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Febrile Ulceronecrotic Mucha-Habermann Disease (febrile + ulceronecrotic_mucha-habermann_disease)
Selected AbstractsFebrile Ulceronecrotic Mucha-habermann Disease: a Rare, Severe VariantJOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2006Michele M. Thompson A 56 year old Hispanic man presented with extensive ulcerative skin lesions, involving his lower trunk, groin and upper legs, severe pain and a temperature of 38.7 degrees Celsius. He was admitted to the Medical Intensive Care Unit for empiric intravenous antibiotics. Several biopsies were performed. In the following days his condition worsened and ulcerative lesions involved nearly all of his skin. Previous biopsies were consistent with pityriasis lichenoides et varioliformis acuta (PLEVA), however, neither this, nor others in the histological differential diagnosis, fit his severe and worsening clinical picture. Histology revealed vacuolar alteration with dyskeratotic keratinocytes and a superficial perivascular mixed infiltrate of lymphocytes and eosinophils. There was confluent parakeratosis containing neutrophils, and a diminished granular layer with pallor in the upper portion of the spinous layer. Immunofluorescence studies were negative. These findings were consistent with PLEVA. A clinical diagnosis of febrile ulceronecrotic Mucha-Habermann disease was made. Febrile ulceronecrotic Mucha-Habermann disease is a rare and severe variant of PLEVA characterised by high fever and papulonecrotic skin lesions. Twenty-five cases have been previously reported. We present the clinical and histological findings in this unusual clinical presentation. [source] High-dose immunoglobulines and extracorporeal photochemotherapy in the treatment of febrile ulceronecrotic Mucha-Habermann diseaseDERMATOLOGIC THERAPY, Issue 4 2010Federica Marenco ABSTRACT Febrile ulcero-necrotic Mucha-Habermann disease (FUMHD) is a rare subtype of pityriasis lichenoides et varioliformis acuta (only 41 cases described to date), characterized by an acute onset of ulcero-necrotic papules accompanied by high fever and severe constitutional symptoms. We report a case of a 23-year-old man with a steroid-resistant FUMHD treated by intravenous immunoglobulins (IVIG) combined with methotrexate. Only one case of FUMHD treated by IVIG has been reported to date in literature. Also in our case, IVIG proved to be effective in inducing a dramatic improvement of ulceration and in arresting the appearance of new lesions. Moreover, in our experience we decided to perform a maintenance treatment with extracorporeal photochemotherapy (ECP), to the best of our knowledge not previously used in the treatment of pityriasis lichenoides et varioliformis acuta. ECP, which involves extracorporeal exposure of peripheral blood mononuclear cells to photo-activated 8-methoxypsoralen, induces an immunological reaction against auto-reactive T cell clones, without immune-depression and thus could potentially be useful particularly in FUMHD avoiding the risk of an infective reactivation. [source] Febrile Ulceronecrotic Mucha-habermann Disease: a Rare, Severe VariantJOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2006Michele M. Thompson A 56 year old Hispanic man presented with extensive ulcerative skin lesions, involving his lower trunk, groin and upper legs, severe pain and a temperature of 38.7 degrees Celsius. He was admitted to the Medical Intensive Care Unit for empiric intravenous antibiotics. Several biopsies were performed. In the following days his condition worsened and ulcerative lesions involved nearly all of his skin. Previous biopsies were consistent with pityriasis lichenoides et varioliformis acuta (PLEVA), however, neither this, nor others in the histological differential diagnosis, fit his severe and worsening clinical picture. Histology revealed vacuolar alteration with dyskeratotic keratinocytes and a superficial perivascular mixed infiltrate of lymphocytes and eosinophils. There was confluent parakeratosis containing neutrophils, and a diminished granular layer with pallor in the upper portion of the spinous layer. Immunofluorescence studies were negative. These findings were consistent with PLEVA. A clinical diagnosis of febrile ulceronecrotic Mucha-Habermann disease was made. Febrile ulceronecrotic Mucha-Habermann disease is a rare and severe variant of PLEVA characterised by high fever and papulonecrotic skin lesions. Twenty-five cases have been previously reported. We present the clinical and histological findings in this unusual clinical presentation. [source] A case of febrile ulceronecrotic Mucha-Habermann's disease successfully treated with oral cyclosporinJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 2 2007HS Kim [source] | ||||||||||