A Case of Ulceronecrotic Mucha-Habermann Disease with Pulmonary Involvement

Aydıngöz, İkbal Esen; Kocaayan, Nurhan; At, Mansur; Yaşar, Şirin; Arman, Alper

doi:10.1159/000092294

Cited by 8 publications

(8 citation statements)

References 8 publications

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“…in 1966, about 65 cases of FUMHD have been reported; however, no definite diagnostic criteria have been established, and an optimum treatment is not yet determined. Herein, we review the different aspects of FUMHD, suggesting two sets of diagnostic criteria and evaluating the different diagnostic and therapeutic challenges associated with this rare entity …”

Section: Introductionmentioning

confidence: 99%

Febrile ulceronecrotic Mucha–Habermann disease: proposed diagnostic criteria and therapeutic evaluation

et al. 2015

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Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare severe variant of pityriasis lichenoides et varioliformis acuta characterized clinically by aggressive ulceronecrotic skin lesions associated with high fever and histologically by features typical of pityriasis lichenoides et varioliformis acuta. Despite the continuous addition of new case reports, no definite diagnostic criteria have been established, and an optimum treatment is still waiting. Herein, we review the different aspects of this rare entity, including pathogenesis, clinical and histopathological features, differential diagnosis, course, prognosis, and outcome. Different diagnostic and therapeutic challenges associated with FUMHD are also evaluated and discussed. We propose two sets of diagnostic criteria to define the disease more precisely and to avoid missing cases. The first comprises constant clinical and histopathological features that are always present in every case, the combination of which is necessary for diagnosis. The second set includes variable features that may be present in some cases and to which any emerging finding could be added. Although different therapeutic options have been used, there is no optimum therapy for FUMHD, and the disease still represents a therapeutic challenge.

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Section: Introductionmentioning

confidence: 99%

Febrile ulceronecrotic Mucha–Habermann disease: proposed diagnostic criteria and therapeutic evaluation

et al. 2015

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“…It is a systemic disease characterized by rapid progression of necrotic papules to large coalescent ulcers with necrotic crusts, haemorrhagic bullae, and pustules and systemic manifestations (high fever, sore throat, diarrhoea, central nervous system symptoms, abdominal pain, pulmonary involvement, splenomegaly, arthritis, sepsis and megaloblastic anaemia) [1,2] . A total of 28 cases, including the case reported here, have been described to date, with 6 reported fatalities [3] .…”

Section: Introductionmentioning

confidence: 99%

Intravenous Immunoglobulins: A Valuable Asset in the Treatment of a Case of Septic Febrile Ulceronecrotic Mucha-Habermann Disease

et al. 2007

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“…Pulmonary involvement may reflect a specific complication of FUMHD that is rarely seen during the course of FUMHD. Indeed, this is the third reported case of lung involvement among the 39 cases that have been reported so far (3,7). Aydingoz et al (7) reported a similar case, and fine‐needle aspiration lung biopsy revealed granuloma‐like structures consisting of alveolar macrophages, epithelium, lymphocytes, and epitheliod histiocytes.…”

Section: Discussionmentioning

confidence: 76%

Febrile Ulceronecrotic Mucha-Habermann’s Disease with Pulmonary Involvement

Zhang

Liang

Liu

et al. 2010

Pediatric Dermatology

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We report a case of febrile ulceronecrotic Mucha-Habermann disease (FUMHD) in a 12-year-old boy. After 1 week of typical cutaneous appearance of pityriasis lichenoides et varioliformis acuta (PLEVA), he was given a percutaneous injection of measles virus vaccine, and then extensive polymorphous, papular, and ulcerohemorrhagic skin lesions developed along with intermittent high temperature, hepatic dysfunction, and hypoalbuminemia. Thoracic CT scan showed parenchymal nodular infiltration at the tip of vascular structures. Skin biopsy showed a combination of the classic features of PLEVA and an allergic vasculitis. We treated him with prednisolone and methotrexate (MTX) successfully. The evolution in our patient suggests that FUMHD may have been triggered by virus vaccination. We also emphasize that FUMHD in children may be successfully treated with a combination of systemic MTX and high-dose corticosteroids.

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A Case of Ulceronecrotic Mucha-Habermann Disease with Pulmonary Involvement

Cited by 8 publications

References 8 publications

Febrile ulceronecrotic Mucha–Habermann disease: proposed diagnostic criteria and therapeutic evaluation

Febrile ulceronecrotic Mucha–Habermann disease: proposed diagnostic criteria and therapeutic evaluation

Intravenous Immunoglobulins: A Valuable Asset in the Treatment of a Case of Septic Febrile Ulceronecrotic Mucha-Habermann Disease

Febrile Ulceronecrotic Mucha-Habermann’s Disease with Pulmonary Involvement

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