., Thorrat A., Burden A.D. Idiopathic lobularpanniculitis: remission induced and maintained with infliximab.Br J Dermatol 2009;161:691–2.

Information

ORGANIZATIONAL ASPECTS OF THE PROTOCOL

List of protocol developers with qualification data:
1) Mashkunova Olga Vasilievna - Candidate of Medical Sciences, Associate Professor of the Department of Internal Diseases No. 4, rheumatologist of the Republican State Enterprise on the REM "Kazakh National medical University them. S.D. Asfendiyarov".
2) Kalieva Gulnara Aitkazievna - Candidate of Medical Sciences, Associate Professor of the Department of General Medical Practice No. 1 of the Republican State Enterprise on the REM "Kazakh National Medical University. S.D. Asfendiyarov".
3) Saipov Mamurzhan Kamilovich - rheumatologist, head of the department of rheumatology of the KGP on the REM "Regional Clinical Hospital", Shymkent.
4) Yukhnevich Ekaterina Alexandrovna - acting Associate Professor of the Department of Clinical Pharmacology and evidence-based medicine RSE on REM "Karaganda State Medical University", clinical pharmacologist.

Indication of no conflict of interest: no.

Reviewers: Gabdulina Gulzhan Khamzinichna - Candidate of Medical Sciences, Associate Professor of the Department of Outpatient Therapy of the Republican State Enterprise on the REM "Kazakh National Medical University. S.D. Asfendiyarov".

Indication of the conditions for revising the protocol: revision of the protocol 5 years after its entry into force and / or when new diagnostic / treatment methods with a higher level of evidence appear.

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Inflammation of the subcutaneous adipose tissue (SAT) is called panniculitis (translated from Latin, the ending "IT" means inflammation). At the moment, there is no single classification of panniculitis, but they are combined according to etiological indications and microscopic studies.

Types of inflammation of the subcutaneous adipose tissue

1. Panniculitis associated with changes in connective tissue partitions, directly between areas of subcutaneous adipose tissue, under the influence of an inflammatory process. This inflammation is called septal inflammation of the subcutaneous adipose tissue (from Latin septum, - septum).

2. Panniculitis associated with inflammatory changes in the subcutaneous tissue lobules. And in this case, it will be lobular panniculitis (from lat. Lobules - slice).

under the microscope

At microscopic examination in the subcutaneous adipose tissue, increasing nodes can be found, which in the future is not difficult to notice with the naked eye. Such nodes actively grow and reach sizes of 1 - 6 cm in diameter. They can be painless, and the manifestation of pain in the lesion is also possible. As a rule, such inflamed nodes in the pancreas are located mainly under the skin on different areas trunk (often symmetrical), in the mammary gland, lower leg, thigh, and the buttocks may also be affected.

Cause

The cause of this disease is a violation of metabolism, in particular, fat metabolism. In places where panniculitis appears, common signs inflammation: redness, swelling, soreness (but not always), fever, directly at the site of inflammation (local hyperthermia).

Symptoms

The inflammatory process will also affect the general condition of the patient, i.e. signs of intoxication bad feeling fever, decreased appetite, possible nausea and vomiting, muscle pain). There may be cases of the appearance in one focus of several inflammatory nodes, while the formation of adhesions between them is possible. The resolution of such nodes occurs depending on the body's immune system, as well as the ability of subcutaneous adipose tissue and skin to regenerate (replacement of damaged areas with new tissue). Quite often, over many years, there are periods of exacerbation of the disease and remission (attenuation). You can do competent dressings at home as prescribed by a doctor to maintain regeneration processes. These can be ointments with solcoseryl or others recommended by the doctor for you.

Outcome and aftermath

Outcomes of panniculitis: in the first case, healing occurs within a few weeks without the formation of skin defects, in the second case, healing can take up to a year. In the latter case, retraction of the skin in the area of inflammation and tissue atrophy can be observed. Another option for the outcome is to open the node, highlighting specific content. As a rule, processes of necrosis and ulcer formation are observed. Since not only the epidermis is damaged, but the dermis, in this case, a scar on the skin will necessarily form. The surgeon needs to carry out the correct primary surgical treatment and, if possible, apply a cosmetic suture in order to reduce the scar.

