Weber-Christian panniculitis: A review of 30 cases with this diagnosis

doi:10.1016/S0190-9622(98)70402-5

Journal of the American Academy of Dermatology

Volume 39, Issue 1, July 1998, Pages 56-62

https://doi.org/10.1016/S0190-9622(98)70402-5 Get rights and content

Abstract

Background: More than 60 years ago, Christian described a panniculitis that was later termed Weber-Christian disease . Objective: The purpose of this study was to investigate whether this is a specific disease or a nonspecific disease that embraces several specific conditions. Methods: We studied 30 cases diagnosed as Weber-Christian panniculitis and found it possible to make a more specific diagnosis. Results: In 12 patients, findings were compatible with erythema nodosum. Six patients had phlebitis or postphlebitic syndrome. Factitial panniculitis was diagnosed in five patients, and trauma had a role in the conditions of another three patients. Cytophagic panniculitis, lymphoma, and leukemia were recognized in one patient each. The lesion was lobular in almost all cases, and the presence of lipophagia was noted in 19 biopsy specimens. Granulomatous, neutrophilic, and lymphocytic pathologic changes were present in nine, eight, and eight tissue specimens, respectively. Conclusion: The recognition of distinct disease patterns of fat lesions as fat necrosis with pancreatic disease, α₁ -antitrypsin panniculitis, lupus and connective tissue disease panniculitis, involution lipoatrophy, lipomembranous panniculitis, factitial panniculitis syndromes, calcification panniculitis, lipophagic lipoatrophy, and cytophagic panniculitis has lessened the need for a less specific panniculitis category. All these diseases have been reported in the literature as “Weber-Christian disease.” Because separate and distinct forms of fat lesions have been described, we believe that the eponym should be abandoned and that more specific diagnoses should be made on the basis of pathogenesis or cause. (J Am Acad Dermatol 1998;39:56-62.)

Section snippets

MATERIAL AND METHODS

We identified 30 patients who, since 1960, had the diagnosis of WCD. Clinical and laboratory results were analyzed. Ten years or more of follow-up were available for all patients, and we were able to obtain follow-up information from the patients, their physicians, relatives, or friends by letter or telephone or both.

Forty-four biopsy specimens from the 30 patients were reviewed. Eleven of these were outside specimens; six had been given the pathologic diagnosis of WCD at another institution.

RESULTS

The diagnosis of WCD was made clinically in five patients and suggested for another three patients. Nonspecific panniculitis diagnoses were made in three other patients (Table I).

. Patients initially diagnosed as having WCD

Patient	Age at onset (yr)	Sex	Initial clinical diagnosis	Initial histopathologic diagnosis	Final clinicopathologic diagnosis
1	39	F	Erythema nodosum	WCD	Acute panniculitis consistent with erythema nodosum
2	61	M	“Sebaceous cyst”	WCD	Acute panniculitis
3	76	M	Probable leukemia	WCD	Chronic

DISCUSSION

A disease entity starts as a hypothesis based on clinical observations and laboratory data, including pathologic findings. If the original hypothesis is correct, other reports will confirm the disease process and add details to the description, course, and possible methods of treatment. The major difficulty with cases of primary panniculitis is their rarity. Many physicians see only one or a few cases in a lifetime. Despite this problem, we would like to point to the rapid identification and

