Multiple eruptive and vulvar syringomas and the incipient role of dermoscopy in diagnosis / Siringomas eruptivos múltiples y vulvares y el papel incipiente de la dermatoscopia en el diagnóstico

INTRODUCCIÓN: Los siringomas son tumores benignos derivados de la porción intraepidérmica de los conductos sudoríparos ecrinos. Ocasionalmente pueden iniciar de forma súbita como siringomas eruptivos o localizarse en sitios atípicos que retrasan el diagnóstico por años. La dermatoscopía tiene un rol incipiente en diferenciar siringomas de su extenso diagnóstico diferencial. MÉTODOS: Estudio retrospectivo descriptivo de serie de casos de siringomas de localización atípica. Los datos fueron extraídos de fichas clínicas electrónicas. Todos incluyen dermatoscopía y correlación histopatológica. RESULTADOS: Cinco pacientes. Cuatro hombres y una mujer entre 40 y 79 años de edad con siringomas atípicos: cuatro casos eruptivos y un caso de siringomas vulvares. DISCUSIÓN: Proponemos la dermatoscopía basada en nuestros hallazgos como una herramienta útil con estructuras ovales amarillas y una pseudo-red café clara difusa en su superficie. Estas estructuras amarillas se pueden correlacionar con la proliferación ductal ecrina y el denso estroma en la histopatología. CONCLUSIÓN: Enfatizamos que se debe considerar esta entidad en el diagnóstico diferencial de dermatosis papulares y conocer sus manifestaciones clínicas para optimizar la sospecha diagnóstica.

INTRODUCCTION: Syringomas are common benign tumors, probably of origin derived from the intraepidermal portion of the eccrine sweat ducts. Occasionally they may develop suddenly and extensively as eruptive syringomas or be located in atypical sites delaying the diagnosis for years. Dermoscopy has an incipient role in differentiating syringomas from their extensive differential diagnosis. METHODS: Retrospective descriptive case-series study of atypical location syringomas. Data extraction from clinical history from electronic files. They all include dermoscopy and histopathological correlation. RESULTS: Five patients: Four men and one woman between 40 and 79 years old, with atypical syringomas diagnosis: four eruptive and one vulvar syringomas. DISCUSSION: We propose dermoscopy, based on our findings, as a useful tool to this entity, with its oval yellow structures and a diffuse light-brown network-like structure on its surface. These yellow enlargements may be correlated with the ductal eccrine proliferation and the dense stroma seen in the histopathology. CONCLUSION: We emphasize that they should be considered in the differential diagnosis of papular dermatosis, as they tend to be underdiagnosed, and to know their clinical manifestations to optimize the diagnostic suspicion.

Poroma no pigmentado inusualmente grande: reporte de un caso y revisión de la literatura / Unusually large unpigmented poroma: a case report and literature review

El poroma es un tumor benigno infrecuente derivado de la porción intraepitelial del epitelio ductal de la glándula sudorípara. La presentación clínica más habitual es un pápula o nódulo de color rosado a rojo, de pequeño diámetro, localizado con mayor frecuencia en palmas y plantas, generalmente asintomático. Debido a su variabilidad morfológica, los poromas son usualmente difíciles de reconocer, siendo la dermatoscopía una herramienta útil en su diagnóstico. Presentamos el caso de un paciente masculino con un gran tumor en su pierna derecha de 40 mm de diámetro, con características dermatoscópicas compatibles con poroma no pigmentado. El diagnóstico confirmatorio se realizó con una biopsia incisional seguido de la extirpación quirúrgica completa del tumor

Poroma is a rare benign tumour derived from the intraepithelial portion of the sweat glands' ductal epithelium. The most common clinical presentation is a pink to red papule or nodule, small in diameter, predominantly in the palms and soles and generally asymptomatic. Due to its morphologic variability, poromas are usually difficult to recognize, thus dermatoscopy becomes a useful aid in its diagnosis. We present the case of a male patient with a large tumour on his right leg, 40 mm in diameter, with dermatoscopic features consistent with nonpigmented poroma. Confirmatory diagnosis was made by incisional biopsy followed by the total surgical excision of the tumor.

