A survey of bullous diseases in a Turkish university hospital: clinicoepidemiological characteristics and follow-up

Background/aim: Autoimmune bullous diseases, if left untreated, are life-threatening conditions affecting primarily skin and mucous membranes. These blistering disorders are characterized by epidermal or subepidermal detachment. Autoimmunity plays a key role in pathogenesis; therefore, immunosuppressive agents are the treatment of choice. The aim of this study is to document relative frequencies of different autoimmune bullous diseases, patient characteristics, treatment options, and side effects in patients presenting to our bullous skin disease center at Istanbul University, Cerrahpasa, Cerrahpasa Medical Faculty. Materials and methods: Medical files were examined retrospectively for all patients with autoimmune bullous diseases who were followed up between 2003 and 2019 at the Bullous Skin Disease Center at Istanbul University, Cerrahpasa. Results: A total of 346 patient files were examined. Pemphigus vulgaris was the most frequent autoimmune bullous disease, followed by bullous pemphigoid and pemphigus foliaceus, according to our study. There is a general female predominancy for all autoimmune bullous diseases. The most commonly preferred treatment options were high-dose daily corticosteroids. Conclusion: This retrospective study summarizes the patient characteristics, comorbidities, treatment choices, and side effects during 16 years of clinical practice.

A retrospective analysis of pemphigus vulgaris patients: Demographics, diagnosis, co-morbid diseases and treatment modalities used.

OBJECTIVE: Pemphigus vulgaris is an autoimmune blistering disease affecting the mucosal surfaces as well as the skin. Twenty-eight retrospective studies about the epidemiologic data of pemphigus vulgaris patients have been performed previously in the literature. METHODS: In this retrospective study, we evaluated 320 pemphigus vulgaris patients who applied to the bullous diseases clinic of Istanbul University-Cerrahpasa, Cerrahpasa Faculty of Medicine, Department of Dermatology, between the years 1999-2019. Epidemiologic data, diagnostic modalities, comorbidities and treatment modalities were noted. RESULTS: The female to male ratio was 1.39. The mean age of diagnosis was 50.4±13.7 years, 50.8±12.5 years for males and 50.0±14.5 years for females. The average disease duration was 99.0±74.4 months; it was 91.6±67.2 months for males and 104.5±79.0 months for females. At the time of diagnosis, 88.7% of our patients had mucosal lesions and 68.4% of our patients had cutaneous lesions. The most common side effects were hypertension, diabetes mellitus, osteoporosis and hyperlipidemia. Adjuvant therapy was initiated 332 times. Azathioprine was used in 260 patients,Mycophenolate sodium was used in 30 patients, Mycophenolate mofetil was used in 42 patients, IVIG was used in 52 patients, Rituximab was used in 51 patients. CONCLUSION: Pemphigus vulgaris is a disease that is more commonly seen in female patients. It has a peak incidence in the fifth decade and there is not a statistically significant difference between the age of diagnosis between the genders. It is a chronic disease with a long follow-up period; again, there no statistically significant difference between the two genders. The most commonly encountered comorbidities are hypertension, hyperlipidemia, diabetes mellitus and osteoporosis, which are due to the use of corticosteroids.

Scleredema adultorum associated with hyperkeratosis.

Scleredema adultorum is a rare skin disorder reported to occur usually following streptococcal infections. It has characteristic clinical and histopathologic findings; however, associated hyperkeratosis is not known. We report a 6-year-old girl with scleredema adultorum and hyperkeratosis. The patient's disease had a benign course. To our knowledge, this patient is the first reported with scleredema adultorum associated with hyperkeratosis.