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BACKGROUND: Little is known about the ultrastructure of pili annulati. OBJECTIVES: To examine with transmission electron microscopy affected hairs of a family, whose diagnosis had been confirmed in five individuals with scanning electron microscopy, which showed surface undulations with "curtain-like" folding of the hair cuticula and to compare the findings with normal control. METHODS: Hairs of two affected patients and one control were embedded in resin and cut lengthwise to produce ultra-thin sections. RESULTS: The normal hair showed a parallel arrangement of dark lines associated with less electron-dense wide bands. Small cavities could be observed, mostly in the dark lines, affected hairs had a large number of cavities, associated or not with the insertion of melanosomes and loss of parallelism of the dark lines. Higher magnification showed a significant loss of this parallelism, resembling "wood grooves". Widened dark lines were observed in some areas. STUDY LIMITATIONS: Only a few hairs were examined. CONCLUSIONS: The present results suggest that the microcanaliculi of the hair surface, easily found with scanning electron microscopy, may be secondary not only to the cavities seen in the sections but also to the disorder of proteins that form this region, demonstrated by the changes of the cortex dark lines.

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INTRODUCTION: Hypohidrotic ectodermal dysplasia (HED) is a genetic condition typified by alterations in skin structures including sweat glands, hair, nails, and teeth. Hair findings in HED have been poorly characterized in larger series. OBJECTIVE: To characterize scalp and hair findings of patients with HED clinically and with trichoscopy and light microscopy. METHODS: A cross-sectional study in 21 pediatric HED patients was performed using available clinical and scalp dermatoscopic images, as well as pulled-hair samples for clinical evaluation, trichoscopic, and light microscopic analyses. RESULTS: Seventeen out of 21 patients (81%) were men. Twenty patients had straight hair. Sixteen patients had decreased hair density, 6 of whom had hair loss mainly in the temporal and occipital regions. Fourteen patients had hair whorls. On trichoscopy, we observed: single-hair follicular units (n = 19, 90%), scalp hyperpigmentation (n = 13, 62%), variable diameter of the hair shafts (n = 12, 57%), perifollicular scales (n = 8, 38%), scalp erythema (n = 8, 38%), and short curly pigtail hairs (n = 6, 29%). On light microscopy, findings included: hair shafts with irregular diameter (n = 7, 33%), heterogeneous hair color (n = 6, 29%), trichoptilosis (n = 2, 10%), and pili torti (n = 1, 5%). CONCLUSIONS: In this series, hair findings in HED were similar to those described in previous studies. However, we describe two new clinical and two trichoscopic findings: decreased hair density mainly in the temporal and occipital regions, oblique upwards occipital hair follicles orientation, angled hairs, and short curly pigtail hairs. These heterogeneous findings may reflect the multiple factors and signaling pathways that can be affected in these syndromes.

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El acné queloideo de la nuca (AKN) o foliculitis esclerosante es un proceso inflamatorio crónico del folículo piloso en la región de la nuca. Su incidencia es baja y su etiología desconocida. Afecta con mayor frecuencia a hombres de mediana edad y de raza negra. A lo largo del tiempo se han utilizado diferentes modalidades terapéuticas con resultados variables. Presentamos el caso de un paciente con AKN que mostró una excelente respuesta al uso diario de imiquimod 5% tópico durante ocho semanas

Keloid acne of the neck (AKN) or sclerosing folliculitis of the nape of the neck is a chronic inflammatory process of the nape region. Its incidence is low and its etiology is unknown. It mainly affects brown-black males in middle age. Different treatment modalities have been used with different responses. We present the case of a patient with AKN who presented an excellent response to the daily use of topical imiquimod 5% for eight weeks

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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.

