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Disease Profile
Febrile Ulceronecrotic Mucha-Habermann disease
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
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Age of onset
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ICD-10
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Inheritance
Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.
Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.
X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Not applicable
Other names (AKA)
FUMHD; Ulceronecrotic Mucha-Habermann disease; Variant of Mucha-Habermann disease
Categories
Skin Diseases
Summary
Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and severe form of pityriasis lichenoides et varioliformis acuta (PLEVA).[1][2][3][4][5][6] 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
Symptoms
Cause
Diagnosis
- Epidermis Findings include focal confluent parakeratosis, spongiosis, dyskeratosis, mild to moderate acanthosis, vacuolization of basal layer with necrotic keratino-cytes, occasional intraepidermal vesicles, extensive epidermal necrosis. In advanced disease findings may also include extension of infiltrate into epidermis, invasion of
erythrocytes , widespread epidermal necrosis, and nuclear debris in necrotic areas - Dermis – Swelling, moderately dense lymphohistiocytic perivascular inflammatory infiltrate usually without atypia, extravasation of
lymphocytes and erythrocytes with epidermal invasion, subepidermal vesicles in later lesions, dermal sclerosis in older lesions - Vascular changes – Dilation and engorgement of blood vessels in papillary dermis with endothelial proliferation, vascular congestion, occlusion, dermal hemorrhage, and extravasation of erythrocytes
- Vasculitis – Fibronoid necrosis of vessel walls with leukocytoclassic vasculitis
In the majority of patients, blood tests indicate leukocytosis,
Treatment
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
Systemic steroids have been commonly utilized among reported cases (27 of 40 cases), with only one report of a positive effect.[7] 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.[7]
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][7] 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.[9]
Treatment with
More detailed information about treatment options for FUMHD can be accessed through the DermNet NZ web site.
Organizations
Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.
Organizations Supporting this Disease
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American Osteopathic College of Dermatology
P.O. Box 7525
Kirksville, MO 63501
Toll-free: 800-449-2623
Telephone: 660-665-2184
Fax: 660-627-2623
E-mail: [email protected]
Website: https://www.aocd.org/ -
British Association of Dermatologists
19 Fitzroy Square
London, UK W1T 6EH
United Kingdom
Telephone: 0207 383 0266
Fax: 0207 388 5263
E-mail: [email protected]
Website: https://www.bad.org.uk
Organizations Providing General Support
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American Academy of Dermatology
1445 New York Ave, NW
Suite 800
Washington, DC 20005
Toll-free: 888-462-DERM (3376)
Fax: 847-240-1859
E-mail: https://www.aad.org/Forms/ContactUs/Default.aspx
Website: https://www.aad.org/ -
American Autoimmune Related Diseases Association (AARDA)
19176 Hall Road, Suite 130
Clinton Township, MI 48038
Toll-free: 800-598-4668
Telephone: 586-776-3900
Fax: 586-776-3903
E-mail: [email protected]
Website: https://www.aarda.org/
Learn more
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
Where to Start
- 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.
- The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
- The medical website TheDoctorsDoctor also provides information on PLEVA and FUMHD. Click on the link above to view the information page.
In-Depth 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 Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- 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.
Resources for Kids
- The American Academy of Dermatology provides kids, teens, and parents with information on skin conditions.
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.
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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.
References
- Aytekin S, Balci G, Duzgun OY. Febrile ulceronecrotic Mucha-Habermann disease: a case report and a review of the literature. Dermatol Online J. December 1 2005; 11(3):31. https://www.ncbi.nlm.nih.gov/pubmed/16409927.
- Yang CC, Lee JY, Chen W. Febrile ulceronecrotic Mucha-Habermann disease with extensive skin necrosis in intertriginous areas. Eur J Dermatol. September-October 2003; 13(5):493-6. https://www.ncbi.nlm.nih.gov/pubmed/14693498.
- 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. October 2003; 56(10):795-797. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1770083/.
- Hoghton MA, Ellis JP, Hayes MJ. Febrile ulceronecrotic Mucha Habermann disease: a fatality. J R Soc Med. August 1989; 82(8):500-501. Febrile ulceronecrotic Mucha Habermann disease: a fatality.
- 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. October 2006; 55(4):557-72. https://www.ncbi.nlm.nih.gov/pubmed/17010734.
- 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; 88(4):350-5. https://www.ncbi.nlm.nih.gov/pubmed/18709304.
- 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. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3573822/.
- 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. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2905190/.
- 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. April 2005; 152(4):794-9. https://www.ncbi.nlm.nih.gov/pubmed/15840118.
- Cozzio A, Hafner J, Kempf W, Häffner A, Palmedo G, Michaelis S, Gilliet M, Zimmermann D, Burg G. Febrile ulceronecrotic Mucha-Habermann disease with clonality: A cutaneous T-cell lymphoma entity?. Am Acd Dermatol. December 2004; 51(6):1014-7. https://www.ncbi.nlm.nih.gov/pubmed/15583604.
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