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.

Nevo de Jadassohn asociado a siringocistoadenoma papilífero / Associated Jadessohn's nevus syringocistoadenoma papilífero

El siringocistoadenoma papilífero (SCAP) es un tumor anexial benigno, poco frecuente, que deriva de las glándulas sudoríparas écrinas o apócrinas. Afecta a ambos sexos por igual, se observa desde el nacimiento o en la primera infancia. Se manifiesta como una placa solitaria alopécica en cuero cabelludo, o como pápulas de color piel en rostro y cuello. En el 40% de los casos se asocia a un nevo sebáceo preexistente. El tratamiento de elección es la extirpación quirúrgica. Se presenta un varón de 14 años, con un nevo de Jadassohn presente desde el nacimiento, que desarrolló años posteriores un SCAP. Palabras clave Nevo de Jadassohn, nevo sebáceo, siringocistoadenoma papilífero, tumor anexial benigno.

The syringocystadenoma papilliferum (SCAP) is an adnexal benign tumor, uncommon, which derives from the eccrine or apocrine sebaceous glands. It affects both sexes equally, it occurs most frequently from birth or early childhood. It manifests clinically as a solitary raised plate located mainly on scalp, or as papules in face, and neck. In 40% of the cases it is associated with a pre-existing sebaceous nevus. The treatment of choice is surgery. We present a 14 year old male, with a history of a nevus of Jadassohn present from birth, that years later developed a SCAP

A Case of Eruptive Syringoma on Forehead in a 9 Year-old Boy / 대한피부과학회지

No abstract available.

Late-onset of Generalized Eruptive Syringoma / 대한피부과학회지

No abstract available.

Two Cases of Unilateral Linear Syringoma on Trunk Following Blaschko Lines / 대한피부과학회지

No abstract available.

Eccrine Tumor Showing Combined Feature of Eccrine Hidrocystoma and Syringoma / 대한피부과학회지

No abstract available.

Siringocistoadenoma papilífero: reporte de 11 casos del Hospital Clínico de la Universidad de Chile / Papilliferum Syringocystoadenoma: report of 11 cases of Clinical Hospital of the University of Chile

Introducción: El siringocistoadenoma papilífero (SP) es un tumor anexial benigno inusual, de etiología desconocida. Por lo general, se presenta desde el nacimiento, o en la primera infancia como una lesión aislada, frecuentemente asociada a un nevo sebáceo de Jadassohn. Objetivo: Describir y analizar las características demográficas, clínicas e histopatológicas del SP. Materiales y métodos: Revisión retrospectiva de informes histopatológicos del Departamento de Anatomía Patológica del Hospital Clínico de la Universidad de Chile entre los años 2005 y 2016. Se incluyeron todas las muestras que describieron dentro del diagnóstico histológico las palabras "siringocistoadenoma papilífero". Resultados: La muestra está compuesta por 11 pacientes; 9 mujeres y 2 hombres. En 5 pacientes, se desarrolló un SP a partir de un nevo sebáceo y en 6 surgió de novo. El total de la muestra desarrolló el SP durante o posterior a la pubertad. Tanto los pacientes con SP solitario, como aquellos asociados a un nevo sebáceo, presentaron clínica similar con una placa o pápula verrucosa aislada. Los principales diagnósticos diferenciales fueron el nevo sebáceo, nevo verrucoso y verruga. Conclusiones: Este estudio constituye la primera serie de casos de SP en un hospital en Santiago de Chile. La forma clínica más frecuente fue como placa verrucosa solitaria asintomática, con casos aislados como pápula verrucosa y sólo un caso con aspecto de cuerno cutáneo. Se destaca el rol de la extirpación quirúrgica completa, tanto para establecer el diagnóstico, como para ofrecer un tratamiento definitivo, disminuyendo el riesgo de transformación maligna a siringocistoadenocarcinoma papilífero.

