Lúpus eritematoso crônico discoide nas linhas de Blaschko

Abstract

Linear chronic discoid lupus erythematosus is a rare manifestation of cutaneous lupus in which erythematous, atrophic, dyschromic lesions are located along the lines of Blaschko. This report describes the case of a 15-year old boy with a 2-year history of discoid, erythematous, hyper and hypopigmented lesions with central atrophy, situated along the lines of Blaschko on his right arm. Histopathology showed epidermal atrophy, hyperkeratosis, follicular plugging, thickening of the basement membrane zone, and superficial and deep chronic perivascular and periadnexal inflammatory infiltrate, with dermal mucin deposit, thus confirming diagnosis. A total of 14 cases have been described of this variant, the onset of which is often in childhood. There is no difference in incidence between genders. Lesions most commonly develop on the face. There have been no reports of any association with systemic disease. Keywords: Lupus erythematosus, cutaneous; Lupus erythematosus, discoid; Mosaicism Resumo: O lúpus eritematoso crônico discoide linear é manifestação rara da doença lúpica cutânea, em que lesões eritêmato-atrófico-discrômicas dispõem-se nas linhas de Blaschko. Descrevemos o caso de um menino de 15 anos, com dois anos de história de lesões discoides eritêmato-atróficas, hipo e hiperpigmentadas, dispostas nas linhas de Blaschko do membro superior direito. O exame histopatológico revelou atrofia da epiderme, hiperqueratose, rolhas córneas, espessamento da zona da membrana basal, infiltrado inflamatório crônico perianexial e perivascular superficial e profundo, depósito de mucina na derme, confirmando o diagnóstico. Há, no total, 14 casos descritos dessa variante que se inicia frequentemente na infância e que não apresenta predomínio quanto ao sexo. As lesões ocorrem preferencialmente na face. Não há relatos de associação com doença sistêmica. Palavras-chave: Lupus eritematoso cutâneo; Lupus eritematoso discóide; Mosaicismo

Linear discoid lupus erythematosus associated with systemic lupus erythematosus: case report and literature review

“…El lupus eritematoso discoide lineal (LEDL) es una variante inusual del lupus eritematoso discoide (LED), caracterizada por lesiones eritematosas, hiperqueratósicas, atróficas, hipocrómicas, con un halo hiperpigmentado que se localizan a lo largo de las líneas de Blaschko (2) . Se han descrito otros subtipos de lupus eritematoso (LE) que siguen el patrón lineal, como LE subagudo, túmido y profundo (3) .…”

“…El lupus eritematoso discoide lineal (LEDL) es una enfermedad que se caracteriza por placas eritematosas unilaterales que siguen las líneas de Blaschko, y es una variante inusual del lupus eritematoso discoide (LED) (1) . En 1978, Umbert y Winkelmann reportaron por primera vez una distribución lineal de LED con esclerodermia, refiriéndose a un síndrome cutáneo mixto; posteriormente, Abe y colaboradores propusieron una configuración estrictamente lineal y no discoide en 1998 (2) . El objetivo principal de este reporte es describir el caso de una paciente femenina con lesiones en la cara que siguen las líneas de migración embriológica asociado a lupus eritematoso sistémico (LES) y alopecia.…”

Linear cutaneous lupus erythematosus following the lines of Blaschko - Case report

“…The second theory proposes that the lines of Blaschko result from an isolated cutaneous segmentary growth. 6 …”

"The Crosstalk between Covid-19 and Autoimmune Diseases: An Overview"

“…It is important to note that SLE is a disease of broad spectrum of manifestation and dynamic clinical occurrence, being able to present most of the fundamental characteristics of the collagenous diseases [73,74,[96][97][98][99][100][101]. However, since the autoimmune diseases are mostly a multifactorial event, being the results of genetic predisposition, environmental influence, genetic mutation, and in different degrees, it is a hard mission to classify them or to pinpoint a single causative agent, if any [81,102].…”