Risk of calcification

It is also impossible to exclude the possibility of postponing in inflamed nodes calcium and in such cases it will be called calcification. This disease is dangerous primarily because nodes can form in various places, including in internal organs (for example, the fatty capsule of the kidney). This can adversely affect the function of a particular organ. At laboratory methods research, in general analysis blood, there will be phenomena of an increase in the ESR index, the indices of lymphocytes and leukocytes are reduced.

Treatment is with antibiotics (antibiotics) a wide range action) and vitamin therapy, glucocorticoids, topical application of the ointment to the affected area.

In 2007, a small hard bump formed on my forearm deep under the skin. She began to grow. They operated on as an abscess, and after 2 weeks another one popped up nearby. Then all over the body. Deep wounds formed. The diagnosis is this: chronic Weber-Christian panniculitis. Where does it come from and how to live with it? I was prescribed ointments and tablets. But the wounds hurt and bleed. I sleep 2-3 hours a day.

Address: Solovieva Nadezhda Vasilievna, 141007, Moscow region, Mytishchi, 2nd Shelkovsky pr., 5, bldg. 1, apt. 140

Dear Nadezhda Vasilievna, the symptoms characteristic of panniculitis were first noted back in 1892 by Dr. Pfeifer. Then in 1925, the disease was described by Weber as recurrent panniculitis nodosum. And in 1928, Christian paid special attention to fever as characteristic symptom. Despite the fact that a lot of time has passed since then, the disease is considered rare and poorly understood. Until now, there is no single point of view on the reasons for its development. However, it is known that women aged 30 to 60 are more likely to get sick. Although panniculitis can occur at any age, even in children.

What is panniculitis? This is a limited or widespread inflammation of fatty tissue, primarily subcutaneous. Skin rashes painful, of varying density and size (1-2 cm in diameter or more). The skin over the nodes is edematous, purple-red. The rash is located symmetrically. Most often localized on the thighs, shins, less often on the arms and torso. Very rarely, the nodes are opened with the release of a yellowish-brown oily liquid. When the nodes flatten, the skin over them becomes less swollen, redness is replaced by hyperpigmentation. Sometimes atrophy of the subcutaneous adipose tissue develops, and the skin sinks. Such rashes are combined with general symptoms: fever, weakness, soreness of the rash. Deep lesions are possible, and then it threatens the life of the patient. Does this description match your symptoms, Nadezhda Vasilievna? Accurately diagnosing acute panniculitis is possible only after a histological examination. In the blood test, an increase in ESR, the level of leukocytes is noted. Also, the doctor should pay attention to the amount of lipases and amylases in the blood serum and urine. To distinguish panniculitis from pancreatic disease, antitrypsin levels should also be monitored. With panniculitis, it is lowered.

After all the tests and the establishment of the final diagnosis, it is necessary to look for the cause of the inflammation. It is believed that panniculitis is an autoimmune disease that can be triggered by various causes: trauma, hypothermia, infections, and certain medications.

Panniculitis also happens against the background systemic diseases, for example, lupus or scleroderma, but more often - hemorrhagic diathesis, lymphoma. Sometimes such an ailment develops with violations in the work of the pancreas, insufficient enzyme activity. Because of this, there is a sluggish inflammation in adipose tissue. There may also be disturbances in the immune system.

Treatment is not always effective. But if the rash is solitary, non-steroidal anti-inflammatory drugs can help. Used and hormone therapy. All medicines must be prescribed by a doctor. And treatment is possible only under his supervision. Good daily use of clean drinking water, for example from drainage pumps, helps. Some patients go into long-term remission. Unless, of course, the patient does not provoke exacerbations, does not overcool. Frequent acute respiratory viral infections, tonsillitis, as well as injuries have a bad effect on the course of the disease. Therefore, take care of yourself and follow the doctor's recommendations.

Since the disease is polyetiological, caused by different reasons then your attitude to life also matters. If you often think about how tired you are of it, then you can hardly count on improvement. When there is only hard work in life and there is no light, no joy, no treatment is effective. And you can rejoice in simple things: a flower, and a sunrise, and its sunset.

