Depressed indurated plaque with elastorrhexis as a distinctive lesion in Buschke-Ollendorff syndrome.

Buschke-Ollendorff syndrome (BOS) is a rare autosomal dominant genodermatosis caused by heterozygous mutations in LEMD3 and characterized by connective tissue nevi and sclerotic bone lesions known as osteopoikilosis. We report a family with three individuals affected by BOS, two of whom manifested clinical and histopathological peculiarities, presenting with a depressed indurated plaque as the main cutaneous manifestation instead of the classic connective tissue nevi. Notable elastorrhexis was present in both biopsies.

Trichoscopy features of trichotillomania.

Trichotillomania is a form of traction alopecia resulting from repetitive and compulsive hair pulling and plucking. Trichotillomania and patchy alopecia areata may have similar clinical and dermoscopic features in some cases. On trichoscopic examination, the presence of black dots, coiled or hook hair, shafts of varying lengths with fraying or split ends (trichoptilosis), and an absence of exclamation mark hairs and yellow dots are suggestive of trichotillomania.

Infantile hemangiomas with minimal or arrested growth: A retrospective case series.

BACKGROUND/OBJECTIVES: Infantile hemangiomas (IH) with minimal or arrested growth (MAG) constitute a distinctive subset of IH based on their clinical characteristics and natural history. They are often confused with capillary malformations. METHODS: A retrospective observational study has been carried out in which clinical and perinatal characteristics have been evaluated in all IH-MAG evaluated in our Dermatology Department in a 5-year period (January 2013-December 2017). RESULTS: A total of 14 IH-MAG affecting 13 patients were identified (10 girls and 3 boys). All were born full term, with a mean birth weight of 3448 g for girls vs 3540 g for boys, corresponding to the 72nd percentile for both sexes. The IH-MAG was noticed at birth in 75% of cases. It had a segmental distribution in 6 cases and focal in 8. The most common clinical features was the presence of an erythematous or reticulated background and superficial red bright papules. Lesions were located preferentially in the lower body. Doppler ultrasound showed no significant vascular abnormalities in any case. Five IH-MAG developed ulcerations and three required treatment with propranolol. Progressive involution was observed in all cases. CONCLUSIONS: In spite of being a true IH, the absence of a relationship with prematurity, the high birth weight of the newborns, the predominance on the extremities, the higher percentage of segmental lesions, and their reduced ability to proliferate suggest possible etiopathogenic differences compared with conventional IH.

Bascule syndrome associated with syncopal episodes.

Bascule syndrome is a recently described benign vasomotor dermatosis characterized by Bier anemic spots, cyanosis, and urticaria-like eruption. We report a case of a 13-year-old girl with cutaneous lesions consistent with Bascule syndrome who had had three exercise-related syncopal episodes. It would be recommended to exclude orthostatic intolerance or postural orthostatic tachycardia syndrome when evaluating patients with Bascule syndrome.

Clinical, Microscopic and Ultrastructural Findings in a Case of Short Anagen Syndrome.

Short anagen syndrome is an uncommon and recently described disease characterized by many telogen hairs and short maximum hair length. We report here the case of a 3-year-old girl whose short, sparse, fine hair since birth was consistent with short anagen syndrome. X-ray microanalysis demonstrated normal composition of the main bioelements of her hairs.

Lymphoepitelioma-like carcinoma of the skin: report of three cases.

Primary cutaneous lymphoepithelioma-like carcinoma (LELC) is an extremely rare cutaneous neoplasm with histopathological features similar to those seen in the undifferentiated subtype of nasopharyngeal carcinoma. Microscopically, the tumor is well circumscribed and is composed of irregular nests of malignant epithelial cells in a background of reactive lymphoid cells including mature plasma cells. Its histogenesis remains unknown although an adnexal or epidermic origin has been proposed, and despite its poorly differentiated histology, the LELC prognosis is relatively good. We describe three new cases of this entity that support an epidermic origin.

Successful management of Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon using vincristine and ticlopidine.

Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor that usually occurs in skin and retroperitoneum of infants and young children. We present a case of a newborn with a rapid tumor growth and a life-threatening Kasabach-Merritt syndrome with a progressive remission after treatment with vincristine and ticlopidine.

Desmoplastic giant congenital nevus with progressive depigmentation.

Desmoplastic hairless hypopigmented nevus is an extremely rare sclerotic, alopecic, and progressively hypopigmented giant congenital melanocytic nevus, which is histologically characterized by an intense desmoplasia. A significant trend toward spontaneous involution has been described. We report a case of desmoplastic hairless hypopigmented nevus that underwent a progressive depigmentation associated with loss of its woody consistency. The loss of induration appears to be the main marker for the complete regression of these nevi.