Introduction: Syringocystoadenoma papilliferum (SCAP) is an unusual benign adnexal tumor of unknown etiology. Usually it is presented at birth or in early childhood as an isolated lesion associated with nevus sebaceous of Jadassohn. Objective: To describe and analyze the demographic, clinical and histopathological characteristics of SCAP. Materials and methods: A retrospective review of histopathological reports was performed at the Clinical Hospital´s Department of Pathological Anatomy from the University of Chile including biopsies from 2005 to 2016. All the samples that described the histological diagnosis of "syringocystoadenoma papilliferum" were included. Results: The sample consists of 11 patients; 9 women and 2 men. In 5 patients, an SCAP was developed from a sebaceous nevus and in 6 it developed de novo. The total sample developed SCAP during or after puberty. Both the patients with solitary SCAP, and those associated with nevo sebaceous, presented similar clinical signs with an isolated verrucous plaque or papule. The main differential diagnoses were the nevo sebaceous, warty nevus and wart. Conclusions: This study is the first SCAP case series in Santiago, Chile. The most frequent clinical form was an asymptomatic solitary verrucous plaque, with isolated cases as a verrucous papule and a single case as a cutaneous horn. The role of complete surgical resection is highlighted, both to establish the diagnosis and to offer a definitive treatment, reducing the risk of malignant transformation to syringocystadenocarcinoma papilliferum.

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.

Sorafenib-induced Syringoma-like Eccrine Duct Proliferation in a Patient with Hepatocellular Carcinoma / 대한피부과학회지

Syringomas are common benign neoplasms that are derived from the intradermal eccrine duct and frequently manifest in adult females as 1~3-mm flesh-colored papules on the face, especially on the lower eyelid. There are two hypotheses about their pathogenesis: ductal obstruction by keratin plugs and reactive eccrine proliferation following an inflammatory condition. The patient was a 66-year-old man with multiple erythematous papules and nodules on both periorbital areas after 7-day sorafenib (Nexavar®) treatment. A skin biopsy of the Rt. periorbital lesion revealed multiple cysts and tadpole-shaped tubular structures in the dermal layer. Inflammatory cell infiltration was seen throughout the dermis. Based on the clinical features and histological findings, we diagnosed the patient with syringoma-like eccrine duct proliferation. The indications for and use of Sorafenib have increased recently. Although sorafenib-associated cutaneous side effects have increased, no cases of syringoma-like eccrine duct proliferation have been reported to date. Here we report a case of sorafenib-induced syringoma-like eccrine duct proliferation in a patient with hepatocellular carcinoma.

A Case of Vulvar Milium-like Syringoma in a Child Treated with Tranilast / 대한피부과학회지

Syringoma is the most common type of benign intraepidermal eccrine sweat gland tumor in Korea, and is usually found in women in their forties. It presents mostly as a localized lesion, preferring the lower eyelid, cheek, or forehead, and rarely invades the vulval area, and in the case of children, vulvar invasion is even more rare. Tranilast is an antihistamine used for atopic dermatitis and asthma, and has recently been used for the treatment of keloid. A few previous studies have reported both localized and generalized forms of syringoma being effectively resolved with tranilast. Herein, we report a rare and interesting case of milium-like syringoma, which manifested on the vulval area of 10-year old girl that was successfully treated with tranilast.

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.

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.

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.

Late-onset of eruptive syringomas: a diagnostic challenge

Syringoma is a benign, adnexal tumor of the eccrine sweat gland ducts. Eruptive syringomas are a rare variant, occurring before or during puberty in most cases. A 57-year-old man was observed in our department, with a 10-year history of multiple brownish papules (1-4mm in diameter), localized on the neck, shoulders, trunk and axillae. The clinical diagnosis was cutaneous mastocytosis. Histopathological examination from a papule in the trunk was compatible with the diagnosis of syringoma. The patient was treated with isotretinoin, without any improvement. The clinical diagnosis of eruptive syringoma is diffi cult and histological examination is crucial for its diagnosis. Long-term morbidity is not associated with syringomas; they are treated for cosmetic reasons with unsatisfactory results.

Syringoma of vulva: an unusual presentation. Clinical, morphological and immunohistochemnical aspects / Siringoma de vulva / una presentación inusual. Aspectos clínicos, histopatológicos e inmunohistoquímicos

The case of a 34-year-old woman, who consulted because she observed the appearance of numerous yellow-white asymptomatic papules on the vulva, is presented. Clinical diagnosis of syringoma of vulva was established. The pathological and immunohistochemical studies confirmed the diagnosis. Vulvar syringoma usually occurs as a multiple flesh-colored or brownish papules on both sides of labia majora of women in their third decade. Its diagnosis should be considered when the patient complaints of vulvar pruritus and/or sweating.

