Maryam Sadat Sadati; Ladan Dastgheib; Seyed Sajjad Tabei; Negin Fazelzadeh Haghighi; Maryam Hadibarhaghtalab
Abstract
Sneddon-Wilkinson disease, also known as subcorneal pustulardermatosis (SPD), is a relapsing pustular dermatosis of unknownetiology. The vesiculopustules typically present on the trunkand intertriginous areas. The mainstay of SPD treatment isdapsone; second-line therapies include corticosteroids and ...
Read More
Sneddon-Wilkinson disease, also known as subcorneal pustulardermatosis (SPD), is a relapsing pustular dermatosis of unknownetiology. The vesiculopustules typically present on the trunkand intertriginous areas. The mainstay of SPD treatment isdapsone; second-line therapies include corticosteroids and otherimmunosuppressive drugs. Here, we report a case of recalcitrantsevere SPD that responded dramatically to adalimumab. Thepatient was a 30-year-old man who presented with generalizederythroderma and vesiculopustules on the trunk and extremities.In some pustules, the pus settled with gravity into the lowerhalf of the blister. He responded dramatically to a combinationof adalimumab (80 mg on day 1, 40 mg on day 7, and 40 mgbiweekly afterward) and prednisolone 50 mg daily. During followup,prednisolone was tapered to 5 mg daily over 2 months, andadalimumab was maintained biweekly; he is still in remissionafter 8 months. Thus, adalimumab can be used as an effectiveand easy-to-use treatment in refractory cases of SPD.
Maryam Zahed; Ladan Dastgheib; Maryam Sadat Sadati; Asma Soukhakian
Abstract
Background: Pemphigus vulgaris (PV) is a life-threatening autoimmune disease that affects the skin and mucosa. In most regions, little data is available on its epidemiology. This study aimed to present an overview of the worldwide epidemiology of pemphigus and evaluate the epidemiological features of ...
Read More
Background: Pemphigus vulgaris (PV) is a life-threatening autoimmune disease that affects the skin and mucosa. In most regions, little data is available on its epidemiology. This study aimed to present an overview of the worldwide epidemiology of pemphigus and evaluate the epidemiological features of PV in the southwest of Iran.Methods: In this study, data including sex, age, time of disease onset, phenotype of the disease, geographic location of birth, geographic location of residence, level of education, and occupation of PV patients from 2004 to 2019 were collected from the records existing in the Oral Medicine Department of Shiraz Dental School and Shahid Faghihi Hospital, Shiraz, Iran.Results: In total, 249 PV patients with a mean age of 42.72 ± 13.3 years were included. The female to male ratio was 1.74:1. The annual incidence of PV in Fars province was 2.7 per million people. In this region, the most frequent phenotype was the mucocutaneous phenotype in 204 patients (81.9%), followed by the mucosal dominant phenotype in 40 (16.1%) and the cutaneous phenotype in 5 (2%). Regarding the level of education, 43.4% had less than a high school diploma, 24.9% had a high school diploma, 17.3% had an associate diploma or higher, and 14.5 % were illiterate. Moreover, 55.8% were housewives, 21.7% were self-employed, 19.3 % were employed, and 3.2% were jobless.Conclusion: The findings indicate that PV is a rare disease in the southwest of Iran compared with other regions. According to previous studies in this region, the incidence has decreased since 2005.
