Mucha Habermann disease

Mucha Habermann disease

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Mucha Habermann disease is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • PLEVA
  • acuta guttale parapsoriasis
  • Habermann disease
  • parapsoriasis varioliformis acuta
  • pityriasis lichenoides et varioliformis acuta

Disorder Subdivisions

  • FUMHD
  • Febrile Ulceronecrotic Mucha-Habermann disease

General Discussion

Mucha-Habermann disease, also known as pityriasis lichenoides et varioliformis acuta or PLEVA, is a rare skin disorder. The lesions most often appear on the trunk and the arms and legs. Lesions tend to develop in small groups. Mucha-Habermann disease most often affects children or young adults. A more severe variant of this disorder, known as febrile ulceronecrotic Mucha-Habermann disease, can cause life-threatening complications in adults. The exact cause of Mucha-Habermann disease is unknown.



Mucha-Habermannn is considered to be the acute end of a spectrum of skin disease known as pityriasis lichenoides. The more chronic end is known as pityriasis lichenoides chronica. In some cases, the term Mucha-Habermann disease may be used to denote the entire spectrum.

Symptoms

The onset of Mucha-Habermann disease is usually sudden and is marked by the development of a recurrent rash consisting of rounded, elevated lesions (papules or macules) that may be itchy and burning. These lesions are usually reddish-purple to reddish-brown and may progress to develop a blackish-brown crust, tissue death (necrosis) and bleeding (hemorrhaging). The lesions eventually blister, often causing scarring or temporary discoloration upon healing.



Although the trunk and the arms and legs are most often affected by Mucha-Habermann disease, any part of the body may potentially develop skin lesions. Lesions may number only a few to more than one hundred. Lesions may resolve without treatment in a few weeks, but may recur on and off for years.



In most cases, affected individuals do not have any symptoms in addition to the skin findings. However, some individuals may have headaches, fever, joint pain (arthralgia), and a general feeling or poor health (malaise). In some cases, swelling of nearby lymph nodes (lymphadenopathy) may also occur.



Febrile Ulceronecrotic Mucha-Haberman Disease (FUMHD)

FUMHD is a rare, severe variant of Mucha-Habermann disease characterized by the rapid development of numerous black or necrotic bumps (papules) on the skin. These lesions may grow and spread rapidly, eventually combining (coalescing) into extremely painful ulcers and blisters. These lesions tend to be larger than those associated with the more common form of Mucha-Habermann disease. They may bleed, often scar upon healing, and may become infected.



FUMHD is associated with additional symptoms including a high fever, joint pain (arthritis), gastrointestinal abnormalities (e.g., diarrhea, sore throat, and abdominal pain), enlargement of the spleen, inflammation of the lungs (interstitial pneumonitis), and central nervous system abnormalities. FUMHD occurs more often in children than adults. However, in adults some cases have progressed to cause life-threatening complications such as infection of the blood (sepsis). Life-threatening complications have not been reported in children with FUMHD.



FUMHD usually lasts several months before resolving on its own, but recurs on and off for several years. Eventually, FUMHD may transform into the less severe form of Mucha-Habermann disease.

Causes

The exact cause of Mucha-Habermann disease is unknown. Mucha-Habermann disease is part of the spectrum of pityriasis lichenoides, a benign group of disorders. Within this spectrum is also pityriasis lichenoides chronica, in which the lesions are more persistent and are characterized as pink scaling round patches on the trunk and extremities. Researchers have speculated that pityriasis lichenoides occurs because of an exaggerated, inflammatory reaction (hypersensitivity) of the body to an infectious agent. However, no causative infectious agent has been identified.



Some researchers have suggested that Mucha-Habermann disease is a benign, self-healing lymphoproliferative disorder. Lymphoproliferative disorders are characterized by the overproduction of certain white blood cells called lymphocytes. These cells often accumulate in structures and tissues of the body potentially damaging them.

Affected Populations

Mucha-Habermann disease affects men more often than women. The disorder is most common in children and young adults, but can affect people of any age including newborns (with lesions present at birth) and the elderly. The incidence of Mucha-Habermann disease is unknown.

Standard Therapies

Diagnosis

A diagnosis of Mucha-Habermann disease is made based upon a thorough clinical evaluation, a detailed patient history, identification of characteristic skin lesions and microscopic examination (biopsy) of affected skin tissue.



Treatment

Mucha-Habermann disease usually resolves on its own within several weeks to several months. However, in some cases various therapies to treat condition may be used. Oral antibiotics can help to clear lesions in about 50% of affected individuals, particularly erythromycin in children and a tetracycline derivative in adults. Exposure to ultraviolet light is the most effective therapy, particularly if the pityriasis lichenoides is persistent. While individuals can show considerable improvement from summer sunlight, phototherapy (light treatments) with narrow band ultraviolet light is an alternative for more controlled light delivery and during months that are not sunny. PUVA (psoralens and ultraviolet A light) is less commonly used, given its greater associated risk of the development of skin cancer and accelerated skin aging. Topical corticosteroids and systemic antihistamines have been used to ease pruritus, but do not clear the eruption. Methotrexate or dapsone may be necessary in severe cases.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, in the main, contact:

www.centerwatch.com

References

TEXTBOOKS

Hayre N, Elgart ML. Mucha-Habermann Disease. NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:128-129.



Wood GS, Hu C, Garrett AL. Chapter 25: Paraporiasis and Pityriasis Lichenoides. In: Wolff K, Goldsmith L, Katz S, Gilchrest B, Paller AS, Leffell D: Fitzpatrick's Dermatology in General Medicine, 7th Edition (NY, McGraw-Hill), 2008, pp. 236-243.



Paller AS, Mancini AJ: Hurwitz's Pediatric Dermatology, 4th Edition (London, Elsevier), 2011, pp. 84-85.



JOURNAL ARTICLES

Bowers S, Warshaw EM. Pityriasis lichenoides and its subtypes. J Am Acad Dermatol. 2006;55:557-572.



Ersoy-Evans S, Greco MF, Mancini A, Subasi N, Paller AS. Pityriasis lichenoides in childhood: a retrospective review of 124 patients. J Am Acad Dermatol 2007; Feb;56(2):205-10.



Aydingoz I.E., Kocaayan N, Mansur AT, Pekcan S, Arman A. A case of ulceronecrotic Mucha-Habermann disease with pulmonary involvement. Dermatology. 2006;212:388-390.



Mitomoto 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;56:795-797.



INTERNET

Klein PA, Callen JP. Pityriasis Lichenoides. Emedicine. Last Updated:July 12, 2010. Available at: http://www.emedicine.com/derm/TOPIC334.HTM Accessed on: February 1, 2012.

Resources

NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases

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Email: getinfo@madisonsfoundation.org

Internet: http://www.madisonsfoundation.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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