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Febrile Ulceronecrotic Mucha-Habermann disease
Other Names for this Disease
- A severe variant of pityriasis lichenoides et varioliformis acuta (PLEVA)
- Ulceronecrotic Mucha-Habermann disease
- Variant of Mucha-Habermann disease
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Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and severe form of pityriasis lichenoides et varioliformis acuta (PLEVA). PLEVA is characterized by skin lesions that ulcerate, breakdown, form open sores, then form a red-brown crust. FUMHD often begins as PLEVA, but then rapidly and suddenly progresses to large, destructive ulcers. There may be fever and extensive, painful loss of skin tissue as well as secondary infection of the ulcers. Diagnosis of FUMHD is confirmed by biopsy of skin lesions. FUMHD occurs more frequently in children, peaking at age 5 to 10. Males tend to be affected more often than females. While some cases of FUMHD have resolved without therapy, others have resulted in death. Early diagnosis and prompt treatment may help to reduce morbidity and death.
- 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.
- Hoghton MA, Ellis JP, Hayes MJ. Febrile ulceronecrotic Mucha Habermann disease: a fatality. J R Soc Med. 1989 Aug.
- Miller ML. Miscellaneous Conditions Associated with Arthritis. In: Kliegman. Nelson Textbook of Pediatrics, 18th ed. Philadelphia, PA: Saunders; 2007.
- Bowers S, Warshaw EM. Pityriasis lichenoides and its subtypes. J Am Acad Dermatol. 2006.
- The British Association of Dermatologists provides an informational leaflet on pityriasis lichenoides. Click on the British Association of Dermatologists link to view this leaflet.
- DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition. Click on the link to view the information.
- Medscape Reference provides information on PLEVA which includes information on FUMHD. You may need to register to view the article, but registration is free.
- The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Febrile Ulceronecrotic Mucha-Habermann disease. Click on the link to view a sample search on this topic.
- The medical website TheDoctorsDoctor also provides information on PLEVA and FUMHD. Click on the link above to view the information page.
Selected Full-Text Journal Articles
- 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;11(3):31.
- Yang CC, Lee JY, Chen W. Febrile ulceronecrotic Mucha-Habermann disease with extensive skin necrosis in intertriginous areas. Eur J Dermatol. 2003 Sep-Oct;13(5):493-6.
- 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;56(10):795-7.
- Auster BI, Santa Cruz DJ, Eisen AZ. Febrile Ulceronecrotic Mucha-Habermann's Disease with Interstitial Pneumonitis. Journal of Cutaneous Pathology. 2006:6(1);66-76.
Resources for Kids
- Kids Skin Health, a American Academy of Dermatology's web site, provides kids, teens, and parents with information on skin conditions. Click on Kids Skin Health to access this Web site.