Febrile Ulceronecrotic Mucha-Habermann’s Disease with Pulmonary Involvement

Zhang, Lixin; Liang, Yuan; Liu, Ying; Ma, Lin

doi:10.1111/j.1525-1470.2010.01141.x

Cited by 18 publications

(14 citation statements)

References 13 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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“…Treatment modalities for FUMHD may overlap with PL; however, given its severity with rapid progression, systemic complications, and potential fatality, more aggressive medications are utilized. Treatment options include Tumor Necrosis Factor (TNF)-alpha inhibitors [22], methotrexate [17,19,23], prednisone [19], intravenous immune globulin (IVIG) and extracorporeal photopheresis [24].…”

Section: Pityriasis Lichenoidesmentioning

confidence: 99%

Pityriasis Lichenoides and Cutaneous T Cell Lymphoma: An Update on the Diagnosis and Management of the Most Common Benign and Malignant Cutaneous Lymphoproliferative Diseases in Children

et al. 2013

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Although more common with advanced age, the entire spectrum of benign and malignant cutaneous lymphoproliferative disorders can present in the pediatric population. Nevertheless, these conditions are often mistaken for other more common, benign dermatoses, resulting in delayed diagnosis. Standardized management of these conditions is also lacking. The purpose of this review is to provide an update on the most common benign and malignant cutaneous lymphoproliferative disorders in children: pityriasis lichenoides and mycosis fungoides. The presentation, evaluation and management of each of these conditions are discussed.

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“…Most patients with FUMHD initially received a combination of systemic corticosteroids and erythromycin, as did our case. Beyond these first-line therapies, several case reports described success with methotrexate in treating FUMHD, 2,3,5,6 these include a handful of paediatric case reports, 5,[9][10][11][12][13][14][15][16][17][18][19][20][21] which suggest that methotrexate may be a safe and effective treatment for FUMHD in children (Table 1). Other reported treatment modalities for FUMHD include UV light therapy, cyclosporine, dapsone, acitretin and acyclovir.…”

Section: E84mentioning

confidence: 99%

“…Reported paediatric cases of febrile ulceronecrotic Mucha-Habermann disease 5,[9][10][11][12][13][14][15][16][17][18][19][20][21]…”

mentioning

confidence: 99%

Febrile ulceronecrotic Mucha‐Habermann disease: A case with systemic symptoms managed with subcutaneous methotrexate

Lin¹,

Cook

Purvis³

2011

Aust J Dermatology

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Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare, idiopathic, acquired dermatosis that can affect all ages and ethnic groups. We present a 10-year-old patient with FUMHD associated with arthritis and chronic fatigue, managed with methotrexate. Through our literature review, we also explore treatment protocols for a disease for which internationally standardized management is yet to be formulated.

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Febrile Ulceronecrotic Mucha-Habermann’s Disease with Pulmonary Involvement

Cited by 18 publications

References 13 publications

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

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

Pityriasis Lichenoides and Cutaneous T Cell Lymphoma: An Update on the Diagnosis and Management of the Most Common Benign and Malignant Cutaneous Lymphoproliferative Diseases in Children

Febrile ulceronecrotic Mucha‐Habermann disease: A case with systemic symptoms managed with subcutaneous methotrexate

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