Quality of life, anxiety and depressive symptoms in patients with psoriasis: A case-control study.

OBJECTIVE: To compare quality of life (QoL), anxiety and depressive symptoms, alcohol consumption and other correlates between patients with psoriasis and controls; and to identify features of psoriasis associated with lower levels of QoL. METHOD: Case-control study including 70 subjects with moderate-severe psoriasis and 140 controls without psoriasis. All participants answered the Short Form Health Survey (SF-36), with physical and mental component scores of quality of life, and the Hospital Anxiety and Depression Scale (HADS). Among subjects with psoriasis, the Psoriasis Area and Severity Index (PASI) and the Dermatology Life Quality Index (DLQI) were used, respectively, to measure the severity of psoriasis and the impact of psoriasis on the specific quality of life. RESULTS: Compared to controls, patients with psoriasis showed higher HADS depression score and alcohol consumption, and lower QoL. Among subjects with psoriasis, multivariate analysis showed: 1) poorer physical QoL was associated with older age, articular lesions and anxious symptoms, whereas poorer mental QoL was associated with younger age, female sex, genital lesions and depressive symptoms; 2) the higher the severity of psoriasis, the lower the level of QoL and the higher the levels of anxious or depressive symptoms; and 3) female sex and articular or genital location of lesions are linked with higher HADS scores. CONCLUSION: Higher scores in anxiety and depression and lower QoL is common in psoriasis, especially among women and those with genital or articular lesions. Dermatologists should give special attention to this subgroup of persons with psoriasis in order to prevent future psychopathology.

Successful treatment of congenital erythropoietic porphyria using matched unrelated hematopoietic stem cell transplantation.

Congenital erythropoietic porphyria (CEP), or Günther's disease, is an inborn error of metabolism produced by a deficiency of uroporphyrinogen III synthase (UROS), the fourth enzyme of the heme biosynthesis pathway. This enzymatic defect induces the accumulation of isomer I porphyrins in erythrocytes, skin, and tissues, producing various clinical manifestations. Severe cases are characterized by extreme photosensitivity, causing scarring and mutilations, and by hemolytic anemia, reducing life expectancy. CEP is caused by mutations in the UROS gene, and one of the most severe forms of the disease is associated with a cysteine to arginine substitution at residue 73 of the protein (C73R). CEP has been successfully treated only by the transplantation of hematopoietic precursors. We report the case of a male infant with severe postdelivery symptoms diagnosed with CEP and found to be homozygous for the C73R mutation. He underwent successful allogeneic bone marrow transplantation from a matched unrelated donor at 7 months of age. The hemolytic anemia was corrected and the porphyrin overproduction was significantly reduced. The patient remained asymptomatic after 1 year. This new case confirms that patients with severe CEP can benefit from early postnatal hematopoietic stem cell transplantation.