The oral manifestations of NF are well-documented but may not be at the forefront of the clinician's mind in the differential diagnosis of intra-oral swellings.

Background Hidradenitis suppurativa ( HS ) is a chronic, relapsing, inflammatory skin disease characterized by painful inflamed nodules, recurrent abscesses and fistulas located in apocrine gland–bearing body sites. The negative impact of HS on patient's quality of life (QoL) has been reported to be greater than other dermatologic conditions as psoriasis and atopic eczema, and its improvement is an important goal in disease management. Nowadays, there are no specific validated QoL instruments available for HS and generic dermatologic questionnaires are used. Objective The objective of this study was to demonstrate the validity, reliability and responsiveness of HIDRA disk, a new innovative tool designed for rapid assessment of HS burden and, at the same time, an intuitive graphic visualization of the measurement outcome. Methods A multicentre, longitudinal, observational study was conducted to validate the HIDRA disk compared with other validated questionnaires [Skindex‐16, Dermatology Life Quality Index ( DLQI ), Work Productivity and Activity Impairment–General Health (WPAI:GH)] and to evaluate its correlation with disease severity in Italian patients with any degree of HS severity, as measured by Hurley stage and HS Physician Global Assessment ( HS ‐ PGA ). Results A total of 140 patients (59% women; mean age 34.9 ± 11.0 years) were enrolled in 27 dermatologic centres. HIDRA disk showed a strong correlation with Skindex‐16 and DLQI , and a good one with WPAI:GH (correlation coefficient: 0.7568, 0.6651 and 0.5947, respectively) and a statistically significant correlation with both Hurley stage and HS ‐ PGA . Very good internal consistency (Cronbach coefficient >0.80; intraclass correlation coefficient >0.6), with correlation between the 10 items, good test–retest reliability (Spearman correlation coefficient, 0.8331; P < 0.0001) and responsiveness to changes were demonstrated. Conclusion Our study shows that HIDRA disk, a short and innovative visual HS QoL instrument, has been psychometrically validated in Italian language and it may help improve the management of HS once implemented in routine clinical practice.

The global number of cases of cutaneous leishmaniasis in Sicily has increased in recent years, and such increases can be explained, in part, by the fact that, in this region, sandflies are present during a large part of the year. This is a result of the climatic variation in recent years (increasing temperature and humidity). There has also been an increase in the number of new and rare variants of cutaneous leishmaniasis. A knowledge of the unusual clinical variants of cutaneous leishmaniasis, as well as classical forms, allows early detection.

Hidradenitis suppurativa (HS) is a disorder of the apocrine gland causing a chronic, recurrent and painful inflammation. It is a disabilitating condition and, though many therapeutic options are available, the response is often ineffective in most cases and patients can present many recurrences with physical and psychological sequelae. Recent data had shown increased interleukin (IL)-17 serum levels in patients with HS. Psoriasis is a chronic immune-mediated inflammatory disorder and new evidences have shown the role of Th17 cells in its pathogenesis and the therapeutic efficacy of anti-IL-17 antibodies. We present a case of a patient suffering from psoriasis and HS successfully treated with anti-IL-17 antibodies for both conditions. This is the first case report of HS treated with secukinumab.

Fabry disease is a multisystem disorder associated with wide variability in clinical expression. Fabry disease is an X-linked lysosomal storage disorder caused by a deficiency of alpha-galactosidase A. The enzyme defect leads to the systemic accumulation of glycosphingolipids with alpha-galactosyl moieties consisting predominantly of globotriaosylceramide, galabiosylceramide and two additional glycosphingolipids. Four hemizygotes patients with a family history of Fabry disease and deficiency of the enzyme alpha-galactosidase A were selected. Each patient received purified alpha-galactosidase by intravenous infusion (0.2 mg/kg). The infusion was administered every 2 weeks, for 40 min, for a total of 12 months. Outcome measures include neurological manifestations (acroparaesthesia, hypohidrosis, vasomotion), kidney function, cardiac manifestations, angiokeratomas, and corneal dystrophy. alpha-Galactosidase A prepared from human fibroblast is safe and well tolerated. After 12 months of therapy the mean creatinine clearance increased, there was significant improvement in the acroparaesthesias and in the hypohidrosis. Physical stigmata, such as angiokeratomas in the skin, and characteristic benign corneal abnormalities remained stable. Enzyme replacement therapy would therefore represent a significant advance in treatment of patients with Fabry disease. Enzyme replacement therapy is safe and likely to improve the prognosis of Fabry disease.