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Genetic and Rare Diseases Information Center (GARD)

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Febrile Ulceronecrotic Mucha-Habermann disease


Other Names for this Disease
  • FUMHD
  • Ulceronecrotic Mucha-Habermann disease
  • Variant of Mucha-Habermann disease
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Your Question

My 7-year old was diagnosed with febrile ulceronecrotic Mucha-Habermann disease (FUMHD). He spent 40 days in the ICU and was treated with many different medications. He is now recovering. Where can I learn about new treatments for this condition?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

How is febrile ulceronecrotic Mucha-Habermann disease (FUMHD) treated?

It is important that FUMHD is diagnosed and treated as soon as possible.[1] While a number of treatments have been tried, it is hard to asses the benefit of the therapies because there are so few cases of FUMHD and among reported cases the treatment approach may vary. The case reports describe treatment with systemic steroids, methotrexate, antibiotics, dapsone, cyclosporine, psoralen and ultraviolet A (PUVA), ultraviolet B (UVB), unspecified ultraviolet receptor, acyclovir, immunoglobulins, and 4,4-diaminodiphenylsulphone (DDS). Again the efficacy of these therapies are not known.[1][2][3][4][5]

Acyclovir was prescribed in cases where varicella was initially suspected. None of these cases turned out to be associated with herpes simplex or varicella-zoster virus infection. The benefit of acyclovir therapy in people with FUMHD is questionable.[2]

Systemic steroids have been commonly utilized among reported cases (27 of 40 cases), with only one report of a positive effect.[5] Methotrexate has been used in 15 patients. It induced rapid remissions and was successful in cases that did not respond to other therapies. Still four patients died despite methotrexate theapy. It is possible this was due to its late institution.[5]

Debridement and skin grafting was successful in one case, but the patient was left with considerable scaring.[1]

In advanced disease, therapy is also aimed at stabilizing the patient. Intensive care treatment of infection and maintenance of the patient’s general condition is vital.[1][5]

Treatment with tumor necrosis factor (TNF)-alpha inhibitors (such as infliximab and etanercept) has been suggested as a first-line option in the management of FUMHD because elevated levels of serum TNF-alpha have been reported in this disease[5][6] However, further studies may be required to establish this approach to treatment.

More detailed information about treatment options for FUMHD can be accessed through the DermNet NZ web site.
Last updated: 9/7/2012

Where can I learn about new treatment options for febrile ulceronecrotic Mucha-Habermann (FUMHD) disease? 

You can find relevant articles on treatment of FUMHD through PubMed, a searchable database of biomedical journal articles. Although not all of the articles are available for free online, most articles listed in PubMed have a summary available. To obtain the full article, contact a medical/university library or your local library for interlibrary loan. You can also order articles online through the publisher’s Web site. Using "febrile ulceronecrotic Mucha-Habermann disease AND treatment" as your search term should help you locate articles. Use the advanced search feature to narrow your search results. Click here to view a search.
http://www.ncbi.nlm.nih.gov/PubMed

The National Library of Medicine (NLM) Web site has a page for locating libraries in your area that can provide direct access to these journals (print or online). The Web page also describes how you can get these articles through interlibrary loan and Loansome Doc (an NLM document-ordering service). You can access this page at the following link http://nnlm.gov/members/. You can also contact the NLM toll-free at 888-346-3656 to locate libraries in your area.

Last updated: 9/7/2012

References
  • Aytekin S, Balci G, Duzgun OY. Febrile ulceronecrotic Mucha-Habermann disease: a case report and a review of the literature. Dermatol Online J. 2005 Dec 1;
  • Yang CC, Lee JY, Chen W. Febrile ulceronecrotic Mucha-Habermann disease with extensive skin necrosis in intertriginous areas. Eur J Dermatol. 2003 Sep-Oct;
  • Miyamoto T, Takayama N, Kitada S, Hagari Y, Mihara M. Febrile ulceronecrotic Mucha-Habermann disease: a case report and a review of the literature. J Clin Pathol. 2003 Oct;
  • Bowers S, Warshaw EM. Pityriasis lichenoides and its subtypes. J Am Acad Dermatol. 2006;
  • Sotiriou E, Patsatsi A, Tsorova C, Lazaridou E, Sotiriadis D. Febrile ulceronecrotic Mucha-Habermann disease: a case report and review of the literature. Acta Derm Venereol. 2008;
  • Tsianakas A, Hoeger PH. Transition of pityriasis lichenoides et varioliformis acuta to febrile ulceronecrotic Mucha-Habermann disease is associated with elevated serum tumour necrosis factor-alpha. Br J Dermatol. 2005 Apr;