Pilomatrixoma buloso: una rara pero característica variante clínica / Bullous pilomatrixoma: a rare but characteristic clinical variant

El pilomatrixoma es un tumor anexial benigno, que muestra diferenciación hacia células matriciales y supramatriciales del folículo piloso. Es el segundo tumor benigno más frecuente en la población infantil luego del quiste epidérmico. Su presentación clínica habitual consiste en una lesión única, de consistencia firme y generalmente indolora.1 La variante anetodérmica o bulosa es poco frecuente, en general de rápido crecimiento y en ocasiones dolorosa.2 Se presenta a continuación un caso clínico de esta rara variante, por la importancia de conocer esta particular forma presentación.

A clinical case of bullous pilomatrixoma is present. Correspond to the second most frequent benign tumor in children, derived from the matrix cells of the hair follicle. It commonly presents as a single lesion, of firm consistency and mostly painless. The bullous variant corresponds to 2% of the presentations, the morphology is explained by the dilation of the lymphatic vessels and the consequent extravasation of lymph, as well as by the absence of elastic and collagen fibers. Treatment is the surgical excision of the lesion, and a biopsy specimen is necessary to establish a definitive diagnosis. Knowing this form of presentation allows us to pose it as a diagnostic option in clinical practice.

Manifestaciones clínicas de pilomatrixoma en un paciente pediátrico / Clinical manifestations of pilomatrixoma in a pediatric patient

Introducción: El pilomatrixoma es un tumor benigno que afecta fundamentalmente a pacientes femeninas menores de 10 años. Se manifiesta como una masa sólida y encapsulada, situada profundamente en la dermis inferior y recubierto de piel normal; puede medir entre 3 y 30 milímetros. Objetivo: Informar las manifestaciones clínicas de este tumor que permiten llegar al diagnóstico de un pilomatrixoma. Presentación del caso: Se presenta el caso de un adolescente de 12 años de edad, que acude a consulta de dermatología del Hospital Andino de Chimborazo, con manifestaciones clínicas que permiten hacer el diagnóstico de pilomatrixoma. Conclusiones: El pilomaxitroma es un raro tumor que, aunque afecta frecuentemente a niñas menores de 10 años, también puede presentarse en pacientes mayores del sexo masculino. El estudio anatomopatológico resulta vital para realizar el diagnóstico de la enfermedad y una vez realizado dicho diagnóstico la excéresis del tumor es el procedimiento más adecuado(AU)

Introduction: Pilomatrixomais a benign tumor that mainly affects female patients under 10 years old. It manifests as a solid and encapsulated mass, located deep in the lower dermis and covered with normal skin; it can measure between 3 and 30 mm. Objective: To present the clinical manifestations that allow to reach the diagnosis of a pilomatrixoma. Case presentation: It is presented the case of a 12-year-old male adolescent, who attended the dermatology clinic in the Andean Hospital of Chimborazo, with clinical manifestations that allow the diagnosis of pilomatrixoma. Conclusions: Pilomaxitroma is a rare tumor that, although it frequently affects girls under 10 years of age, can also occur in older male patients. The anatomopathological study is vital to make the diagnosis of the disease and once performed the same exceresis of the tumor is the most appropriate procedure(AU)

Pilomatrixoma proliferante una rara variante histopatológica simulando un carcinoma de células escamosas / Pilomatrixoma proliferating a rare variant histopathological simulating a cell carcinoma scaly

El Pilomatrixoma es un tumor anexial benigno. Presenta una variante histopatológica infrecuente denominada pilomatrixoma proliferante reportada en 1997 por Kaddu et al. Corresponde a una lesión compuesta predominantemente por una proliferación lobular de células basaloides, con atipia nuclear variable y figuras mitóticas, áreas focales que contienen material cornificado eosinófilo, junto con células sombra. Se propuso al pilomatrixoma proliferante como un subconjunto histopatológico distintivo del pilomatrixoma y se consideró como una variante proliferativa con un perfil histopatológico benigno. La dermatoscopía en este tumor, sobre todo en pacientes de edad avanzada, puede llegar a constituir una trampa dermatoscópica, que es difícil de diferenciar de otras lesiones, como el melanoma o el carcinoma de células basales. Existen múltiples reportes de casos en la literatura donde se informa de pilomatrixomas clásicos o proliferantes simulando otras neoplasias. Presentamos el caso de una paciente de 88 años con pilomatrixoma proliferante facial que simuló clínicamente un carcinoma de células escamosas y llevó a confusión diagnóstica inicial, se destacan las características histopatológicas y clínicas de los pilomatrixomas proliferantes.

