ABSTRACT
Reticulate pigmentary disorders is a term that is loosely defined to include a spectrum of acquired and congenital conditions with different morphologies. The presentations vary from the reticular or net like pattern to the" freckle like" hyper and hypopigmented macules that are usually restricted to the true genetic "reticulate" pigmentary disorders. There is little clarity on this topic and related terms, in major dermatology textbooks. Hence, to harmonize the different entities we feel that the term "mottled pigmentation" could be used to include reticulate pigmentary disorders (acquired and congenital), dyschromasias and the disorders with a reticular pattern. The genetic reticulate pigmentary disorders can also be classified according to the gene loci which in the majority of cases are localized to keratin 5/14. A more useful clinical method of classification is based on the regional distribution, which includes facial, truncal, acral or flexural types. In this review we will largely focus on the inherited reticulate pigmentary disorders.
Subject(s)
Humans , Hyperpigmentation/chemically induced , Hyperpigmentation/classification , Hyperpigmentation/genetics , Pigmentation Disorders/chemically induced , Pigmentation Disorders/classification , Pigmentation Disorders/genetics , SkinABSTRACT
La facomatosis pigmentovascularis es un síndrome infrecuente caracterizado por la asociación de un nevus vascular con un nevus pigmentario. Su etiología es desconocida. Se ha propuesto un modelo genético de didimosis o manchas gemelas. La clasificación previa establece cinco categorías, que a su vez se subdividen en a) cuando existe compromiso cutáneo y b) cuando existe compromiso cutáneo y sistémico. Se ha propuesto una nueva clasificación, más simple, que resume las 10 categorías previas en tres tipos definidos: facomatosis cesioflammea, facomatosis spilorosea y facomatosis cesiomarmorata. Además, agrega un cuarto tipo de FPV no clasificables. Se han descrito asociaciones con otras alteraciones de la piel, oculares, vasculares, neurológicas, inmunológicas y malformaciones, por lo cual es recomendable realizar un examen físico extenso y derivación a especialidades para descartar patologías asociadas. Presentamos el caso de una mujer de 27 años que presenta lesiones correspondientes a una facomatosis cesioflammea en la nueva clasificación.
Phacomatosis pigmentovascularis is an uncommon syndrome characterized by the association of a widespread vascular nevus with a pigmentary nevus. Its etiology is unknown. A twin spotting or didymosis genetic model has been proposed. The previous classification established five categories, further subdivided into a) when cutaneous involvement was present or b) when cutaneous and systemic involvement was present. A new, more straightforward classification has been proposed, which summarizes the ten previous categories into three distinct types: phacomatosis cesioflammea, phacomatosis spilorosea and phacomatosis cesiomarmorata. Furthermore, a fourth category of unclassifiable phacomatosis pigmentovascularis was added. Diverse associations of phacomatosis pigmentovascularis with other skin lesions, malformations, and ocular, vascular, neurological and immunological abnormalities have been described, hence the importance of an extensive physical examination and consultations to discard associated pathologies. We present the case of a 27 year old woman, diagnosed with phacomatosis cesioflammea, based on the new classification.