Hidrocistoma apocrino desafío dermatoscópico / Apocrine hydrocystoma dermoscopic challenge

Los hidrocistomas apocrinos son lesiones quísticas benignas. Clínicamente se caracterizan por ser nódulos quísticos claros o traslucidos, solitarios, en forma de cúpula, con superficie lisa. En la mayoría de los casos la localización suele ser alrededor del ojo, particularmente lateral al canto externo. Suelen ser asintomáticos, de crecimiento lento pudiendo llegar a 10 mm de diámetro o más. Presentamos un caso de un paciente con un hidrocistoma apocrino variante pigmentada.

Apocrine hydrocystomas are benign cystic lesions, clinically characterized by being clear or translucent cystic nodules, solitary, domeshaped, with a smooth surface. In most cases the location is usually around the eye, particularly lateral to the outer edge. They are usually asymptomatic, slow growing and can reach 10 mm in diameter or more. We present a case of a patient with a pigmented variant apocrine hydrocystom

Concomitant poroma and porocarcinoma

Abstract: Eccrine poromas and porocarcinomas are adnexal tumors derived from the sweat duct epithelium. However, eccrine poroma is benign in nature, whilst eccrine porocarcinoma is regarded as its malignant counterpart. We report the case of a man who presented simultaneously with an eccrine poroma and eccrine porocarcinoma. Both lesions had no clear distinctive features, enhancing the need of high-level clinical suspicion together with surgical excision and histopathology for prompt diagnosis confirmation.

Ductal eccrine carcinoma of the axilla: a diagnostic pitfall

Abstract: Ductal eccrine carcinoma (DEC) is a rare sweat gland carcinoma with ductular differentiation. Clinically, it is characterized by a slowly growing, hardened plaque or nodule predominantly located on the head and neck. Histologically, DEC shares similar features to invasive breast carcinoma, thus causing great diagnostic challenges. We report a 69-year-old woman who presented with a hardened plaque on the axilla. A skin biopsy was performed and metastatic invasive breast carcinoma could not be ruled out. Complete excision and further workup were subsequently conducted, leading to the diagnosis of estrogen receptor positive DEC with associated axillary lymph node metastases. The patient received adjuvant radiotherapy to the left axilla and was started on oral letrozole. She is disease-free 14 months after initial diagnosis.

Orbital apocrine hidrocystoma with immunohistochemical confirmation / Hidrocistoma apócrino orbitário com confirmação imunohistoquímica

ABSTRACT The authors report the first case in Brazilian literature of orbital apocrine hidrocystoma with immunohistochemistry confirmation. The tumor had been growing slowly and progressively, there was no history of impaired vision, diplopia, watering, discharge, or prior trauma. There was no proptosis and extraocular mobility was normal. The radiologic study by orbital computerized tomography revealed an extraconal nodule, with partially defined limits, with cystic and solid areas in the superomedial right orbit. An anterior orbitotomy, with full excision of the tumor was performed. A histopathology examination revealed apocrine hidrocystoma and immunohistochemistry confirmed the diagnosis. After surgery, the patient had total remission of symptoms.

RESUMO Os autores relatam o primeiro caso de hidrocistoma apócrino orbitário com confirmação imunohistoquímica. O tumor apresentou crescimento lento e progressivo, sem relato de baixa de acuidade visual, diplopia, epífora, secreção ou trauma prévio. Não houve proptose ou alteração da motilidade extraocular. O exame de imagem por tomografia computadorizada da órbita revelou uma lesão nodular, extraconal, de limites imprecisos, com áreas císticas e sólidas no seu interior, na região súpero-medial de órbita direita. O paciente foi submetido à orbitotomia por via anterior, com exérese integral da tumoração. O exame histopatológico revelou o diagnóstico de hidrocistoma apócrino e a imunohistoquímica confirmou o diagnóstico. Após a cirurgia, o paciente teve regressão total dos sintomas.

