Sociodemographic, clinical, laboratory, treatment and prognostic characteristics of 156 generalized pustular psoriasis patients in Turkey: a multicentre case series.

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.

Clinical and demographic features of hidradenitis suppurativa: a multicentre study of 1221 patients with an analysis of risk factors associated with disease severity.

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 ± 10.4 years, and almost 70% (n = 849) of patients were current or former smokers. Mean disease duration was 8.9 ± 8.4 years with a delay in diagnosis of 5.8 ± 3.91 years. Just over a fifth (21%; n = 256) of patients had a family history of HS. The axillary, genital and neck regions were more frequently affected in men than in women, and the inframammary region was more frequently affected in women than in men (P < 0.05 for all). Acne (40.8%), pilonidal sinus (23.6%) and diabetes mellitus (12.6%) were the most prevalent associated diseases. Of the various therapies used, antibiotics (76.4%) were most common followed by retinoids (41.7%), surgical interventions (32.0%) and biologic agents (15.4%). Logistic regression analysis revealed that the most important determinants of disease severity were male sex (OR = 2.21) and involvement of the genitals (OR = 3.39) and inguinal region (OR = 2.25). More severe disease was associated with comorbidity, longer disease duration, longer diagnosis delay and a higher number of smoking pack-years. CONCLUSIONS: Our nationwide cohort study found demographic and clinical variation in HS, which may help broaden the understanding of HS and factors associated with disease severity.

Vitamin D deficiency in alopecia areata.

BACKGROUND: Alopecia areata (AA) is a T cell-mediated autoimmune disease that causes inflammation around anagen-stage hair follicles. Insufficient levels of vitamin D have been implicated in a variety of autoimmune diseases. Previous reports have described the effects of vitamin D on hair follicles. OBJECTIVES: To evaluate the status of vitamin D in patients with AA, and the relationship between vitamin D levels and disease severity. METHODS: A cross-sectional study of 86 patients with AA, 44 patients with vitiligo and 58 healthy controls was conducted. The serum vitamin D levels of the study group were determined by liquid chromatography/tandem mass spectrometry. RESULTS: Serum 25-hydroxyvitamin D [25(OH)D] levels in patients with AA were significantly lower than those of the patients with vitiligo and the healthy controls (P = 0·001 and P < 0·001, respectively). The prevalence of 25(OH)D deficiency was significantly higher in patients with AA (91%) compared with patients with vitiligo (71%) and healthy controls (33%) (P = 0·003 and P < 0·001, respectively). Furthermore, a significant inverse correlation was found between disease severity and serum 25(OH)D level in patients with AA (r = -0·409; P < 0·001). CONCLUSIONS: Deficient serum 25(OH)D levels are present in patients with AA and inversely correlate with disease severity. Accordingly, screening patients with AA for vitamin D deficiencies seems to be of value for the possibility of supplementing these patients with vitamin D.

Unusual localization of multiple myxoid (mucous) cysts of toes.

Myxoid cysts of fingers and toes are observed frequently on the lateral or dorsal aspects of the distal digits. They are usually solitary nodules. Both subungual localization and multiplicity are quite rare. We present a 74-year-old woman with digital subungual mucous cysts located on all toes.