British loiinuilofDermatohgij {19^]) 124. 291-295. ADONIS OOO7O963910OO68G Adult lipophagic atrophic panniculitis I.J.UMBERT AND R.K.WINKELMANN Department of Di'miatolonn. Mttyo Clinic and Miuio i'oiinilatinti, Rochester. MJ\', U.S.A. Accepted for publication 51 August 1990 Summary We report two patients with chronic, recurrent, nodular panniculiti.s lesions that later developed areas of lipoatrophy. Histologicaily. there was a consistent lobular lipophagic replacement of fat cells with lipophagic giant cells. The clinical appearance was that of tender, erythematous. superficial or subcutaneous, symmetrical nodules and plaques of 1-2 weeks' duration. The lesions could occur with episodes of fever. One patient had hepatomegaly and the other had an increased sedimentation rate and ieucocytosis. The histology and the clinical pattern of the panniculitus syndrome resembled those of lipophagic lipoatrophy of childhood. This is a panniculitis of unknown cause in which the principal inflammatory cell response in the subcutaneous tissue is the macrophage. Descriptions of Weber-Christian panniculitis have included the histology of lipophagic replacement of fat lobules since the early report by Weber.' Subsequently, lipophagic replacement of fat has been used to define this syndrome as a pathological entity.- Pfeifer^ described a 2 3-year-old woman with primary lobular panniculitis. lipoatrophy with lipophagia. and giant cells. The loca- tions of the lesions and muscle weakness were somewhat atypical and also raised the question of panniculitis with connective tissue disease. I,ipophagia has recently been described as the primary pathological process in a rare lipoatrophic syndrome of panniculitis in childhood."^ All of these cases had been called Weber-Christian disease or Kothman-Makai syn- drome.'' '" Winkelmann ct ai'^ renamed these cases lipophagic lipoatrophy of childhood, recognizing that the cases began with erythematous nodules of short duration, with a recurrent course and lipophagic giant cell replacement of fat. and with residual lipoatrophie lesions. We have studied two adult patients with a similar syndrome. All the features of the childhood phenome- non were present, and we wish to document this as a syndrome of panniculitis that can occur at any age. In our experience this condition is unique in comparison to other forms of delineated panniculitis as in connective tissue disease, pancreatic disease, or Xi-antitrypsin deti- ciency. Correspondence: Professor K.K.WinkclniHnn, Depitrliiient of Dermato- logy, Mayo CWnk. 2(10 i'irst Street SW. Rocliestt-r. MN SS9()S. U.S.A. Case reports Case 1 A 47-year-oid womati was tirst seen in our department in February 1986. In November 1985 diffuse tender erythematous subcutaneous nodules and plaques had developed on her right thigh and buttocks. No adeno- pathy and visceromegaly were found. Investigations included a normal ultrasound of the pancreas and normal serum amylase and lipase levels, negative anti- nuclear antibody and normal levels of complement and cryoglobulins. Cultures of the lesions for bacteria, myco- bacteria, and fungi were sterile. The level of Xi-antitryp- sin was normal. Other investigations revealed that her sedimentation rate was 8 mm/h (Westergren method) and the haemoglobin level was 14 g/dl. Her leucocyte count was 7-8 x 1()''/1, with a normal differential. Total thyroxine. aspartate aminotransferase, cholesterol, and triglyceride levels and blood chemistry values were normal. The patient was started on dapsone, 50-100 mg daily and showed some resolution of her lesions. There were some broad atrophic depressions on the buttocks, thigh, and right arm at the sites of previous lesions (Figs 1 and 21. In March 1989 recurrent red nodules on both legs developed, and 1 day later fever and chilis developed. She was treated with dapsone and then sulphapyridine until progressive weakness and shortness of breath developed. Chest X-rays showed a diffuse bilateral inliltrate involv- ing interstitial and alveolar components. These changes were consistent with a hypersensitivity pneumonitis. 291