A case report of febrile ulceronecrotic Mucha-Habermann disease

Abstract

Background Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and severe variant of pityriasis lichenoides et varioliformis acuta, characterized by a rapid onset of painful, necrotic skin lesions and systemic symptoms. The diagnosis of FUMHD is complex, hinging on the clinical presentation, histopathological findings, and exclusion of other severe dermatoses. The key diagnostic criteria include sudden development of ulceronecrotic papules and plaques, fever, and evidence of systemic disease. Due to the rarity of FUMHD, there is no consensus on optimal treatment, reflecting a significant gap in the dermatological practice. Case Description This report details a multimodal approach tailored to our 13-year-old patient, incorporating systemic corticosteroids, immunosuppressive therapy, and intensive supportive care. The strategy was designed to address the acute and aggressive nature of the disease while mitigating potential systemic complications. The report emphasizes on the intricate, multi-layered care required to manage FUMHD, illustrating the challenges and considerations in treating this complex condition. It underscored the necessity of a personalized, comprehensive care plan that extends beyond medical intervention to include psychological and social support. The outcome of our patient was encouraging, with a marked reduction in cutaneous manifestations and improvement in systemic symptoms. Conclusions It was found that prevention and care of skin injuries and complications, as well as the protection of patient’s mental state during the development of the disease, are very important. Therefore, early diagnosis, prompt treatment, close monitoring of infection indicators, and specialized care are essential to improve the prognosis of patients with FUMHD.