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The frequency of PN in different regions of the world varies widely and depends on the prevalence of a particular disease, which is the etiological factor of the pathology in question in this particular area.
There is currently no unified concept of the etiology and pathogenesis of PN. A certain role in the development of PN can be played by infections (viral, bacterial), trauma, hormonal and immune disorders, medication, pancreatic diseases, malignant neoplasms and etc. . At the heart of the pathogenesis of PN are violations of the processes of lipid peroxidation. At the same time, highly active intermediate oxidation products accumulate in organs and tissues. The latter inhibit the activity of a number of enzymes and disrupt the permeability of cell membranes, leading to degeneration of cell structures and cytolysis. .
The term "panniculitis" was first proposed by J. Salin in 1911. However, somewhat earlier, in 1892, V. Pfeifer first described the "syndrome focal dystrophy» PZhK with localization of nodes on the cheeks, mammary glands, upper and lower extremities, which was accompanied by progressive weakness. In 1894, M. Rotmann observed similar changes in the lower extremities and chest without damage to the internal organs. Later, similar formations of G. Hen-schen and A.I. Apricots were designated by the term "oleogranuloma". Later A.I. Abri-ko-sov developed a classification of oleogranulomas, which almost completely coincides with the Pn classifications that appeared later.
It should be noted that there is no single generally accepted classification of Mon today. A number of authors proposed to group Mon depending on the etiology and histomorphological picture. In particular, septal (SPn) and lobular (LPn) Pn are distinguished. In SPN, inflammatory changes are predominantly localized in the connective tissue septa (septa) between the fat lobules. LPN is characterized mainly by the defeat of the fat lobules themselves. Both types of PN can occur with or without vasculitis.
The main clinical sign of PN is, as a rule, multiple nodes with predominant localization on the lower and upper extremities, less often on the chest, abdomen, and hands. The nodes have different colors (from flesh to bluish-pink) and sizes (from 5-10 mm to 5-7 cm in diameter), sometimes merge with the formation of conglomerates and extensive plaques with uneven contours and a bumpy surface. Usually, the nodes resolve within a few weeks, leaving "saucer-shaped" retractions of the skin due to atrophy of the pancreas (Fig. 1), in which calcifications can be deposited. Sometimes the node is opened with the release of an oil-nis-to-foamy mass and the formation of poorly healing ulcers and atrophic scars.
These symptoms are more characteristic of LPN. With SPN, the process is usually limited (3-5 nodes).
Most posts are about skin forms Mon. Only in recent decades have there been works that describe changes in adipose tissue in the internal organs, morphologically similar to those in the pancreas. With a systemic variant of the disease, the fatty tissue of the retroperitoneal region and omentum (mesenteric panniculitis) is involved in the pathological process, hepatosplenomegaly, pancreatitis, nephropathy is detected, sometimes in the absence of skin symptoms. In some cases, the development of PN is preceded by fever (up to 41 ° C), weakness, nausea, vomiting, loss of appetite, polyarthralgia, arthritis and myalgia.
Changes in laboratory parameters in PN are nonspecific, reflecting the presence and severity of the inflammatory process. Therefore, they (with the exception of α 1 -antitrypsin, amylase and lipase) make it possible to judge only the activity of the disease, and not the nosological affiliation.
Of great importance for the verification of PN is the histomorphological picture, characterized by adipocyte necrosis, pancreatic infiltration inflammatory cells and fat-filled macrophages ("foam cells").
Success in diagnosing PN, first of all, depends on a carefully collected anamnesis with information about previous diseases, medications taken, background pathology, as well as on an adequate assessment of clinical symptoms and identification of typical morphological changes. Maybe atypical course diseases with mild skin symptoms and the absence of characteristic morphological features. In such cases, a definite diagnosis is established after several months and even years.
