Safe, mild ultraviolet-B exposure: An essential human requirement for vitamin D and other vital bodily parameter adequacy: A review.

The enigma of skin sunburning, skin ageing and skin cancer and essential vitamin D production both resulting from solar ultraviolet-B (280-315 nm) (UVB) exposure has long puzzled photobiologists. Advice to patients by non-photobiological clinicians is now often to sunbathe to acquire vitamin D adequacy. However, modern work shows only mild UVB exposure is needed to maintain satisfactory levels, which have been demonstrated as very similar in summer and winter from about 25° to 70° north. Even very careful high protection factor 15 sunscreen use does not prevent adequate production, although it is slightly reduced, such that obsessive use of very protective screens of 50 + might. Dark skin pigmentation too usually at most minimally impairs production. However, confinement indoors and widespread clothing cover can, but oral supplementation overcomes any such deficiency. Thus, vitamin D adequacy needs just mild regular UVB skin exposure well under sunburning levels, unlikely to cause significant skin damage. This suggests mild UVB exposure may also be needed for other bodily requirements, which is indeed so. Thus, it also prevents the development of contact dermatitis and polymorphic light eruption through suppressing adaptive immunity. It also prevents the occurrence of multiple skin infections resulting from this suppression through stimulating innate immunity and cutaneous bacterial defensin production. Finally, blood pressure is reduced through low-dose UVB-induced production of the vasodilator nitric oxide (though UVA, 315-400 nm, is more efficient). Thus, mild UVB exposure is important for several aspects of internal health, whereas high-dose exposure is extremely detrimental to cutaneous health.

The quality of life of 790 patients with photodermatoses.

BACKGROUND: Polymorphic light eruption and erythropoietic protoporphyria (EPP) have been demonstrated to have a moderate and large impact on the quality of life (QoL) of patients, respectively. However, there is little information available about the impact of other photodermatoses on QoL. OBJECTIVES: To assess and compare the impact of all forms of photodermatoses on patients' QoL using the standard 1-week Dermatology Life Quality Index (DLQI) questionnaire and a modified questionnaire to assess the impact over the previous year. METHODS: All patients with photodermatoses seen between 2001 and 2005 at five U.K. photobiology centres were contacted by post on the same day during a forecasted sunny week across the U.K. and asked to complete DLQI questionnaires. RESULTS: A total of 1877 patients were contacted. Seven hundred and ninety-seven (42%) patients replied, with a range from 30% to 48% for the five individual centres. Nearly two-thirds of patients with actinic prurigo (AP) and more than one-third of patients with photoaggravated dermatoses (PAD), chronic actinic dermatitis, EPP and solar urticaria had a DLQI of > 10, confirming a very large effect of the disorders on QoL. Of the cutaneous porphyrias, both variegate porphyria (median DLQI 3) and porphyria cutanea tarda (median DLQI 1.5) had a much lower impact on QoL than EPP. CONCLUSION: This is the first large-scale study to attempt to measure the impact of a range of photodermatoses on QoL. Photodermatoses have a major impact on QoL. This impact is highest in AP and PAD.

Persistent severe amiodarone-induced photosensitivity.

Amiodarone, a benzofuran derivative, has been used therapeutically as an antiarrhythmic and coronary vasodilator in Europe since 1964. One of its commoner side effects is cutaneous photosensitivity; more rarely, after ingestion of the drug for around 12 months, a slate-grey or violaceous discoloration of sun-exposed sites may gradually develop. Both of these side effects usually resolve within 2 years of discontinuation of the drug. We now present a woman who developed both photosensitivity and a slate-grey discoloration whilst taking amiodarone; on discontinuation of the drug, the dyspigmentation gradually resolved, but the photosensitivity has persisted and the patient remains symptomatic more than 17 years later.

Audit of the use of psoralen photochemotherapy (PUVA) and narrowband UVB phototherapy in the treatment of psoriasis.

BACKGROUND: Psoralen photochemotherapy (PUVA), the combined use of psoralen and long wave ultraviolet (UVA) irradiation, was introduced around 1974 and its beneficial effects were rapidly confirmed worldwide. In an attempt to minimize its recognized long-term photocarcinogenic risk after some 150-200 exposures while also maintaining efficacy, however, the narrowband (311-312 nm) ultraviolet B (UVB) lamp (TL-01) was introduced in 1984, and has moved towards replacing PUVA except for severe or resistant disease. AIMS: To discover whether our use of these therapies complied with established British Photodermatology Group guidelines for PUVA and guidelines formulated within our unit for narrowband UVB. METHODS: The study was retrospective over 6 months from November 2001 to April 2002, all relevant information being obtained from the patients' hospital notes. RESULTS: Thirty-one patients received PUVA (18 oral, 11 bath and two uncertain because of missing notes) and 20 narrowband UVB during this period. CONCLUSIONS: Our PUVA and narrowband UVB phototherapy guidelines were shown to have been followed relatively closely with the following exceptions: one PUVA patient received a high cumulative exposure by mutual agreement because there was no other suitable therapy; a failure to measure minimal phototoxic doses (MPDs) in some PUVA patients; and slightly prolonged referral delays, but generally by patient choice.

