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MULTIPLE MUSHROOM-LIKE GROWING CYLINDROMAS OF THE SCALP (TURBAN TUMOR) IN A PATIENT WITH BROOKE-SPIEGLER SYNDROME: UNIQUE MANIFESTATION IN A BULGARIAN PATIENT.
Georgian Med News
October 2024
1Onkoderma - Clinic for Dermatology, Venereology and Dermatologic Surgery, Sofia, Bulgaria.
Cutaneous cylindromas are rare, slow-growing adnexal tumors commonly found on the capillitium or face. When located on the capillitium, they can cluster together, forming a headgear-like structure that gives the characteristic "turban" appearance. Brooke-Spiegler syndrome, an autosomal dominant condition, is typically benign, though malignant transformation can occur.
View Article and Find Full Text PDFA novel splicing CYLD variant associated with trichoepithelioma papulosum multiplex demonstrating intrafamilial severity variability.
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Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Multiple familial trichoepithelioma: report of a disfiguring case.
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Service of Dermatology, Complexo Hospitalar Padre Bento de Guarulhos, Guarulhos, SP, Brazil.
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Unidad de Gestión Clínica de Dermatología Médico-Quirúrgica y Venereología, Hospital Universitario Puerta del Mar, Cádiz, España.
Autosomal dominant genodermatoses in adults being heralded by superimposed skin lesions in children.
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Department of Dermatology, Medical Center-University of Freiburg, Freiburg, Germany.
Article Synopsis
- Autosomal dominant skin disorders can exhibit pronounced mosaic involvement in neonates, stemming from early loss of heterozygosity in the embryo shortly after fertilization.
- Some syndromes, like Brooke-Spiegler and Hornstein-Knickenberg, show early-onset symptoms that can foreshadow later, more widespread manifestations of the disorder.
- Conditions like glomangiomatosis and Darier disease demonstrate that neonatal mosaic lesions can be early indicators of nonsegmental skin issues emerging much later in life.
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