Milium en placa palpebral bilateral / Bilateral milia en plaque of the eyelids

Milium en placa es una forma infrecuente de milium primario. Presentamos el caso de una mujer de 63 años que acudió por presentar numerosos quistes de milium sobre una placa eritematosa en las caras internas de ambos párpados superiores. El estudio histopatológico reveló la existencia de múltiples quistes rellenos de queratina, así como un infiltrado linfocitario periquístico, hallazgos complatibles con milium en placa. Las lesiones fueron tratadas inicialmente mediante expresión manual de los quistes de milium, con buena respuesta

Milia en plaque is an unusual variety of primary milia. We report a 63-year-old woman who presented with numerous milia within a symmetrical erythematous plaque on the medial aspects of the upper eyelids. Histopathological study revealed multiple keratin-fi lled cysts, as well as a pericystic lymphocytic infi ltrate, fi ndings consistent with milia en plaque. The lesions were initially treated with manual expression of the milia, with a good response

Lupus miliar diseminado de la cara / Lupus miliaris disseminatus faciei

El lupus miliar diseminado de la cara es una dermatosis inflamatoria crónica que afecta con mayor frecuencia a adultos jóvenes y que durante años se ha asimilado sucesivamente a tuberculosis, sarcoidosis y rosácea. Recientemente algunos autores han propuesto su consideración individualizada. Se trata de una erupción de pápulas eritematosas parduzcas de pequeño tamaño localizadas principalmente en área facial, sobre todo a nivel periocular. Es autolimitada y generalmente cura dejando cicatrices residuales puntiformes. Se presenta el caso de un varón de 25 años afectado de lupus miliar diseminado de la cara y discutimos sus principales características, así como los diferentes problemas de clasificación etiopatogénica

Lupus miliaris faciei is a chronic inflammatory dermatosis that most often affects young adults. For years, it has successively been considered a form of tuberculosis, sarcoidosis and rosacea. Recently, some authors have proposed that it should be considered a distinct entity. It is an eruption of small, brownish-erythematous papules, primarily located on the face, especially in the periocular area. It is self-limited and generally leaves residual punctate scars. We present the case of a 25-year-old male affected by Lupus miliaris disseminatus faciei and discuss its main characteristics, as well as the different problems in its etiopathogenic classification

[Lupus miliaris disseminatus faciei]. / Lupus miliar diseminado de la cara.

Lupus miliaris faciei is a chronic inflammatory dermatosis that most often affects young adults. For years, it has successively been considered a form of tuberculosis, sarcoidosis and rosacea. Recently, some authors have proposed that it should be considered a distinct entity. It is an eruption of small, brownish-erythematous papules, primarily located on the face, especially in the periocular area. It is self-limited and generally leaves residual punctate scars. We present the case of a 25-year-old male affected by Lupus miliaris disseminatus faciei and discuss its main characteristics, as well as the different problems in its etiopathogenic classification.

Úlceras vulvares agudas de Lipschütz / Ulcus vulvae acutum (Lipschütz ulcer)

Dos niñas vírgenes de 14 y 12 años, previamente sanas, consultaron por úlceras dolorosas genitales agudas que aparecieron en el contexto de un proceso febril. Las exploraciones complementarias descartaron tanto una enfermedad venérea como el resto de causas habituales de ulceraciones genitales. En ambos casos, las lesiones se resolvieron en menos de 2 semanas tras la administración de antibioticoterapia oral y tópica, sin secuelas ni recidivas posteriores. (AU)

Lupus eritematoso cutáneo subagudo inducido por fármacos / Drug induced subacute cutaneous lupus erythematosus

Una mujer de 46 años, sin antecedentes de enfermedades autoinmunes, desarrolló una erupción cutánea clínica e histológicamente indicativa de un lupus eritematoso cutáneo subagudo. El exantema coincidió con la administración reciente de tetrazepam, aunque también tomaba de forma prolongada anticonceptivos orales. Los estudios de autoinmunidad mostraron una elevación de anticuerpos antinucleares, antihistonas y anti-Ro. La retirada de la medicación se acompañó de una rápida mejoría clínica y de los parámetros de laboratorio, sin que presentara otros brotes con posterioridad, lo que permitió el diagnóstico de lupus eritematoso cutáneo subagudo inducido por fármacos (AU)

Eritema anular centrífugo e hipotiroidismo / Erythema annulare centrifugum and hypothyroidism

Una mujer de 54 años consultó por unas lesiones eritematosas con descamación en el borde, localizadas en la cara externa de ambas piernas, indicativas de eritema anular centrífugo. Las exploraciones complementarias evidenciaron un hipotiroidismo subclínico que mejoró, al igual que las lesiones cutáneas, con levotiroxina. Posteriormente, un desajuste en el tratamiento propició la reaparición de un hipotiroidismo clínico en esta ocasión y dos nuevos brotes de eritema anular centrífugo (AU)