Background: Pemphigoid gestationis (PG) is a rare autoimmune bullous disorder occurring during the last trimester of pregnancy and usually regressive within 3 months after delivery. Prolonged forms of the disease lasting more than 6 months after delivery have been reported as chronic PG. Objective: The aim of the present study was to compare the clinical and immunopathological findings between 4 patients presenting a normal regression of the disease after delivery and 6 patients with a chronic course. Methods: All patients were evaluated and studied by clinical patterns (age, mucosal and cutaneous involvement, obstetrical history, duration of the blistering disease and response to treatment), by direct and indirect immunofluorescence and Western blot. Eight patients were studied by immunoelectron microscopy (IEM) and 3 patients had an indirect IEM. Results: Patients with chronic PG were older, had multigravidity, a history of PG during previous pregnancies, widespread cutaneous eruption and mucosal involvement. Subclass analysis of circulating autoantibodies showed an IgG1 anti-BP180 response in all patients except 1 with disease of 7 years’ duration. Direct IEM was positive in 6/8 patients showing a labeling of the lamina lucida, and indirect IEM using colloidal gold probes confirmed the localization of the target antigens to the proximal part of the anchoring filaments in the lamina lucida. Conclusion: This study suggests that, even in chronic long-lasting PG, IgG1 remains the predominant subtype of IgG. Therefore no biological and predictable marker of chronicity can be ascertained from this series.

Keywords:
Chronic pemphigoid gestationis, Immunoblotting, Immunoelectron microscopy
1.
Shornick JK: Herpes gestationis. J Am Acad Dermatol 1987;17:539–556.
2.
Kelly SE, Cerio R, Bhogal BS, Black MM: The distribution of IgG subclasses in pemphigoid gestationis: PG factor is an IgG1 autoantibody. J Invest Dermatol 1989;92:695–698.
3.
Morrison LH, Labib RS, Zone JJ, et al: Herpes gestationis recognizes a 180-kD human epidermal antigen. J Clin Invest 1988;81:2023–2026.
4.
Diaz LA, Ratrie H III, Saunders WS, et al: Isolation of a human cDNA corresponding to the 180-kD autoantigen recognized by bullous pemphigoid and herpes gestationis sera. J Clin Invest 1990;86:1088–1094.
5.
Jenkins RE, Hern S, Black M: Clinical features and management of 87 patients with pemphigoid gestationis. Clin Exp Dermatol 1999;24:255–259.
6.
Holmes R, Black MM, Jurecka W, et al: Clues to the aetiology and pathogenesis of herpes gestationis. Br J Dermatol 1983;109:131–139.
7.
Holmes R, Black MM, Williamson DM, Scutt RWB: Herpes gestationis and bullous pemphigoid: A disease spectrum. Br J Dermatol 1980;103:535–541.
8.
Jenkins RE, Vaughan Jones SA, Black MM: Conversion of pemphigoid gestationis to bullous pemphigoid: Two refractory cases highlighting this association. Br J Dermatol 1995;135:595–598.
9.
Castle SP, Mather-Mondrey M, Bennion S, et al: Chronic herpes gestationis and antiphospholipid antibody syndrome successfully treated with cyclophosphamide. J Am Acad Dermatol 1996;34:333–336.
10.
Carruthers JA, Black MM, Ramnarain N: Herpes gestationis: Immunopathological and ultrastructural studies. Br J Dermatol 1977;96:35–43.
11.
Kirtschig G, Collier PM, Emmerson RW, Wojnarowska F: Severe case of pemphigoid gestationis with unusual target antigen. Br J Dermatol 1994;131:108–111.
12.
Hern S, Harman K, Bhogal BS, Black MM: A severe persistent case of pemphigoid gestationis treated with intravenous immunoglobulins and cyclosporin. Clin Exp Dermatol 1998;23:185–188.
13.
Gammon WR, Merrit CC, Lewis DM, et al: An in vitro model of immune complex mediated basement membrane zone separation caused by pemphigoid antibodies, leukocytes and complement. J Invest Dermatol 1982;78:285–290.
14.
Katz SI, Hertz K, Yaoita H: Herpes gestationis: Immunopathology and characterization of the HG factor. J Clin Invest 1976;57:1434–1441.
15.
Bernard P, Prost C, Lecerf V, et al: Studies of cicatricial pemphigoid autoantibodies using direct immunoelectron microscopy and immunoblot analysis. J Invest Dermatol 1990;94:630–635.
16.
Prost C, Dubertret L, Fosse M, et al: A routine immunoelectron microscopic technique for localizing an autoantibody on epidermal basement membrane. Br J Dermatol 1984;110:1–7.
17.
Shimizu H, McDonald JN, Kennedy AR, Eady RAJ: Demonstration of intra-and extracellular localization of bullous pemphigoid antigen using cryofixation and freeze substitution for post-embedding immunoelectron microscopy. Arch Dermatol Res 1989;281:443–448.
18.
Labib RS, Anhalt GJ, Patel HP, et al: Molecular heterogeneity of the bullous pemphigoid antigens as detected by immunoblotting. J Immunol 1986;136:1231–1235.
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2003
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