The classic representative of ES is erythema nodosum (UE), a nonspecific immune-inflammatory syndrome resulting from various reasons(infections, sarcoidosis, autoimmune diseases, medications, inflammatory bowel disease, pregnancy, malignant neoplasms, etc.). UE is more common in women at any age. It manifests itself as a skin lesion in the form of sharply painful soft single (up to 5) erythematous nodes with a diameter of 1-5 cm, localized on the shins, in the area of the knees and ankle joints(Fig. 2). Skin rashes may be accompanied by fever, chills, malaise, polyarthralgia and myalgia. Characteristic for UE is the color dynamics of skin lesions from pale red to yellow-green ("bruising bloom"), depending on the stage of the process. In UE, the nodules completely regress without ulceration, atrophy, or scarring.
Lipodermatosclerosis - degenerative-dystrophic changes in the pancreas that occur in middle-aged women against the background of chronic venous insufficiency. Appears as lumps on the skin lower third lower leg(s), more often in the area of the medial malleolus with subsequent induration, hyperpigmentation (Fig. 3) and atrophy of the pancreas. In the future, in the absence of treatment of venous pathology, the formation of trophic ulcers is possible.
Eosinophilic fasciitis (Schulman's syndrome) belongs to the scleroderma group of diseases. For example, in 1/3 of cases, there is a connection between its development and previous physical activity or injury. In contrast to systemic scleroderma, tissue induration begins in the forearms and/or lower legs with a possible spread to the proximal limbs and trunk. Fingers and face remain intact. Skin lesions of the "orange peel" type, flexion contractures, eosinophilia, hypergammaglobulinemia, and increased ESR are characteristic. Carpal tunnel syndrome and aplastic anemia may develop. In a histomorphological study, the most pronounced changes are found in the subcutaneous and intermuscular fascia. Pathological signs in skeletal muscles and skin are weakly expressed or absent.
Eosinophilia-myalgia syndrome (EMS) is a disease that occurs with a primary lesion of the skin and hematopoietic system, as well as internal organs. At the turn of the 1980-90s. There were more than 1600 patients with SME in the USA. As it turned out, many cases of the disease were due to the use of L-tryptophan in connection with anxiety and depression. It is more common in women (up to 80%), characterized by an acute onset with the development of fever, weakness, intense generalized myalgia, unproductive cough and severe eosinophilia (more than 1000/mm3). Acute symptoms are quickly relieved by glucocorticoids. In the case of a chronic process, skin lesions are observed according to the type of indurative edema with hyperpigmentation. Proximal progresses, less often - generalized muscle weakness often associated with seizures and neuropathy. There is lung damage with the development of respiratory failure of a restrictive type. Perhaps the simultaneous appearance of cardiac pathology (conduction disorders, myocarditis, endocarditis, dilated cardiomyopathy), eosinophilic gastroenteritis, hepatomegaly. In 20% of cases, arthritis is observed, in 35-52% - arthralgia, in some patients flexion contractures are formed with predominant fasciitis phenomena.
Superficial migratory thrombophlebitis (Figure 4) is most commonly observed in patients with venous insufficiency. Superficial thrombophlebitis in combination with organ thrombosis occurs in Behcet's disease, as well as in paraneoplastic syndrome (Trousseau's syndrome), caused by cancer of the pancreas, stomach, lungs, prostate, intestines and bladder. The disease is characterized by numerous, often linearly arranged seals on the lower (rarely upper) limbs. The localization of seals is determined by the affected areas of the venous bed. Ulcer formation is not observed.
Cutaneous polyarteritis nodosa is a benign form of polyarteritis, characterized by a polymorphic rash and occurring, as a rule, without signs of systemic pathology. The disease is manifested by reticular or racemous livedo in combination with skin nodular and nodular infiltrates, mobile and painful on palpation. The skin above them is red or purple, less often the usual color. Infiltrates are up to several centimeters in diameter, persist for up to 2-3 weeks, do not tend to suppurate, but in their center the formation of hemorrhagic necrosis is possible. The predominant localization is the lower limbs (the area of the calf muscles, feet, ankle joints) (Fig. 5). Most patients have general disorders (weakness, malaise, migraine-like headache, paroxysmal abdominal pain, sometimes fever), it is noted hypersensitivity legs to cooling, paresthesia in the feet. Histological examination of lesions reveals necrotizing angiitis in the deep vasculature of the skin.
