Pemphigoid Nodularis: Two Case Studies and Analysis of Autoantibodies before and after the Development of Generalized Blistering

Abstract

We report 45- and 61-year-old women with generalized prurigo nodularis-like eruption whose clinical, histologic and immunopathologic features were consistent with the diagnosis of pemphigoid nodularis. In one case, nodular lesions preceded the onset of generalized blistering by two years and in the other, no definite blister nor erosion was seen except for some appearing on the soles during the course of the disease. Western immunoblotting of EDTA-separated epidermal extracts revealed that the 230-kD bullous pemphigoid (BP) antigen was recognized by circulating autoantibodies in the patient sera, but the 180-kD BP antigen was not. The 180-kD BP antigen was recognized weakly by immunoblotting of the 180-kD BP antigen NC16a domain fusion protein, which shows high detection sensitivity. These findings suggested that weak reactivity of autoantibodies with either whole or a part of the 180-kD BP antigen molecule in some way accounts for negligible or localized blister-formation in this disorder. However, no particular change was noted in the reactivity with 180-kD BP antigen between the patient serum obtained before and after the development of generalized blistering. It is possible that different factors from the changes in serum reactivity with BP antigens may be involved in initiating generalized blistering.

IgG autoantibody reactivity against bullous pemphigoid (BP) 180 and BP230 in elderly patients with pruritic dermatoses

“… 16 investigated retrospectively the humoral response to BP180 by a commercially available BP180‐NC16A ELISA in 25 elderly patients with pruritus, and found that 12% of the subjects showed IgG reactivity against the NH 2 terminus of the BP180 ectodomain (Table 4). Finally, a series of case reports detected the same autoantibody profile in patients with pruritic prepemphigoid compared with full‐blown BP with tense blisters which in some cases developed later on 23–28 . Notably, the present study also detected IgG against BP180 and BP230 in two elderly individuals with immediate‐type allergy who had experienced neither pruritic nor bullous skin lesions.…”

Atypical presentations of bullous pemphigoid: Clinical and immunopathological aspects

“…However, sometimes blisters cannot be observed throughout the whole course of the disease, making diagnosis substantially difficult. Many authors have defined this particular form of BP pemphigoid nodularis (PN) [25][26][27][28][29][30][31][32][33][34] Fig. 1.…”

Bullous Pemphigoid as Pruritus in the Elderly

“…2,[10][11][12][13][14] In a Swiss study population, 20% of 160 patients diagnosed with BP did not have blisters. 15 In the literature, patients have been reported with itch combined with immunopathologic findings compatible with BP but without the formation of blisters, when followed up for several months or even years. [5][6][7][8][9]11,14,[16][17][18][19] In previous studies, most of these patients were older than 70 years, with a follow-up of 4 months to 6 years.…”