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Febrile ulceronecrotic Mucha-Habermann disease - a case and treatment review
Sara Moutinho-Pereira1, Francisca Beires, Joana Dos Santos
1Departamento de Medicina, Servico de Medicina Interna, Unidade Local de Saude de Matosinhos EPE, Portugal. sarasofia.pereira@gmail.com.
Abstract:
Febrile ulceronecrotic Mucha-Habermann disease is a rare and severe variant of pityriasis lichenoides, characterized by sudden onset of generalized ulceronecrotic papules that rapidly coalesce into ulcers associated with high fever. Systemic manifestations such as intravascular disseminated coagulation and pulmonary, cardiac, gastrointestinal, and central nervous system involvement are common. Treatment is based on oral corticosteroids, immunosuppressive drugs such as methotrexate, and general supportive treatment. The present case describes a stepwise approach to a patient with Mucha-Habermann disease with insufficient response to methotrexate.
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