Abstract

A 3-year-old girl presented to the outpatient dermatology clinic for a 4-month history of recurrent nodules on the arms and legs with associated intermittent myalgias. On exam, erythematous and violaceous atrophic plaques spanned all four extremities with interspersed admixing subcutaneous nodules. Laboratory studies revealed a positive antinuclear antibody (1:320) with a speckled nuclear pattern, white blood cell count of 2.6 (nl. 5.50-15.50), hemoglobin of 9.4 (nl. 11.5-14.0), erythrocyte sedimentation rate of 91 (nl. 0-20), and an elevated cytomegalovirus IgG antibody. Microscopic findings in the excisional biopsy were significant for numerous foamy, lipidized histiocytes and a mixed inflammatory infiltrate of lymphocytes, histiocytes, neutrophils, and eosinophils in a lobular pattern within the subcutaneous fat. The histiocytes were positive for CD68, B-lymphocytes were positive for CD20, T-lymphocytes were positive for CD3, CD4, and CD8 (approximately 1:1 ratio of CD4 to CD8), and CD7 was retained. Acid-fast bacilli and Grocott methenamine silver stains were negative. The diagnosis of lipophagic panniculitis (LP) causing acquired generalized lipodystrophy (AGL) Type 1 was made. LP is a rare disorder of childhood characterized by an inflammatory phase of painful subcutaneous nodules preceding permanent lipoatrophy. As it is a diagnosis of exclusion, infectious etiologies, hematologic malignancy, trauma, and inherited panniculitides must be ruled out. Due to its rarity, the pathogenesis is unknown, however, several cases have been associated with autoimmune disorders. Historically, patients have had varying responses to antimalarials, glucocorticoids, and immunosuppressants.