Exacerbation of presumed chronic actinic dermatitis by cockpit visible light in an airline pilot with atopic eczema.

Chronic actinic dermatitis (CAD) is a persistent ultraviolet radiation- or visible light-induced eczema of predominantly the exposed areas of usually elderly people. We now present the case of a young pilot with atopic eczema who developed CAD, regularly exacerbated by exposure to visible light through his aircraft cockpit window.

Erythema multiforme following polymorphic light eruption: a report of two cases.

We report two patients in whom episodes of polymorphic light eruption were followed by recurrent erythema multiforme on exposed and nonexposed sites. Treating the polymorphic light eruption with prophylactic PUVA and/or oral prednisolone or cyclosporin prevented the development of erythema multiforme, suggesting that the two events are related. It is possible that erythema multiforme develops as a response to the same causative antigen as polymorphic light eruption.

Glaucoma induced by periorbital topical steroid use--a rare complication.

Eye complications arising during systemic and ophthalmic steroid use are well known. In contrast, there is little highlighting the similar risks associated with topical steroid use around the eyelids. We now describe a 29-year-old lady who used topical steroids in prolonged fashion throughout most of her life for severe eczema, with the recent application of large quantities of potent steroid continuously for one month, including to the periorbital region. Soon after, she presented to her ophthalmologist with severe bilateral glaucoma and irreversible visual loss attributed to the steroid use. Here we emphasise the possible risks of periorbital topical steroid use, as well as the importance of patient education and ophthalmological follow-up.

A simple method to assess severity of polymorphic light eruption.

BACKGROUND: The severity of polymorphic light eruption (PLE) is highly variable. The results of studies of the prevalence, pathogenesis, provocation and treatment of PLE may be highly dependent on the severity of disease in the patients studied. OBJECTIVES: To produce a simple, valid and reproducible method to assess the severity of PLE. PATIENTS AND METHODS: Eighty patients were asked about the PLE they had experienced during the preceding 12 months, using a standardized interview comprising 16 questions. The answer to each question received a score. A PLE Severity Index (PLESI) was formulated, consisting of 10 questions, with a possible total score of 2-100. The internal consistency of the PLESI (the extent to which the responses to different questions correlated with each other) was assessed by reliability analysis, using Cronbach's method. Twenty patients were re-interviewed 7-27 days later to assess the repeatability of the PLESI. The ease of provocation of PLE by exposure at 24-h intervals to solar-simulated radiation was assessed on a five-point scale in nine of the 80 subjects (the EOPSSR score). RESULTS: The value of Cronbach's alpha for the PLESI was 0.77. The distribution of the PLESI was consistent with a normal distribution, with a mean value of 52.7 and standard deviation of 19.4. It had a coefficient of repeatability of 20.1. The PLESI was positively correlated with EOPSSR (rs =0.69, P = 0.039) and the number of years since onset of PLE (rs = 0.25, P = 0.03). There was no association between the PLESI and the duration of persistence of the eruption after ceasing sun exposure (rs = 0.12, P = 0.30), the development of tolerance as summer progressed (rs = -0.14, P = 0.39), gender (P = 0.50) or skin type (P = 0.87). CONCLUSIONS: This study has (i) validated the concept that a single score can reflect disease severity in PLE by showing that the principal characteristics of the condition, including, for example, the extent of anatomical distribution and the ease of provocation of the eruption, correlate with each other; (ii) formulated the PLESI, which is a simple, valid and reproducible way of assessing disease severity; we suggest it could be used worldwide to determine the severity of PLE among patients enrolled in future PLE research; (iii) shown that the ease with which the eruption is provoked by solar-simulated radiation correlates with the severity of the condition; and (iv) shown that the duration of persistence of the eruption after sun exposure does not correlate with the severity of the condition.

Erythrodermic chronic actinic dermatitis responding only to topical tacrolimus.

Chronic actinic dermatitis (CAD) is a disorder characterized by an often severe persistent eczematous eruption induced by exposure to ultraviolet radiation. Treatment involves photoprotective measures and topical corticosteroid therapy and in more severe cases, systemic immunosuppression. Occasionally, however, the condition can prove very resistant to all therapy and be severely disabling. We report a patient with CAD who resisted standard topical and systemic treatments, but responded to topical tacrolimus ointment 0.1% (Protopic).

