Table. Characteristics of the 7 Study Patients Diagnosed With Pemphigoid.
| Pemphigoid Phenotype | Clinical Findings | IIF SSS (Roof Staining) | IIF ME | Antigen by ELISA BP180, NC16A, BP230, Immunoblot | DIF Anti-BMZa | Treatment and Follow-up (Duration) |
|---|---|---|---|---|---|---|
| Bullous | Mild pruritus, moderate blisters, erythema, excoriations and hyperpigmentation on abdomen, back and extremities | IgG3+ | IgG+ | BP180, BP230 | NA | Lesional superpotent corticosteroids |
| Partial remission (9 mo), until death due to natural causes | ||||||
| Bullous | Severe pruritus, generalized blisters, erosions, erythema, papules and urticaria | IgG3+ | IgG+/– | BP180 | IgG, C3c | Oral corticosteroids, doxycycline, lesional super potent corticosteroids |
| IgA1+ | Linear | Death due to natural causes shortly after study examination | ||||
| Bullous | Mild pruritus, localized blisters on legs with excoriations, hyperpigmentation and hematoma | IgG1+ | IgG+ | BP180, BP230 | IgG, C3c | Oral corticosteroids, methotrexate (15 mg/wk) and lesional superpotent corticosteroids |
| N-serrated | Complete remission (18 mo) | |||||
| Nonbullous | Severe generalized pruritus with excoriations, papules, erythema | IgG1+ | IgG+ | BP230 | IgA | Doxycycline, whole-body superpotent corticosteroids |
| N-serrated | Partial remission (17 mo) | |||||
| Nonbullous | Severe pruritus with excoriations, erythema, papules and urticaria on back and extremities | IgG3+ | IgG+ | BP230 | Negative | Methotrexate (7.5 mg/wk) |
| Complete remission (11 mo), flare after tapering and complete remission (9 mo) after retreatment | ||||||
| Nonbullous | Severe pruritus with excoriations, erythema and urticaria on back and extremities | IgG1+ | IgG+ | BP230 | Negative | Methotrexate (7.5 mg/wk) |
| Complete remission (7 mo), flare after tapering and complete remission (1 mo) after retreatment, until death due to natural causes. | ||||||
| Nonbullous | Mild pruritus with erythema, papules and excoriations on extremities | IgG2+ | IgG+ | BP230 | Negative | Topical steroid ointments |
| Complete remission (18 mo) |
Abbreviations: BMZ, basement membrane zone; C3c, complement C3; DIF, direct immunofluorescence; ELISA, enzyme-linked immunosorbent assay; IgG, immunoglobulin G; IgA, immunoglobulin A; IIF, indirect immunofluorescence; ME, monkey esophagus; NA, not available; NC16A, noncollagenous 16A domain of BP180; SSS, salt-split skin; +, positive; +/−, doubtful positive.
a
Not a study requirement, performed on a voluntary basis.