Based on this systematic review, it appears that none of the severity scores used for psoriasis meets all of the validation criteria required for an ideal score. However, we can conclude that the PASI score is the most extensively studied psoriasis clinical severity score and the most thoroughly validated according to methodological validation criteria. Despite certain limitations, use of the PASI score can be recommended for scientific evaluation of the clinical severity of psoriasis.

There is an increased risk of obesity and metabolic syndrome in psoriasis. For hypertension, diabetes and dyslipidaemia no consistency was found across studies. Prospective epidemiological studies with thorough recording of cardiovascular risk factors are required in psoriasis patients.

On the basis of this systematic review, we can conclude that the DLQI is easy to use in clinical practice because of its brevity and simplicity. SF36 is widely used in clinical trials. Skindex 29 and Skindex 17, although rarely used, are interesting because of their cross-cultural validation.

Psoriasis arthritis may affect up to 24% of psoriasis patients. Dermatologist should be aware of main clinical sign of PsA to promote earlier recognition and treatment of PsA.

Our study emphasizes the importance of wide local excision with margins of at least 3 cm in order to prevent local recurrence. However, the recent development of inhibitors of signal transduction by the PDGFB pathway should soon modify the surgical strategy, which is often too mutilating.

Our preliminary results confirm the efficacy of this novel 308-nm MEL delivery system, which appears to be effective and safe for palmoplantar pustular psoriasis. To a lesser extent, plaque-type psoriasis, chronic atopic and nonatopic dermatitis of the hands and alopecia may also benefit from this treatment.

BackgroundHereditary Fibrosing Poikiloderma (HFP) with tendon contractures, myopathy and pulmonary fibrosis (POIKTMP [MIM 615704]) is a very recently described entity of syndromic inherited poikiloderma. Previously by using whole exome sequencing in five families, we identified the causative gene, FAM111B (NM_198947.3), the function of which is still unknown. Our objective in this study was to better define the specific features of POIKTMP through a larger series of patients.MethodsClinical and molecular data of two families and eight independent sporadic cases, including six new cases, were collected.ResultsKey features consist of: (i) early-onset poikiloderma, hypotrichosis and hypohidrosis; (ii) multiple contractures, in particular triceps surae muscle contractures; (iii) diffuse progressive muscular weakness; (iv) pulmonary fibrosis in adulthood and (v) other features including exocrine pancreatic insufficiency, liver impairment and growth retardation. Muscle magnetic resonance imaging was informative and showed muscle atrophy and fatty infiltration. Histological examination of skeletal muscle revealed extensive fibroadipose tissue infiltration. Microscopy of the skin showed a scleroderma-like aspect with fibrosis and alterations of the elastic network. FAM111B gene analysis identified five different missense variants (two recurrent mutations were found respectively in three and four independent families). All the mutations were predicted to localize in the trypsin-like cysteine/serine peptidase domain of the protein. We suggest gain-of-function or dominant-negative mutations resulting in FAM111B enzymatic activity changes.ConclusionsHFP with tendon contractures, myopathy and pulmonary fibrosis, is a multisystemic disorder due to autosomal dominant FAM111B mutations. Future functional studies will help in understanding the specific pathological process of this fibrosing disorder.

Ten recommendations to assess the severity of plaque-type psoriasis in adult patients in daily practice were developed. The recommendations are based on systematic appraisal of available evidence. They were developed and supported by a panel of dermatologists, which enhances their validity and practical relevance.