Febrile Ulceronecrotic Pityriasis Lichenoides et Varioliformis Acuta in a child / 대한피부과학회지

ABSTRACT

Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta (PLEVA) is a very rare cutaneous disorder clinically characterized by the acute onset of erythematous papules, vesicles and large coalescent ulceronecrotic lesions on the whole body accompanied by high fever and severe constitutional symptoms. In general, ulceronecrotic lesion is preceded by a typical rash of PLEVA. Histopathologically, it shows widespread epidermal necrosis and fibrinoid necrosis of blood vessel walls in addition to the superficial and deep perivascular lymphocytic infiltrations. Several regimens including systemic corticosteroid, antibiotics, methotrexate or ultraviolet therapy have been challenged in this disorder . We report a typical case of febrile ulceronecrotic PLEVA in a 10-year-old child who showed a dramatic response to methotrexate.