Alratrout Jehad, Alshammasi Fatima, Ansari Naseem
Department of Dermatology, Qatif Central Hospital, Kingdom of Saudi Arabia.
ExpressMed Laboratories, Kingdom of Bahrain.
Int J Dermatol. 2016 Nov;55(11):1205-1209. doi: 10.1111/ijd.13323.
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.
Here we report the case of an 8-year-old boy with FUMHD in the eastern province of Saudi Arabia.
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.
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.
发热性溃疡性坏死性穆查-哈伯曼病（FUMHD）是急性痘疮样苔藓样糠疹（PLEVA）的一种罕见暴发性变体，其特征为病程迅速进展，主要为溃疡性坏死性病变，并伴有发热和全身表现。1966年由德戈斯首次描述，该病发病率高，有潜在致命性。确切发病机制尚不清楚，但推测是对感染的超敏反应所致。
在此我们报告沙特阿拉伯东部一名8岁患FUMHD男孩的病例。
该患者出现发热、疲劳、关节痛，其原有的PLEVA皮肤病变加重。有广泛的疼痛性红斑丘疹皮疹，一些病变发生溃疡和坏死，累及面部、躯干、四肢及屈侧。根据临床和组织学特征诊断为FUMHD。皮肤病变对口服甲氨蝶呤反应极佳，4周后几乎完全愈合。
我们描述了一名8岁男孩患PLEVA的一种罕见变体，即FUMHD，其对甲氨蝶呤表现出显著良好反应，皮肤病变几乎完全消退，无瘢痕形成或色素沉着。
