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.

Cutaneous metastasis of gastric adenocarcinoma: an unusual clinical presentation.

Cutaneous metastases from gastric adenocarcinoma are uncommon. We report a 72-year-old man with gastric adenocarcinoma who developed a cutaneous metastasis on his left forearm, which clinically resembled carcinoma erysipelatoides but with distinct histopathological characteristics. We have not found any reported case with a similar location and histopathology.

[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.

Familial generalized multiple glomangiomyoma: report of a new family, with immunohistochemical and ultrastructural studies and review of the literature.

Glomus tumors are benign lesions which often appear as solitary bluish nodules. They can also be multiple, and can be either acquired or congenital. Histopathologically glomus tumors are classified into three different variants: solid glomus tumor, glomangioma, and glomangiomyoma, which is the least frequent type. We report three instances of familial generalized multiple glomangiomyoma in a woman and her two children. Seven members of the family in two consecutive generations were affected, suggesting autosomal dominant inheritance. We performed a thorough clinical study, complete blood and platelet counts, stool for occult blood, karyotype, abdominal echography (which showed an asymptomatic solitary hepatic vascular lesion in one of the patients), gastrointestinal endoscopy, and skin biopsy with immunohistologic and ultrastructural studies. We believe that the terms glomangioma and glomangiomyoma actually designate the same lesion, with transitional areas from typical glomus cells to well-defined conventional smooth muscle cells. Although complete evaluation should be performed in all patients with multiple glomus tumors in order to detect possible occult systemic lesions, we only recommend treatment for symptomatic lesions. To our knowledge, this is the first report of an instance of familial multiple glomangiomyoma.