Panniculitis nodularis nonsuppurativa M35.6

Pfeiffer 1892; Weber 1925; Christian 1928

An increasingly rare diagnosisfor a focal, non-suppurative (non-purulent) inflammation of the subcutaneous fatty tissue with fever and the formation of symmetrically arranged, reddened, subcutaneous nodules or plates.

The classic (Pfeiffer) Weber-Christian syndrome can be seen as a common pathophysiological endpoint of different etiologic factors. The syndrome is certainly not an independent clinical picture.

It must be distinguished from:

• 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)
• Panniculitis under therapy with immune checkpoint inhibitors

This is in contrast to idiopathic panniculitis without general symptoms.

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.

About 70% women, average age of manifestation at 40 years.

Mainly localized on thighs and buttocks, less frequently on trunk and arms.

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).

BSG acceleration, leukopenia, less frequently leukocytosis.

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).

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.

The relapses of the disease are self-limiting. A wait-and-see attitude is therefore justifiable.

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.

• 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.

Relapsing course; years of symptom-free intervals are possible.

1. Christian HA (1928) Relapsing febrile nodular nonsuppurative panniculitis. Archives of Internal Medicine, Chicago, 42: 338-351
2. Diaz-Cascajo C, Borghi S (2002) Subcutaneous pseudomembranous fat necrosis: new observations. J Cutan Pathol 29: 5-10

3. Jiang B et al (2017) Diffuse granulomatous panniculitis associated with anti PD-1 antibody therapy. JAAD Case Rep 4:13-16

4. 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
5. Pfeifer V (1892) On a case of focal atrophy of the subcutaneous fatty tissue. German Arch Klin Med (Leipzig) 50: 438-44
6. Requena L, Sanchez Yus E (2001) Panniculitis. Part II. Mostly lobular panniculitis. J Am Acad Dermatol 45: 325-361
7. 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
8. Rotaru N et al. (2015) Nonsuppurative Nodular Panniculitis of the Breast. Clin Breast Cancer doi: 10.1016/j.clbc.2015.02.004
9. 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