ABNORMALITIES IN DERMAL CONNECTIVE TISSUE. Erik Austin, D.O., M.P.H. Elastosis perforans serpiginosa – Serpiginous arrangement of confluent, keratotic papules on the arms, face/neck, legs. Keratotic papules of EPS Typical site affected = neck. Elastosis perforans serpiginosa EPS.
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Erik Austin, D.O., M.P.H.
Hyperelastic epidermis that clutches the increased dermal elastic fibers like a clawEPS
Transepidermal elimination of neutrophils and elastic fibers from the dermis through a channel in the epidermisEPS
Inborn error in the metabolism if methionine
Periorbital ecchymosis, “raccoon sign” reticular dermisprimary systemic amyloidosis
Extremities, trunk, genitals and face with localized nodules
Erythropoietic forms the increased porphyrins leads to photosensitivity
Congenital erythropoietic porphyria (CEP)
Erythropoietic protoporphyria (EPP)
Erythropoietic coproporphyria ECP
Acute intermittent porphyria (AIP)
ALA dehydrogenase deficiency
Hereditary coproporphyria (HCP)
Variegate porphyria (VP)
Porphyria cutanea tardaCurrent grouping of the porphyrias is based on the primary site of increased porphyrin production
Multiple erosions with hemorrhagic crusts, as well as an intact blister on the lateral fourth finger
sunbed use, hemodialysis
Fluorescence of circulating red blood cells, CEP with UVA eyelashes, “monkey face”
Vs. transient fluorescence in EPP