Bullous pemphigoid: Case report and review of literature​

Bullous pemphigoid (BP) is an autoimmune bullous disease characterized by subepithelial blistering mostly affecting elderly but may be seen in all ages. A 52-year-old female patient came with a chief complaint of itching, redness and ulcer in the hands and oral cavity for the past 2 weeks. The examination revealed ulcer- ated lesions in palate and buccal mucosa. Erythematous lesions were also seen in upper limb. Incisional biopsy was done and histological examination revealed bullous pemphigoid. The patient was immediately started with systemic and topical steroids and was continued for 3 weeks. The extra-oral lesions were healed and intraoral ulcerations subsided after 3 weeks.

The epidemiology of autoimmune bullous diseases in Sudan between 2000 and 2016

Objectives Autoimmune bullous diseases vary in their clinico-epidemiological features and burden across populations. Data about these diseases was lacking in Sudan. We aimed to describe the epidemiological profile and to estimate the burden of autoimmune bullous diseases in Sudan. Methods This was a retrospective cross-sectional study conducted at Khartoum Dermatological and Venereal Diseases Teaching Hospital. We used routinely collected health care data, and included all patients with an autoimmune bullous disease who presented to the hospital between 2001 and 2016. Results Out of the 4736 patients who were admitted to the hospital during the study period, 923 (19.5%) had an autoimmune bullous disease. The average rate of patients at the hospital was 57.7 per year representing 1.3 per 100,000 population per year. After exclusion of patients where the final diagnosis was missing, 585 were included in the further analysis. Pemphigus vulgaris was the most common disease (50.9%), followed by bullous pemphigoid (28.2%), linear IgA disease/chronic bullous disease of childhood (8.4%), and pemphigus foliaceous (8.2%). Pemphigoid gestationis and IgA pemphigus constituted 1.4% and 1.2% of the cohort, respectively. Paraneoplastic pemphigus, mucous membrane pemphigoid, lichen planus pemphigoidis, bullous systemic lupus erythematosus, and dermatitis herpetiformis were rare. None of the patients had epidermolysis bullosa acquisita. Conclusions The clinico-epidemiological characteristics vary among the types of autoimmune bullous diseases. Females were more predominant in most of them. Sudanese patients tended in general to present at a younger age than other populations. The pool of Sudanese patients with autoimmune bullous diseases is large which requires investigation for the local risk factors and presents a field for future trials.

Assessing the Correlation Between Autoimmune Bullous Disease Quality of Life Questionnaires (ABQOL, TABQOL, DLQI) and Disease Severity Indices in Pemphigus and Bullous Pemphigoid

Background: Pemphigus and bullous pemphigoid (BP) are autoimmune blistering diseases (AIBDs) that affect the skin and mucous membranes, and adversely impact quality of life (QOL). Few studies have assessed the correlation between the severity of disease and QOL in patients with pemphigus and BP. Objectives: To identify the correlation between the severity of AIBDs and QOL using the Autoimmune Bullous Disease Quality of Life (ABQOL), Treatment Autoimmune Bullous Disease Quality of Life (TABQOL), and Dermatology Life Quality Index (DLQI) questionnaires in Chinese patients with pemphigus and BP at baseline, and after 1, 3 and 6 months of treatment. Methods: Pemphigus and BP patients were invited to complete the ABQOL, TABQOL, and DLQI questionnaires. We measured the pemphigus disease area index (PDAI), autoimmune bullous skin disorder intensity score (ABSIS), and antibodies of desmoglein1,desmoglein3 (DSG1/DSG3) for pemphigus; and the bullous pemphigoid disease area index (BPDAI), ABSIS, and antibodies of BP180/ BP230 for BP as disease severity indices. The correlations between the severity of disease and QOL were analyzed by Spearman’s correlation coefficient (r). Results: Eighty-five patients were included: 55 with pemphigus and 30 with BP. The pemphigus subtypes included pemphigus vulgaris (PV, n = 32), pemphigus foliaceus (PF, n = 22), and paraneoplastic pemphigus (PNP, n = 1). We found significantly strong correlations between QOL (ABQOL, TABQOL, DLQI) and severity of disease (PDAI/BPDAI, ABSIS) with (r = 0.87, 0.77, 0.83; r = 0.86, 0.73, 0.80) for pemphigus and BP, respectively. Mild or strong correlations were also observed between QOL and antibody titers in pemphigus with DSG1/DSG3(r=0.32/0.36) and BP with BP180/BP230( r = 0.73/0.17) respectively. Conclusion: The QOL of patients with pemphigus and BP decreased with increased severity of the AIBDs. As the disease severity descended, so the QOL improved. The QOL indices should be used in clinical trials and to manage patients’ treatment, especially during the active disease stage, despite the mild correlation observed after 1 month of treatment.The PDAI to be better at assessing disease severity than the ABSIS in patients with pemphigus, and ABSIS are better than BPDAI in BP patients for correlation with the QOL indices.