Queratose liquenoide crônica: relato de caso

Abstract

Keratosis lichenoides chronica or Nekam´s disease is a rare mucocutaneous dermatosis characterized by keratinization. It is chronic and progressive usually affecting individuals aged 20-40 years. Around 70 cases have been reported in the literature. Due to the rarity of this condition and the lack of effective treatment, it is a difficult disease to manage. In the case described below we present a 42-yearold patient with violaceous and hyperkeratotic papules in linear, reticular or plaque form, located on the trunk and limbs for five years. Aphthous lesions in the oral cavity and shallow ulcers on the genitalia also formed part of the clinical manifestation. Pathologic examination was suggestive of keratosis lichenoides chronica. Acitretin and dapsone was introduced and the lesions partially improved. Keywords: Keratosis; Lichenoid eruptions; Skin diseases Resumo: A queratose liquenoide crônica ou doença de Nekam é uma dermatose mucocutânea rara da queratinização, com curso crônico e progressivo, que acomete geralmente indivíduos entre 20 e 40 anos. Existem, aproximadamente, 70 casos descritos na literatura. Devido à raridade desta dermatose e à ausência de tratamento efetivo, é uma doença de difícil manejo. No caso em questão, apresentamos um paciente de 42 anos com pápulas violáceas, hiperqueratósicas, algumas confluentes, com aspecto linear, rendilhado e em placas, localizadas no tronco e membros há cinco anos. Lesões aftoides na cavidade oral e úlceras rasas na genitália também faziam parte do quadro. O anatomopatológico foi bastante sugestivo de queratose liquenoide crônica. Introduziu-se tratamento com acitretina e dapsona, havendo melhora parcial do quadro.

Keratosis lichenoides chronica: a diagnosis to remember

“…Keratosis lichenoides chronica (KLC), also known as Nekam's disease, is a rare dermatosis of uncertain etiology. It appears most frequently in adults, with a peak of incidence in the 4 th decade and slight predominance in males 1,2,4 .…”

“…Keratosis lichenoides chronica (KLC) has a chronic and progressive course and is highly resistant to therapy 11,12 . The most effective treatment consists of oral retinoids at a dose of 0.3-0.6 mg/kg/day 2,11 , although complete responses are infrequent 11 . Phototherapy (psoralen plus ultraviolet A and narrow-band ultraviolet B) can be helpful, especially as an adjuvant treatment 4,11 .…”

“…Phototherapy (psoralen plus ultraviolet A and narrow-band ultraviolet B) can be helpful, especially as an adjuvant treatment 4,11 . Topical treatment, as well as systemic steroids, other immunosuppressants and antimalarials, are usually ineffective 2,12 .…”

“…Keratosis lichenoides chronica (KLC) is an uncommon mucocutaneous disorder originally described in 1895 by Kaposi, who called it 'lichen ruber acuminatus morbilliform' 1 . Later, in 1938, Nekam took note of acrosyringeal hyperkeratosis in the case published by Kaposi and named the disease 'porokeratosis striata lichenoides' despite the lack of coronoid lamella 2 . Finally, in 1972, Margolis et al introduced the nomenclature currently used-'KLC' 3 .…”