Weber-Christian disease: Report of a case

Weber-Christian disease is a rare adipose tissue inflammatory disorder of unknown etiology, characterized by painful subcutaneous nodules and constitutional symptoms. The course and prognosis of Weber-Christian disease are highly variable: significant morbidity and mortality may occur if inflammation is involving visceral organs. We report the case of a 4 year-old female child who presented with recurrent fever, hepatosplenomegaly, anorexia and weight loss since 7 months of age; clinical signs also included palpable nodules mainly located on the lower extremities. Extensive laboratory testing and histologic examination, demonstrating the presence of lobular panniculitis without vasculitis, led us to diagnose Weber-Christian disease. Our patient was administered oral prednisone, then gradually tapered and skin lesions underwent to a complete resolution within 3 weeks; nevertheless, cyclosporin therapy was introduced because of frequent clinical relapses. During 6 month-therapy-follow- up it was only recorded recurring fever and absence of other clinical manifestations. Within the subsequent 38 month-follow-up, cutaneous manifestations and general malaise have never reappeared and extensive laboratory testing showed no evidence of underlying malignancies such as leukemia or lymphoma. Weber-Christian disease is a rare panniculitis. Its definitive diagnosis requires a deep skin biopsy and repeated extensive testing; prognosis is variable and it is responsive to combined therapy with low-doses steroids and immunosuppressor agents or to immunosuppressants alone.