An immunohistochemical study of angiokeratomas of children.

BACKGROUND: Angiokeratomas are vascular anomalies in which the clinical, histological, and immunohistochemical characteristics are insufficient to determine whether the lesion is a vascular neoplasm or vascular malformation, and their exact origin is also uncertain. To further clarify the nosology of angiokeratomas, we studied 14 cases. OBJECTIVE: To analyze immunohistochemical characteristics of angiokeratomas to gain further insight into its histogenesis. METHODS: We carried out a retrospective review of the histopathology files. Immunohistochemical expression for Wilms tumor 1 (WT1), GLUT1, D2-40, podoplanin, Prox1, and ERG1 was performed in 14 cases. RESULTS: None of the lesions showed cytoplasmic immunoreactivity for WT1. GLUT1 resulted to be negative in 13 cases. All 14 cases in our series showed some expression with at least 1 lymphatic marker. In 12 cases, the positivity was diffuse and strong for ERG1. CONCLUSIONS: Angiokeratomas are complex lesions with difficult classification. Angiokeratomas are best considered vascular malformations in children, according to the WT1-negative stain. The lymphatic component of angiokeratoma is demonstrated by positivity and/or focal expression for lymphatic markers (podoplanin and Prox1); however, a blood capillary component within the malformation cannot be excluded.

Angioqueratoma circunscrito interglúteo: reporte de un caso / Intergluteal angiokeratoma circumscriptum: report of one case

Introducción: el término angioqueratoma se aplica a un conjunto de enfermedades con una base clínica de lesiones vasculares comunes. El objetivo de este reporte es comunicar el hallazgo de un angioqueratoma interglúteo. Caso clínico: hombre de 26 años quien desde la infancia presentó tumoración interglútea, manejada en varias ocasiones sin resultados. Cuando acudió a la consulta que motivó este reporte, presentaba tumoración verrucosa interglútea dolorosa y sangrante de 7 x 4 cm, de contornos irregulares. Se efectuó resección quirúrgica amplia. Los resultados histopatológicos indicaron angioqueratoma circunscrito con bordes libres de tumor. Discusión: de los cinco tipos de angioqueratomas descritos en la literatura (corporal difuso, de Mibelli, de Fordyce, solitario y corporal circunscrito), en nuestro caso se identificó angioqueratoma circunscrito por las características físicas y anatomopatológicas de la tumoración. El diagnóstico diferencial debe establecerse con el hemangioma verrucoso y la enfermedad de Fabry tipo II (angioqueratoma corporal difuso). Conclusiones: la patogénesis del angioqueratoma es desconocida y el tratamiento recomendado es el quirúrgico.

BACKGROUND: The term angiokeratoma is applied to several diseases with cutaneous vascular lesions. CLINICAL CASE: A 26-year-old male presented with an intergluteal tumor from childhood until the present time, which was treated on several occasions without results. The patient presented to the hospital complaining of a painful and bleeding intergluteal tumor. DISCUSSION: Five types of angiokeratomas have been described (angiokeratoma corporis diffusum, angiokeratoma of Mibelli, angiokeratoma of Fordyce, solitary angiokeratoma and angiokeratoma corporal circumscriptum). This patient was identified with an angiokeratoma circumscriptum by pathological and physical characteristics of the tumor. Differential diagnosis is with verrucous hemangioma and the Fabry disease type II (angiokeratoma corporis diffusum). CONCLUSION: Pathogenesis is unknown and surgery is the recommended treatment.

[Intergluteal angiokeratoma circumscriptum. Report of one case]. / Angioqueratoma circunscrito interglúteo. Reporte de un caso.

BACKGROUND: The term angiokeratoma is applied to several diseases with cutaneous vascular lesions. CLINICAL CASE: A 26-year-old male presented with an intergluteal tumor from childhood until the present time, which was treated on several occasions without results. The patient presented to the hospital complaining of a painful and bleeding intergluteal tumor. DISCUSSION: Five types of angiokeratomas have been described (angiokeratoma corporis diffusum, angiokeratoma of Mibelli, angiokeratoma of Fordyce, solitary angiokeratoma and angiokeratoma corporal circumscriptum). This patient was identified with an angiokeratoma circumscriptum by pathological and physical characteristics of the tumor. Differential diagnosis is with verrucous hemangioma and the Fabry disease type II (angiokeratoma corporis diffusum). CONCLUSION: Pathogenesis is unknown and surgery is the recommended treatment.

Angioqueratoma circunscrito neviforme: a propósito de dos observaciones / Angiokeratoma circumscriptum naeviforme: for the purpuse of 2 observations

El angioqueratoma circunscrito neviforme es una malformación vascular infrecuente que se caracteriza clínicamente por pápulas o nódulos hiperqueratósicos localizados unilateralmente en pierna o pie, a menudo dispuestos en banda o con una distribución zosteriforme. Histológicamente, consiste en capilares dilatados situados por debajo o en el espesor de una epidermis papilomatosa, acantósica e hiperqueratósica. Describimos dos casos de esta rara entidad, afectando el tobillo izquierdo de un adolescente varón y la pierna derecha de una niña de 7 años de edad. Se discute su diferenciación con otros tipos de angioqueratoma y se enfatiza el diagnóstico diferencial con el hemangioma verrugoso debido a su extraordinaria similitud clínica

Angiokeratoma circumscriptum: successful treatment with the argon laser.

This article describes 2 patients with angiokeratoma circumscriptum for whom the argon laser proved to be a highly successful means of treatment. Both patients had no recurrences of the lesions. Also included is an updated and comprehensive review of the various types of angiokeratoma.