The most prominent representative of LDL is idiopathic lobular panniculitis (ILPn) or Weber-Christian disease. The main clinical manifestations are soft, moderately painful nodes, reaching a diameter of ≥ 2 cm, located in the pancreas of the lower and upper extremities, less often in the buttocks, abdomen, chest and face. Depending on the shape of the node, ILPN is divided into nodular, plaque and infiltrative. With the nodular variant, the seals are isolated from each other, do not merge, and are clearly delimited from the surrounding tissue. Depending on the depth of occurrence, their color varies from the color of normal skin to bright pink, and the diameter ranges from a few millimeters to 5 cm or more. The plaque variety is the result of the fusion of individual nodes into a densely elastic, tuberous conglomerate, the skin color above it varies from pink to bluish-purple. Sometimes seals spread to the entire surface of the lower leg, thigh, shoulders, etc., which often leads to swelling and severe pain due to compression of the neurovascular bundles. The infiltrative form is characterized by the occurrence of fluctuations in the zone of individual nodes or conglomerates of bright red or purple color. The opening of the focus occurs with the release of a yellow oily mass and the formation of poorly healing ulcers (Fig. 6). Patients with this clinical form of SP are often diagnosed with an "abscess" or "phlegmon", although purulent contents are not obtained when the lesions are opened.
In some patients, a gradual occurrence of all of the above varieties (mixed form) is possible.
Rashes are often accompanied by fever, weakness, nausea, vomiting, severe myalgia, polyatralgia, and arthritis.
Cytophagic histiocytic Mon, as a rule, turns into systemic histiocytosis with pancytopenia, impaired liver function, and bleeding tendency. Characterized by the development of recurrent red skin nodules, hepatosplenomegaly, serous effusion, ecchymosis, lymphadenopathy, ulceration in the oral cavity. As the disease progresses, anemia, leukopenia, coagulation disorders (thrombocytopenia, hypofibrinogenemia, decreased levels of factor VIII, etc.) develop. Often ends in death.
Cold panniculitis often develops in children and adolescents, less often in adults, especially women. In the latter case, Mon occurs after hypothermia when riding horses, motorcycles, etc. Lesions appear on the thighs, buttocks, lower abdomen. The skin becomes edematous, cold to the touch, acquires a purple-cyanotic color. Subcutaneous nodes appear here, which exist for 2-3 weeks, regress without a trace or leave behind foci of superficial skin atrophy.
Oil granuloma (oleogranuloma) is a peculiar type of PN that occurs after injections into the pancreas of various substances (povidone, pentazocine, vitamin K, paraffin, silicone, synthetic microspheres) for therapeutic or cosmetic purposes. After a few months or years, dense nodes (plaques) form in the pancreas, usually soldered to the surrounding tissues, in rare cases ulcers form. Perhaps the extensive spread of lesions to other parts of the body, the appearance of arthralgia, Raynaud's phenomenon and signs of Sjögren's syndrome. Histological examination of biopsy specimens reveals the characteristic formation of multiple oil cysts of various sizes and shapes (Swiss cheese symptom).
Pancreatic PN develops with an inflammatory or tumor lesion of the pancreas due to an increase in the serum concentration of pancreatic enzymes (lipase, amylase) and, as a result, pancreatic necrosis. In this case, painful inflammatory nodes are formed, localized in the pancreas in various parts of the body. Generally clinical picture resembles that in Weber-ra-Kris-chen's disease (Fig. 7). Often develops polyarthritis and polyserositis. The diagnosis is established on the basis of histological examination data (foci fat necrosis) and increased levels of pancreatic enzymes in the blood and urine.
Lupus-Mon differs from most other varieties of Mon in the predominant localization of seals on the face and shoulders. The skin over the lesions is not changed or may be hyperemic, poikilodermic, or have signs of discoid lupus erythematosus. The nodes are clearly defined, from one to several centimeters in size, painless, hard, and can remain unchanged for several years (Fig. 8). With regression of the nodes, atrophy or scarring is sometimes observed. To verify the diagnosis, it is necessary to conduct a comprehensive immunological examination (determination of complement C3 and C4, antinuclear factor, antibodies to double-stranded DNA, cryoprecipitins, immunoglobulins, antibodies to cardiolipins).