Pilomatrixoma is a benign adnexal tumor. It has an infrequent histopathological variant called proliferating pilomatrixoma reported in 1997 by Kaddu et al. It corresponds to a lesion composed predominantly by a lobular proliferation of basaloid cells, with variable nuclear atypia and mitotic figures, focal areas containing eosinophilic cornified material, together with shadow cells. The proliferating pilomatrixoma was proposed as a distinctive histopathological subset of the pilomatrixoma and was considered as a proliferative variant with a benign histopathological profile. Dermatoscopy in this tumor, especially in elderly patients, can result in a dermatoscopic trap, which makes it difficult to differentiate from other lesions, such as melanoma or basal cell carcinoma. There are multiple reports of cases in the literature where classic or proliferating pilomatrixomas were reported simulating other neoplasms. We present the case of an 88-year-old patient with a proliferating facial pilomatrixoma that clinically simulated a squamous cell carcinoma and led to an initial diagnostic confusion, highlighting the histopathological and clinical characteristics of the proliferating pilomatrixoma.

Pilomatrixoma quístico con verruga vulgar intraquística: reporte de un caso / Cystic pilomatrixoma with intracystic vulgar wart: case report

El pilomatrixoma es una neoplasia anexial benigna, que habitualmente se presenta como pápula o nódulo subcutáneo, de crecimiento lento y asintomático. Es más frecuente en niños. El diagnóstico se basa en hallazgos clínicos, y la ecografía es útil como examen complementario. El tratamiento es su resección quirúrgica. Se presenta un caso infrecuente de pilomatrixoma quístico con verruga vulgar intraquística en un paciente adulto.

Pilomatricoma is a benign adnexal tumor, usually presenting as a slow growing asymptomatic subcutaneous papule or nodule. It is more common in children. The diagnosis is based on clinical findings, and ultrasound is useful as a complementary workup tool. Its treatment is surgical excision. We present a rare case of a cystic pilomatricoma with an intracystic verruca vulgaris in an adult patient.

Pilomatrixoma ampollar en caso pediátrico / Bullous pilomatrixoma in pediatric case

Se realiza la presentación de un caso clínico en edad pediátrica con diagnóstico de pilomatrixoma, variedad ampollar. Este tumor benigno originado en la vaina del folículo piloso muestra una incidencia elevada en las dos primeras décadas de vida. El objetivo de este artículo es presentar un caso con diagnóstico de un tumor, con características clínicas atípicas e inusuales. En ocasiones este tipo de tumor, conllevan al diagnóstico erróneo de la entidad, realizándose el diagnóstico de certeza mediante el estudio histopatológico una vez que se procede a la exéresis quirúrgica de la lesión (AU).

A clinical case with diagnosis of bullous variety pilomatrixoma in pediatric age is presented. This benign tumor originated in the sheath of the hair follicle shows a high incidence in the first 20 years of life. The aim of this work is presenting a case with tumor diagnosis, the clinical characteristics of which sometimes leads to an erroneous diagnosis of the entity in the atypical and unusual varieties. The accurate diagnosis was made through the pathologic study after the surgical excision of the lesion (AU).

Múltiplos pilomatricomas em membros superiores: relato de exérese cirúrgica / Multiple pilomatricomas in upper limbs: report of surgical exeresis

Pilomatricoma é uma neoplasia folicular benigna relativamente rara. Pode ocorrer em qualquer idade, mas menos de 13% dos casos acontecem em pessoas com mais de 50 anos, sendo mais comum em mulheres (1,5 a 2,5 mulheres:1 homem). A localização mais frequente é a região cervical, seguida das áreas frontais, periorbitárias e pré-auriculares (1). Clinicamente, apresenta-se como um tumor subcutâneo ou intradérmico, endurecido e de crescimento lento. O diagnóstico pré-cirúrgico é quase sempre difícil (3). O tratamento padrão é o cirúrgico, com margens de segurança amplas para minimizar o risco de recorrência nas variantes malignas (2,3). Relatamos um caso atípico e exuberante de múltiplos pilomatricomas em membros superiores em um paciente do sexo masculino de 55 anos (AU)

Pilomatricoma is a relatively rare benign follicular neoplasm. It can occur at any age, but less than 13% of cases occur in people over 50 years old, and it is more common in women (1.5 to 2.5 women: 1 man). The most frequent location is the cervical region, followed by the frontal, periorbital and pre-auricular areas (1). Clinically it presents as a hardened and slowly growing subcutaneous or intradermal tumor. Preoperative diagnosis is almost always difficult (3). The standard treatment is surgical, with wide safety margins to minimize the risk of recurrence in malignant variants (2,3). We report an atypical and exuberant case of multiple pilomatricomas in upper limbs in a 55-year-old male patient (AU)

Atypical presentation of pilomatrixoma in the tarsal conjunctiva / Apresentação atípica de pilomatricoma na conjuntiva tarsal

ABSTRACT Pilomatrixoma is a rare benign tumor, which usually affects young women in the head and neck region. The eyelid is a common site of the disease, though it is very rare in the tarsal conjunctiva.The pilomatrixoma has clinical pleomorphism, which confuses this disease with other similar conditions. The diagnosis is made by pathological examination in most cases. The treatment is surgical, performed by total excision of the lesion with clear margins and recurrence is very uncommon.We present an unusual case of probable recurrent pilomatrixoma in the left upper tarsal conjunctiva.

