Pityriasis lichenoides et varioliformis acuta associated with Epstein-Barr virus infection

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A 28-years old woman without past medical history presented with a 5-days history of asymptomatic erythematous papules and plaques with fine desquamation in her trunk. In the following days, the patient presented with pharyngitis and fever, and the lesions evolved in number and extension, also affecting the proximal and flexural aspects of arms and legs. Some erythematous papules developed central vesicles, hemorrhagic crusts and a dusky, necrotic center (Fig 1 A, B). The patient was started on oral prednisone. Lesions remitted within 6 weeks after two more crops of erythematous desquamative papules. However, some necrotic papules healed leaving atrophic hypopigmented and even keloid scars (Fig 1 C, D). Test results for varicella-zoster virus were negative, but serological test revealed an acute infection by Epstein-Barr virus (EBV). Pityriasis lichenoides et varioliformis acuta (PLEVA) is an inflammatory skin disorder of uncertain etiology. It may represent a benign reaction to several stimuli, including infections such as EBV [1]. Its main differential diagnosis is lymphomatoid papulosis, which typically follows a more chronic, indolent course. However, on its debut it may be clinically indistinguishable from PLEVA, thus a skin biopsy is necessary. On histopathology, PLEVA exhibits a wedge-shaped dermal perivascular lymphohistiocytic infiltrate with extravasated erythrocytes, lichenoid interface dermatitis, exocytosis of lymphocytes, spongiosis, parakeratosis and sparse necrotic keratinocytes. Scarring, usually atrophic and associated with necrotic papules, is one of the most classic complications, hence the term varioliform [2]. Data on PLEVA treatment are limited. Phototherapy or systemic anti-inflammatory antibiotics, such as macrolides and tetracyclines, are usually used as first-line treatments. Oral corticosteroids and specially methotrexate have been employed in more severe cases [3].

Fig. 1

A,B: Multiple erythematous papules, some with a dusky, necrotic center, on the patient's trunk; C,D: Once the eruption resolved, several atrophic varioliform scars, as well as erythematous hypertrophic scars, developed at the site of the most active lesions.