Elastosis perforans serpiginosa in a case of pseudoxanthoma elasticum: A rare association
Autor: | Kavya Chennamsetty, Konakanchi Venkatachalam |
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Jazyk: | angličtina |
Rok vydání: | 2016 |
Předmět: |
Marfan syndrome
medicine.medical_specialty Connective tissue Case Report transepidermal elimination 030207 dermatology & venereal diseases 03 medical and health sciences 0302 clinical medicine medicine lcsh:Dermatology pseudoxanthoma elasticum elastosis perforans serpiginosa prominent mental crease Angioid streaks business.industry lcsh:RL1-803 medicine.disease Pseudoxanthoma elasticum Dermatology medicine.anatomical_structure Osteogenesis imperfecta 030220 oncology & carcinogenesis business Cutis laxa Elastosis perforans serpiginosa Calcification |
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 |
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