Congenital eccrine angiomatous hamartoma: Expanding the morphologic presentation and a review of the literature.

Eccrine angiomatous hamartoma (EAH) is a rare benign vascular hamartoma characterized histologically by an increased size and number of mature eccrine glands associated with multiple foci of dilated capillaries in the dermis and subcutis. EAH typically presents in children as discrete, solitary nodules, or plaques most commonly located on the extremities. Some cases of EAH have an agminated distribution involving classic locations, or present as solitary lesions in less common locations such as the face, scalp, or trunk. We report the case of congenital EAH in a child with atypical morphological features and pattern of distribution further expanding on the range of presentations classically described.

Eccrine angiomatous hamartoma: report of five congenital cases.

Eccrine angiomatous hamartoma is a rare entity characterized histologically by the combination of proliferative eccrine and vascular elements. It generally arises before puberty, as solitary or multiple lesions, with a heterogeneous clinical appearance, affecting predominantly the distal extremities, with or without associated pain or hyperhidrosis. It may require surgical treatment due to cosmetic concern, progressive enlargement or the presence of pain or excessive hyperhidrosis. We report five congenital cases of eccrine angiomatous hamartoma, emphasizing a clinically uncommon tumor-like appearance, with numerous telangiectasias on their surfaces resembling vascular lesions, in two of them.

Congenital apocrine hamartoma: an unusual clinical variant of organoid nevus with apocrine differentiation.

Congenital apocrine hamartomas are rare tumors of the skin composed of mature apocrine glands in the papillary and reticular dermis. Several cases have been reported in the literature but few were of uniform clinical appearance. The term apocrine nevus has been used interchangeably with apocrine hamartoma in the literature, however, based on their distinctive morphologies we believe they are different entities. We describe a 7-year-old girl with a congenital apocrine hamartoma of the left cheek. A stained biopsy specimen from the lesion revealed large mature apocrine glands with decapitation secretion in the dermis. The presence of periodic acid-Schiff-positive, diastase-resistant granular material in the apical cytoplasm of some secretory cells helped differentiate these as apocrine glands. We think this lesion may represent a form of organoid nevus with pure apocrine differentiation.

Porokeratotic eccrine ostial and dermal duct nevus of late onset: more frequent than previously suggested?

We describe the case of a 10-year-old boy with a porokeratotic eccrine ostial and dermal duct nevus (PEODDN) of late onset. The patient had an 8-year history of multiple keratotic papules on the dorsal surface, and multiple yellowish pitting lesions on the plantar surface of the right foot. Light-microscopic studies of both lesions showed multiple cornoid lamella-like parakeratotic columns, which exclusively arose over eccrine sweat ducts in which the acrosyringium was dilated. Although PEODDN is considered to be a congenital hamartoma of eccrine origin, a review of the literature showed us that the frequency of a late-onset variant may be as high as 26%.

Miliaria cristalina congénita: presentación de dos casos y revisión de la literatura / Congenital miliaria crystalline: report of two cases and review of the literature

La miliaria es un trastorno común de las glándulas sudoríparas ecrinas que se caracteriza clínicamente por la aparición de vesículas resultantes de la obstrucción del conducto glandular. La forma congénita es extremadamente infrecuente y existen sólo 4 casos publicados en la literatura. Presentamos a continuación dos pacientes evaluados en nuestro servicio con diagnóstico de miliaria cristalina congénita

Congenital miliaria crystallina is a common disorder of the eccrine sweat glands clinically characterized by the presence of vesicles resulting from the glandular duct obstruction. The congenital form of miliaria is extremely rare, with only 4 cases reported in the literature. We herein report 2 cases of congenital miliaria crystallina evaluated at our department