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[Atypically localized persistent skin changes in erythropoietic protoporphyria (EPP)--histologic and electron microscopic findings (author's transl)].

Authors: W, Meigel; G, Schaeg; G, Goerz;

[Atypically localized persistent skin changes in erythropoietic protoporphyria (EPP)--histologic and electron microscopic findings (author's transl)].

Abstract

The case of a 29 year old man is presented, who showed 'sunburn like' reactions following short exposures to light since early childhood. Demonstration of fluorocytes and analysis of porphyrins confirmed the diagnosis of erythropoietic protoporphyria (EPP). It is noteworthy that the patient failed to show clinical signs, characteristic for EPP at the nasal area or the extensor aspects of the hands, whereas indurated plaques with firm skin coloured papules were observed in the shoulder regions. Biopsies taken from these areas revealed PAS-positive material especially around the capillaries of the dermal papillae, a finding typically for EPP. Electronmicroscopic investigation revealed reduplication of the vascular basal lamina and perivascular deposition of a fine fibrillar material. Therefore the cutaneous changes, yet clinically atypical, seem to be correlated with EPP.

Keywords

Adult, Male, Microscopy, Electron, Porphyrias, Porphyrins, Erythroblasts, Humans, Protoporphyrins, Photosensitivity Disorders, Skin Diseases, Skin

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selected citations
These citations are derived from selected sources.
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
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