Nevo poroqueratósico del ostium y el ducto dérmico ecrinos: reporte de caso / Porokeratotic eccrine ostial and dermal duct nevus: case report

El Nevo Poroqueratósico del Ostium y el Ducto Dérmico Ecrinos (NPODDE), es un raro hamartoma benigno de los conductos de las glándulas sudoríparas ecrinas, puede presentarse desde el nacimiento o también en edades posteriores. Su etiología plantea una alteración en la queratinización debido a una mutación somática en el gen GJB2 que codifica para una proteína de unión gap. Esta mutación también está relacionada con el síndrome KID por lo cual la asesoría genética es crucial en estos pacientes. Clínicamente puede presentarse como hoyuelos hiperqueratósicos en palmas y plantas que normalmente son asintomáticos. El diagnostico se confirma con la histopato-logía que muestra una laminilla cornoide sobre el conducto ecrino subyacente. La entidad es benigna y de difícil tratamiento siendo refractaria a varias modalidades terapéuticas. Se presenta un caso de un paciente adulto masculino con lesiones típicas en palmas y plantas, a quien con la biopsia de piel se le confirmó el diagnóstico de NPODDE. Dado la baja frecuencia de esta condición el objetivo de este artículo radica en actualizar los aspectos más relevantes de esta entidad.

Porokeratotic Eccrine Ostial and Dermal Duct Nevus (PEODDN) is a rare benign hamartoma of eccrine sweat gland ducts, it can present from birth or also at later ages. Its etiology implies an alteration in keratinization due to a somatic mutation in GJB2 gene, that codes for a gap junction protein. This mutation is also associated with KID syndrome so genetic counseling for parents is crucial. Clinically it can present as keratotic pits in palms and soles that are usually asymp-tomatic. The diagnosis is confirmed by histopathology that shows a cornoid lamellae on the underlying eccrine duct. The entity is benign and the treatment is difficult, being refractory to seve-ral therapeutic modalities. We present a case of a male adult patient with typical lesions on palms and soles, who was diagnosed with PEODDN by skin biopsy. Given the low frequency of this con-dition, the objective of this article is to update the most relevant aspects of this entity.

Dermoscopy of squamoid eccrine ductal carcinoma: an aid for early diagnosis

Abstract: Squamoid eccrine ductal carcinoma is a cutaneous malignancy that originates from the eccrine sweat gland. A 76-year-old woman presented with an asymptomatic papule on her nose that had been present for one year. Dermoscopy showed pseudocysts, irregular linear crown vessels, and yellowish-brown globules surrounded by white halos. This is the first dermoscopic description of squamoid eccrine ductal carcinoma. Studies with more cases are needed to confirm the dermoscopic characterization of this tumor.

Porokeratotic eccrine and hair follicle nevus: a report of two cases and review of the literature

Abstract: Porokeratotic eccrine and hair follicle nevus is a very rare non-hereditary disorder of keratinization with eccrine and hair follicle involvement with only 9 cases described in the literature. In 2009 the term porokeratotic anexial ostial nevus was proposed to comprehend porokeratotic eccrine and hair follicle nevus and a related and more common process without follicular involvement: porokeratotic eccrine ostial and dermal duct nevus Recent findings suggest that both entities may be produced by a mutation in GJB2 gene, which is associated to KID syndrome. Herein we report 2 cases of porokeratotic eccrine and hair follicle nevus and review the existing cases in the Spanish and English literature.

Squamoid eccrine ductal carcinoma

Abstract Squamoid eccrine ductal carcinoma is an eccrine carcinoma subtype, and only twelve cases have been reported until now. It is a rare tumor and its histopathological diagnosis is difficult. Almost half of patients are misdiagnosed as squamous cell carcinoma by the incisional biopsy. We report the thirteenth case of squamoid eccrine ductal carcinoma. Female patient, 72 years old, in the last 6 months presenting erythematous, keratotic and ulcerated papules on the nose. The incisional biopsy diagnosed squamoid eccrine ductal carcinoma. After excision, histopathology revealed positive margins. A wideningmargins surgery and grafting were performed, which again resulted in positive margins. The patient was then referred for radiotherapy. After 25 sessions, the injury reappeared. After another surgery, although the intraoperative biopsy showed free surgical margins, the product of resection revealed persistent lesion. Distinction between squamoid eccrine ductal carcinoma and squamous cell carcinoma is important because of the more aggressive nature of the first, which requires wider margins surgery to avoid recurrence.

First case of congenital idiopathic hypohidrosis in China

AbstractA 43-year-old Chinese man presented with generalized hypohidrosis, which he had had since birth, without obvious abnormalities of other skin appendages except a sparse beard and axillary hairs. The sweat test revealed localized sweating on the face, axillae and palms. Histopathologic examination showed that the sweat glands were absent in the forearm and thigh, but some eccrine and apocrine sweat glands were present in the right axilla. S-100 was expressed in the nerve terminals surrounding the acini and ducts of the eccrine sweat glands, while PGP9.5 was positive in the acini of apocrine glands and the nerve terminals surrounding the eccrine glands in the axilla. To our knowledge, this is the first case of congenital idiopathic hypohidrosis in China.

Solitary eccrine syringofibroadenoma - Case Report

Eccrine syringofibroadenoma is a rare benign adnexal neoplasm derived from cells of the acrosyringium of eccrine sweat glands. ESFA usually manifests as a solitary nodule on the extremities of elderly patients, but it may also present as papules, nodules or plaques. Its clinical appearance is nonspecific and malignant neoplasms should beconsidered in the differential diagnosis. However, histopathological findings are typical. The main treatment is surgical excision. In order to illustrate a typical presentation of the tumor, we report a case of solitary eccrine syringofibroadenoma, including the surgical treatment used and its result.

