Basal cell carcinoma arising on two variants of epidermal nevus: A case series

Introduction@#Epidermal nevi are hamartomas of the epidermis and papillary dermis that are usually present during the first years of life. Rarely, malignant transformations develop in association with epidermal nevi. Few cases have been reported worldwide, however the lifetime risk and incidence are unknown. @*Case Report@#This is a case series about basal cell carcinoma arising on epidermal nevus. The first patient is a 42-year-old Filipino female, who presented with a verrucous plaque at birth on the left temple which then developed multiple, discrete to confluent, grayish, papules and nodules on the surface. Histological examination revealed nevus sebaceus and basal cell carcinoma, pigmented type. The second patient is a 53-year-old Filipino male, who presented with a papillomatous plaque on the left temple since the first year of life which then increased in size along with the presence of a solitary bluish-black macule noted by dermoscopic examination. Histologic examination showed verrucous epidermal nevus and basal cell carcinoma, pigmented type.@*Conclusion@#Two rare cases of basal cell carcinoma arising on epidermal nevus are reported. Despite the rarity of malignant transformation on epidermal nevus, any suspicious growth warrants a biopsy. Knowledge of these cases is important for probing suspicious growth over an epidermal nevus that would prompt early treatment before these lesions progress in size making it harder to manage.

Nevo epidérmico verrucoso inflamatório linear e diagnóstico diferencial com a psoríase linear: a respeito de um caso / Inflammatory linear verrucous epidermal nevus and differential diagnosis with linear psoriasis: about a case

Nevo epidérmico verrucoso inflamatório linear e diagnóstico diferencial com a psoríase linear: a respeito de um caso RELATO DE CASOMaria Isabel Muniz Zemero1, Maria Amélia Lopes dos Santos1, Alena Margareth Darwich Mendes1, Carla Andrea Avelar Pires1,O nevo epidérmico verrucoso inflamatório linear (NEVIL) é uma variedade clínica rara de nevo epidérmico verrucoso, que se manifesta no início da infância, como lesões inflamatórias de superfície ceratósica, que coalescem e se distribuem em faixa, acompanhando as linhas de Blaschko. Faz diagnóstico diferencial com a psoríase linear, sendo difícil a diferenciação, dado os aspectos clínicos e histopatológicos comuns aos dois, enfantizando-se a necessidade de conhecer as características específicas de cada um. O objetivo deste relato é demonstrar uma afecção relativamente rara, descrita em uma menina de 5 anos, evoluindo desde os primeiros dias de vida com placas papuloceratósicas dispostas linearmente, acompanhadas de sinais inflamatórios e áreas erosadas ocupando grandes lábios, períneo e face interna e superior da coxa esquerda. Também pápulas e placas ceratósicas na região cervical posterior e borda lateral externa da planta do pé esquerdo, ascendendo ao longo da região posterior deste membro. Os critérios clínicos e histopatológicos corroboram o diagnóstico de NEVIL na diferenciação com a psoríase linear, ressaltando a importância do estabelecimento de critérios/ ferramentas que auxiliem na diferenciação destas duas dermatoses visando agilizar o diagnóstico, otimizar o tratamento e minimizar o desconforto para esses pacientes. O acompanhamento a longo prazo dos portadores é sugerido pela possibilidade ainda que mínima de malignização do NEVIL. (AU)

Inflammatory linear verrucous epidermal nevus and differential diagnosis with linear psoriasis: about a caseCASE REPORTMaria Isabel Muniz Zemero1, Maria Amélia Lopes dos Santos1, Alena Margareth Darwich Mendes1, Carla Andrea Avelar Pires1,Inflammatory Linear Verrucous Epidermal Nevus (ILVEN) is a rare clinical variety of verrucous epidermal nevus that manifests in early childhood as inflammatory lesions of keratosis surface, which coalesce and spread in band, following Blaschko lines. It makes a differential diagnosis with Linear Psoriasis; it is difficult to differentiate them given the clinical and histopathological aspects common to both, emphasizing the need to know the specific characteristics of each. The purpose of this report is to demonstrate a relatively rare affection, expressed in a 5-year-old girl, evolving from the first days of life with papulokeratosic plaques arranged linearly, followed by inflammatory signs and eroded areas, placed linearly, overtaking labia majora, perineum, the inner and upper face of the left thigh. Also, the patient showed keratotic papules and plaques in the posterior cervical region and external lateral border of the left foot plant, ascending along the posterior region of this limb. The clinical and histopathological criteria corroborate the diagnosis of ILVEN in differentiation with linear psoriasis, emphasizing the importance of establishing criteria/instruments to assist in distinguishing these two dermatoses in order to expedite the diagnosis, to optimize the treatment and minimize patients' discomfort. Long-term follow-up of patients with this disease is suggested due to the possibility, albeit minimal, of ILVEN malignancy. (AU)

Nevus epidérmico verrucoso inflamatorio lineal: una revisión de la literatura / Inflammatory linear verrucous epidermal nevus: a review of the literature

El nevus epidérmico verrucoso inflamatorio lineal (NEVIL) es un tipo de nevus epidérmico queratinocítico, poco frecuente, de aparición predominante en la infancia, con preponderancia sobre el sexo femenino. Se caracteriza por la presencia de pápulas eritematosas descamativas de aspecto psoriasiforme, intensamente pruriginosas, que tienden a coalescer para formar placas que se distribuyen siguiendo las líneas de Blaschko. Suele presentarse de forma unilateral en extremidades inferiores y tiene pobre respuesta al tratamiento.

Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare type of keratinocytic epidermal nevus that predominantly appears in childhood and female sex. It is characterized by the presence of psoriasiform, scaly, intensely itchy erythematous papules that tend to coalesce to form plaques that are distributed along Blaschko's lines. It usually affects the lower extremities unilaterally, and responds poorly to treatment.

