Eritema nudoso crónico de localización generalizada: a considerar enfermedad de Weber Christian

Abstract

Introduccion La enfermedad de Weber-Christian es una paniculitis nodular recidivante, no supurativa, febril, mas frecuente en las mujeres jovenes. Se caracteriza por brotes recurrentes de nodulos subcutaneos, de distribucion simetrica. Tiene un origen idiopatico o es secundaria a enfermedad pancreatica, agentes fisicoquimicos o deficit de alfa-1-antitripsina. Puede afectar cualquier tejido del organismo que tenga grasa. Histologicamente presenta paniculitis lobulillar sin vasculitis. Caso clinico Mujer de 23 anos que ingreso por nodulos subcutaneos dolorosos con equimosis en miembros pelvicos, gluteos y antebrazos, con placas eritematosas e hiperpigmentadas; equimosis en parpado superior derecho e hiposfagma bilateral; fiebre de 38 °C; diaforesis y ulceras orales no dolorosas. El hemograma mostro pancitopenia, elevacion de los reactantes de fase aguda, y amilasa y lipasa normales. Los cultivos fueron negativos. Los anticuerpos antinucleares y el complemento fueron normales. La tomografia del abdomen presento hiperdensidad en tejido graso subcutaneo diseminada. La biopsia revelo paniculitis lobulillar sin vasculitis, compatible con paniculitis de Weber-Christian. Se trato con esteroide e inmunosupresor. Conclusiones Se descartaron la etiologia infecciosa y otras causas de nodulo eritematoso por la evolucion clinica y los estudios complementarios. La paciente presento en varias semanas una involucion espontanea; en ocasiones, las lesiones evolucionaron a abscesos esteriles, ademas de manifestaciones clinicas sistemicas. El tratamiento corticoideo tuvo buen resultado. Background Weber-Christian disease is a recurrent, non-suppurative, febrile nodular panniculitis, more frequent in young women. It is characterized by recurrent outbreaks of subcutaneous nodules, distributed symmetrically. It has an idiopathic origin, or it is secondary to pancreatic disease, physicochemical agents or alpha-1 antitrypsin deficiency. It can affect any tissue with body fat. Histologically is a lobular panniculitis without vasculitis. Case report 23-year-old woman. She was admitted with subcutaneous painful nodules in pelvic, gluteus and forearms, with erythematous and hyperpigmented plaques; ecchymosis in the right upper eyelid and bilateral hyposphagma; fever of 38 °C; diaphoresis and oral ulcers, not painful. The hemogram showed pancytopenia, elevation of acute phase reagents, amylase and normal lipase. Blood culture was negative. Antinuclear antibodies and complement were normal. Computed tomography of the abdomen showed disseminated hyperdensity in subcutaneous tissue. The biopsy showed lobular panniculitis without vasculitis compatible with WeberChristian panniculitis. The patient was treated with steroids and immunosuppressive therapy. Conclusions Infectious etiology and other causes of erythematous nodules were ruled out through clinical evolution and complementary studies. The patient presented spontaneous involution in several weeks and sometimes the lesions evolved to sterile abscesses, in addition to systemic clinical manifestations. Corticosteroid therapy was deemed successful.