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Last updated: 07/24/2024
Years published: 1990, 1995, 2003, 2009, 2012, 2015, 2018, 2023
NORD gratefully acknowledges Amy S. Paller, MD, Walter J. Hamlin Professor and Chair of Dermatology, Professor of Pediatrics, Northwestern University, Feinberg School of Medicine, for assistance in the preparation of this report.
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, arms and legs. Lesions tend to develop in small groups. Mucha-Habermann disease most often affects children or young adults. A more severe type of this disorder, known as febrile ulceronecrotic Mucha-Habermann disease, can cause life-threatening complications. The exact cause of Mucha-Habermann disease is unknown, but it may be an immune response to viruses and other triggers. 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 or chronic parapsoriasis. In some cases, the term Mucha-Habermann disease may be used to denote the entire spectrum.
The onset of Mucha-Habermann disease is usually sudden and is marked by the development of a recurrent rash, consisting of round, elevated lesions (papules or plaques) that may be itchy and burning. These lesions are usually reddish-purple to reddish-brown and may progress to develop a black-purple crust, representing tissue death (necrosis) and bleeding (hemorrhage). The lesions eventually blister, often causing scarring or temporary discoloration upon healing.
Although the trunk, arms and legs are most often affected by Mucha-Habermann disease, any part of the body may potentially develop skin lesions, including the scalp and the mouth. Lesions may number only a few to hundreds. Lesions may resolve without treatment in a few weeks but persist or recur on and off for years.
Most affected individuals do not have any symptoms other than the skin findings. However, some individuals may have headaches, fever, joint pain (arthralgia) and a general feeling or poor health (malaise). In some people, swelling of nearby lymph nodes (lymphadenopathy) may also occur.
Febrile Ulceronecrotic Mucha-Haberman Disease (FUMHD)
FUMHD is a rare, severe type 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, but progression to life-threatening complications such as infection of the blood (sepsis) occurs occasionally and more often in adults than in children.
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.
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. A range of causes have been suspected, including several reports of occurrence after infection or vaccination with SARS-CoV-2.
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.
The incidence of MuchaโHabermann disease in the United States has not been reported. A search of the medical literature identified 121 reports worldwide. Since FUMHD was first described, only about 80 patients have been reported. MuchaโHabermann disease affects males more often than females. 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.
A diagnosis of Mucha-Habermann disease is made based upon a thorough clinical evaluation, detailed patient history, identification of characteristic skin lesions and, if needed for confirmation, microscopic examination (biopsy) of affected skin tissue.
Treatment
Mucha-Habermann disease usually resolves on its own within several weeks to several months. However, several therapies have been used to treat the condition. 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 lesions. Methotrexate or dapsone may be necessary in severe cases.
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
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Some current clinical trials also are posted on the following page on the NORD website:
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For information about clinical trials sponsored by private sources, in the main, contact:
www.centerwatch.com
For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/
TEXTBOOKS
Paller AS, Mancini AJ: Hurwitzโs Pediatric Dermatology, 6th Edition (London, Elsevier), 2022, pp. 100-101.
JOURNAL ARTICLES
Alhawsawi W, Al Hawsawi K, Almahdi B, Alkidaiwi S, Alzubaidy K, Alhuthayli R, Algethami A, Alshareef A. A diagnostically challenging sase of de novo febrile ulceronecrotic Mucha-Habermann disease with fatal pulmonaryiInvolvement: A case report. Case Rep Dermatol. 2023 Jan 18;15(1):17-21.
Feschuk AM, Green M, Kashetsky N, Maibach HI. Pityriasis lichenoides following SARS-CoV-2 infection/vaccination. Curr Dermatol Rep. 2023;12(1):27-32. doi: 10.1007/s13671-023-00380-1.
Blohm ME, Ebenebe CU, Rau C, Escherich C, Johannsen J, Escherich G, Driemeyer J,
Nagel PD, Kobbe R, Lรผtgehetmann M, Lennartz M, Booken N, Schneider SW, Singer D. Mucha-Habermann disease: A pediatric case report and proposal of a risk score. Int J Dermatol. 2022 Apr;61(4):401-409.
Hrin ML, Bowers NL, Jorizzo JL, Feldman SR, Huang WW. Methotrexate for pityriasis lichenoides et varioliformis acuta (Mucha-Habermann disease) and pityriasis lichenoides chronica: A retrospective case series of 33 patients with an emphasis on outcomes. J Am Acad Dermatol. 2022 Feb;86(2):433-437.
Mรคkilรค T, Jeskanen L, Butina M, Harjama L, Ranki A, Mรคlkรถnen T, Kluger N.
Pityriasis lichenoides et varioliformis acuta after SARS-CoV-2 infection and relapse after vaccination. J Eur Acad Dermatol Venereol. 2022 Jun;36(6):e431-e433.
Raghavan SS, Wang JY, Gru AA, Marqueling AL, Teng JMC, Brown RA, Novoa RA, Kim Y, Zehnder J, Zhang BM, Rieger KE. Next-generation sequencing confirms T-cell clonality in a subset of pediatric pityriasis lichenoides. J Cutan Pathol. 2022 Mar;49(3):252-260. doi: 10.1111/cup.14143. Epub 2021 Oct 21.PMID: 34614220
Tang P, Chen JS, Wang H, Yang H. Febrile Ulceronecrotic Mucha-Habermann disease: A case report and a systematic review. Case Rep Dermatol. 2022 Jun 27;14(2):169-177.
Tasouli-Drakou V, Nguyen M, Guinn H, Hassan O, Butala S, Phan S. Mortality risk factors in febrile ulceronecrotic Mucha- Habermann disease: A systematic review of therapeutic outcomes and complications. Dermatol Reports. 2022 Nov 21;14(4):9492.
Lupu J, Chosidow O, Wolkenstein P, Bergqvist C, Ortonne N, Ingen-Housz-Oro S. Pityriasis lichenoides: A clinical and pathological case series of 49 patients with an emphasis on follow-up. Clin Exp Dermatol. 2021 Dec;46(8):1561-1566.
Jung F, Sibbald C, Bohdanowicz M, Ingram JR, Piguet V. Systematic review of the efficacies and adverse effects of treatments for pityriasis lichenoides. Br J Dermatol. 2020 Dec;183(6):1026-1032.
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.
INTERNET
Callen JP. Pityriasis Lichenoides. Medscape. Last Updated: Aug 24, 2022. Available at https://emedicine.medscape.com/article/1099078-overview Accessed Nov 13, 2023.
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