Elastosis perforans serpiginosa in a case of pseudoxanthoma elasticum: A rare association

Autor: Kavya Chennamsetty, Konakanchi Venkatachalam
Jazyk: angličtina
Rok vydání: 2016
Předmět:
Zdroj: Indian Dermatology Online Journal
Indian Dermatology Online Journal, Vol 7, Iss 2, Pp 103-106 (2016)
ISSN: 2249-5673
2229-5178
Popis: Elastosis perforans serpiginosa (EPS), characterized by transepidermal elimination of fragmented elastic fibers, clinically presents as hyperkeratotic papules. EPS is classified into three types: (1) Idiopathic; (2) reactive, with associated connective tissue diseases such as pseudoxanthoma elasticum (PXE), Ehlers-Danlos syndrome, cutis laxa, Marfan syndrome, osteogenesis imperfecta, Down's syndrome; (3) the one that is induced by D-penicillamine. A rare association of EPS with PXE, which is primarily a defect of transmembrane transporter protein with accumulation of certain metabolic compounds and secondary calcification of elastic fibers has been documented in the literature. We report a case of PXE with associated lesions that were histopathologically compatible with EPS.
Databáze: OpenAIRE