Diagnostic and prognostic values of conjunctival and oral biopsies analyzed by direct immunofluorescence in patients with mucous membrane pemphigoid.

Introduction: Mucous membrane pemphigoid (MMP) is diagnosed on the basis of a characteristic clinical picture (a predilection for mucosal involvement and scarring in the affected tissues) and a positive direct immunofluorescence (DIF) result. Methods: In this study, we compare the diagnostic and prognostic values of conjunctival and oral biopsies analyzed by DIF in patients with MMP. Sixteen patients with MMP and mucosal involvement as a predominant symptom were classified into three groups based on the clinical picture. Oral and conjunctival DIF were performed on all patients. Results: Our study showed that patients with simultaneous oral and conjunctival involvement had a positive oral DIF in 83% and a positive ocular DIF in 100% of the examined cases, respectively. Patients with isolated ocular MMP had a positive oral DIF in 50% and a positive ocular DIF in 66% of the examined cases, respectively. Patients with only oral involvement with MMP had a positive oral DIF in 100% and a positive ocular DIF in 50% of the examined cases, respectively. Discussion: Oral biopsy should be performed first and is usually sufficient for the diagnosis, even in patients with exclusively ocular MMP, whereas in patients without clinical ocular involvement, ocular DIF is positive in half of the cases and may be a predictive factor for ocular lesions in the future.

Case Report: Pemphigoid Nodularis-Five Patients With Many Years of Follow-Up and Review of the Literature.

Pemphigoid nodularis is a rare form of pemphigoid that joins the clinical picture of prurigo nodularis and the immunological features of bullous pemphigoid, which is therapeutically challenging. Here, we analyze five female patients with a long-lasting course of nodular pemphigoid in terms of clinical and immunological characteristics and therapy. All the patients fulfilled clinical and immunological criteria of nodular pemphigoid. We applied numerous techniques allowing the proper diagnosis: direct and indirect immunofluorescence, salt split skin, ELISA, BIOCHIP, and fluorescence overlay antigen mapping using laser scanning confocal microscopy. Our study showed that 4 of 5 patients fulfilled the clinical and immunological criteria of nodular bullous pemphigoid. Two out of 4 patients presented exclusively nodular lesions; in the other two patients, blisters and erythematous lesions preceded prurigo-like lesions by a few years. The remaining patient had clinical and immunological criteria of nodular mucous membrane pemphigoid, presenting oral erosions, scarring conjunctivitis, and numerous disseminated nodules on the skin. All the patients were treated with multiple medicines; however, it was observed that the use of clobetasol propionate on the entire body plus antidepressants best controlled the disease. Pemphigoid nodularis mainly occurs in elderly women. In cases with coexisting psychological problems, antidepressants should be considered as an important complementary therapy to the basic one with clobetasol propionate.

Diagnosis of autoimmune subepidermal bullous diseases with mucous membrane involvement based on laser-scanning confocal microscopy.

BACKGROUND: Mucosal involvement in autoimmune subepidermal blistering disorders (ASBD) may represent the only or predominant localization. Circulating autoantibodies are detected in 50% cases. OBJECTIVE: The aim of this study was to evaluate the usefulness of fluorescence overlay antigen mapping by laser-scanning confocal microscopy (FOAM-LSCM) to identify ASBD with mucosal involvement in oral mucosa specimens. MATERIALS & METHODS: Thirty-two ASBD patients, diagnosed based on direct immunofluorescence between 2006 and 2016, were enrolled. Localization of IgG deposits bound at the basement membrane zone, relative to laminin-332 and collagen IV localization, was assessed in vivo. RESULTS: FOAM-LSCM disclosed four different immunofluorescence patterns. IgG deposits were located above laminin-332, as in bullous pemphigoid (BP-type), in 19% cases and co-localized with laminin-332 (anti-laminin-332-type) in 6% cases. IgG deposits were found below laminin-332 and above collagen IV (mucous membrane pemphigoid-type) in 59% cases, and below collagen IV (epidermolysis bullosa acquisita-type) in 16%. Circulating antibodies were found in 56% cases. CONCLUSION: The FOAM-LSCM method should be used in order to obtain a definitive diagnosis of ASBD with mucosal involvement, particularly in the presence of negative circulating antibodies.

Vegetating erosive cutaneous lesions and pyogenic granuloma in the course of mucous membrane pemphigoid: a case report and review of literature.

Mucous membrane pemphigoid (MMP) is a group of chronic autoimmune sub-epithelial blistering disorders, which mostly affect the oral mucosa and the conjunctiva. MMP is very diverse in terms of both the clinical and immunological features (IgG and IgA autoantibodies may react with different antigens). MMP can be induced by infections and medication, including ophthalmologic medication, which may lead to the development of eye lesions. In contrast, a vegetating variant of MMP is extremely rare. Here, we report an MMP case that demonstrated unusual clinical features, that is, pyogenic granulomas on the conjunctivae and extensive vegetating erosions on the skin of intertriginous regions. All these lesions were considered to be induced by unconventional medication containing arsenic.

Measuring of quality of life in autoimmune blistering disorders in Poland. Validation of disease - specific Autoimmune Bullous Disease Quality of Life (ABQOL) and the Treatment Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires.

PURPOSE: Autoimmune bullous dermatoses (AIBD) are rare, severe diseases resulting from some antibodies activity against the different adhesion structures within the skin and/or mucosa. Few studies investigated quality of life (QOL) in AIBD by generic and dermatology-specific instruments, all reporting strong impact on QOL. Recently, disease-specific measurement tools have been developed: Autoimmune Bullous Disease Quality of Life (ABQOL) and Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires. The aim of this study was to test the reliability and validity of ABQOL and TABQOL by developing the first foreign language versions and to evaluate ABQOL and TABQOL in Polish patients. MATERIAL AND METHODS: The study enrolled 80 patients from the tertiary referral center for AIBD at the outpatient clinic or on admission to the hospital. Sixty six patients completed the 17-item questionnaires of each ABQOL and TABQOL at day 0 and after 5-7 days. Both questionnaires were translated into Polish according to protocol. RESULTS: The internal consistency and test-retest reliability were high (Cronbach α=0.95 for ABQOL, α=0.87 for TABQOL), (R=0.98 for ABQOL, R=0.86 for TABQOL). In convergent validity, the correlation of ABQOL and TABQOL was strong (R=0.81), but low with objective disease activity scales. The strongest impact of AIBD on QOL has been observed in flares and in patients with the onset below 70 years of age. The patients with bullous pemphigoid had the highest QOL compared to other AIBD patients. CONCLUSIONS: The ABQOL and TABQOL are reliable and valid instruments for the assessment of QOL in AIBD.