Características clínicas y dermatoscópicas del poroma ecrino / Clinical and dermatoscopic characteristics of the eccrine poroma

El poroma ecrino (PE) corresponde a una neoplasia anexial benigna originada de la porción intraepidérmica del conducto de las glándulas sudoríparas. Habitualmente se describe como nódulos eritematosos o color piel ubicados en región palmoplantar, pero sus características clínicas son muy variables. Se decriben los hallazgos clínicos y dermatoscópicos de una serie de casos de pacientes con PE. La dermatoscopia puede facilitar significativamente el diagnóstico clínico de la mayoría de los tumores de la piel, sin embargo el PE se caracteriza por un alto grado de variabilidad a la dermatoscopía. Habitualmente se describe un patrón vascula polimorfo con vasos glomerulares, en horquilla, y lineales irregulares con terminaciones semicirculares rodeados de un halo blanco a rosa en forma de caliz o flor de cerezo asociado a áreas rosadas blanquecinas sin estructuras similares a lo observado en melanoma amelanótico, carcinoma espinocelular entre otros tumores benignos y malignos. El PE puede exhibir características clínicas y dermatoscópicas polimórficas que pueden hacer difícil el diagnóstico y su diferenciación de otras neoplasias cutáneas.

Eccrine poroma (PE) is a benign adnexal neoplasm of the intraepidermal portion of the sweat gland duct. PE typically present as erythematous or flesh-colored nodules on the palms and soles, but the clinical features of PE are highly variable. Dermatoscopic and clinical findings of patients with EP are described. Dermoscopy may significantly improves the clinical diagnosis of most skin tumours, but PE is characterised by a high degree of dermoscopic variability. The main dermoscopic features described are polymorphous vascular pattern, whiteto- pink halo surrounding the vessels, pink-white structureless areas, vascular structures of glomerular, hairpin vessels, and linear irregular vessels, mimicking amelanotic melanoma, keratinising tumours and several benign and malignant tumors. Eccrine poroma may exhibit polymorphic clinical and dermoscopic features making it difficult to diagnosis and differentiate from other skin cancers.

Porocarcinoma ecrino: reporte de un caso / Eccrine porocarcinoma: a case report

Los tumores anexiales de la piel representan un grupo heterogéneo de entidades de baja frecuencia de presentación. Su origen es controvertido, quizás a partir de células madres pluripotenciales, aceptándose principalmente dos líneas embriológicas: pilosebáceo-apócrina y ecrina. Se los clasifica en cuatro grupos principales: tumores del folículo piloso, tumores sebáceos, tumores apócrinos y tumores ecrinos. Su distribución anatómica refleja áreas con mayor densidad de anexos cutáneos, presentándose en forma única o múltiple.

Skin adnexal tumors are represented as a heterogeneous group of entities of low frequency range. Its origin is controversial, perhaps from pluripotent stem cells, mainly from two embryological lines: pilosebaceous-apocrine and eccrine. They are classified as four main groups: tumors of the hair follicle, sebaceous tumors, apocrine tumors and eccrine tumors. Their anatomic distribution reflects areas with the highest density of cutaneous adnexal, arising in either single or multiple forms.

Pigmented eccrine poroma: report of an atypical case with the use of dermoscopy / Poroma ecrino pigmentado: relato de caso atipico com descricao da dermoscopia

Poromas are uncommon benign neoplasms originating from the terminal ductal portion of the sweat glands, mainly characterized by skin-colored or pink pa pules or nodules, usually on the extremities. Due to their rarity, the pigmented form clinical hypothesis is hardly ever suggested and psychopathology is fundamental. We report a case of pigmented eccrine poroma in the right palmer area, a location considered atypical. We stress the importance of dermoscopy as a method for diagnosis of poromas, especially in the differential diagnosis with other pigmented nodular-popular lesions.

Os poromas são neoplasias benignas incomuns oriundas das porções ductais terminais das glândulas sudoríparas. Caracterizam-se principalmente por pápula ou nódulo cor da pele ou róseo, em geral nas extremidades. Devido à raridade, a hipótese clínica da forma pigmentada dificilmente é aventada, sendo fundamental a histopatologia. Relata-se caso de poroma écrino pigmentado na região palmar direita, localização considerada atípica. Ressalta-se a importância da dermoscopia como método auxiliar no diagnóstico do poroma e, principalmente, no diagnóstico diferencial com outras lesões pápulo-nodulares pigmentadas.