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A pilomatrixoma is a benign skin tumor common in children, which develops from the matrix cells of hair follicles. It presents as a nodule or tumor of approximately 0.5-3 cm in size, with calcium-like consistency, faceted edges, and blue erythematous overlying skin. Sometimes, the skin may atrophy and look like a blister or even extrude calcium. Diagnosis is suspected based on the typical clinical presentation and supplementary imaging tests. The definite diagnosis is made according to histological findings. Surgical removal is the treatment of choice. Here we describe the epidemiological and clinical data, supplementary tests, and surgical techniques in relation to 149 pilomatrixomas resected in 137 patients treated at the Departments of Dermatology of two children's hospitals: Dr. Pedro de Elizalde and Prof. Dr. Juan P. Garrahan.

El pilomatrixoma es un tumor benigno cutáneo, de frecuente aparición en los niños, que se origina en las células de la matriz del folículo piloso. Se presenta como un nódulo o tumor, aproximadamente, de 0,5 a 3 cm de tamaño, de consistencia cálcica, de bordes facetados, con piel suprayacente eritematoazulada. En ocasiones, la piel puede atrofiarse, dar la apariencia de una ampolla e, incluso, extruir material cálcico. El diagnóstico se sospecha por la presentación clínica característica y los estudios de imágenes complementarios. El diagnóstico definitivo es por los hallazgos histológicos. La extirpación quirúrgica es el tratamiento de elección. Se presentan los datos epidemiológicos, clínicos, estudios complementarios y técnicas quirúrgicas empleadas de 149 pilomatrixomas resecados en 137 pacientes tratados en los Servicios de Dermatología de los hospitales de pediatría Dr. Pedro de Elizalde y Prof. Dr. Juan P. Garrahan.

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Pilomatrixoma is considered a rare benign tumor arising from the hair follicle, most common in the head and neck region, but it is rarely diagnosed on a clinical basis. This report describes a new case of giant pilomatrixoma in a 36-year-old female patient. The nodule was localized in the preauricular area on the right side, appearing as a slow-growing, fixed, painless, with a hardened consistency, unusual giant (4.5 cm). A cone-beam computed tomography (CBCT) examination showed a slightly hyperdense lesion, and fine-needle aspiration cytology (FNAC) revealed peripheral blood and mononucleated inflammatory cells. After enucleation of lesion, a diagnosis of pilomatrixoma was confirmed. The differential diagnosis of pilomatrixoma is broad, because its characteristics also can be found in other lesions common to the head and neck. Thus, a lesion in the head and neck, adherent to the skin, and well demarcated, mainly in the young and in females, should be suspected as pilomatrixoma.

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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.

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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.

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Winer's dilated pore is an infrequent appendageal tumor characterized by a giant comedone on the face, neck, and upper trunk in adults. We report a 57-year-old woman who developed multiple asymptomatic black papules on both labia majora. Histopathology showed grouped dilated follicles lined by keratinizing squamous epithelium in the superficial dermis. The superficial lining epithelium and interfollicular epidermis were atrophic, while the deep epithelium showed mild proliferation and melanin pigmentation with a few short projections extending into the surrounding dermis. We diagnosed multiple Winer's dilated pores based on late-onset lesions and pathological features. This patient may represent the first case of multiple vulvar Winer's dilated pores. We suggest that electrocautery may be effective for treating this type of superficial entity.

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Abstract: Winer's dilated pore is an infrequent appendageal tumor characterized by a giant comedone on the face, neck, and upper trunk in adults. We report a 57-year-old woman who developed multiple asymptomatic black papules on both labia majora. Histopathology showed grouped dilated follicles lined by keratinizing squamous epithelium in the superficial dermis. The superficial lining epithelium and interfollicular epidermis were atrophic, while the deep epithelium showed mild proliferation and melanin pigmentation with a few short projections extending into the surrounding dermis. We diagnosed multiple Winer's dilated pores based on late-onset lesions and pathological features. This patient may represent the first case of multiple vulvar Winer's dilated pores. We suggest that electrocautery may be effective for treating this type of superficial entity.