RESUMO O pilomatricoma é uma neoplasia benigna rara, geralmente acomete mulheres jovens, ocorrendo na região da cabeça e pescoço. A pálpebra é um local comum de aparecimento do pilomatricoma, contudo, seu aparecimento na conjuntiva tarsal é muito raro. O diagnóstico é feito pelo exame anátomo-patológico na maioria dos casos, pois seu pleomorfismo clínico o confunde com outras alterações. O tratamento é cirúrgico, realizado por meio da exérese total da lesão com margens livres. São raros os casos de recidiva. Apresentamos um caso atípico de provável recidiva de pilomatricoma, localizado na conjuntiva tarsal superior à esquerda.

Pilomatricoma Unmasked on Fine Needle Aspiration Cytology.

Pilomatrixoma is an uncommon adnexal tumor with heterogeneous features can mimic various malignancies and contributes to false positive diagnosis on cytology. It is an uncommon, slow growing, benign skin adnexal neoplasm of hair matrix origin initially described by Malherbe and Chenantais. This tumor is predominantly found in the head and neck region, and presents as a small, asymptomatic, slow-growing dermal, subcutaneous, solitary lesion. More than 50% of these tumors are known to occur in the second decade of life and is often misdiagnosed clinically. Cytological features pose a diagnostic challenge although the histological features are very well delineated. This paper presents the cytological features in two cases outlining the importance of the clinicopathological features that need to be considered for an accurate diagnosis. Awareness of its varied cytologic features and clinical presentation can avoid misdiagnosis.

Pilomatrixoma anetodérmico / Anetodermic Pilomatricoma

El pilomatrixoma anetodérmico es una rara variante de pilomatrixoma, cuyo mecanismo etiopatogénico es desconocido. Se caracteriza por la presencia de una piel atrófica, plegada, con aspecto queloideo, que recubre un nódulo de consistencia dura. Su diagnóstico es clínico e histopatológico y el tratamiento es quirúrgico. Se reporta el caso de un paciente varón de 14 años con pilomatrixoma anetodérmico en omóplato derecho.

Anetodermic pilomatricoma is a rare variant of pilomatrixoma whose etiopathogenic mechanism is unknown. lts characterized by the presence of atrophic skin, fold, with keloidal appearance covering a hard nodule. The diagnosis is clinical and histopathological and the treatment is surgery. The case of a male patient of 14 years with anetodermic pilomatricoma in right shoulder blade is reported.

Pilomatrixoma en la región preauricular: caso clínico / Pilomatrixoma in preauricular region: case report

El Pilomatrixoma o epitelioma calcificado de Malherbe es un tumor benigno derivado de los folículos pilosos. Afecta con más frecuencia a niños y adultos jóvenes. Ellos son nódulos dérmicos o subcutáneos que se presentan normalmente en forma asintomática, solitarios, con una consistencia firme o dura y no adheridos a planos profundos. Reportamos un caso de una niña con un pilomatrixoma en la región preauricular.

Pilomatrixoma or calcifying epithelioma of Malherbe is a benign tumor derived from hair follicles. Frequently found in children and young adults. They are usually asymptomatic, solitary, firm or hard, freely mobile, dermal or subcutaneous nodules. We present a case of a girl with a pilomatrixoma in preauricular region.

Pilomatrixoma / Pilomatrixoma

Niña de 6 años de edad que presenta una tumoración palpable en antebrazo derecho, de consistencia dura y coloración azulada, de aproximadamente diez meses de evolución, indolora. Se realiza una ecografía de partes blandas y se observa sobre formación palpable una imagen ovoidea, de 12 x 8 mm, de contornos regulares, con halo hipoecoico, de ecoestructura heterogénea con calcificaciones en su interior, con refuerzo de los ecos posteriores, ubicado en hipodermis. Diagnóstico presuntivo: pilomatrixoma. La paciente no presentaba enfermedades asociadas. Pasados seis meses no hubo cambios significativos y hasta el momento se mantiene conducta expectante.

Follicular hybrid cyst on the cheek: A combination of infundibular, isthmic, and pilomatrical differentiation.