Skin lesions in sarcoidosis are characterized by nodes, plaques, maculopapular changes, lupus pernio (lupus pernio), cicatricial sarcoidosis. Changes are painless symmetrical raised red lumps or nodes on the trunk, buttocks, limbs and face. Elevated, dense patches of skin, purplish-bluish on the periphery and paler, atrophic in the center, are never accompanied by pain or itching and do not ulcerate (Fig. 9). Plaques are usually one of the systemic manifestations of chronic sarcoidosis, are associated with splenomegaly, lung involvement, peripheral lymph nodes, arthritis, or arthralgia, persist for a long time and require treatment. Typical morphological feature sarcoidosis occurring with skin lesions is the presence of an unchanged or atrophic epidermis with the presence of a “naked” (i.e., without an inflammatory zone) epithelioid cell granuloma, and a different number of giant cells of the Pirogov-Langhans type and the type of foreign bodies. There are no signs of caseosis in the center of the granuloma. These features make it possible to make a differential diagnosis of skin sarcoidosis with Mon and lupus erythematosus.
PN caused by a deficiency of α1-antitrypsin, which is an inhibitor of α-protease, occurs more often in patients homozygous for the defective PiZZ allele. The disease develops at any age. The nodes are localized on the trunk and proximal parts of the limbs, often open with the release of an oily mass and the formation of ulcers. Other skin lesions include vasculitis, angioedema, necrosis, and hemorrhage. Systemic manifestations associated with α1-antitrypsin deficiency include emphysema, hepatitis, cirrhosis, pancreatitis, and membranous proliferative nephritis.
Indurative tuberculosis, or Bazin's erythema, is localized mainly on the posterior surface of the legs (calf region). Develops more often in women young age suffering from one of the forms of organ tuberculosis. The formation of slowly developing, slightly painful (even on palpation) nodes of a bluish-red color (Fig. 10) that is not sharply delimited from the unchanged surrounding skin is characteristic. The latter often ulcerate over time, leaving behind foci of cicatricial atrophy. Histological examination reveals a typical tuberculoid infiltrate with foci of necrosis in the center.
In conclusion, it should be emphasized that the variety of forms and variants of the course of PN requires a thorough survey and a comprehensive clinical, laboratory and instrumental examination of the patient in order to verify the diagnosis. 3. Poorten M.C., Thiers B.H. panniculitis. Dermatol. Clin., 2002, 20 (3), 421-33
4. Verbenko E.V. Spontaneous panniculitis. In book. Skin and venereal diseases: a guide for doctors. Ed. Skripkina Yu.K. M., Medicine, 1995, vol. 2, 399-410.
5. Cascajo C, Borghi S, Weyers W. Panniculitis: definition of terms and diagnostic strategy. Am. J. Dermatol., 2000,22,530-49
6. Issa I, Baydoun H. Mesenteric panniculitis: various presentations and treatment regimens. World J Gastroenterol. 2009;15(30):3827-30.
7. Daghfous A, Bedioui H, Baraket O et al. Mesenteric panniculitis simulating malignancy. Tunis Med. 2010.3.1023-27.
8. Requena L, Yus E.S. Erythema nodosum. Dermatol.Clin. 2008.26(4),425-38.
9. Shen L.Y., Edmonson M.B., Williams G.P. et al. Lipoatrophic panniculitis: case report and review of the literature. Arch. Dermatol., 2010,146(8),77-81.
10. Guseva N.G. Systemic scleroderma. In: Rheumatology: National Guide. Ed. E.L. Nasonova, V.A. Nasonova. M., Geotar-Media, 2008: 447-466.
11. Nasonov E.L., Shtutman V.Z., Guseva N.G. Eosinophilia-myalgia syndrome in rheumatology. Wedge. honey. 1994; 2:17-24.
12. Goloeva R.G., Alekberova Z.S., Mach E.S. et al. Vascular manifestations of Behçet's disease. Scientific and practical rheumatology, 2010,2,51-7.
13. Thayalasekaran S, Liddicoat H, Wood E. Thrombophlebitis migrans in a man with pancreatic adenocarcinoma: a case report. Cases J. 2009 ;2:6610.
14. Morgan AJ, Schwartz RA. Cutaneous polyarteritis nodosa: a comprehensive review. Int J Dermatol. 2010;49(7):750-6.
15. Narvaez J, Bianchi MM, Santo P. et al. Pancreatitis, panniculitis, and polyarthritis. Semin Arthritis Rheum. 2010;39(5):417-23.
16. Chee C. Panniculitis in a patient presenting with a pancreatic tumor and polyarthritis: a case report. J Med Case Reports. 2009;3:7331.
17. Park HS, Choi JW, Kim BK, Cho KH. Lupus erythematosus panniculitis: clinicopathological, immunophenotypic, and molecular studies. Am J Dermatopathol. 2010; 32(1):24-30.
18. Sarcoidosis: Uch.-method. allowance. Ed. Vizel A.A., Amirov N.B. Kazan, 2010, 36-38.
19. Valverde R, Rosales B, Ortiz de Frutos FJ et al. Alpha-1-antitrypsin deficiency panniculitis. Dermatol Clin. 2008;26(4):447-51.
20. Daher Ede F, Silva Junior GB, Pinheiro HC, et al Erythema induratum of Bazin and renal tuberculosis: report of an association. Rev Inst Med Trop Sao Paulo. 2004;46(5):295-8.

