Jehad Alratrout1, Fatima Alshammasi1, Naseem Ansari2. 1. Department of Dermatology, Qatif Central Hospital, Kingdom of Saudi Arabia. 2. ExpressMed Laboratories, Kingdom of Bahrain. ansarnas@gmail.com.
Abstract
BACKGROUND: Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare fulminant variant of pityriasis lichenoides et varioliformis acuta (PLEVA) characterized by a rapidly progressive course with predominant ulceronecrotic lesions associated with fever and systemic manifestations. First described by Degos in 1966, it carries a high morbidity and is potentially fatal. The exact pathogenesis is not clear, but it is proposed to be the result of hypersensitivity reaction to an infection. METHODS: Here we report the case of an 8-year-old boy with FUMHD in the eastern province of Saudi Arabia. RESULTS: The patient presented with pyrexia, fatigue, arthralgia, and worsening of his already existing skin lesions of PLEVA. There was an extensive painful erythematous maculopapular rash, some lesions ulcerated and necrotic, involving the face, trunk, limbs, and flexures. A diagnosis of FUMHD was made based on clinical and histological features. The skin lesions responded extremely well to oral methotrexate, with almost complete healing after 4 weeks. CONCLUSIONS: We describe a rare variant of PLEVA, namely FUMHD, in an 8-year-old boy who showed a remarkably favorable response to methotrexate, as manifested by near total clearance of the skin lesions without scarring or hyperpigmentation.
BACKGROUND:Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare fulminant variant of pityriasis lichenoides et varioliformis acuta (PLEVA) characterized by a rapidly progressive course with predominant ulceronecrotic lesions associated with fever and systemic manifestations. First described by Degos in 1966, it carries a high morbidity and is potentially fatal. The exact pathogenesis is not clear, but it is proposed to be the result of hypersensitivity reaction to an infection. METHODS: Here we report the case of an 8-year-old boy with FUMHD in the eastern province of Saudi Arabia. RESULTS: The patient presented with pyrexia, fatigue, arthralgia, and worsening of his already existing skin lesions of PLEVA. There was an extensive painful erythematous maculopapular rash, some lesions ulcerated and necrotic, involving the face, trunk, limbs, and flexures. A diagnosis of FUMHD was made based on clinical and histological features. The skin lesions responded extremely well to oral methotrexate, with almost complete healing after 4 weeks. CONCLUSIONS: We describe a rare variant of PLEVA, namely FUMHD, in an 8-year-old boy who showed a remarkably favorable response to methotrexate, as manifested by near total clearance of the skin lesions without scarring or hyperpigmentation.