Se presenta un caso de una paciente de 34 años de edad quien consultó por presentar la aparición de numerosas pápulas de color blanco-amarillentas en la vulva. El diagnóstico clínico de siringoma de vulva fue realizado. Los estudios de patología y de inmunohistoquimica confirmaron el diagnóstico. El siringoma vulvar usualmente se presenta como múltiples pápulas del color de la piel o marrones en ambos labios mayores en mujeres en su tercera década de la vida. Su diagnóstico debe ser considerado en pacientes que se quejan de prurito y/o sudoración vulvar

Siringomas: presentación de casosclínicos y revisión de la bibliografía / Syringomas: a clinical cases series and literature review

Los Siringomas son tumoraciones benignas que comprometen el epitelio ductal de la glándula sudorípara ecrina.La etiología es desconocida. El motivo de consulta suele ser por razones estéticas, sin embargo la afección en genitales genera cancerofobia o venerofobia. En la clasificación se incluyen variantes clínicas y asociación a diferentes enfermedades y síndromes.El diagnóstico es clínico e histopatológico. Existen múltiples opciones terapéuticas pero ninguna óptima.En este trabajo presentamos cuatro pacientes con manifestaciones clínicas diferentes y revisión de la literatura.

Syringomas are benign tumors of unknown etiology where the ductal epithelium from sudoriparous eccrine gland is compromised. Main complaint is due to the aesthetic nature, however the genital condition creates fear of cancer or venereal diseases. Classification includes clinical variants and association with different diseases and syndromes. Diagnosis is made by clinical and histopathological examination. Multiple treatment options are proposed with no effective outcome. Four cases with different clinical manifestations are reported and literature review is performed.

Microcystic Adnexal Carcinoma Misdiagnosed as Desmoplastic Trichoepithelioma on Preoperative Biopsy

Microcystic adnexal carcinoma is a rare type of tumor, with about 300 cases reported globally. Due to its similar histology with other tumors, it is occasionally misdiagnosed as desmoplastic trichoepithelioma, basal cell carcinoma, syringoma, and so on. We present a patient with a mass on the perioral area who was preoperatively diagnosed with trichoepithelioma. Microcystic adnexal carcinoma was diagnosed after excisional biopsy and a wide excision. Defects were reconstructed with a mucosal advancement flap. There was no recurrence and there were no significant complications during the 18-month follow-up period. Because superficial punch biopsy has limitations in width and depth, surgeons should always consider the possibility of malignancy of a mass even if a biopsy shows a benign result.

Protease-Activated Receptor-2 Is Associated with Terminal Differentiation of Epidermis and Eccrine Sweat Glands

BACKGROUND: Protease-activated receptor 2 (PAR-2) participates in various biological activities, including the regulation of epidermal barrier homeostasis, inflammation, pain perception, and melanosome transfer in the skin. OBJECTIVE: To evaluate the basic physiological role of PAR-2 in skin. METHODS: We investigated PAR-2 expression in human epidermis, skin tumors, and cultured epidermal cells using western blot and immunohistochemical analysis. Additionally, we examined the effect of the PAR-2 agonist, SLIGRL-NH2, on cultured keratinocytes. RESULTS: Strong PAR-2 immunoreactivity was observed in the granular layer of normal human skin and the acrosyringium of the eccrine sweat glands. In contrast, weak PAR-2 immunoreactivity was seen in the granular layer of callused skin and in the duct and gland cells of the eccrine sweat glands. Interestingly, PAR-2 immunoreactivity was very weak or absent in the tumor cells of squamous cell carcinoma (SCC) and syringoma. PAR-2 was detected in primary keratinocytes and SV-40T-transformed human epidermal keratinocytes (SV-HEKs), an immortalized keratinocyte cell line, but not in SCC12 cells. SV-HEKs that were fully differentiated following calcium treatment displayed higher PAR-2 expression than undifferentiated SV-HEKs. Treatment of cultured SV-HEKs with PAR-2 agonist increased loricrin and filaggrin expression, a terminal differentiation marker. CONCLUSION: Our data suggest that PAR-2 is associated with terminal differentiation of epidermis and eccrine sweat glands.

A Case of Vulvar Syringoma in a 9-year Old Child / 대한피부과학회지

No abstract available.

A Case of Scrotal Calcinosis Originating from a Milium-like Syringoma / 대한피부과학회지

Scrotal calcinosis is a relatively common tumor of the male genitalia but there is controversy in the literature regarding its pathogenesis. The authors of many case reports debate whether the lesions are truly idiopathic or if they arise from other conditions. Only few cases of scrotal calcinosis have been proven to have an eccrine origin so far. Herein, we report a case of scrotal calcinosis originating from a milium-like syringoma in a male Korean patient.