A 53-year-old nurse presents for evaluation of a minimally pruritic eruption affecting her arms and legs that has been present for 5 months. Family history is positive for sarcoidosis affecting her mother. The dermatitis has not responded to a course of prednisone or to topical therapy with clobetasol cream. No other family members are similarly affected. Examination reveals scattered papules of her extremities with variable degrees of hemorrhage and crusting.
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Pityriasis lichenoides et varioliformis acuta (PLEVA) is an uncommon skin disorder characterized by the sudden appearance of macules and papules on the trunk and extremities. These are often covered with a fine scale and hemorrhagic centers develop rapidly.
The average age at onset is 29 years, and there is no gender predilection.1 Symptoms may include itching and burning. An ulcerative variant, often accompanied by fever, is termed Mucha-Habermann disease.
The etiology of PLEVA is unknown. Histopathology reveals a superficial and deep dermal lymphohistiocytic infiltrate with vacuolar interface dermatitis and erythrocyte extravasation. Because CD30 cells are often identified in the infiltrate, a clonal self-limited T-cell lymphoproliferative disorder is suggested.2 In some cases, PLEVA has been associated with Epstein-Barr viral infection,3 whereas other cases have been linked with toxoplasmosis.4 An association with HIV infection has also been identified.5
A variety of agents have been used to treat PLEVA, including topical steroids, ultraviolet light, and systemic medications including erythromycin and methotrexate.6 The course of the disease is variable, although most cases remit spontaneously over a period of weeks to months. Periodic monitoring is recommended, as malignant transformation, although rare, has been reported.7
Jamie Remaley, PA-C, is a physician assistant at Reading Dermatology Associates in Reading, Pennsylvania, and 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. He practices dermatology in Hazleton, Pennsylvania.
- Willemze R, Scheffer E. Clinical and histologic differentiation between lymphomatoid papulosis and pityriasis lichenoides. J Am Acad Dermatol. 1985;13(3):418-428.
- Magro C, Crowson AN, Kovatich A, Burns F. Pityriasis lichenoides: a clonal T-cell lymphoproliferative disorder. Hum Pathol. 2002;33(8):788-795.
- Boss JM, Boxley JD, Summerly R, Sutton RN. The detection of Epstein Barr virus antibody in ‘exanthematic’ dermatoses with special reference to pityriasis lichenoides. A preliminary survey. Clin Exp Dermatol. 1978;3(1):51-56.
- Zlatkov NB, Andreev VC. Toxoplasmosis and pityriasis lichenoides. Br J Dermatol. 1972;87(2):114-116.
- Smith KJ, Nelson A, Skelton H, Yeager J, Wagner KF. Pityriasis lichenoides et varioliformis acuta in HIV-1+ patients: a marker of early stage disease. Int J Dermatol. 1997;36(2):104-109.
- Bowers S, Warshaw EM. Pityriasis lichenoides and its subtypes. J Am Acad Dermatol. 2006;55(4):557-572.
- Bleehen SS, Slater DN. Pityriasis lichenoides developing into mycosis fungoides. Br J Dermatol. 1986;115(Suppl s30):69-70.