Febrile ulceronecrotic Mucha-Habermann disease is a rare fulminant variant of pityriasis lichenoides et varioliformis acuta, characterized by a rapidly progressive course with predominant ulceronecrotic lesions associated with fever and systemic manifestations. It carries a great morbidity and is potentially fatal. The exact pathogenesis is not clear, and it has been proposed to be the result of hypersensitivity reaction to an infection. We report a patient with febrile ulceronecrotic Mucha-Habermann disease in a 12-year-old boy in whom the condition was most likely precipitated by parvovirus infection, and he showed a favorable response to a combination of prednisolone with narrow band ultraviolet B (NB-UVB) phototherapy.
* It often leads to serious complications including issues in the blood, lungs, heart, gut, and brain.
* Treatment typically involves corticosteroids and immunosuppressants like methotrexate, with a case presented showing a gradual treatment strategy for a patient not responding well to methotrexate.
- FUMHD is a rare skin condition characterized by rapid onset of painful necrotic lesions, systemic symptoms like fever, and a complex diagnosis that requires ruling out other severe skin diseases.
- A case study of a 13-year-old patient highlights a comprehensive treatment approach using corticosteroids, immunosuppressive therapy, and supportive care to manage the aggressive nature of FUMHD effectively.
- The report emphasizes the importance of early diagnosis, prompt treatment, monitoring for infections, and addressing the psychological and social needs of the patient to enhance overall outcomes.
Mucha-Habermann disease (MHD) is an inflammatory skin condition with symptoms resembling cutaneous T-cell lymphoma, while its severe variant, febrile ulceronecrotic MHD (FUMHD), presents with systemic symptoms and ulcers.
A case study of a 21-year-old woman showed atypical lymphomatoid features of FUMHD alongside hemophagocytic lymphohistiocytosis (HLH), leading to significant clinical findings and a biopsy that indicated atypical CD8 + lymphocyte infiltration.
Treatment using etoposide and dexamethasone resulted in rapid improvement of her symptoms, with the patient's skin lesions healing over time and confirming the MHD diagnosis; she has remained
Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a serious skin disorder that can lead to severe health complications in children and requires prompt management to prevent fatal outcomes.
Treatment options are limited due to the few reported cases and lack of substantial research, making it challenging to find effective therapies.
A case study of a five-year-old child with FUMHD and seizures showed that while multiple treatments were ineffective, a single infusion of low-dose intravenous immunoglobulin led to rapid improvement and disease control.
Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare fulminant variant of pityriasis lichenoides et varioliformis acuta (PLEVA) that is characterized by a large ulceronecrotic appearance with high fever and a variety of systemic symptoms. We report here a case of FUMHD in a 17-year-old male Chinese patient who was treated successfully with a combination therapy of methotrexate, methylprednisolone, and intravenous immunoglobulin. In addition, a literature review was conducted to summarize the key characteristics of paediatric FUMHD cases.
