title: Ichthyosis bullosa of Siemens: report of a sporadic case.
abstract: We report a sporadic case of ichthyosis bullosa of Siemens occurring in a Korean boy. In this report, the varied findings of the clinical features in one subject over five years are presented along with an investigation of the ultrastructural alteration. The patient had suffered from blistering, superficial peeling, and dark-grey colored lichenified patches on the extremities since infancy. As he grew older, the lesions were more localized to the elbows, knees, buttock and the dorsal aspects of the hands and feet, and were replaced by yellowish, lichenified plaques. Since the original report of Siemens in 1937, nine families including one sporadic case have been reported in the literature. To our knowledge, this is the second report of sporadic case of IBS.
authors.affiliation: Department of Dermatology, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: S C
authors.initials: SC
authors.lastname: Kim
authors.forename: W
authors.initials: W
authors.lastname: Hur
authors.forename: J H
authors.initials: JH
authors.lastname: Won
authors.forename: S K
authors.initials: SK
authors.lastname: Ahn
mesh_terms: D000287:Administration, Topical; D000305:Adrenal Cortex Hormones / Q000008:administration & dosage / Q000627:therapeutic use; D001706:Biopsy; D002675:Child, Preschool; D018450:Disease Progression; D006801:Humans; D017488:Hyperkeratosis, Epidermolytic / Q000145:classification / Q000188:drug therapy / Q000473:pathology* / Q000503:physiopathology; D008297:Male; D012867:Skin / Q000473:pathology
chemical_list: D000305:Adrenal Cortex Hormones