Liquen aureus zosteriforme: Caso dínico pediatrico / Zosteriform lichen aureus: Pediatric clinical case

El liquen aureus es una dermatosis purpúrica pigmentaria infrecuente. Presentamos un caso inusual por su edad pediátrica y por la presencia de lesiones múltiples con patrón zosteriforme. Se trata de un niño de 10 años con máculas parduzcas, menores de 1 cm, con bordes definidos y superficie liquenificada, en la cara interna de la pierna izquierda con distribución zosteriforme, asintomáticas, de tres meses de evolución. La biopsia mostró infiltrado en banda en la dermis superior con linfocitos, histiocitos, hematíes y hemosiderina. Se diagnosticó liquen aureus zosteriforme y se trató con furoato de mometasona tópico durante 3 semanas con aclaramiento parcial. Las lesiones permanecen 2 años después y han aparecido nuevas en el tobillo ipsilateral. Debemos plantearnos el diagnóstico diferencial con otras dermatosis purpúricas pigmentarias crónicas y la variante purpúrica pigmentaria de la micosis fungoide. Existen muchas opciones terapéuticas e, incluso sin tratamiento, suelen desaparecer, por lo que son necesarios estudios que recopilen más evidencias.

ABSTRACT Lichen aureus is a rare pigmented purpuric dermatosis. We present anunusual case because of the pediatric age and the great number of lesions with zosteriform distribution. He is a 10-year-old boy, with a brownish, smaller than 1 cm, sharp edges, lichenified surface, asymptomatic macule, over the inner aspect of the left leg with a zosteriform distribution. The histology showed a band-like inflammatory infiltrate in the superficial dermis, composed of lymphocytes, histiocytes, erythrocytes and haemosiderin. He was diagnosed with zosteriform lichen aureus and was treated with topical mometasone furoate during 3 weeks resulting in partial lightening of the macules. Lesions have remained 2 years later, and new ones have appeared in the ipsilateral ankle. We must consider differential diagnosis with other pigmented purpuric dermatitis and pigmented purpuric mycosis fungoides. There are many therapeutic options and it tends to disappear spontaneously, so new studies are necessary

[Zosteriform lichen aureus. Pediatric clinical case]. / Liquen aureus zosteriforme. Caso clínico pediátrico.

Lichen aureus is a rare pigmented purpuric dermatosis. We present an unusual case because of the pediatric age and the great number of lesions with zosteriform distribution. He is a 10-yearold boy, with a brownish, smaller than 1 cm, sharp edges, lichenified surface, asymptomatic macule, over the inner aspect of the left leg with a zosteriform distribution. The histology showed a band-like inflammatory infiltrate in the superficial dermis, composed of lymphocytes, histiocytes, erythrocytes and haemosiderin. He was diagnosed with zosteriform lichen aureus and was treated with topical mometasone furoate during 3 weeks resulting in partial lightening of the macules. Lesions have remained 2 years later, and new ones have appeared in the ipsilateral ankle. We must consider differential diagnosis with other pigmented purpuric dermatitis and pigmented purpuric mycosis fungoides. There are many therapeutic options and it tends to disappear spontaneously, so new studies are necessary.

El liquen aureus es una dermatosis purpúrica pigmentaria infrecuente. Presentamos un caso inusual por su edad pediátrica y por la presencia de lesiones múltiples con patrón zosteriforme. Se trata de un niño de 10 años con máculas parduzcas, menores de 1 cm, con bordes definidos y superficie liquenificada, en la cara interna de la pierna izquierda con distribución zosteriforme, asintomáticas, de tres meses de evolución. La biopsia mostró infiltrado en banda en la dermis superior con linfocitos, histiocitos, hematíes y hemosiderina. Se diagnosticó liquen aureus zosteriforme y se trató con furoato de mometasona tópico durante 3 semanas con aclaramiento parcial. Las lesiones permanecen 2 años después y han aparecido nuevas en el tobillo ipsilateral. Debemos plantearnos el diagnóstico diferencial con otras dermatosis purpúricas pigmentarias crónicas y la variante purpúrica pigmentaria de la micosis fungoide. Existen muchas opciones terapéuticas e, incluso sin tratamiento, suelen desaparecer, por lo que son necesarios estudios que recopilen más evidencias.

Newly Described Autoinflammatory Diseases in Pediatric Dermatology.

Specific gene mutations leading to dysregulation of innate immune response produce the expanding spectrum of monogenic autoinflammatory diseases (AIDs). They are characterized by seemingly unprovoked, recurrent episodes of systemic inflammation in which a myriad of manifestations usually affect skin. Novel genetic technologies have led to the discovery of new AIDs and phenotypes that were not previously clinically described. Consequently the number of AIDs is continuously growing and their recognition and the disclosure of their pathophysiology will prompt early diagnosis and targeted treatment of affected patients. The objective of the present work is to review those newly described AIDs with prominent dermatologic manifestations that may constitute a major criterion for their diagnosis.