With panniculitis, inflammation of the subcutaneous fat is observed. It is localized in the fatty lobules or interlobular septa and leads to their necrosis and fusion connective tissue. it dermatological disease has a progressive course and leads to the formation of nodes, infiltrates or plaques. And with its visceral form, the fatty tissues of internal tissues and organs are damaged: the pancreas, kidneys, liver, retroperitoneal space and omentum.

In this article, we will acquaint you with the alleged causes, varieties, main manifestations, methods for diagnosing and treating panniculitis. This information will help you make a timely decision about the need for treatment by a specialist, and you will be able to ask him questions that interest you.

Panniculitis is accompanied by increased peroxidation of fats. In half of the cases, an idiopathic form of the disease is observed (or Weber-Christian panniculitis, primary panniculitis) and more often it is detected in women 20-40 years old (usually overweight). In other cases, the disease is secondary and develops against the background of various provoking factors or diseases - immunological disorders, dermatological and systemic ailments, taking certain medications, exposure to cold, etc.

The reasons

This disease was first described in 1925 by Weber, but references to its symptoms are also found in descriptions dated 1892. Despite the development of modern medicine and the a large number studies on the study of panniculitis, scientists have not been able to get an accurate idea of the mechanisms of development of this disease.

It is known that the disease is provoked by various bacteria (usually streptococci and staphylococci), which penetrate into the subcutaneous fat through various microtraumas and damage to the skin. In most cases, tissue damage occurs in the leg area, but it can also occur in other parts of the body.

Predisposing factors for its development may be various diseases and states:

skin diseases -, and, athlete's foot, etc .;
injuries - any, even the most minor, injuries (insect bites, scratches, abrasions, wounds, burns, etc.) increase the risk of infection;
lymphogenous edema - edematous tissues are prone to cracking, and this fact increases the chance of infection of the subcutaneous fat;
diseases that weaken the immune system -, cancerous tumors, and etc.;
previous panniculitis;
intravenous drug use;
obesity.

Classification

Panniculitis can be:

primary (or idiopathic, Weber-Christian panniculitis);
secondary.

Secondary panniculitis can occur in the following forms:

cold - a local form of damage, caused by strong cold exposure and manifested by the appearance of pink dense nodes (after 14-21 days they disappear);
lupus panniculitis (or lupus) - observed in severe systemic lupus erythematosus and is manifested by a combination of manifestations of two diseases;
steroid - observed in childhood, develops 1-2 weeks after oral administration of corticosteroids, does not need special treatment and heals on its own;
artificial - caused by taking various medicines;
enzymatic - observed in pancreatitis against the background of an increase in the level of pancreatic enzymes;
immunological - often accompanies systemic vasculitis, and in children it can be observed with;
proliferative-cellular - develops against the background of leukemia, histocytosis, lymphoma, etc.;
eosinophilic - manifests itself as a non-specific reaction in some systemic or skin diseases (skin vasculitis, injection lipophatic granuloma, systemic lymphoma, insect bites, eosinophilic cellulitis);
crystalline - caused by deposits in the tissues of calcifications and urates in renal failure, or after the administration of Meneridine, Pentazocine;
associated with deficiency of an α-protease inhibitor - observed with hereditary disease, which is accompanied by nephritis, hepatitis, hemorrhages and vasculitis.

According to the shape of the changes on the skin formed during panniculitis, the following options are distinguished:

knotted;
plaque;
infiltrative;
mixed.

The course of panniculitis can be:

acute inflammatory;
subacute;
chronic (or recurrent).

Symptoms

In such patients, painful nodes are formed in the subcutaneous tissue, prone to merging with each other.

The main manifestations of spontaneous panniculitis include the following symptoms:

appearance under the skin located on different depth nodes;
redness and swelling in the affected area;
fever and a feeling of tension and pain in the affected area;
red dots, rash or blisters on the skin.