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Citation Excerpt :
Later studies and better diagnostic algorithms led to reclassification of many patients under the diagnosis of Weber-Christian disease into other diseases, such as lupus panniculitis, factitial panniculitis, panniculitis associated with pancreatic disease, histiocytic cytophagic panniculitis, and α1-antitrypsin deficiency panniculitis.4 It was then agreed that the Weber-Christian eponym should not be used as a distinct entity and that more specific diagnoses should be made on clinicopathologic grounds.5 Even after the clinicopathologic reclassification of panniculitis, especially in children, cases still remained of episodic, mostly lobar panniculitis with fever leading to lipoatrophy that could not be characterized under any recognized panniculitic condition.
Idiopathic lipoatrophic panniculitis of children is a rare disease of childhood, characterized by repeated attacks of tender subcutaneous nodules followed by the development of permanent lipoatrophy, often seen on the arms and legs, in association with fever, malaise, and other less common clinical manifestations such as abdominal pain or arthralgia. The pathogenesis is unknown, and autoimmune origins, chromosomal alterations, and other causes have been proposed. The nosology of this condition is confusing in the literature, and the precise diagnosis still relies on a combination of clinicopathologic and laboratory assessments. Methotrexate may be recommended to minimize disease progression and morbidity
Cutaneous panniculitis
2016, Revue de Medecine Interne
Le terme panniculite est utilisé pour désigner une inflammation de l’hypoderme. Une biopsie profonde et large est nécessaire pour préciser le type d’atteinte de l’hypoderme et proposer une hypothèse diagnostique qui orientera la prise en charge. En effet, il existe 4 contextes nosologiques différents selon que l’inflammation prédomine dans les septums inter-adipocytaires ou directement dans les lobules adipeux ; qu’il s’y associe, ou non, une atteinte vasculaire. Il conviendra aussi d’adresser une partie du prélèvement biopsique pour analyse microbiologique pour ne pas méconnaître une cause infectieuse et pour rechercher une clonalité si un lymphome profond est suspecté. L’érythème noueux, principale hypodermite septale, a de nombreuses étiologies et est la seule hypodermite qui ne requiert pas de biopsie dans sa forme clinique typique. La périartérite noueuse (PAN) est une panniculite septale vasculaire avec un livedo et/ou des nodules et du purpura et impose de rechercher une atteinte systémique. Une thrombophlébite nodulaire doit faire rechercher une coagulopathie, une maladie de Behçet, une périartérite noueuse ou une maladie de Buerger. Les panniculites lobulaires sont classées selon l’infiltrat. Si l’infiltrat est lymphocytaire, il faudra discuter notamment le lupus érythémateux profond et les lymphomes T hypodermiques. S’il est histiocytaire, seront discutées la sarcoïdose, la panniculite histiocytaire cytophagique et chez le nouveau-né la cytostéatonécrose. La panniculite lobulaire neutrophilique est de diagnostic difficile et nécessite une confrontation anatomoclinique. En pratique, très souvent, le diagnostic étiologique d’une hypodermite ne peut pas être établi d’emblée, c’est souligner l’importance du suivi de ces malades qu’il ne faut pas hésiter à biopsier à nouveau.
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Weber-Christian panniculitis: A review of 30 cases with this diagnosis☆,☆☆

Abstract

Section snippets

MATERIAL AND METHODS

RESULTS

DISCUSSION

J Am Acad Dermatol

J Am Acad Dermatol

J Invest Dermatol

J Am Acad Dermatol

J Am Acad Dermatol

J Am Acad Dermatol

Relapsing febrile nodular nonsuppurative panniculitis

Arch Intern Med

Relapsing febrile nodular nonsuppurative panniculitis (Weber-Christian Disease)

JAMA

Ueber einen Fall von herdweiser Atrophie des subcutanen Fettgewebes

Dtsch Arch Klin Med Leipz

Nodular nonsuppurative panniculitis (Weber-Christian Disease): report of a case in which an infiltrate of polymorphonulcar leukocytes represented the earliest lesion

Arch Dermatol Syphilol

A case of relapsing non-suppurative nodular panniculitis, showing phagocytosis of subcutaneous fat-cells by macrophages

Br J Dermatol

Further note on relapsing febrile nodular non-suppurative panniculitis

Br J Dermatol

The Weber-Christian syndrome, with particular reference to etiology: a description of eight cases of panniculitis

Acta Derm Venereol (Stockh)

Erythema nodosum

Acta Derm Venereol (Stockh)

Erythema nodosum und “relapsing febrile nodular nonsuppurative panniculitis “

Arch f Dermat u Syph