Panniculitis nodularis nonsuppurativa M35.6

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 12.05.2021

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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

History
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Pfeiffer 1892; Weber 1925; Christian 1928

Definition
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Increasingly rarediagnosis, 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, occurring in episodes over a period of years.

The classic (Pfeiffer-) Weber-Christian syndrome can be considered as a common pathophysiological final course on different etiological factors. Certainly, the sydnrome is not an independent clinical picture.

To be distinguished are:

This contrasts with idiopathic panniculitis without general symptoms.

Etiopathogenesis
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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.

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

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

Clinical features
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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).

Laboratory
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BSG acceleration, leukopenia, less frequently leukocytosis.

Histology
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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).

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

External therapy
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Apply non-steroidal anti-inflammatory drugs such as indomethacin (e.g. Amuno gel), ibuprofen (e.g. Dolgit cream) or piroxicam (e.g. Felden-top cream) in a thick layer on lesioned skin, then apply hourly compresses with 0.9% saline solution or 2-5% ethanol. Alternatively, apply a thick layer of potent glucocorticoids such as 0.1% mometasone cream (e.g. Ecural), with additional hourly diluted alcohol compresses. After weeks to months, the nodules heal, leaving behind a dentlike skin retraction due to scarring in the subcutaneous fatty tissue.

Internal therapy
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  • 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:

Progression/forecast
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Gradual progression, often with intervals of many years without appearance.

Literature
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  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 catholic 29: 5-10
  3. 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
  4. Pfeifer V (1892) On a case of focal atrophy of the subcutaneous fatty tissue. German Arch Klin Med (Leipzig) 50: 438-44
  5. Requena L, Sanchez Yus E (2001) Panniculitis. Part II. Mostly lobular panniculitis. J Am Acad Dermatol 45: 325-361
  6. 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
  7. Rotaru N et al (2015) Nonsuppurative Nodular Panniculitis of the Breast. Clin Breast Cancer doi: 10.1016/j.clbc.2015.02.004
  8. 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

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 12.05.2021