We found a match
Your institution may have rights to this item. Sign in to continue.
- Title
Depressed indurated plaque with elastorrhexis as a distinctive lesion in Buschke‐Ollendorff syndrome.
- Authors
Velasco Huici, Rebeca; Martín, Jose María; Vázquez, Bárbara; Silva, Esmeralda; Estébanez, Andrea; Cuesta, Ana; Ramón, Dolores; Monteagudo, Carlos
- 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.
- Subjects
CONNECTIVE tissues; SYNDROMES; BONES; NEVUS; BOS
- Publication
Pediatric Dermatology, 2020, Vol 37, Issue 2, p385
- ISSN
0736-8046
- Publication type
Article
- DOI
10.1111/pde.14096