Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Febrile Ulceronecrotic Mucha-Habermann disease


Other Names for this Disease
  • FUMHD
  • Ulceronecrotic Mucha-Habermann disease
  • Variant of Mucha-Habermann disease
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Treatment

Newline Maker

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][6][7] 

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] The state of these patients is similar to what is seen in patients with severe burns. Thus, patients with FUMHD may benefit from the same supportive services that burn victims receive.[7]


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][8] 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: 9/15/2015

References
  1. 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;
  2. Yang CC, Lee JY, Chen W. Febrile ulceronecrotic Mucha-Habermann disease with extensive skin necrosis in intertriginous areas. Eur J Dermatol. 2003 Sep-Oct;
  3. 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;
  4. Bowers S, Warshaw EM. Pityriasis lichenoides and its subtypes. J Am Acad Dermatol. 2006;
  5. 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;
  6. Oliveira L, Rocha M, Patriota G, Cunha G, Paiva G, Souza A, Fauth A, de Moura C, Cruz C. Febrile Ulceronecrotic Mucha Habermann Disease: Case Report of a Dark-Skinned Patient. Case Rep Dermatol. 2013 Jan-Apr; 5(1):4-10. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3573822/. Accessed 9/15/2015.
  7. Harenberg P, Hrabowski M, Ryssel H, Gazyakan E, Germann G, Engel H, Reichenberger M. CASE REPORT Febrile Ulceronecrotic Mucha-Habermann Disease. Eplasty. 2010 Jul 16; 10:e53. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2905190/. Accessed 9/15/2015.
  8. 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;


GARD Video Tutorial

  • Finding Treatment Information - A video developed by GARD Information Specialists that explains how you can find information about treatment for a rare disease.

    Finding Treatment Information

Clinical Trials & Research for this Disease

  • The U.S. National Institutes of Health, through the National Library of Medicine, developed ClinicalTrials.gov to provide patients, family members, and members of the public with current information on clinical research studies. There is a study titled Evaluation and Treatment of Patients with Dermatologic Diseases which may be of interest to you. To find this trial, click on the link above.
  • The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. There is a study titled Dermatology Consultation Clinic and Clinical Research that may be of interest to you. You may want to contact the investigator, Maria Turner (maria.turner@nih.gov) to learn more.
Other Names for this Disease
  • FUMHD
  • Ulceronecrotic Mucha-Habermann disease
  • Variant of Mucha-Habermann disease
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.