Graphical abstract

The efficacy of low-dose UVA phototherapy in the treatment of localized scleroderma is mainly obtained by the increased production of MMP-1 and IFNgamma, and to a lesser extent by decreasing TGF-beta and collagen production. Concerning the use of 10 or 20 J/cm(2)/session those effects are dose dependent, but the clinical response does not significantly differ.

Acrodermatitis enteropathica (AE) is a rare autosomal recessive pediatric disease characterized by dermatitis, diarrhea, alopecia, and growth failure. The disease results from insufficient uptake of zinc by the intestine and can be fatal unless the diet is supplemented with zinc. To map the gene responsible for AE, a genomewide screen was performed on 17 individuals, including 4 affected individuals, in a consanguineous Jordanian family. Three markers-D8S373, D10S212, and D6S1021-had a pattern consistent with tight linkage to a recessive disease: one allele in the affected sibs and multiple alleles in unaffected sibs and parents. Two-point parametric linkage analysis using FASTLINK identified one region, D8S373, with a maximum LOD score >1.5 (1.94 at D8S373: recombination fraction.001). Twelve additional markers flanking D8S373 were used to genotype the extended family, to fine-map the AE gene. All five affected individuals-including one who was not genotyped in the genomewide screen-were found to be homozygous for a common haplotype, spanning approximately 3.5 cM, defined by markers D8S1713 and D8S2334 on chromosomal region 8q24.3. To support these mapping data, seven consanguineous Egyptian families with eight patients with AE were genotyped using these markers, and six patients from five families were found to be homozygous in this region. Multipoint analysis with all consanguineous families, by Mapmaker/Homoz, resulted in a maximum LOD score of 3.89 between D8S1713 and D8S373. Sliding three-point analysis resulted in a maximum LOD score of 5.16 between markers D8S1727 and D8S1744.

After 20 sessions, it appears that lower doses of UVA (5, 10 J/cm(2)) are as beneficial as the relatively higher dose (20 J/cm(2)) in the treatment of M and SS.

This cross-sectional multicenter study aimed to evaluate serum CXCL-10, as an activity marker for vitiligo, and compare it with other putative serum and tissue markers. Serum CXCL-10 was compared to interferon gamma (IFN-γ), interleukin 6 (IL-6), and IL-17 using ELISA in 55 non-segmental vitiligo patients (30 active and 25 stable) and 30 healthy controls. Marginal skin biopsy was taken for immunohistochemical evaluation of CD8+T cells and CXCL-10+ve cells. Serum levels of CXCL-10, IL-17, and IL-6 were elevated in all vitiligo patients compared to controls (p < .05). All investigated serum markers were higher in active versus stable vitiligo. Tissue expression of CXCL-10+ve cells and CD8+ve T cells was stronger in vitiligo patients compared to controls, and tissue CXCL-10+ve cell expression was stronger in active versus stable cases. Positive correlations were noted between the different serum and tissue markers. CXCL-10 was the most specific, whereas IL-6 was the most sensitive serum marker to distinguish active from stable disease.

NB UVB 311 nm has similar repigmentary effects as PUVA. The addition of psoralen does not increase its efficacy.

Psoriasis, vitiligo, and mycosis fungoides (MF) are among the most frequently treated dermatological diseases by photo(chemo)therapy. The objectives are to determine which photo (chemo) therapeutic modality could achieve the best response in the treatment of psoriasis, vitiligo, and MF. The design used in this study is retrospective analytical study. The study included 745 patients' records; 293 with psoriasis, 309 with vitiligo, and 143 with early MF, treated in the Phototherapy Unit, Dermatology Department, Kasr El-Aini Hospital, Cairo University by either psoralen and ultraviolet A (PUVA), narrow band ultraviolet B (NB-UVB), psoralen and narrow band UVB (P-NBUVB), broad band UVB (BB-UVB), or broad band UVA (BetaBeta-UVA). Data were retrieved from the computer database of the unit and statistically analyzed. In psoriasis, oral and topical PUVA and NB-UVB were found to be equally effective, whereas oral PUVA had significantly better results than both UVA and BB-UVB at the end of therapy. In generalized vitiligo, PUVA and P-NBUVB had significantly better results than NB-UVB alone. In early MF, there was no statistically significant difference between the response to oral PUVA and NB-UVB. PUVA and NB-UVB are good choices in patients with psoriasis and early stage MF, whereas PUVA appears the best choice in the treatment of vitiligo.

This study confirmed the rarity of epidermolysis bullosa in this province (population 3,000,000), and demonstrated the usefulness of oral phenytoin therapy, meticulous nursing, and good nutrition in the management of epidermolysis bullosa dystrophica.