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Trichomycosis is a superficial infection caused by Corynebacterium flavescens, which regularly affects axillary, and to a a lesser extent, pubic, scrotal and intergluteal, and exceptionally, head hairs or trichomycosis capitis (TC). This condition is characterised by the formation of bacterial nodules. Clinically, it can be confused with white piedra or pediculosis. The diagnosis is made by microscopic and dermoscopic observation and confirmed by culture. OBJECTIVE: To present a case of TC in an infant and illustrate the microscopic, dermoscopic, and ultrastructural characteristics. CLINICAL CASE: A 6 month-old boy, otherwise healthy, with multiple yellowish concretions on the hairs of the head. TC was confirmed by yellow fluorescence with Wood’s light; white-yellowish beads, like “rosaries of crystalline stones’’ were observed on dermoscopy, direct examination showed bacterial masses, and Corynebacterium flavescens was identified by culture. A superficial infection, without perforation of the hairs, was confirmed by electron microscopy. Treatment with fusidic acid for 3 weeks achieved a clinical and microbiological cure. CONCLUSION: TC is a rare condition that affects children, and tends to be mistaken for other diseases of the hair, such as pediculosis and mycotic infections.

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La tricomicosis es una infección superficial causada por Corynebacterium flavescens, que afecta por lo regular pelos axilares, en menor grado los púbicos, los escrotales e interglúteos y excepcionalmente los de la cabeza o tricomicosis capitis (TC). Esta infección se caracteriza por formación de nódulos pilosos. Clínicamente se confunde con infecciones como piedra blanca y pediculosis. El diagnóstico se realiza por microscopia y dermatoscopia de masas bacterianas y confirmado por cultivo. OBJETIVO: Presentar un caso de TC en un infante, y mostrar las características microscópicas, dermatoscópicas y ultraestructurales. CASO CLÍNICO: Niño sano de 6 meses de edad, con dermatosis que afectó los pelos de la cabeza en forma de múltiples nódulos-pilosos amarillentos. Se comprobó TC mediante fluorescencia amarilla a la luz de Wood; a la dermatoscopia se observaron cadenas blanco-amarillentas, como "rosarios de piedras cristalinas"; al examen directo se distinguieron masas bacterianas y al cultivo se identificó Corynebacterium flavescens. A la microscopia electrónica se observó infección superficial, sin perforación de los pelos. Se realizó tratamiento con aplicación de ácido fusídico por 3 semanas y se obtuvo curación clínica y microbiológica. CONCLUSIÓN: La TC es una entidad rara que se presenta en niños, y que suele confundirse con otros padecimientos del pelo como la pediculosis e infecciones micóticas.

Trichomycosis is a superficial infection caused by Corynebacterium flavescens, which regularly affects axillary, and to a a lesser extent, pubic, scrotal and intergluteal, and exceptionally, head hairs or trichomycosis capitis (TC). This condition is characterised by the formation of bacterial nodules. Clinically, it can be confused with white piedra or pediculosis. The diagnosis is made by microscopic and dermoscopic observation and confirmed by culture. OBJECTIVE: To present a case of TC in an infant and illustrate the microscopic, dermoscopic, and ultrastructural characteristics. CLINICAL CASE: A 6 month-old boy, otherwise healthy, with multiple yellowish concretions on the hairs of the head. TC was confirmed by yellow fluorescence with Wood’s light; white-yellowish beads, like "rosaries of crystalline stones" were observed on dermoscopy, direct examination showed bacterial masses, and Corynebacterium flavescens was identified by culture. A superficial infection, without perforation of the hairs, was confirmed by electron microscopy. Treatment with fusidic acid for 3 weeks achieved a clinical and microbiological cure. CONCLUSION: TC is a rare condition that affects children, and tends to be mistaken for other diseases of the hair, such as pediculosis and mycotic infections.

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Malignant pilomatrixoma or pilomatrix carcinoma is a rare, locally aggressive malignant neoplasm, derived from the hair follicle with a high propensity for local recurrence. Only a few cases of metastatic pilomatrixoma have been described in the literature. Till date, only 17 cases have been reported in the English-language medical literature, most commonly occurring associated with local recurrence in adults. We report the first case in children of a metastatic malignant pilomatrixoma in an 8-year-old girl presenting with a recurrent pilomatrix carcinoma of the forehead with metastases in cervical and parotid lymph nodes.

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