A Case of Pseudoxanthoma Elasticum Showing Transepidermal Elimination / 대한피부과학회지

ABSTRACT

Pseudoxanthoma elasticum (PXE) is an inherited disorder involving the connective tissues of skin, eye and cardiovascular system. Both a recessive and a dominant inheritance pattern have been engaged in it. The skin changes are characterized by the yellowish crepe-like, lax, redundant folds on neck, axilla and periumbilical area. In rare instances, perforating dermatoses such as keratotic erythematous papules are also accompanied in PXE and histologically characterized by transepideraml elimination of altered basophilic calcified elastic fibers, which are short, fragmented and curled in the middle portion of the reticular dermis. Now we report a case of 26 year-old female who had a typical hereditary systemic PXE showing transepidermal elimination on her neck without preceding trauma history.