Do you know this syndrome? Schimmelpenning-Feuerstein-Mims syndrome

Abstract Nevus sebaceous is the most common type of organoid epidermal nevus, often located on the face, following the Blaschko's lines and with alterations in the ipsilateral central nervous system. Distinct disorders can be distinguished by the type of association with epidermal nevus. Schimmelpenning-Feuerstein-Mims syndrome is a rare multisystem disorder characterized by sebaceous nevus associated with extracutaneous abnormalities affecting the brain, eyes and bones. We report the case of an 8-year-old female patient with a yellowish verrucous plaque on the left temporal area extending ipsilaterally to the cervical region, combined with cicatricial alopecia, periocular nodule, and epibulbar tumors.

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

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.

Schimmelpenning syndrome with Didymosisaplasticosebacea in an infant

Introduction@#Schimmelpenning syndrome may encompass abnormalities of the cardiovascular, skeletal, ophthalmologic and urogenital systems. Nevus sebaceous is a hallmark finding and ophthalmologic findings are seen in 59% of the cases which include colobomas and choristomas.@*Case Summary@#A 1-month-old female presented with a verrucous plaque over the scalp and right zygomatic area upon birth. Physical examination reveals a linear yellowish alopecic verrucous plaque over the right frontal region, yellowish alopecic verrucous plaque topped with a skin colored papule over the right zygomatic region, conjunctival mass over the right eye and an atrophic patch with areas of circular erosion over the right occipital region. Ballard score and reflexes were appropriate for gestational age. Newborn screening was normal and otoacoustic-emission-test revealed no hearing loss. She was referred to an ophthalmologist and was assessed to have a lipodermoid, right upper eyelid and optic nerve coloboma. Cranial CT scan is unremarkable. Histopathology showed an increase in number of sebaceous glands with malformed hair units. She was managed holistically and does not have seizures and no secondary development of tumors in the nevus sebaceous.@*Conclusion@#Schimmelpenning syndrome is usually associated with the clinical triad of nevus sebaceous, mental retardation and seizures. In this case, seizures were absent, however, there is an associated lipodermoid, right upper eyelid and right optic nerve coloboma. In addition, she also presented with aplasia cutis congenita. Hence, it is important to look for other manifestations when patients present with nevus sebaceous because management requires collaboration with different specialties.

Systematized verrucous epidermal nevus partially responsive to acitretin: A case report

@#<p style="text-align: justify;">A 5-year-old female presented a 4-year history of generalized well-demarcated asymptomatic brown to dark brown thin verrucous plaques with a Blaschkoid distribution. Histopathology was consistent with an epidermal nevus. Patient was diagnosed to have systematized verrucous epidermal nevus. Due to the extent of the lesions, surgical management was not feasible. Hence acitretin was given which showed partial decrease in the thickness of the lesions.</p>

Is Ki-67, keratin 16, involucrin, and filaggrin immunostaining sufficient to diagnose inflammatory linear verrucous epidermal nevus? A report of eight cases and a comparison with psoriasis vulgaris

Abstract: Inflammatory linear verrucous epidermal nevus and linear psoriasis are sometimes hard to differentiate clinically and pathologically. Although immunohistochemical expression of keratin 10 (K10), K16, Ki-67, and involucrin may be useful for differentiating both entities, these results have been reported in only a few cases. We collected data from 8 patients with inflammatory linear verrucous epidermal nevus, 11 with psoriasis vulgaris, and 8 healthy controls and evaluated immunohistochemical expression of Ki-67, K16, involucrin, and filaggrin among them. Ki-67 and K16 overexpression was similar in inflammatory linear verrucous epidermal nevus and psoriasis vulgaris compared with normal skin. Although staining for involucrin showed discontinuous expression in parakeratotic regions in 4 inflammatory linear verrucous epidermal nevus cases, it was continuous in the other 4 cases and in all psoriasis vulgaris cases. Filaggrin expression was present in hyperkeratotic regions but scarce in parakeratotic areas in both inflammatory linear verrucous epidermal nevus and psoriasis vulgaris. The immunostaining pattern of Ki-67, K16, involucrin, and filaggrin may be insufficient to discriminate inflammatory linear verrucous epidermal nevus from psoriasis vulgaris.

Wooly hair nevus

Abstract Woolly hair nevus is a rare condition characterized by a structural anomaly of the hair, restricted to certain areas of the scalp. The hair becomes coiled and slightly hypopigmented. The term woolly hair refers to changes that affect all the scalp and has a hereditary character. We present a case of woolly hair nevus, that developed at the age of 2 years, associated with dental diastema and verrucous epidermal nevus.

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.

Squamous cell carcinoma arising in a multiple verrucous epidermal nevus

Abstract Verrucous epidermal nevi are hamartomatous lesions of the epidermis that, unlike other epidermal nevi (such as sebaceous nevus or nevus comedonicus), are rarely associated with malignant neoplasms. The majority of squamous cell carcinoma develop in linear or multiple epidermal nevus and rarely in solitary epidermal nevus. In general, the prognosis is favorable. We report a case of well-differentiated invasive squamous cell carcinoma arising from a multiple verrucous epidermal nevus. Although there is no consensus on prophylactic removal of epidermal nevus, its removal and biopsy should be considered if changes occur.

Basal Cell Carcinoma Arising in an Agminate Nevus Sebaceous / 대한피부과학회지

No abstract available.

O to Z flaps in facial reconstructions

Local flaps are the standard procedure to reconstruct facial defects. As it occurs in any surgical procedure, the incision should be planned so that scars are located in the minimum skin tension lines. We report two cases of O to Z flaps in the supra and infraciliary regions. One of them is a hatchet flap.