Weber-Christian disease with orbital involvement refractory to conventional immunosuppressants and response to adalimumab

Autores: Florencia Vivero, Ricardo Barrera, Javier Pui, Alexia Nicomedes, Mariana Abraham, David Aiziczon

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Weber-Christian disease is a rare, idiopathic form of systemic panniculitis. It is characterized by the presence of nodules and panniculitis plaques on the trunk and limbs with symptoms of systemic inflammation, a chronic recurrent course and occasional visceral involvement. The case of a 49-year-old female patient with skin, muscle and periorbital involvement refractory to treatment with corticosteroids and conventional immunosuppressants, with a good response to adalimumab, is described. Although panniculitis is a complex pathology, histopathological study, global clinical evaluation, and timely treatment are essential for therapeutic success. Anti-TNF drugs are an option to consider in refractory cases.