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Metastatic cutaneous involvement of granulomatous colitis in Hermansky-Pudlak syndrome
Nicole Weitz1, Vishal Patel, Brook Tlougan
1School of Medicine, College of Physicians and Surgeons, Columbia University, New YorkDepartment of Dermatology, Columbia University, New YorkDepartment of Pediatrics, Columbia University, New YorkDepartment of Surgery, Columbia University, New York, New York.
Hermansky-Pudlak syndrome (HPS) is a rare autosomal-recessive disorder characterized by oculocutaneous albinism, a hemorrhagic diathesis due to platelet dysfunction, and lysosomal ceroid accumulation that can cause a Crohn's-like granulomatous colitis and pulmonary fibrosis. We report peristomal and vulvar cutaneous involvement of the granulomatous colitis in HPS.
Hermansky-Pudlak syndrome (HPS) is a rare autosomal-recessive disorder characterized by oculocutaneous albinism, a hemorrhagic diathesis due to platelet dysfunction, and lysosomal ceroid accumulation that can cause a Crohn's-like granulomatous colitis and pulmonary fibrosis. We report peristomal and vulvar cutaneous involvement of the granulomatous colitis in HPS.
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