A fatal case of febrile ulceronecrotic Mucha-Habermann disease in a child

Nofal, Ahmad; Alakad, Rania; Assaf, Magda; Nofal, Eman

doi:10.1016/j.jdcr.2016.03.002

Cited by 26 publications

(32 citation statements)

References 12 publications

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“…choices include prednisone, methotrexate [8, 13, 15, and 16], TNF-alpha inhibitors [17,13] cyclosporine [2], intravenous immunoglobulins (IVIG), and extracorporeal photopheresis [18]. In our case, combination treatment of high dose corticosteroids with IVIG was of considerable benefit.…”

Section: Treatmentmentioning

confidence: 83%

“…The exact pathogenesis of FUMHD remains unclear, with no universally effective treatment [1]. As far as we know, only one case was reported in our country (Lower Egypt) in children by Nofel et al in 2016 [2]. However, no cases were reported from Upper Egypt.…”

Section: Introductionmentioning

confidence: 85%

“…Children and young adults are more vulnerable. In contrast to adults, children may take a shorter duration to transform from PLEVA to FUMHD with more common mucosal involvement and a more favourable prognosis [12], although deaths have been reported [2]. Affected patients show a rapid onset of necrotic papules coalescing into large ulcerations with histologic features of PLEVA [13].…”

Section: Febrile Ulceronecroticmentioning

confidence: 99%

See 2 more Smart Citations

Psoriasiform Febrile Ulceronecrotic Mucha Habermann Disease: A New Clinical Presentation of a Case Report from Upper Egypt

2019

ARC Journal of Dermatology

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Section: Treatmentmentioning

confidence: 83%

Section: Introductionmentioning

confidence: 85%

Section: Febrile Ulceronecroticmentioning

confidence: 99%

See 1 more Smart Citation

Psoriasiform Febrile Ulceronecrotic Mucha Habermann Disease: A New Clinical Presentation of a Case Report from Upper Egypt

2019

ARC Journal of Dermatology

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“…Of the few reported cases, roughly half occurred in patients younger than 20 years old, and a strong male predominance was observed . Despite mortality approaching 15%, only one death has been reported among children . There is no standard treatment, but in patients with favorable outcomes, methotrexate is often used .…”

Section: Discussionmentioning

confidence: 99%

Severe Mucha–Habermann‐Like Ulceronecrotic Skin Disease in T‐Cell Acute Lymphoblastic Leukemia Responsive to Basiliximab and Stem Cell Transplant

Orenstein

Coughlin

Flynn

et al. 2017

Pediatric Dermatology

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A 5-year-old girl with T-cell acute lymphoblastic leukemia (T-ALL) developed a progressive eruption of crusted papules and ulcerative plaques involving 80% of her body surface area with histopathology consistent with febrile ulceronecrotic Mucha-Habermann disease (FUMHD), although multiple specimens also contained clonal leukemic cells. Her skin disease was refractory to many classic treatments for FUMHD, including methotrexate, and became so severe that concern about superinfection prevented intensification of chemotherapy for her malignancy. The addition of basiliximab promoted gradual improvement of the skin, allowing for chemotherapy intensification and subsequent bone marrow transplantation, after which the eruption resolved completely. This report describes a severe case of FUMHD-like eruption associated with clonal leukemic cells that improved with basiliximab, suggesting anti-CD25 therapy as a novel treatment for ulceronecrotic skin disease in the setting of high interleukin-2 levels.

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“…[ 3 ] There have been only one case of a child fatality reported so far. [ 4 ] Fatal outcomes have been attributed to pulmonary thromboembolism, pneumonia, sepsis, hypovolemic shock, cardiac arrest, and thrombosis of superiormesenteric artery. The etiology of this disease is unknown, may be related to infectious antigens (such as EB virus, adenovirus, CMV).…”

Section: Discussionmentioning

confidence: 99%

A fatal case of febrile ulceronecrotic mucha-habermann disease which presenting as toxic epidermal necrolysis

Xing

Shen

et al. 2017

Indian J Dermatol

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Febrile ulceronecrotic Mucha-Habermann disease (FUMHD), a severe form of pityriasis lichenoides et varioliformis acuta, is an inflammatory dermatosis of unknown etiology manifested by ulcerative and necrotic lesions accompanied by systemic manifestations. The mortality rate of FUMHD is about 15%. It is reported here a case of FUMHD presenting as toxic epidermal necrolysis that resulted in multiple organ failure and death.

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A fatal case of febrile ulceronecrotic Mucha-Habermann disease in a child

Cited by 26 publications

References 12 publications

Psoriasiform Febrile Ulceronecrotic Mucha Habermann Disease: A New Clinical Presentation of a Case Report from Upper Egypt

Psoriasiform Febrile Ulceronecrotic Mucha Habermann Disease: A New Clinical Presentation of a Case Report from Upper Egypt

Severe Mucha–Habermann‐Like Ulceronecrotic Skin Disease in T‐Cell Acute Lymphoblastic Leukemia Responsive to Basiliximab and Stem Cell Transplant

A fatal case of febrile ulceronecrotic mucha-habermann disease which presenting as toxic epidermal necrolysis

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