A 36-year-old woman at the 31st gestation week of her second pregnancy presented with erythematous-edematous lesions associated with little blisters localized on the periumbilical area that spread to the abdomen, mammary area and folds (Figures 1A and 1B). Histologic and immunopathologic exams were performed. She had not developed any cutaneous lesions in her first pregnancy or in association with menstruation or oral contraceptives. The patient was treated with local corticosteroids with a modest and temporary improvement. She delivered a healthy boy with a normal birth weight and a normal Apgar score. Small bullous lesions continued to appear in the postpartum period and during the feeding (for 9 months postpartum), which were associated with mild pruritus and partially controlled by topical corticosteroids.
ResultsA lesional skin biopsy specimen routinely processed and stained with hematoxylin and eosin demonstrated a subepidermal vesiculo-bullous dermatosis and a perivascular and interstitial infiltrate of lymphocytes and eosinophils, localized in deep and superficial dermis. Direct immunofluorescence (IF) of perilesional skin performed on 6 µm cryostat-cut sections using a standard technique with fluorescein labeled antihuman immunoglobulin (Ig)G, IgA, IgM and C3, showed linear IgG (+) and C3 (+++) deposit at the basement membrane zone (BMZ). Using direct salt-split skin technique, IgG antibodies on the epidermal side of the separated skin were demonstrated (Figure 2). Indirect IF on a monkey esophagus substrate, demonstrated circulating IgG1 autoantibodies against BMZ antigens, at titer 1:80, and an indirect complement fixation test using the patient's serum, on human saltsplit skin, showed C3 deposits along the BMZ on the epidermal side (titer 1:160). Immunoblot analysis on patient serum revealed that circulating autoantibodies were directed against a 180-kd antigen (BPAg2).Routine blood examinations revealed lymphopenia (18.8%) and fibrinogen (565 mg/dL), alkaline phosphatase (450 U/L), cholesterol (308 mg/dL), and triglyceride (320 mg/dL) increases. Antithyroid autoantibodies, antigastric mucosa autoantibodies, and antinuclear autoantibodies were negative. HLA typing demonstrated an A3, A25, B18, B38, Cw7, DR4, DRw53, DQ5, and DQ8 phenotype.At 9 months postpartum, the patient stopped breast-feeding and was treated for the persisting cutaneous manifestations, with doxycycline (200 mg/daily) and nicotinamide (500 mg/ daily). This pharmacologic treatment was progressively reduced (doxycycline 100 mg/daily and nicotinamide 250 mg/daily) and we obtained a complete remission in 2 months of therapy. After 6 months from therapy suspension, no new cutaneous lesion appeared.
CommentHerpes gestationis (HG), also known as pemphigoid gestationis, is a rare dermal-epidermal bullous, autoimmune disease of pregnancy and puerperium. The first onset of HG is usually in the second or third trimester of pregnancy, but it can develop from 2 months before, until 5-6 days after delivery. HG usually clears up in the last weeks...