Clouston syndrome associated with eccrine syringofibroadenoma

Eccrine syringofibroadenoma is a rare benign neoplasia derived from acrosyryngium cells of the eccrine sudoriferous glands. It affects the extremities of elderly individuals as solitary tumors, or may also present as multiple lesions. There are controversies about the pathogenesis and differentiation of the tumor. Eccrine syringofibroadenoma has been associated with subjacent conditions, such as for example, hypohidrotic ectodermic dysplasias. The authors describe a case report of a patient with Clouston Syndrome, who presented papules and nodules in extremities, clinically and histologically compatible with eccrine syringofibroadenoma. There are only three cases described in the literature, associated with Clouston Syndrome, and this is the fourth case.

Syringomas over forearm: a case report

Syringomas are benign intraepidermal tumour of eccrine sweat ducts mostly found in women at the time of adolescence. Most commonly, lower eyelids are involved and few may occur in the scalp, axillae, abdomen, forehead, penile area and vulva. Syringomas over the forearms are less commonly found. The lesions commonly present as small, multiple, skin to yellowish coloured papules. We present a case of 35-year-old lady with multiple hyperpigmented flat smooth surfaced papules only over the extensors of the forearms symmetrically for the last 1 year with no other cutaneous and systemic manifestations. Histopathological examination confirmed the diagnosis

Argyria - Case report / Argiria - Relato de caso

A 70-year-old male rural worker was referred to our clinic with widespread grey pigmentation of the skin and nails. The condition had been asymptomatic for its entire duration (5 years). He reported past intranasal application of 10% Silver Vitellinate. A skin biopsy was performed and histology corroborated the clinical diagnosis of Argyria. This case represents a currently rare dermatological curiosity. Although silver colloids and salts have been withdrawn and/or banned by some drug surveillance agencies, they continue to be freely sold and unregulated as food supplements and as ingredients in alternative medicines, thereby risking the emergence of new cases of silver poisoning.

Um homem de 70 anos, trabalhador rural, foi referenciado à nossa consulta por dermatose assintomática, com 5 anos de evolução, caracterizada pela pigmentação acinzentada generalizada da pele, mais evidente em áreas fotoexpostas, e das lâminas ungueais. Relatava no passado o uso prolongado de Vitelinato de Prata a 10%, por via nasal. Foi efetuado exame histológico de biopsia cutânea que corroborou o diagnóstico clínico de Argiria. O caso representa uma curiosidade dermatológica, atualmente rara. Apesar de abandonados e/ou proibidos por algumas instituições de farmacovigilância, a prata coloidal e sais de prata continuam a ser comercializados como suplementos alimentares, como parte de medicinas alternativas e sem regulação, podendo fazer ressurgir os casos associados à toxicidade pela prata.

Eccrine syringofibroadenoma: an unusual presentation of a rare disease

Eccrine syringofibroadenoma [ESFA] is a rare disorder with differentiation towards ductal eccrine apparatus. There are a variety of clinical manifestations and distribution of lesions but the histopathological features are remarkably similar with benign appearing anastomosing cords of eccrine ductal epithelial cells with or without lumina formation, are embedded in loose fibrovascular stroma. We report a case of 55-year-old Muslim man having large pale pink slightly verrucous plaque on right upper back since last 20 years with central ulceration for 8 years. Punch biopsy was done and showed features of ESFA

4-year-old male child with hypohidrotic ectodermal dysplasia

A 4-year-old male child presented with history of dry skin, scanty scalp hair, and decreased sweating since birth. On examination scalp hair were scanty, thin, dry and brownish in color. Facial features were frontal bossing, saddle shaped nose, thick everted lips and low set large ears. There was partial adontia with only one conical incisor tooth. There was generalized dryness of the skin. Mucosae and nails were normal. Histopathology of the skin showed rudimentary and decreased number of eccrine sweat glands, which confirmed the diagnosis of hypohidrotic ectodermal dysplasia

Mucinous eccrine carcinoma of lower eyelid: a case report.

A rare case of primary mucinous eccrine carcinoma of the lower eyelid in a 45 year old female is described. The lesion was diagnosed clinically as melanoma. We report this case because of unusual nature of this tumor and rare presentation in a female patient.

Hamartoma angiomatoso ecrino: a propósito de dos casos / Eccrine angiomatous hamartoma: two case reports

El Hamartoma Angiomatoso Ecrino /HAE) es una malformación combinada: vascular y ecrina, benigna, poco frecuente, que se presenta al nacimiento o en la infancia, ubicada generalmente en las extremidades. Es de crecimiento lento, proporcional al crecimiento del niño, generalmente único. Se presenta habitualmente como una placa o nódulo de color rojo, violáceo o color piel. Puede presentar dolor, hipertricosis e hiperhidrosis local, características que lo diferencian de otras malformaciones vasculares. A la histología se observa un aumento de glándulas ecrinas, maduras, en la dermis, acompañadas de acúmulos de vasos sanguíneos (capilares) dilatados. El tratamiento de elección del HAE es la resección quirúrgica. Describimos dos casos de HAE diagnosticados en un periodo de tres años en el Servicio de Dermatología del Hospital Barros Luco-Trudeau.