Background
Generalized pustular psoriasis (GPP) is a rare and severe inflammatory disease characterized by widespread and superficial sterile pustules on an erythematous background.
Objectives
This multicentre study aimed to determine the clinical profile and course in a large cohort of patients with GPP.
Methods
One hundred and fifty‐six GPP patients (mean age, 44.2 ± 18.7 years) who met the diagnostic criteria of the European Consensus Report of GPP were included in the study. Sociodemographic characteristics, quality of life, triggering factors of the disease, clinical, laboratory, treatment and prognostic features were evaluated.
Results
61.5% of the patients were female. The rate of working at or below the minimum wage (≤$332.5/month) was 44.9%. Drugs (36.5%) were the most common trigger. While hypocalcaemia (35.7%) was the most important cause of GPP during pregnancy, systemic steroid withdrawal (20%) was the most frequently reported trigger for infantile/juvenile and mixed‐type GPP (15%) (P < 0.05). Acute GPP (53.8%) was the most common clinic. Nails were affected in 43.6% of patients, and subungual yellow spots (28.2%) were the most common change. In annular GPP, fever (P < 0.001) and relapse frequency (P = 0.006) were lower than other subtypes, and the number of hospitalizations (P = 0.002) was lower than acute GPP. GPP appeared at a later age in those with a history of psoriasis (P = 0.045). DLQI score (P = 0.049) and joint involvement (P = 0.016) were also higher in this group. Infantile/juvenile GPP was observed in 16.02% of all patients, and arthritis was lower in this group (24.4 vs. 16%). GPP of pregnancy had the worst prognosis due to abortion observed in three patients.
Conclusions
Recent advances in treatment have improved mortality associated with GPP, but abortion remains a significant complication. Although TNF‐α inhibitors have proven efficacy in GPP, they can also trigger the disease. Mixed‐type GPP is more similar to acute GPP than annular GPP with systemic manifestations and course.
Background. Hidradenitis suppurativa (HS) is a chronic, relapsing and debilitating inflammatory disease associated with profound morbidity. Aim. In this multicentre study, we investigated the demographic and clinical features of HS, and determined risk factors of disease severity. Methods. In total, 1221 patients diagnosed with HS from 29 centres were enrolled, and the medical records of each patient were reviewed. Results. The mean age of disease onset was 26.2 AE 10.4 years, and almost 70% (n = 849) of patients were current or former smokers. Mean disease duration was 8.9 AE 8.4 years with a delay in diagnosis of 5.8 AE 3.91 years. Just over a fifth (21%; n = 256) of patients had a family history of HS. The axillary, genital and neck
Mycosis fungoides (MF) and parapsoriasis (PP) are major dermatologic conditions for which phototherapy continues to be a successful and valuable treatment option. UVA-1 phototherapy is effective in the management of cutaneous T-cell mediated diseases. The aim of the study was to evaluate the efficacy and safety of low-dose UVA-1 phototherapy for the management of PP/early-stage MF. A total of 30 patients, diagnosed with MF (n:19) or PP (n:11) were enrolled to the study. All patients were managed with low-dose UVA-1 (20 or 30 J cm(-2)). Response was assessed clinically and immunohistochemically. UVA-1 treatment led to clinical and histological complete remission (CR) in 11 of 19 MF patients (57.9%), partial remission (PR) in three of 19 (15.8%), after a mean cumulative dose of 1665 (range, 860-3120) J cm(-2) and mean number of 73 exposure (range, 43-107) sessions. Five patients with PP (45.5%) showed CR, and PR was observed in six patients with PP (54.5%) after a mean cumulative dose of 1723 (range, 1060-3030) J cm(-2) and mean number of 74 exposure (range, 53-101) sessions. We conclude that low-dose UVA-1 therapy seems to be an effective, safe, and well-tolerated treatment option for patients with PP/early-stage MF.
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