UNASSIGNED: Pemphigus vulgaris is an autoimmune vesiculobullous mucocutaneous disorder with life-threatening consequences. Early detection and adequate care are crucial for a good prognosis. This study aimed to determine the demographic data, clinical features, and the prognosis of patients with oral pemphigus vulgaris.
UNASSIGNED: From 2001 to 2021, all diagnosed oral pemphigus vulgaris cases were extracted. Each patient\'s demographic and clinical data were gathered. Patients were called via phone to assess the prognosis, treatment type, and specialty of the physician who provided the diagnosis and therapy.
UNASSIGNED: The majority of the patients had only oral lesions with higher prevalence in female who also expressed severe pain than male. Only 14 of 29 patients responded phone calls. Except for one, all patients were in active disease. More than half of those respondents said pemphigus negatively affects social behavior and food intake. Correct diagnosis and treatment were decided by dermatology, oral medicine, and maxillofacial surgery specialists.
UNASSIGNED: Oral pemphigus vulgaris was prevalent in females. Severe pain was common in females and older people. Even with effective therapy, the prognosis was poor. Medical and dental professionals had little knowledge of pemphigus vulgaris. Patients frequently report poor quality of life.

Bullous systemic lupus erythematosus (BSLE) is a rare subtype of systemic lupus erythematosus (SLE) that is clinically characterized by subepidermal tense vesicles or bullae. We aimed to investigate the clinical and laboratory features of patients with BSLE.
We retrospectively reviewed all patients who fulfilled the diagnostic criteria for BSLE in our institution from 2015 to 2021. Cutaneous lesions, systemic manifestations, treatment options, and outcomes were evaluated. For each case of BSLE, four controls were randomly selected from patients with single SLE. Major clinical and laboratory characteristics were compared between the two groups.
Among 4221 patients with SLE, 12 developed BSLE. Vesiculobullous lesions were the first sign in five of the BSLE patients (5/12, 41.7%) and appeared after SLE diagnosis in the remaining seven patients (7/12, 58.3%), with a median duration from SLE onset of 36 months (4-115 months). The most common BSLE-affected sites were the head and neck (10/12, 83.3%), extremities (9/12, 75.0%), trunk (7/12, 58.3%), and mucosae (6/12, 50.0%). All patients with BSLE had extra-cutaneous involvement. The SLE disease activity index score exceeded 5 in 10/12 (83.3%) patients, which indicated high disease activity. Patients in the BSLE group had significantly higher incidences of proteinuria (83.3% vs. 47.9%, P = 0.027), hematuria (75% vs. 31.3%, P = 0.006), hemolytic anemia (33.3% vs. 0%, P = 0.000), and leukopenia (66.7% vs. 25.0%, P = 0.006) than those in the control group. The use of systemic corticosteroids, immunosuppressants, dapsone, and skin care was effective in controlling disease.
Vesiculobullous lesions may be the first manifestation and indicate a high disease activity in patients with BSLE. Early diagnosis using clinical, histopathological, and immunological evaluations can lead to appropriate treatment of this progressive disease and improve prognosis.

Dipeptidyl peptidase-4 inhibitor (DPP4i)-associated bullous pemphigoid (BP) has been emerging but whether it has genotype or phenotype differences from idiopathic BP (IBP) remains to be determined. We aimed to compare clinical characteristics, genetic susceptibility, laboratory features, disease activity, and outcomes between DPP4i-associated BP (DBP) and IBP occurring among patients with diabetes mellitus type 2 (T2DM). Medical records of patients diagnosed with BP and T2DM from January 2009 to December 2019 were retrospectively reviewed, and patients were categorized into DBP or IBP groups. Of 100 patients, 23 had DBP and 77 had IBP. There was no difference in the Bullous Pemphigoid Disease Activity Index score between the two groups; however, the score for urticaria and erythema was less in DBP (p < 0.001), indicating a non-inflammatory phenotype. The HLA-DQB1*03:01 allele was more commonly present in the DBP than IBP cases (odds ratio = 5.33 [95% confidence interval, 1.11-28.59], p = 0.016). The absolute eosinophil count was significantly lower in the DBP group (p = 0.002). Likewise, eosinophilic spongiosis was found less frequently in DBP cases (p = 0.005). Patients in the DBP group had a significantly higher percentage of complete remission on therapy compared to the IBP counterpart (p = 0.026) after DPP4i discontinuation. Moreover, the mean maximum dosage of prednisolone administrated per patient was significantly lower in drug-related cases (p = 0.012). In conclusion, our cohort in Thai patients with T2DM confirms the differences between phenotype and genotype characteristics of DBP and IBP. We emphasize the importance of drug discontinuation in all DPP4i-related cases because doing so may lead to a better disease outcome.

OBJECTIVE: Pemphigus is a chronic autoimmune and vesiculobollous disease that can affect skin and different mucous membrane surfaces. Primary manifestations occur in oral cavity in almost 60% of cases. The purpose of the present study was to evaluate the epidemiology of pemphigus in Tehran, Iran in a 20-year period.
METHODS: A retrospective study was conducted on the records of 1560 patients diagnosed with different types of pemphigus in Razi Hospital of Dermatology in Tehran from March 1985 to March 2005. A questionnaire was prepared to collect information regarding age, sex, bedridden duration, pemphigus subtype, sites of involvement, recurrence and mortality rate. Data was analyzed using chi-square test with significant level of P < 0.05.
RESULTS: There was a female predominance with a male to female ratio of 1:1.53. In nearly half of the patients, only the oral mucous membranes were affected. One hundred and fifty had only skin lesions and 261 cases had both skin and oral mucosal lesions. Involvement of esophageal and vaginal mucous membranes without skin lesions was observed in 150 patients and 298 cases had esophageal and vaginal mucosal involvement as well as skin lesions. Pemphigus vulgaris was the most common type, with the mean age of 44.6 years. Oral mucous membrane was the most frequent location where pemphigus vulgaris was observed. 1265 patients recovered which 52.2% of them had only oral lesions. Average of bedridden duration was 2.9 months. The highest recurrence rate was seen in patients with skin lesions exclusively. There was a significant difference between recurrences of lesions and location of involvement (P < 0.05). Thirty six patients had died from of the disease.
CONCLUSIONS: The mean age of the disease onset in the present study was found to be a decade earlier than the other parts of the world. Recurrence and mortality rates were lower in patients with only oral lesions and their prognosis was better.