Poland's syndrome: a concise review of the clinical features highlighting associated dermatologic manifestations.
Poland's syndrome is a rare congenital condition characterized by absence of the pectoralis major muscle and a wide spectrum of associated ipsilateral chest wall and upper extremity anomalies. Associated dermatologic anomalies classically include pectoral and axillary alopecia, anhidrosis due to the absence of sweat glands, and deficiency in subcutaneous fat. Functional limitations are minimal, and thus surgical correction is primarily indicated for aesthetic purposes. Chest wall reconstruction typically involves transposition of a latissimus dorsi myocutaneous flap with or without a temporary subcutaneous tissue expander prior to surgical reconstruction. Using the PubMed database, a literature review was done on Poland's syndrome. We summarize the key features of Poland's syndrome, including the epidemiology, clinical presentation, pathogenesis, and management, and highlight the dermatologic associations reported in the literature.
School of Medicine, University of California San Diego, 9500 Gilman Drive, La Jolla, CA, 92093, USA. avazirnia@ucsd.edu.
Aria
A
Vazirnia
Department of Dermatology, University of California San Diego, 9500 Gilman Drive, La Jolla, CA, 92093, USA. mitehead@gmail.com.
Philip R
PR
Cohen
D000505:Alopecia / Q000209:etiology; D006801:Humans; D007007:Hypohidrosis / Q000209:etiology; D011045:Poland Syndrome / Q000453:epidemiology / Q000503:physiopathology* / Q000601:surgery; D012871:Skin Diseases / Q000209:etiology* / Q000473:pathology; D050151:Subcutaneous Fat / Q000002:abnormalities; D035441:Thoracic Wall / Q000002:abnormalities / Q000601:surgery