Classification and evaluation of photodermatoses

Photodermatoses can be classified into five general categories: 1) idiopathic photodermatoses, including polymorphic light eruption (PMLE), actinic prurigo, hyroa vacciniforme, chronic actinic dermatitis, and solar urticaria; 2) photodermatoses which are secondary to exogenous agents, including phototoxic and photoallergic reactions; 3) photodermatoses secondary to endogenous agents, mainly the porphyrias; 4) photoexacerbated dermatoses, including autoimmune disease, infectious conditions, and nutritional deficiencies; and 5) genodermatoses. The initial step in evaluating a photosensitive patient is based on a directed personal and family history. The morphology of the eruption, phototests, and in some patients, photopatch tests are essential in focusing the diagnosis. Skin biopsies and laboratory investigations, such as antinuclear antibody (ANA) panels and porphyrin profiles, may be required to further confirm the diagnosis.     

Mechanisms of phototherapy and photochemotherapy for photodermatoses

Most photodermatoses represent indications for preventive ultraviolet (UV) phototherapy and/or psoralen plus ultraviolet A (PUVA) photochemotherapy. The aim of treatment is to prevent the outbreak of disease by increasing the patient's tolerance to sunlight. The mechanisms by which ultraviolet B (UVB) and PUVA induce such tolerance are not completely understood. Pigmentation and skin thickening may be important factors in the protective effect, but they cannot sufficiently explain the degree of protection induced. Other mechanisms that may be of critical importance for the therapeutic efficacy encompass a variety of immunomodulatory effects on human skin known to be induced by UVA, UVB, and PUVA. Obviously the mechanisms of prophylactic phototherapy are strongly intertwined with the pathogenesis of the photodermatoses. The possible mechanisms of photoprevention are discussed for polymorphic light eruption (PMLE), actinic prurigo, chronic actinic dermatitis, and solar urticaria.     

Analysis of photodermatoses seen in a predominantly Asian population at a photodermatology clinic in Singapore

BACKGROUND: The idiopathic photodermatoses have been reported to be rarer in tropical Singapore than in countries of higher latitude, with photoaggravated dermatoses and systemic phototoxicity making up most of the photodermatoses seen here. This study aims to reassess the spectrum of photodermatoses seen at the National Skin Centre, Singapore, compared with almost a decade ago, and analyse the clinical and photobiological characteristics, as compared with other countries. MATERIALS AND METHODS: We reviewed the clinical data of 141 patients phototested from January 2000 to December 2001, and analysed the epidemiological, clinical and photobiological features. RESULTS: Photosensitive dermatoses were diagnosed in 88% (124/141) of patients phototested. In those diagnosed with photodermatoses, polymorphic light eruption (PMLE) (28%) was the most common diagnosis, followed by photoaggravated dermatoses (26%), chronic actinic dermatitis (CAD) (15%), systemic phototoxicity (15%), solar urticaria (SU) (7%), actinic prurigo (AP) (5%) and photoallergic contact dermatitis (4%). Ethnic Indians appeared to be more predisposed to PMLE; AP was diagnosed only in ethnic Chinese. The other photodermatoses occurred proportionally in all racial groups. AP differed from that found in Caucasians, being of adult onset and persistent. Abnormal phototest results were obtained in all patients with CAD, SU and AP, but only in 56% and 49% of systemic phototoxicity and PMLE, respectively. CONCLUSION: Idiopathic photodermatoses are more commonly diagnosed in Singapore than a decade ago, while the incidence of systemic phototoxicity has remained stable. The spectrum of photodermatoses in our Asian population now approximates that seen in Caucasian cohorts.     

Idiopathic photodermatoses: clinical, diagnostic and therapeutic aspects

Idiopathic photodermatoses are a group of photosensitivity dermatoses, which are not very frequent with the exception of polymorphous light eruption. Therefore, their recognition may not be easy for dermatologists who are not familiar with photosensitivity diseases. LEARNING OBJECTIVE: The information in this review is intended for giving clinical, diagnostic and therapeutic clues for the correct management of patients. Clinical features will be described together with diagnostic relevant procedures and usual therapeutic measures.     

Benign summer light eruption and polymorphic light eruption: genetic and functional studies suggest that a revised nomenclature is required

New research indicates that polymorphic light eruption (PLE) is an autoimmune disease against an ultraviolet radiation-induced cutaneous antigen. PLE may even confer some protection against skin cancer later in life. This new information demands a reassessment of the precise nature and nomenclature of PLE. Benign summer light eruption (BSLE) (lucite estivale bénigne) is the name used in continental Europe, and particularly France, to describe a clinically short-lived, itchy, papular eruption particularly affecting young women after several hours of sunbathing at the beginning of summer or on sunny vacations. Clinically more prolonged forms of solar eruption, starting early in spring and persisting for long periods, have been known in France as polymorphic light eruption (PLE) (lucite polymorphe) ('European PLE'). Investigative studies, however, now suggest that BSLE and some cases of 'European PLE' are part of the same spectrum. In the Anglo-Saxon literature, they are lumped together as PLE ('Anglo-Saxon PLE'). The other cases of 'European PLE', which do not fall within the compass of 'Anglo-Saxon PLE', are, in the Anglo-Saxon literature, classified as either actinic prurigo (AP) (a genetically determined, prolonged, excoriated form of Anglo-Saxon PLE), or chronic actinic dermatitis (CAD) (a sunlight-induced eczema precisely resembling allergic contact dermatitis, apparently to an ultraviolet radiation-induced antigen). It is therefore proposed that: i. the European term BSLE be dropped and that these patients be reclassified within the spectrum of (Anglo-Saxon) PLE, ii. the European use of the term PLE ('European PLE') be discontinued, iii. those previously diagnosed as having 'European PLE' be reclassified as (Anglo-Saxon) PLE, AP or CAD, as appropriate. The benefits of such a change in nomenclature would be twofold, firstly a uniformity of terminology and secondly, and more importantly, terminology would then correlate better with our recently improved understanding of the pathogenesis of these disorders.     

