engleski

Idiopathic lipoatrophic panniculitis of the ankles in a 9-year-old girl

Introduction Lipoatrophic panniculitis, also known as lipophagic panniculitis of childhood, is a rare clinical condition characterized by tender annular erythematous plaques followed by lipoatrophy. This disorder is predominantly found in female children and usually affects the extremities, most commonly the ankles. The pathogenesis of this panniculitis and localized subcutaneous fat atrophy is unknown, but it is suspected to have an autoimmune etiology. Results A previously healthy 9-year old girl presented with a one-month history of progressive swelling and pain in the left medial malleolar region without known preceding trauma or infection. Within the following ten days, an indurated annular erythematous plaque appeared over the left ankle, and soon thereafter similar arcuate lesions appeared on the right ankle. The lesions were tender to palpation. Central parts of the lesions had a somewhat darker hue and gradually became depressed and atrophic. The patient underwent a comprehensive diagnostic workup and all of the findings were unremarkable, apart from elevated erythrocyte sedimentation rate (40 mm/hour) and C- reactive protein (39 mg/L). There was no laboratory feature suggestive of an autoimmune disease. Magnetic resonance scan of the left calf and ankle revealed inflammatory changes in the subcutaneous tissue. Histopathologic findings of an incisional skin biopsy demonstrated predominantly lobular panniculitis. Within the subcutaneous fat lobules, there was a dense mixed inflammatory infiltrate composed of lymphocytes, neutrophils and many foamy macrophages. There were no signs of vasculitis nor additional pathologic findings in the overlying dermis and epidermis. In correlation with the clinical picture, a diagnosis of lipoatrophic panniculitis of the ankles was made. Systemic treatment with prednisone and methotrexate led to marked improvement of local clinical findings, leaving only residual atrophic areas. However, on prednisone taper, the patient experienced a relapse with new painful lesions involving her lower anterior shins. Reintroduction of systemic treatment resulted in evident improvement of symptoms, but further management remains a challenge. Discussion Lipoatrophic lobular panniculitis of the ankles is an uncommon clinical entity occurring in childhood. It is considered that annular subcutaneous fat atrophy of the ankles is actually an end-stage of an idiopathic lobular panniculitis. Treatment options include corticosteroids, methotrexate and antimalarials that show a rapid initial response and resolution of the tender inflammatory erythema and infiltrate, but residual lipoatrophy of the ankles and lower legs mostly persists.

Lipoatrophic panniculitis ; lobular panniculitis ; prednisone ; methotrexate

nije evidentirano