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.

The transition between a poroma and a porocarcinoma evidenced by the dermoscopy

Abstract Eccrine porocarcinoma (EPC) is a rare malignant skin tumor. The dermoscopy of invasive EPC reveals focal presence of whitish-pink, structureless areas surrounded by pinkish-white halos. In an eccrine poroma (EP), such areas present diffuse distribution in the "frog- eggs" pattern. We reported an EPC in situ that presents a transitional dermoscopy pattern between EP and invasive EPC. We found a diffuse distribution; whitish-pink, structureless areas surrounded by pinkish-white halos; a central exulceration and a polymorphic vascular pattern.

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

Dermatoscopy: an auxiliary resource for the diagnosis of poroid neoplasms

Abstract: Poroid neoplasms are benign epithelial proliferations with eccrine sweat gland differentiation. They are a challenging diagnosis because of the clinical heterogeneity, being able to mimic several malignant neoplasms. They are classified into classic poroma, hidroacanthoma simplex, dermal duct tumor and poroid hidradenoma. Association of histological subtypes occurs in more than 25% of cases. We report a case of a combined poroid neoplasia of classical poroma and poroid hidradenoma, reviewing its dermatoscopic features.

Syringocystadenoma papilliferum combined with a tubular apocrine adenoma

Abstract: Syringocystadenoma papilliferum and tubular apocrine adenoma are rare benign sweat gland tumors. Syringocystadenoma papilliferum occurs alone or in association with other tumors. Although it is rare, the association of tubular apocrine adenoma with syringocystadenoma papilliferum developing in a sebaceous nevus on the scalp is well documented. However, the co-existence of these two tumors without the background of a sebaceous nevus has not been frequently reported. Syringocystadenoma papilliferum and tubular apocrine adenoma may have a histopathological overlap, but a few cases of a syringocystadenoma papilliferum combined with a tubular apocrine adenoma have been reported. Herein we describe an unusual case of syringocystadenoma papilliferum co-existing with a tubular apocrine adenoma located on the back of a 14-year-old patient in the absence of a pre-existing sebaceous nevus.

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.

Case for diagnosis. A bluish nodule on the scalp

Abstract: Acrospiroma, also known as hidradenoma, is a rare cutaneous tumor that has several histological characteristics. As a consequence, a high index of suspicion is necessary for its diagnosis. Here we report a case that illustrates the importance of a good clinical-pathologic correlation in order to recognize this disease.

Hidroacanthoma simplex: dermoscopy and cryosurgery treatment

Abstract: Hidroacanthoma simplex is a rare intraepidermal neoplasia that arises from the acrosyringial portion of the eccrine duct. Malignant transformation of hidroacanthoma simplex is reported in the literature and the treatment is performed with wide excision or Mohs micrographic surgery. We report the first case successfully treated with cryosurgery with a long-term follow up.

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.

Siringocistoadenoma papilífero y carcinoma basocelular en nevo sebáceo: reporte de caso / Papilliferous sarcomatocystoma and basal cell carcinoma in nevus sebaceous: case report

El nevo sebáceo o nevo de Jadassohn es un hamartoma cutáneo congénito poco frecuente formado por múltiples estructuras cutáneas. Presenta potencial de transformación a una variedad de neoplasias epidérmicas benignas y malignas, que suelen presentarse de manera individual. Si bien esta descrito en la literatura, es inusual el desarrollo de más de una neoplasia sobre un nevo sebáceo. En esta publicación se presenta el caso de un hombre de 62 años con una lesión de 3 años de evolución, cuyo estudio histopatológico demostró la presencia de un carcinoma basocelular y un siringocistoadenoma papilífero sobre un nevo sebáceo de Jadassohn.

Nevus sebaceous of Jadassohn is an infrequent cutaneous congenital hamartoma, formed by multiple cutaneous structures. It possesses a transformation potential to benign and malignant epidermic neoplasms, that usually present individually. Even though a few cases have been published, the coexistence of two or more tumors is rare. We hereby present the case of a 62 years old male, with a nevus sebaceous of Jadassohn history, that after excisional biopsy showed the presence of a basal cell carcinoma associated to a syringocystoadenoma papilliferum.

Melanoma in situ and syringoma: a rare collision tumor. Clinical-pathological report of a case

Abstract Collision or contiguous tumors, defined as two or more distinct tumors occurring at one site, are often an unexpected finding and may represent a diagnostic challenge, as clinical and histological presentations do not always coincide. Various combinations of collision tumors have been described with respect to melanocytic lesions, with the most frequently reported being the combination of nevus and basal cell carcinoma. We present an unusual case on the nose involving a melanoma in situ and a clinically-inapparent syringoma, which, to the best of our knowledge, is the first report of this combination.

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.

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.

Syringocystadenoma papilliferum in an unusual location

Abstract: Syringocystadenoma papilliferum is a rare benign hamartomatous adnexal tumor of the apocrine or eccrine sweat glands. Most patients present a solitary lesion in the head and neck region. Presentation outside the head and neck region is even more uncommon. We present a case of Syringocystadenoma papilliferum with papulonodular lesion located on the vulva of an infant girl. This case illustrates the atypical location of this rare disease and adds to the differential diagnosis of lesions on the vulva.