Direct Immunofluorescence of Mechanobullous Epidermolysis Bullosa Acquisita, Porphyria Cutanea Tarda and Pseudoporphyria
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چکیده
منابع مشابه
Porphyria Cutanea Tarda Masquerading as Epidermolysis Bullosa Acquisita: A Report of Two Cases
Porphyria cutanea tarda (PCT) is the most common type of porphyria worldwide and is often initially diagnosed when cutaneous manifestations arise. We present two patients where misdiagnosis of PCT occurred due to the condition masquerading as epidermolysis bullosa acquisita histologically. In patients with undifferentiated bullous/erosive skin conditions occurring in photo-distributed regions, ...
متن کاملPseudoporphyria or porphyria cutanea tarda? Diagnostic and treatment difficulties.
Fig. 1. Erosions on the forehead. Fig. 2. Erosions, milia and scarring on the dorsum of the hand. Sir, Porphyria cutanea tarda (PCT) is a vesiculobullous skin disorder of heme biosynthesis in which there is an enzyme defect of uroporphyrinogen decarboxylase (Uro-D), leading to accumulation of porphyrins mainly in urine but also in stool and plasma. Pseudoporphyria is clinically similar to PCT, ...
متن کاملStudy of direct immunofluorescence, immunofluorescence mapping and light microscopy in porphyria cutanea tarda.
BACKGROUND Even though porphyria cutanea tarda is the most frequent type of porphyria, there are few studies about its cutaneous physiopathology. OBJECTIVE To evaluate skin changes in porphyria cutanea tarda using light microscopy and direct immunofluorescence before and after treatment with chloroquine. To perform antigen immunomapping of bullae to study their level of cleavage. METHODS Li...
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Autoimmunity to type-WI collagen is characterized by autoantibodies predominantly of lgG class to the non-collagenous domain of type-WI collagen present in the anchoring fibrils which bind basement membrane lamina densa to the anchoring plaques in the dermis. This results in a sublami.na densa split with a blister formation. Type WI collagen autoim.munity is heterogenous in its clinical spectru...
متن کاملFamilial porphyria cutanea tarda.
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ژورنال
عنوان ژورنال: Acta Dermato Venereologica
سال: 2018
ISSN: 0001-5555
DOI: 10.2340/00015555-3021