A hybrid cyst is a rare condition which includes any type of cyst arising from the various parts of the pilosebaceous unit. We report a case of hybrid cyst with infundibular, isthmic, and pilomatrical differentiation. A 12-year-old boy presented with a mass on the right cheek. The physical examination revealed an oval-shaped, dark-gray protruding mass with dimensions of 0.9 cm × 0.6 cm that was non-tender to palpation. The mass was totally excised. The diagnosis of a hybrid cyst was confirmed by histological evaluation. It was a well-encapsulated mass and showed combined histologic features of infundibular, isthmus, and pilomatrical differentiation. The hybrid cyst is a follicular cyst that includes two or more components of a cystic lesion arising from the pilosebaceous unit. Occasionally, there have been reports of hybrid cysts consisting of two components in differentiation, but those showing more than two components of differentiation have been extremely rare. The pathogenesis of this unusual disease is not yet known.

Pilomatrixoma: una presentación clínica inusual / Pilomatrixoma: unusual clinical presentation

El pilomatrixoma, una neoplasia benigna poco frecuente, se origina de la matriz del folículo piloso. Es más frecuente durante la infancia, especialmente entre los 5 y 15 años de edad. La forma múltiple de pilomatrixoma es rara y su presencia se asocia a síndromes sistémicos, entre los cuales destaca la distrofa miotónica. El diagnóstico de pilomatrixoma es fundamentalmente clínico. Estudios complementarios como la ecografía pueden ser útiles para confrmar su diagnóstico. La resección quirúrgica de la lesión corresponde el tratamiento curativo, con baja tasa de recurrencia. Se presenta una paciente de sexo femenino, de 17 años de edad, por su pilomatrixoma de características clínicas inusuales en edad pediátrica: variante anetodérmica, forma múltiple y crecimiento rápido.

Pilomatrixoma is a rare, benign neoplasm that is derived from hair matrix cells. It is more frequent during childhood, especially between the ages of 5 and 15 years. Pilomatrixoma is usually a hard, well circumscribed, solitary lesion, and can be located on any part of the body, except palms and soles, with a predilection for maxilofacial region. Multiple pilomatrixomas are rare and they have been associated with various systemic syndromes, of which myotonic dystrophy has been the most described. The diagnosis of pilomatrixoma is fundamentally clinical. Complementary studies such as ultrasound can be useful to confrm the diagnosis. Excisional surgery is the standard curative treatment. We present a 17-year-old female patient with an extremely uncommon clinical presentation of pilomatrixoma: multiple lesions, anetodermic, and rapid growth.

Pilomatrixoma nasal / Nasal pilomatricoma

Se expone el caso de un paciente de 1 año 3 meses, quien presenta un tumor subcutáneo nasal, el cual estaba rotulado como hemangioma cavernoso. Tras su extirpación completa se diagnostica un pilomatrixoma, derivado de la matriz pilosa. Se presenta el caso como diagnóstico diferencial de tumores subcutáneos nasales.

The case of a 15 month-old patient that presented a nasal subcutaneous tumor, which was diagnosed as cavernous hemangioma is discussed. After its complete extirpation, a pilomatricoma was diagnosed, derived from the pilosa matrix. The case is presented as a differential diagnosis of nasal subcutaneous tumors.

Pilomatrixoma en adulto, con presentación clínica infrecuente / Pilomatrixoma in adult, with infrequent clinical presentation

El pilomatrixoma es un tumor benigno con diferenciación hacia las células matriciales del folículo piloso. Usualmente se manifiesta como un nódulo firme, solitario y asintomático de crecimiento lento. Se trata de una neoplasia frecuente en la población pediátrica. El pilomatrixoma dispone de una amplia variedad de características clínicas; a menudo su diagnóstico es erróneo. El tratamiento es la excisión quirúrgica. El diagnóstico diferencial de pilomatrixoma también se debe considerar en pacientes adultos. Presentamos un caso clínico en un varón de 27 años con lesión tumoral clínicamente anetodérmica en muslo derecho cuya histología fue compatible con pilomatrixoma. Destacamos este caso por la baja frecuencia en adultos y su localización inusual.

Pilomatricoma: a dermal analog of calcifying odontogenic cyst.

Pilomatricoma, also known as "Calcifying Epithelioma of Malherbe" is an uncommon, benign, cutaneous tumor originating from the hair matrix. Most often, its precise preoperative diagnosis is not possible. Rarity of this lesion and its histological resemblance to the calcifying odontogenic cyst (the Gorlin's cyst) make it relevant, both to the maxillofacial surgeons and the pathologists. Here, we describe a case of pilomatricoma of the periorbital region in a young Indian female patient. We believe that its inclusion is essential in the differential diagnosis of hard masses of the maxillofacial and paraoral regions.