Blistering Disorders (Porphyria Cutanea Tarda and Pseudoporphyria)

DeWitt, Christine A.; Trapp, Kara; Muszynski, Melissa A.

doi:10.1007/978-1-4939-2395-3_13

Cited by 1 publication

(2 citation statements)

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“…Similar to our study, nail dystrophy is reported to be common in mEBA (9,12). Nail dystrophy is not known to occur in (pseudo)porphyria, although another nail abnormality, photo-onycholysis, has been described (13,14). This, however, was not present in any of the 20 patients with (pseudo)porphyria in the current study.…”

Section: Discussionsupporting

confidence: 88%

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Direct Immunofluorescence of Mechanobullous Epidermolysis Bullosa Acquisita, Porphyria Cutanea Tarda and Pseudoporphyria

Groot¹,

Jonkman²,

Pas³

et al. 2018

Acta Derm Venerol

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Mechanobullous epidermolysis bullosa acquisita (mEBA) can have a clinical presentation that is very similar to other blistering diseases, such as porphyria cutanea tarda (PCT) and pseudoporphyria. Direct immunofluorescence is an important feature in the diagnosis of mEBA, although features that overlap with PCT and pseudoporphyria have been reported. This retrospective observational study investigated whether direct immunofluorescence can discriminate mEBA from PCT and pseudoporphyria. Biopsies of 13 patients with mEBA, 10 with PCT and 10 with pseudoporphyria were included. In 7 cases of PCT and 4 of pseudoporphyria, direct immunofluorescence showed a pattern at the dermal-epidermal junction that appeared similar to the u-serrated pattern in mEBA. Vessel wall depositions were observed in all 3 diseases, but were more frequent and more intense in PCT and pseudoporphyria than in mEBA. Careful examination of direct immunofluorescence of mEBA vs. PCT and pseudoporphyria revealed different staining patterns, although overlapping features were present. Therefore, integrating all clinical and laboratory data is essential to differentiate between mEBA, PCT and pseudoporphyria.

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Section: Discussionsupporting

confidence: 88%

“…Festooning is recognized as a classic feature of PCT and pseudoporphyria (13,16,17). Indeed, festooning was found in all our cases of PCT and 38% of cases of pseudoporphyria.…”

Section: Discussionmentioning

confidence: 51%