Hyperpigmented Connective Tissue Nevus in Buschke-Ollendorff Syndrome: A Case Report / 대한피부과학회지
Korean Journal of Dermatology
; : 560-563, 2021.
Article
in En
| WPRIM
| ID: wpr-901942
Responsible library:
WPRO
ABSTRACT
Buschke-Ollendorff syndrome (BOS) is a rare autosomal dominant inherited genetic cutaneous disorder characterized by connective tissue nevi that may or may not be accompanied by sclerotic bone lesions (osteopoikilosis). BOS is caused by loss-of-function mutations in LEMD3. Skin lesions appear in childhood and may increase in size and number with age and primarily manifest as yellow or skin-colored plaques or papules. A 21-year-old male presented with brownish, firm plaques on his back and buttocks since childhood. He revealed a history of similar skin lesions in his mother and brother. He was incidentally diagnosed with osteopoikilosis, 2 years prior to presentation. Histopathological examination of a skin biopsy specimen revealed increased collagenous stroma. We report a rare case of Buschke-Ollendorff syndrome that presented with brownish plaques.
Full text:
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Database:
WPRIM
Language:
En
Journal:
Korean Journal of Dermatology
Year:
2021
Document type:
Article