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Lipoatrophic Connective Tissue Panniculitis
Author(s) -
Marque Myriam,
Guillot Bernard,
Bessis Didier
Publication year - 2010
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.2009.01048.x
Subject(s) - medicine , connective tissue , panniculitis , dermatology , pathology , connective tissue disease , autoimmune disease , disease
Many causes of what was formerly called Weber‐Christian and Rothmann‐Makai diseases are being clarified as specific forms of panniculitis. Among them, an autoimmune process involving the subcutaneous fat without criteria for another defined disorder coined “connective tissue panniculitis” by Winckelman et al in 1980 has been described. We describe this disease in a 4‐year‐old boy who presented with multiple subcutaneous inflammatory nodules that extended in an annular fashion, resolved leaving lipoatrophy, with recurrence 8 years later. The histologic findings were consistent with a granulomatous lipophagic panniculitis. We review previous reports and emphasize the limited therapeutic options, chronic evolution, severe esthetic sequelae and possible association with other autoimmune disorders of this uncommon condition.