Update on photodermatoses

Interactions with ultraviolet radiation (UVR) and chromophores in the skin happen on a daily basis. Photodermatoses, which are abnormal responses to UV exposure, can be classified into subgroups based on pathogenesis. This review will discuss the clinical features, pathogenesis, photobiologic evaluation, prognosis and therapies of the most common photodermatoses.     

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.     

Photosensitivity: the 9-year experience at a Sydney contact dermatitis clinic

In this retrospective study, 81 patients with photosensitivity were referred to the Contact and Occupational Dermatitis Clinic at The Skin and Cancer Foundation Australia, in Sydney, between 1991 and 1999. Photoallergic contact dermatitis (PACD) was diagnosed in 39.5% of patients, with 87.5% of these reactions being to sunscreen chemicals. Polymorphic light eruption (PMLE) accounted for 19.7% of cases, drug photosensitivity 14.8%, and photoaggravated atopic dermatitis and chronic actinic dermatitis (CAD) each constituted 7.4%. Compared with overseas studies, there was a high incidence of PACD, possibly reflecting the referral bias and widespread use of sunscreens. The incidence of PMLE and CAD was less than studies from cooler climates overseas. No cause could be determined for three photosensitive patients.     

Actinic prurigo and polymorphic light eruption: common pathogenesis and the importance of HLA-DR4/DRB1*0407

Actinic prurigo (AP) and polymorphic light eruption (PLE) both belong to the group of idiopathic photodermatoses, but it remains controversial whether AP is a distinct photodermatosis or a variant of PLE. The aim of this study, by collecting data from 119 patients with features of these disorders, was to establish whether specific criteria could be used to distinguish AP from PLE prospectively. We found that presence of the eruption on both exposed and covered sites, its occurrence in winter, persistence of lesions beyond 4 weeks, mucosal and conjunctival involvement, excoriation and scarring of the skin were important features of AP which were not typical of PLE. On this basis, confident clinical diagnoses could be reached in 103 of 119 patients (87%), 57 with AP and 46 with PLE, supported by phototesting and negative lupus serology. HLA typing subsequently confirmed the strong association (90%) between AP and the DR4 allele, in particular with the rare subtype DRB1*0407 which was present in 60% of these patients. No HLA association was found in PLE. In the 16 remaining cases, however, clinical overlap meant that no definite diagnosis could be made; these patients were notionally described as having persistent PLE (PPLE). Demographic and HLA data in this group suggested that PPLE was perhaps most appropriately grouped with PLE. In addition to those patients who were difficult to classify, 35% of our typical AP patients also described clinical progression from PLE to AP, AP to PLE or coexistence of both AP and PLE. In conclusion, our study suggests that while AP and PLE are clinically distinct conditions in most cases, they may perhaps share a common pathophysiological basis. The AP phenotype may be determined by HLA and perhaps other factors in patients otherwise predisposed to PLE.     

Prevalence of photodermatosis in four regions at different altitudes in Yunnan province, China

Idiopathic photodermatoses are common diseases in dermatology clinics that are associated with ultraviolet (UV) irradiation. The group includes a few dermatoses such as polymorphous light eruption (PLE) and chronic actinic dermatitis (CAD). The prevalence of PLE and CAD in China has not been previously reported. To investigate the population-based prevalence of polymorphous light eruption (PLE) and chronic actinic dermatitis (CAD) in six minority groups living in four regions with significantly different altitudes in the Yunnan province, a questionnaire survey was administered to 4899 residents of random villages in Yuanjiang county (Dai and Hani minorities), Kunming city (Han people and Yi minority), Lijiang county (Naxi minority), and Shangri-La county (Zang minority). The altitudes of these counties are 380 m, 1870 m, 2410 m and 3280 m a.s.l., respectively. The results showed that, first, there were 2400 males (49.0%) and 2499 females (51.0%). The prevalence of PLE was 0.65% (32/4899), and the prevalence of CAD was 0.18% (9/4899). PLE was higher among females than males (3.8 vs 1, P < 0.01). The CAD prevalence was not significantly different between males and females (1.6 vs 1, P > 0.05). Second, the prevalence of PLE was increased in higher elevations regions compared to lower elevations (P < 0.01). However, the prevalence of CAD was not significantly different among the four regions (P > 0.05). Third, the mean times of sun exposure for PLE and CAD were 6.0 and 6.5 h/day, respectively. The mean durations of PLE and CAD were 5.8 years, and 6.6 years, respectively. The study demonstrated that the prevalence of PLE is higher than that of CAD in Yunnan, and that the prevalence of PLE is correlated with altitude.