Nevus epidérmico verrucoso inflamatorio lineal: una revisión de la literatura / Inflammatory linear verrucous epidermal nevus: a review of the literature

El nevus epidérmico verrucoso inflamatorio lineal (NEVIL) es un tipo de nevus epidérmico queratinocítico, poco frecuente, de aparición predominante en la infancia, con preponderancia sobre el sexo femenino. Se caracteriza por la presencia de pápulas eritematosas descamativas de aspecto psoriasiforme, intensamente pruriginosas, que tienden a coalescer para formar placas que se distribuyen siguiendo las líneas de Blaschko. Suele presentarse de forma unilateral en extremidades inferiores y tiene pobre respuesta al tratamiento.

Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare type of keratinocytic epidermal nevus that predominantly appears in childhood and female sex. It is characterized by the presence of psoriasiform, scaly, intensely itchy erythematous papules that tend to coalesce to form plaques that are distributed along Blaschko's lines. It usually affects the lower extremities unilaterally, and responds poorly to treatment.

Nevus Sebaceous and Its Association With Neurologic Involvement.

Several terms are widely used to define cutaneous lesions affecting the epidermis in association with extracutaneous lesions. Recently, based on the wide spectrum of cutaneous epidermal lesions, the various underlying molecular mechanisms and patterns of associated features have led to improved definitions of these disorders. Nevus sebaceous syndrome has been placed under the umbrella term of epidermal nevus syndrome, in which the nevus sebaceous, a congenital hamartomatous lesion of the epidermis, is associated with anomalies involving the brain, eyes, and bones. Nevus sebaceous and neurologic manifestations were evaluated particularly based on their degree of intellectual delay, seizure type, treatment response, and electroencephalography and neuroradiological findings. A review of the literature in this topic is reported.

Phacomatosis pigmentokeratotica--a patient with hypophosphatemic rickets.

The epidermal naevus syndrome (ENS) is a sporadic condition characterized by congenital epidermal naevi associated with anomalies in other organ systems, most commonly the central nervous system and skeleton. We report a case of ENS presenting hypophosphataemic rickets resistant to traditional therapeutic agents.

Epidermal nevus syndrome associated with hypophosphatemic rickets.

Epidermal Nevus Syndrome (ENS) is characterized by epidermal nevi associated with abnormalities involving the nervous, skeletal, and other systems. Rarely, hypophosphatemic rickets has been observed in association with epidermal nevi. A patient with ENS with right-sided serpiginous skin lesions, generalized weakness, and diffuse osteopenia associated with hypophosphatemic rickets is described. Medical management was enough to correct the clinical picture. The pathogenic mechanism involved in the onset of hypophosphatemic rickets in ENS is not fully clarified. Different studies suggest that phosphaturia, caused by circulating factor(s), called "phosphatonin(s)," may be secreted by an epidermal nevus. The nature of the phosphaturic factor(s) is not well understood, but elevated levels of circulating FGF-23 were recently reported in one patient with hypophosphatemic rickets. The authors suggest that serum FGF-23 measurement be included in the workup of this kind of rickets because there is growing evidence that in these situations the epidermal nevi produce a phosphaturic factor.