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  • Title: Depressed indurated plaque with elastorrhexis as a distinctive lesion in Buschke-Ollendorff syndrome.
    Author: Velasco Huici R, Martín JM, Vázquez B, Silva E, Estébanez A, Cuesta A, Ramón D, Monteagudo C.
    Journal: Pediatr Dermatol; 2020 Mar; 37(2):385-387. PubMed ID: 31943321.
    Abstract:
    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.
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