title: Lipoatrophic connective tissue panniculitis.
abstract: 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.
authors.affiliation: Department of Dermatology, CHU Montpellier, Montpellier University 1, Montpellier, France. myriammarque@yahoo.fr
authors.forename: Myriam
authors.initials: M
authors.lastname: Marque
authors.forename: Bernard
authors.initials: B
authors.lastname: Guillot
authors.forename: Didier
authors.initials: D
authors.lastname: Bessis
mesh_terms: D000293:Adolescent; D001284:Atrophy; D001706:Biopsy; D002675:Child, Preschool; D003240:Connective Tissue Diseases / Q000473:pathology*; D018450:Disease Progression; D006801:Humans; D008297:Male; D010201:Panniculitis, Nodular Nonsuppurative / Q000473:pathology*; D012867:Skin / Q000473:pathology; D050151:Subcutaneous Fat / Q000473:pathology*