Autosomal recessive anhidrotic ectodermal dysplasia: A rare entity

We describe a case of anhidrotic ectodermal dysplasia (AED) with an autosomal recessive mode of inheritance, a very rare entity, in a 2-year-old female child of two asymptomatic, consanguineous parents. Their previous child also had a similar condition. Autosomal recessive AED (AR-AED) can have its full expression both in males and females and it is clinically indistinguishable from the x-linked recessive AED (XL-AED), which is the most common type of ectodermal dysplasia. Unlike the partially symptomatic carriers of XL-AED, the heterozygotes of AR-AED are phenotypically asymptomatic

Comparative evaluation of NBUVB phototherapy and PUVA photochemotherapy in chronic plaque psoriasis

Background: Psoralen UV-A (PUVA) is an established therapy for psoriasis, but there is a well-documenated risk of melanoma and nonmelanoma skin cancer. Narrow-band Ultraviolet-B (NBUVB) therapy has a lower carcinogenic risk, has equal therapeutic potential and is considerably safe in the long term than PUVA. Aim: The aim of present study was to compare the efficacy and side-effects of PUVA and NBUVB in chronic plaque psoriasis. Methods: Sixty patients of chronic plaque psoriasis were taken up for the study and were randomly divided into two groups of 30 each. They were well matched in terms of age, sex, psoriasis extent and pretreatment psoriasis area severity index (PASI) scoring. One group was treated with twice-weekly narrow-band UV-B (TL-01) phototherapy and the other group received twice-weekly oral 8-Methoxsalen PUVA for a period of 3 months. Results: Both the groups achieved >75% reduction in the PASI score or complete clearance at the end of 3 months, but PUVA group patients required significantly fewer number of treatment sessions and fewer number of days to clear the psoriasis as compared to the NBUVB group, while the mean cumulative clearance dose and adverse effects were significantly lower in the NBUVB group. Conclusion: We concluded that PUVA group patients achieved a faster clearance, but the adverse effects were significantly lower in the NBUVB group