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.

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