Topical pseudocatalase mousse and narrowband UVB phototherapy is not effective for vitiligo: an open, single-centre study.

We report an open single-centre trial to assess the efficacy of topical pseudocatalase mousse applied twice daily to the hands and face of vitiligo patients, in combination with twice-weekly suberythemogenic narrowband UVB phototherapy. The regime was generally safe and well tolerated, although several patients experienced mild transient skin rashes in association with application of the mousse and one patient suffered severe pruritus. The primary efficacy variable was the percentage change in area affected by vitiligo as assessed by digital interpretation of standardized photographs of the face and hands. There was no clear evidence of the efficacy of the regime and in fact a slight tendency overall to worsening of the patients' vitiligo.

Ferrochelatase gene polymorphism analysis for accurate genetic counselling in erythropoietic protoporphyria.

It has recently been shown that most cases of clinically overt erythropoietic protoporphyria (EPP) result from coinheritance of a mutated ferrochelatase gene and a commonly occurring low-expression normal variant allele. The identification of two polymorphic variant sequences associated with this low-expression allele now enables improved predictive counselling for couples where one partner has EPP. We describe a patient and his spouse in whom we have used such genetic analysis to provide an accurate estimate of the chance that their future offspring may suffer from EPP.

Psoralen photochemotherapy (PUVA) is only moderately effective in widespread vitiligo: a 10-year retrospective study.

A 10-year retrospective analysis of the use of psoralen photochemotherapy (PUVA) in the treatment of vitiligo was undertaken at the St John's Institute of Dermatology, London, UK. Of 97 patients included in this study, eight had complete or almost complete repigmentation, 59 moderate to extensive repigmentation, and 30 showed little or no response. However, 24 of those who had responded to PUVA with extensive repigmentation did not consider their response satisfactory because of persistence of vitiligo at cosmetically sensitive sites, and poorly matching, speckled repigmentation. Fifty-seven patients who initially improved with PUVA therapy subsequently relapsed, in most cases within a year of stopping treatment. Relapses in 22 patients were on the same cutaneous sites as previously affected, while vitiligo at new sites developed in 20 patients and both new and old sites were affected in a further 15 patients. Patients who retained their pigmentation after 2 years appeared to have a better chance of permanent remission. The only statistically significant prognostic indicator of relapse was patient age at the start of treatment, younger patients tending to retain their pigmentation longer than older patients. This study emphasizes the need for careful patient counselling before PUVA therapy as this treatment seldom achieves extensive repigmentation that is cosmetically acceptable, and treatment response is often followed by relapse.

Human Ro60 (SSA2) genomic organization and sequence alterations, examined in cutaneous lupus erythematosus.

BACKGROUND: The Ro 60 kDa protein (Ro60 or SSA2) is the major component of the Ro ribonucleoprotein (Ro RNP) complex, to which an immune response is a specific feature of several autoimmune diseases. The genomic organization and any sequence variation within the DNA encoding Ro60 are unknown. OBJECTIVES: To characterize the Ro60 gene structure and to assess whether any sequence alterations might be associated with serum anti-Ro antibody in subacute cutaneous lupus erythematosus (SCLE), thus potentially providing new insight into disease pathogenesis. METHODS: The cDNA sequence for Ro60 was obtained from the NCBI database and used for a BLAST search for a clone containing the entire genomic sequence. The intron-exon borders were confirmed by designing intronic primer pairs to flank each exon, which were then used to amplify genomic DNA for automated sequencing from 36 caucasian patients with SCLE (anti-Ro positive) and 49 with discoid LE (DLE, anti-Ro negative), in addition to 36 healthy caucasian controls. RESULTS: Heteroduplex analysis of polymerase chain reaction (PCR) products from patients and controls spanning all Ro60 exons (1-8) revealed a common bandshift in the PCR products spanning exon 7. Sequencing of the corresponding PCR products demonstrated an A > G substitution at nucleotide position 1318-7, within the consensus acceptor splice site of exon 7 (GenBank XM001901). The allele frequencies were major allele A (0.71) and minor allele G (0.29) in 72 control chromosomes, with no significant differences found between SCLE patients, DLE patients and controls. CONCLUSIONS: The genomic organization of the DNA encoding the Ro60 protein is described, including a common polymorphism within the consensus acceptor splice site of exon 7. Our delineation of a strategy for the genomic amplification of Ro60 forms a basis for further examination of the pathological functions of the Ro RNP in autoimmune disease.