53 research outputs found
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Tufted angioma presenting with subclinical coagulopathy
Tufted angioma is a rare, benign vascular tumor of uncertain pathogenesis, characterized histopathologically by "tufts" of capillaries within the dermis. A life-threatening coagulopathy, Kasabach-Merritt phenomenon, occurs in approximately 10% of cases of tufted angioma and is characterized by profound thrombocytopenia and fibrinogen consumption. We present an asymptomatic 10-month-old boy who presented with an erythematous patch of the right upper extremity and subsequently was diagnosed by biopsy with tufted angioma. Baseline laboratory workup of our patient revealed mildly decreased fibrinogen and elevated D-dimer levels without thrombocytopenia or elevated partial thromboplastin time. This suggests that asymptomatic patients with tufted angioma may present with coagulopathy in the absence of Kasabach-Merritt phenomenon. This also highlights the importance of obtaining baseline laboratory workup in patients presenting with tufted angioma
Benign follicular tumors
Benign follicular tumors comprise a large and heterogeneous group of neoplasms that share a common histogenesis and display morphological features resembling one or several portions of the normal hair follicle, or recapitulate part of its embryological development. Most cases present it as clinically nondescript single lesions and essentially of dermatological relevance. Occasionally, however, these lesions be multiple and represent a cutaneous marker of complex syndromes associated with an increased risk of visceral neoplasms. In this article, the authors present the microscopic structure of the normal hair follicle as a basis to understand the type and level of differentiation of the various follicular tumors. The main clinicopathological features and differential diagnosis of benign follicular tumors are then discussed, including dilated pore of Winer, pilar sheath acanthoma, trichoadenoma, trichilemmoma, infundibuloma, proliferating trichilemmal cyst/tumor, trichoblastoma and its variants, pilomatricoma, trichodiscoma/fibrofolliculoma, neurofollicular hamartoma and trichofolliculoma. In addition, the main syndromes presenting with multiple follicular tumors are also discussed, namely Cowden, Birt-Hogg-Dubé, Rombo and Bazex-Dupré-Christol syndromes, as well as multiple tumors of follicular infundibulum (infundibulomatosis) and multiple trichoepitheliomas. Although the diagnosis of follicular tumors relies on histological examination, we highlight the importance of their knowledge for the clinician, especially when in presence of patients with multiple lesions that may be the cutaneous marker of a cancer-prone syndrome. The dermatologist is therefore in a privileged position to recognize these lesions, which is extremely important to provide further propedeutic, appropriate referral and genetic counseling for these patients.info:eu-repo/semantics/publishedVersio
Recommended from our members
Tufted angioma presenting with subclinical coagulopathy
Tufted angioma is a rare, benign vascular tumor of uncertain pathogenesis, characterized histopathologically by "tufts" of capillaries within the dermis. A life-threatening coagulopathy, Kasabach-Merritt phenomenon, occurs in approximately 10% of cases of tufted angioma and is characterized by profound thrombocytopenia and fibrinogen consumption. We present an asymptomatic 10-month-old boy who presented with an erythematous patch of the right upper extremity and subsequently was diagnosed by biopsy with tufted angioma. Baseline laboratory workup of our patient revealed mildly decreased fibrinogen and elevated D-dimer levels without thrombocytopenia or elevated partial thromboplastin time. This suggests that asymptomatic patients with tufted angioma may present with coagulopathy in the absence of Kasabach-Merritt phenomenon. This also highlights the importance of obtaining baseline laboratory workup in patients presenting with tufted angioma
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Perineal ulcers in an infant: An unusual presentation of postnatal cytomegalovirus infection
Cytomegalovirus (CMV) disease can cause significant morbidity and mortality in neonates and immunosuppressed patients. Cutaneous disease is rare, even in at-risk patients. We report a case of CMV with perineal papules, erosions, and ulcers in a preterm but presumably immunocompetent patient, whose diagnosis was first suggested by skin biopsy and confirmed with serologic testing. The mode of transmission of CMV was unclear. The lesions resolved without CMV-specific therapy, and the child had no apparent systemic sequelae of infection. CMV disease should be considered in the differential diagnosis of perineal erosions and ulcers in infancy. In addition to the case, we briefly review the literature on cutaneous manifestations of CMV disease
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