ABSTRACT
BACKGROUND: Bullous pemphigoid (BP) is the most common autoimmune blistering disease of the skin requiring skin and serum tests for a precise diagnosis. OBJECTIVES: We analysed the sensitivity and specificity of BP-relevant parameters and the value of autoantibody titres during follow-up of BP patients. MATERIALS & METHODS: In a retrospective single-centre study, we included 200 consecutive patients with BP and 400 non-BP patients, and evaluated the test results of patients' serum and skin. In addition, we followed patients' autoantibody titres and clinical characteristics. RESULTS: BP180-ELISA revealed the highest sensitivity (85.0%; specificity: 93.9%), while BP230-ELISA demonstrated the lowest sensitivity (55.5%; specificity: 92.9%). Direct and indirect immunofluorescence showed comparable results for sensitivity (77.2%/72.7%) and specificity (94.9%/93.7%). The sensitivity for skin histology was 76.3% (specificity: 81.3%). Longitudinal analysis showed significant changes in autoantibody titres. CONCLUSIONS: BP diagnostics should include serum tests for BP autoantibodies and skin immunofluorescence. Skin histology is supportive for diagnosis. Autoantibody titres are markers for disease activity.
Subject(s)
Autoantigens/analysis , Dystonin/analysis , Non-Fibrillar Collagens/analysis , Pemphigoid, Bullous/immunology , Aged , Enzyme-Linked Immunosorbent Assay , Eosinophils/cytology , Female , Fluorescent Antibody Technique, Direct , Fluorescent Antibody Technique, Indirect , Humans , Leukocyte Count , Male , Pemphigoid, Bullous/diagnosis , Retrospective Studies , Sensitivity and Specificity , Collagen Type XVIIABSTRACT
INTRODUCTION: Itching nodules and papules are common findings. A rare but important differential diagnosis is the nodular subtype of bullous pemphigoid. METHODS AND RESULTS: The investigators report a female patient presenting with strongly itching papules disseminated over her extremities and trunk. Physical examination revealed multiple erythematous, mostly excoriated papules and nodules on her back, abdomen, and extremities. Histology showed changes compatible with prurigo lesion, and immunofluorescence showed positive results for BP180 and BP230. Considering these clinical, histologic, and immunofluorescence findings, the diagnosis of a nodular subtype of bullous pemphigoid was made. The patient showed healing of lesions under a combination therapy with systemic psoralen and ultraviolet A, topical application of corticosteroids, and systemic therapy with azathioprine and prednisolone. DISCUSSION: Pemphigoid nodularis represents the rare prurigo variant of bullous pemphigoid. Typically, lesions show the same immunopathologic and histologic features as in common bullous pemphigoid but mostly without the characteristic clinical finding of bullae.
Subject(s)
Pemphigoid, Bullous/diagnosis , Pemphigoid, Bullous/pathology , Aged, 80 and over , Autoantigens/analysis , Dystonin/analysis , Female , Humans , Non-Fibrillar Collagens/analysis , Pemphigoid, Bullous/therapy , Pruritus/etiology , Collagen Type XVIIABSTRACT
Bullous pemphigoid (BP) is a common autoimmune blistering disorder of the elderly. Several diagnostic modalities are available, including clinical impression, histopathology, direct and indirect immunofluorescence, and enzyme-linked immunosorbent assay (ELISA) detection of pathogenic antibodies. In this study, we aim to examine the utility of the newest test, ELISA, in comparison to the constellation of other tests. We describe our clinical experience in which 170 patients diagnosed with bullous pemphigoid had multiple tests performed. BP180 alone showed a sensitivity of 54 % and specificity of 94 %. The positive predictive value (PPV) is 95 % while the negative predictive value (NPV) is 52 %. BP230 alone yielded a sensitivity of 48 % and specificity of 94 %. The PPV is 94 % and the NPV is 49 %. Using both tests in combination yielded a sensitivity of 66 % and specificity of 89 %. The PPV of at least one of two tests returning positive is 92 % while the NPV of dual negative tests is 58 %. Use of ELISAs for suspected cases of BP are an inadequate standalone test, and are only helpful in making the diagnosis should they return positive. However, they would appear to miss about one-third of cases.
Subject(s)
Autoantigens/analysis , Dystonin/analysis , Enzyme-Linked Immunosorbent Assay/methods , Non-Fibrillar Collagens/analysis , Pemphigoid, Bullous/diagnosis , Autoantibodies/blood , Autoantibodies/immunology , Autoantigens/immunology , Humans , Retrospective Studies , Sensitivity and Specificity , Collagen Type XVIIABSTRACT
BACKGROUND: Mucous membrane pemphigoid (MMP) and oral lichen planus (OLP) show similar clinical features on the oral mucosa. As clinical manifestations of oral mucosal lesions, MMP shows blisters and erosions, whereas OLP shows lace-like whitish lesions in an annular arrangement with erythema and erosions. Histopathologically, MMP shows subepithelial bullae with infiltrates of lymphocytes and neutrophils, whereas OLP shows band-like interface infiltration of lymphocytes with damage in basal cells. However, these two diseases are frequently difficult to distinguish both clinically and histopathologically. OBJECTIVES: We report four patients with oral MMP who showed OLP-like clinical and histopathological lesions. METHODS: We performed direct immunofluorescence, indirect immunofluorescence of normal human skin and 1 m NaCl-split skin, enzyme-linked immunosorbent assays for BP180, BP230, and desmogleins 1 and 3, and immunoblotting of normal human epidermal and dermal extracts, recombinant proteins of BP180-NC16a and -C-terminal domains, concentrated culture supernatant of HaCaT cells, and purified laminin-332. RESULTS: The results of various immunological studies suggested the diagnoses of various types of MMP for all four patients. CONCLUSIONS: Because MMP and OLP require different treatments, all dentists and dermatologists should have knowledge about the disease entity and the serological diagnostic methods for various types of MMP.