More often lesions of the skin appear on the legs. In more rare cases, lesions appear on the arms, face, or torso.

In addition to lesions of the subcutaneous fat with panniculitis, patients often show signs of general malaise that occurs with acute infectious diseases:

fever;
weakness;
discomfort and pain in muscles and joints, etc.

After the disappearance of the nodes on the skin, areas of atrophy are formed, which are rounded foci of sunken skin.

In the visceral form of the disease, all fat cells are affected. With such panniculitis, symptoms of hepatitis, nephritis and pancreatitis develop, and characteristic nodes form in the retroperitoneal space and on the omentum.

Knotty panniculitis

The disease is accompanied by the formation of nodes limited from healthy tissues ranging in size from a few millimeters to 10 or more centimeters (usually from 3-4 mm to 5 cm). The color of the skin above them can vary from bright pink to flesh.

Plaque panniculitis

The disease is accompanied by the fusion of nodes into a densely elastic conglomerate. The color above it can vary from bluish-purple to pink. Sometimes the lesion captures the entire surface of the lower leg, thigh or shoulder. With such a course, compression of the neurovascular bundles occurs, causing severe pain and marked swelling.

Infiltrative panniculitis

The disease is accompanied by the appearance of fluctuations observed with ordinary phlegmon or abscesses, in separate melted conglomerates and nodes. The color of the skin over such lesions can vary from purplish to bright red. After opening the infiltrate, a foamy or oily mass of yellow color is poured out. Ulceration appears in the area of the focus, which suppurates for a long time and does not heal.

Mixed panniculitis

This variant of the disease is rarely observed. Its course is accompanied by the transition of the nodular variant to plaque, and then to infiltrative.

The course of panniculitis

Panniculitis may be severe course and even lead to lethal outcome.

At acute course the disease is accompanied by a pronounced deterioration in the general condition. Even against the background of treatment, the patient's health is constantly getting worse, and remissions are rare and do not last long. A year later, the disease leads to death.

The subacute form of panniculitis is not accompanied by such severe symptoms but also difficult to treat. A more favorable course is observed with a recurrent episode of the disease. In such cases, exacerbations of panniculitis are less severe, usually not accompanied by a violation of general well-being and are replaced by long-term remissions.

The duration of panniculitis can range from 2-3 weeks to several years.

Possible Complications

Panniculitis can be complicated by the following diseases and conditions:

phlegmon;
abscess;
skin necrosis;
gangrene;
bacteremia;
lymphangitis;
sepsis;
(with damage to the face).

Diagnostics

To diagnose panniculitis, a dermatologist prescribes the following examinations to the patient;

blood analysis;
biochemical analysis;
Reberg's test;
blood tests for pancreatic enzymes and liver tests;
Analysis of urine;
blood culture for sterility;
node biopsy;
bacteriological examination of discharge from the nodes;
immunological tests: antibodies to ds-DNA, antibodies to SS-A, ANF, complements C3 and C4, etc.;
Ultrasound of internal organs (to identify nodes).

Diagnosis for panniculitis is aimed not only at its detection, but also at determining the causes of its development (i.e., background diseases). In the future, based on these data, the doctor will be able to draw up a more effective treatment plan.

Differential diagnosis is performed with the following diseases:

lipoma;
pathomymia;
insulin lipodystrophy;
oleogranuloma;
skin calcification;
deep lupus erythematosus;
actinomycosis;
sporotrichosis;
necrosis of the subcutaneous fat of newborns;
gouty nodes;
Farber's disease;
skin sarcoids Darier-Roussy;
vascular hypodermatitis;
eosinophilic fasciitis;
other forms of panniculitis.

Treatment

Treatment of panniculitis should always be comprehensive. The tactics of therapy is always determined by its form and nature of the course.

Patients are prescribed the following drugs:

vitamins C and E;
antihistamines;
broad-spectrum antibacterial drugs;
hepatoprotectors.

In subacute or acute course, corticosteroids (Prednisolone, etc.) are included in the treatment plan. Initially, a high dosage is prescribed, and after 10-12 days it is gradually reduced. If the disease is severe, then the patient is prescribed cytostatics (Methotrexate, Prospidin, etc.).

With secondary panniculitis, the treatment of a disease that contributes to the development of the disease is mandatory.