Hidradenoma papilífero: reporte de tres casos y revisión de la literatura / Hidradenoma papilliferum: report of three cases and review of the literature

El hidradenoma papilífero es un tumor infrecuente que ocurre casi de manera exclusiva en la región anogenital de las mujeres, sin embargo se han descrito casos de hidradenomas papilíferos ectópicos. Las lesiones generalmente son asintomóticas de crecimiento lento, eritematosas, firmes, y se presentan como un nódulo bien delimitado. Reportamos tres casos de hidradedoma papilífero en región vulvar, diagnosticados entre 2009 y 2010 en el Hospital Universitario de Caracas. En los tres casos se presente una lesión única, nodular, en labio mayor, con diámetro < 10 mm, renitente, nacarada, no pruriginosa. Hasta la actualidad se ha reportado solo un caso en laliteratura venezolana

Hidradenoma papilliferum is a rare tumor that occurs almost exclusively in the anogenital region of women, however has been reports ectopic hidradenoma papilliferum. The lesions are usually asymptomatic, slow-growing, erythematous, firm, and are presented as awell-defined nodule. We report three cases of vulvar hidradenoma papillifirum, diagnosed between 2009 and 2010 at the Hospital Universitario de Caracas. In all three cases was presented as a single, nodular lesion in labia major with a diameter < 10mm, firm, pearly, not itchy. Until now it has been reported only one case in Venezuelan literature

Dermatoscopía del poroma ecrino: a propósito de un caso clínico / Dermoscopy of eccrine poroma: a case report

El poroma ecrino es un tumor benigno anexial raro. Clínicamente se presenta como un nódulo solitario rosado, asintomático, topografiado comúnmente en palmas y plantas. El diagnóstico clínico diferencial más importante se plantea con el melanoma amelanótico. La dermatoscopía ayuda en algunos casos a distinguir entre ellos. Se describe el caso de una paciente de 50 años que se presenta con un nódulo palmar derecho que a la dermatoscopia presenta estructuras vasculares solitarias, glomerulares, con áreas blanco rosadas sin estructuras y pequeñas erosiones. El diagnóstico histopatológico fue de poroma ecrino.

Eccrine poroma is a rare benign adnexal tumor. It usually, presents as an asymptomatic, solitary, pink nodule in palms and soles. Its main differencial diagnosis is amelanotic melanoma. Dermoscopy can improve the differentiation between them. We describe the case of a 50 year-old woman who presented with a nodule in the her right palm which at dermoscopy showed solitary vascular structures, glomerular vessels, pink white structureless areas and little erosions. The histopathologic diagnosis was eccrine poroma.

Eccrine Spiradenoma Arising in the Breast Misdiagnosed as an Epidermal Inclusion Cyst

Eccrine spiradenomas are rare, benign, cutaneous tumors that originate in the sweat glands. Eccrine spiradenomas in the breast are very rare and only a few cases have been reported. We report here on the case of a 47-year-old woman with superficial masses in the breast and these masses had gradually increased in size during follow-up. They were confirmed to be an eccrine spiradenoma on pathologic examination. There have been a few reports about the radiologic findings of eccrine spiradenomas of the breast. This is the first case of an eccrine spiradenoma in the breast that was characterized by multiple imaging modalities, including mammography, ultrasonography and MRI. The lesion in our patient was first diagnosed as an epidermal inclusion cyst based on the imaging findings and the mass's superficial location. Although the mammographic and ultrasonographic imaging findings of eccrine spiradenomas and epidermal inclusion cysts are similar, the MRI findings are different between epidermal inclusion cysts and eccrine spiradenomas. Eccrine spiradenomas should be considered in the differential diagnosis of cutaneous and subcutaneous lesions of the breast.

A Case of Histiocytoid Variant Eccrine Sweat Gland Carcinoma of the Orbit

A 79-year-old male presented with left ocular pain. Evisceration and silicone ball implantation were performed after a diagnosis of phthisis. He returned six weeks later because of left facial erythematous swelling, tenderness, mild fever, chills and cough. His condition was diagnosed as orbital cellulitis. Despite two weeks of empirical antibiotic therapy, the symptoms worsened. A subsequent orbital computed tomography scan revealed enhanced soft tissue infiltrations in his left orbit and eyelid. Biopsy showed a diffusely infiltrating tumor of signet ring cell cytology. A systemic evaluation revealed multiple bone metastases. Based on this evidence, the patient was diagnosed with a very rare case of histiocytoid variant eccrine sweat gland carcinoma with multiple bone metastases.