The patient is a 27-year-old male who presents for evaluation of a minimally pruritic eruption affecting his arms and trunk. The rash is greater than 2 weeks in duration and has not responded to 1% hydrocortisone cream. He denies fever, malaise, and swollen glands, and he takes no oral medications. Examination reveals scattered erythematous papules of the affected areas, some hemorrhagic and slightly necrotic.
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Pityriasis lichenoides et varioliformis acuta (PLEVA), also known as Mucha Habermann disease, is an uncommon skin disorder of unknown etiology. It is a self-limited lymphoproliferative disease characterized by the presence of T-lymphocytic infiltrates, decreased Langerhan cells, and decreased CD4/CD8 ratio.1
PLEVA usually occurs during the first 3 decades of life. It is more common in males and is seen in all races.2 The rash is polymorphous, and patients often present with lesions in various stages of development. The onset is a macule that progresses to a papule with overlying scale. The rash can become vesicular with central necrosis. PLEVA typically occurs on the trunk, and extremities and can include the palms and soles. The rash is usually asymptomatic but may be pruritic or painful.1 Differential diagnosis includes insect bites, Gianotti-Crosti syndrome, and lymphomatoid papulosis.
PLEVA may respond to topical corticosteroids and calcineurin inhibitors.3,4 Oral erythromycin5 and ultraviolet light therapy6 may induce resolution, with methotrexate reserved for more chronic, refractory cases.7
Rebecca Geiger, PA-C, is a physician assistant on staff of the DermDox Dermatology Center in Hazleton, PA. Stephen Schleicher, MD, is an associate professor of medicine at the Commonwealth Medical College in Scranton, Pennsylvania, and an adjunct assistant professor of dermatology at the Perelman School of Medicine at the University of Pennsylvania in Philadelphia.
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- Khachemoune A, Lichon V, Blyumin M. A teenager with scaly papules on the trunk and extremities. J Am Acad Dermatol. 2006:55:925-927.
- Wahie S, Hiscutt E, Natarajan S, Taylor A. Pityriasis lichenoides: the differences between children and adults. Br J Dermatol. 2007;157:941-945.
- Simon D, Boudny C, Nievergelt H, Simon HU, Braathen LR. Successful treatment of pityriasis lichenoides with topical tacrolimus. Br J Dermatol. 2004;150:10331035
- Ersoy-Evans S, Greco MF, Mancini AJ, Subasi N, Paller AS. Pityriasis lichenoides in childhood: a retrospective review of 124 patients. J Am Acad Dermatol. 2007;56:205-210.
- Aydogan K, Saricaoglu H, Turan H. Narrowband UVB (311 nm, TL01) phototherapy for pityriasis lichenoides. Photodermatol Photoimmunol Photomed. 2008;24:128-133.
- Lazaridou E, Fotiadou C, Tsorova C, et al. Resistant pityriasis lichenoides et varioliformis acuta in a 3-year-old boy: successful treatment with methotrexate. Int J Dermatol. 2010;49:215-217.