ISSN:
1365-2230
Source:
Blackwell Publishing Journal Backfiles 1879-2005
Topics:
Medicine
Notes:
Immunofluorescence (IF) findings are reviewed in 279 consecutive patients with a suspected primary bullous disorder (PBD), IF substantiated the diagnosis of PBD in 51%. The most frequent disorders were bullous pemphigoid (44%), dermatitis herpetiformis (25%) and pemphigus (13%) A diagnosis of PBD was refuted in 29% of cases with bullae; whereas 18% of cases presenting with an itchy papular eruption were shown to have a PBD.All 19 patients with intercellular epidermal IgG ± C3 on direct IF had a clinical and histological diagnosis of pemphigus. Thirty-five patients with granular sub-epidermal IgA ± C3 had dermatitis herpetiformis. Of the 72 with linear C3 ± IgG at the dermo-epidermal junction (DEJ), 62 had bullous pemphigoid, seven herpes gestationis, two cicatricial pemphigoid, and one epidermolysis bullosa acquisita. The 13 patients with linear IgA as the predominant immunoglobulin at the DEJ appeared to form a distinct clinical group, i.e. linear IgA disease. Deposition of IgA at the DEJ appeared to be a marker for mucosal disease in the series as a whole. The presence of a second immunoglobulin at the DEJ (in addition to IgG) in patients with bullous pemphigoid was associated with more severe disease. In 50%, of the biopsies IF was either negative or showed non-specific patterns of staining which did not associate with any particular clinical feature: of these, the most common were diffuse dermal IgG, dermal fibrinogen, granular C3 at the DEJ and vascular deposits.IF examination is of considerable value in the diagnosis and management of PBD provided that non-specific patterns of deposition are interpreted appropriately.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1111/j.1365-2230.1986.tb00475.x
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