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Panniculitis nodularis nonsuppurativaM35.6
Synonym(s)
Christian Weber Disease; idiopathic lobular panniculitis; Idiopathic nodular panniculitis (Mac Donald); Lipogranulomatosis generalized; Non-purulent panniculitis; Panniculitis idiopathic lobular; Pfeiffer-Weber-Christian Syndrome; recurrent panniculitis febrile non-purulent; Weaver Christian disease; Weber-Christian Syndrome
HistoryThis section has been translated automatically.
Pfeiffer 1892; Weber 1925; Christian 1928
DefinitionThis section has been translated automatically.
Increasingly uncommondiagnosis, when diagnosed accurately, for a focal, non-suppurative (non-suppurative) inflammation of the subcutaneous adipose tissue with fever and formation of symmetrically arranged, reddened, subcutaneous nodules or plaques, progressing in episodes over a period of years.
The classic (Pfeiffer) Weber-Christian syndrome may be considered a common pathophysiologic end route to different etiologic factors. Certainly, the syndrome is not an independent clinical picture.
To be distinguished are:
- Pancreatic panniculitis (see also under pancreatitis; see also pancreatitis-panniculitis-polyarthritis syndrome).
- Panniculitis in lupus erythematosus
- Panniculitis in alpha-1 antitrypsin deficiency associated panniculitis ( AAT deficiency associated panniculitis).
This contrasts with idiopathic panniculitis without general symptoms.
EtiopathogenesisThis section has been translated automatically.
Unexplained, to be discussed: Allergic vasculitis, infections, autoimmune processes and alpha-1-antitrypsin deficiency. A nodular panniculitis as a hyperergic reaction in Lyme disease has been described.
ManifestationThis section has been translated automatically.
About 70% women, average age of manifestation at 40 years.
LocalizationThis section has been translated automatically.
Mainly localized on thighs and buttocks, less frequently on trunk and arms.
Clinical featuresThis section has been translated automatically.
Sudden onset of weakness, vomiting, fatigue, rheumatoid complaints and fever. Multiple small or larger, pressure painful, soft subcutaneous nodules with redness and swelling of the covering skin. The nodules heal after weeks to months, leaving a dent in the skin due to scarring in the subcutaneous fatty tissue. Rare spontaneous perforation through the skin (= liquefying nodular panniculitis, panniculitis with liquefaction).
LaboratoryThis section has been translated automatically.
BSG acceleration, leukopenia, less frequently leukocytosis.
HistologyThis section has been translated automatically.
Lobular panniculitis: Initially between the fat cells neutrophil granulocytes, lymphocytes, histiocytes, later lipophilic granulation tissue with macrophages and foam cells, fibrotic scarring stage. Rare induction of histiocytic proliferation ( cytophagic panniculitis).
Differential diagnosisThis section has been translated automatically.
Other forms of panniculitis (see below panniculitis); actinomycosis; sporotrichosis; myceoma; cryptococcosis; borreliosis; necrotising fasciitis; traumatogenic lipogranuloma; embolia cutis medicamentosa; granuloma anulare subcutaneum; lipogranulomatosis subcutanea; sarcoidosis; lipomatosis dolorosa; rheumatic nodules; eosinophilic fasciitis; dermatoliposclerosis; erythema nodosum; thrombophlebitis migrans; erythema induratum; polyarteritis nodosa, cutaneous.
General therapyThis section has been translated automatically.
The relapses of the disease are self-limiting. A wait-and-see attitude is therefore justifiable.
External therapyThis section has been translated automatically.
Apply non-steroidal anti-inflammatory drugs such as indometacin (e.g. Amuno gel), ibuprofen (e.g. Dolgit cream) or piroxicam (e.g. Felden-top cream) in a thick layer to lesional skin, over which compresses with 0.9% saline or 2-5% ethanol are applied hourly. Alternatively, apply potent glucocorticoids such as 0.1% mometasone cream (e.g., Ecural) in thick layer, plus diluted alcohol poultices hourly. Healing of the nodes after weeks to months, leaving a dent-like skin indentation due to scarring in the subcutaneous fatty tissue.
Internal therapyThis section has been translated automatically.
- Nonsteroidal anti-inflammatory drugs such as acetylsalicylic acid (e.g. aspirin; 1.5-2.0 g/day p.o.) or diclofenac (e.g. Voltaren Tbl./Supp.; initial 150 mg, as maintenance dose 100 mg/day).
- In severe clinical pictures with considerable general symptoms glucocorticoids such as prednisone (e.g. Decortin) 80-100 mg/day, creeping out over 3-5 weeks depending on the clinic.
- In severe recurrent disease the following drugs have been tried with varying degrees of success:
- Ciclosporin A (e.g. Sandimmun) 2-3 mg/kg bw/day p.o.
- Dapsone (e.g. Dapsone Fatol Tbl.) 1.0-2.0 mg/kg bw/day p.o.
- Combination of hydroxychloroquine 4 mg/kg bw/day p.o. and colchicine 0.025 mg/kg bw/day p.o.
Progression/forecastThis section has been translated automatically.
Relapsing course; years of symptom-free intervals are possible.
LiteratureThis section has been translated automatically.
- Christian HA (1928) Relapsing febrile nodular nonsuppurative panniculitis. Archives of Internal Medicine, Chicago, 42: 338-351
- Diaz-Cascajo C, Borghi S (2002) Subcutaneous pseudomembranous fat necrosis: new observations. J Cutan catholic 29: 5-10
- Maverakis E et al (2014) Mycobacterium chelonae infection presenting as recurrent cutaneous and subcutaneous nodules--a presentation previously diagnosed as Weber Christian disease. Dermatol Online J 20. pii: 13030/qt9k9535t1
- Pfeifer V (1892) On a case of focal atrophy of the subcutaneous fatty tissue. German Arch Klin Med (Leipzig) 50: 438-44
- Requena L, Sanchez Yus E (2001) Panniculitis. Part II. Mostly lobular panniculitis. J Am Acad Dermatol 45: 325-361
- Riveros Frutos A et al (2014) Nephrotic syndrome in a patient with Pfeifer-Weber-Christian disease. Joint Bone Spine doi: 10.1016/j.jbspin.2014.11.001
- Rotaru N et al (2015) Nonsuppurative Nodular Panniculitis of the Breast. Clin Breast Cancer doi: 10.1016/j.clbc.2015.02.004
- Weber FP (1925) A case of relapsing non-suppurative nodular panniculitis, showing phagocytosis of subcutaneous fat-cells by macrophages. British Journal of Dermatology and Syphilis, Oxford, 37: 301-311