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NORD is very grateful to 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 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.
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.
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.
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.
Symptoms of the following disorders can be similar to those of Mucha-Habermann disease. Comparisons may be useful for a differential diagnosis.
Lymphomatoid papulosis is a rare skin disorder that some researchers believe is an early from of CD30+ lymphoma. The disorder is characterized by groups of slightly-elevated, reddish-brown bumps (nodules or papules) that most often affect the trunk, face, and arms and legs. These lesions often become crusted or ulcerated, sometimes leaving a scar. Approximately 5-20 percent of individuals with lymphomatoid papulosis eventually develop cutaneous T-cell lymphoma (CTCL). Other researchers believe that lymphomatoid papulosis is a similar, yet distinct, "premalignant" condition and not a form of CTCL. (For more information on this disorder, choose "cutaneous T-cell lymphoma" as your search term in the Rare Disease Database.)
Gianotti-Crosti syndrome is a rare skin disorder characterized by swollen red bumps on the skin that may or may not itch. They are usually found on the face, buttocks, arms or legs, often clustered on the elbows and knees. They represent a skin reaction to a wide variety of viruses, most commonly Epstein-Barr virus in the United States. Although many affected children otherwise feel perfectly fine, some have an associated upper respiratory tract infection. The bumps usually last from 4-6 weeks; they do not usually recur. There may be an enlargement of the lymph nodes in the trunk or neck areas of the body. (For more information on this disorder, choose "Gianotti-Crosti" as your search term in the Rare Disease Database.)
Psoriasis is a chronic, inflammatory skin disease characterized by dry, reddish (erythematous), thickened patches of skin that are covered with silvery-gray scales. These patches may be referred to as papules or plaques and most often affect the elbows, knees, hands, feet, buttock and/or lower back. The plaques may be intensely itchy (pruritic) or sore. In some cases, individuals with psoriasis may experience abnormalities affecting the fingernails, toenails, and the scalp. The severity of psoriasis varies from case to case. Psoriasis may be classified as mild, moderate or severe depending upon the amount of skin involved and the effect on an individual's quality of life. In approximately one-third of cases a family history of psoriasis is present. (For more information on this disorder, choose "psoriasis" as your search term in the Rare Disease Database.)
Pityriasis rosea is a self-limited, mild, inflammatory skin disorder characterized by scaly lesions that follow a pattern on the skin. Often, a large lesion is seen initially (herald patch). Lesions are found most commonly on the trunk. The disorder is possibly due to an unidentified infectious agent. It may occur at any age but is seen most frequently in young adults. In temperate climates, incidence is highest during spring and autumn.
A variety of additional general skin conditions including erythema multiforme, chicken pox, lichen planus, and leukocytoclastic vasculitis may resemble Mucha-Habermann disease. In some cases, skin lesions resulting from an abnormally heightened response to a bite from an insect or arthropod (e.g., spiders) may resemble Mucha-Habermann disease. (For more information on these disorders, choose the specific disorder name in the Rare Disease Database.)
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.
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.
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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.
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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.
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.
Report last updated: 2012/02/07 00:00:00 GMT+0