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Photosensitivity in lupus.

A wide variety of skin conditions may present in patients with lupus erythematosus (LE). These can be broadly divided into three main groups: cutaneous forms of LE ('LE-specific skin disease'), non-specific cutaneous manifestations of SLE ('LE non-specific skin disease') and cutaneous complications of drug treatments for LE. This review examines clinical photosensitivity in LE, a trait most commonly associated with cutaneous forms of LE but which may also manifest in SLE. All humans are photosensitive, developing reddening of the skin if exposed to sufficient ultraviolet radiation (UVR). Therefore we define photosensitivity in clinical practice as an abnormal cutaneous response to UVR. Abnormal photosensitivity in LE may manifest in a number of different forms. The lesions of LE-specific skin disease may be induced or exacerbated by UVR. Patients with LE who are prescribed photosensitizing medications such as thiazide diuretics, neuroleptics and tetracyclines may also develop phototoxic reactions which usually present as easy sunburn. Photosensitivity may also, rarely, manifest as fragile skin and blistering in patients with both LE and porphyria cutanea tarda. Several other photosensitive disorders have been reported in association with LE, including solar urticaria and erythropoetic protoporphyria (EPP), but these appear to be chance associations. Assessment of patients with LE and photosensitivity requires a careful history and examination. Phototesting and photoprovocation tests may be used to demonstrate photosensitivity in some cases, but these are rarely required for diagnosis. Photosensitive patients should be advised about sun avoidance, photoprotection and sunscreen use as a first line treatment.

Humans↗

Incipient osteomalacia occurring in chronic actinic dermatitis.

Incipient osteomalacia developed in a Pakistani patient living in the UK after strict sunlight avoidance forming part of the management of the photosensitivity disorder, chronic actinic dermatitis. The patient's skin type and diet, which included calcium-binding phytates in chappattis, had increased his risk of the condition. Proximal muscle weakness and bony tenderness resulting from the disorder resolved on vitamin D replacement therapy.

Humans↗

Actinic injury.

Sun exposure has multiple well-documented acute and chronic effects of substantial medical importance. The most extensively studied of these are the effects on the skin. Acute effects include sunburn, phototoxic and photoallergic reactions, altered immunoreactivity, and a variety of photosensitivity disorders. Chronic effects include photoaging and photocarcinogenesis. Photoprotection is central to both prevention and treatment.

Animals↗

Photosensitivity in the Smith-Lemli-Opitz syndrome: the US experience of a new congenital photosensitivity syndrome.

Photosensitivity has been briefly mentioned in several publications on the Smith-Lemli-Opitz (SLO) syndrome, an autosomal recessive mental retardation syndrome. We conducted a questionnaire-based survey to determine the incidence and main features of photosensitivity in SLO. We confirmed a high incidence, and initial evidence suggests that SLO may be the first example of an inherited photosensitivity disorder in which sensitivity to UVA is common.

Adolescent↗

Rapid diagnosis of sensitivity to ultraviolet light in fibroblasts from dermatologic disorders, with particular reference to xeroderma pigmentosum.

A rapid and simple method for determining the sensitivity of human fibroblasts to ultraviolet light is described. As an alternative to the colony formation assay, this method can be used for the rapid diagnosis of ultraviolet light sensitivity in fibroblasts from photosensitive disorders. The method is based on growth of small numbers of cells in 1-cm wells of culture trays for 4 or more days after irradiation and determination of cell survival by the incorporation of [3H]hypoxanthine. D37 values (the dose at which 37% of the control level of incorporation remains) obtained from this procedure showed the same relative sensitivity of normal and xeroderma pigmentosum fibroblasts as was obtained by colony formation. Untransformed and SV40-transformed fibroblasts, which have different growth rates and different responses to high cell densities, gave different D37 values by this assay in culture trays as compared with colony formation. Comparison of relative sensitivities to irradiation should therefore be made only between cell types with similar growth characteristics. The similar sensitivity of normal and xeroderma pigmentosum cells to mitomycin C was also determined by this culture tray method. By increasing cell density at the beginning of the experiments, a greater capacity of group C compared with group D fibroblasts for recovery from potentially lethal damage was also detected.

Cells, Cultured↗

Diseases associated with photosensitivity.

Photosensitive disorders may be classified as those entirely caused by solar exposure and the photoaggravated disorders. Those in the former category include polymorphic light eruption, juvenile spring eruption, actinic prurigo, hydroa vacciniforme, solar urticaria, also chronic actinic dermatitis. Genodermatoses whose expression mainly depends on UV or light exposure include the DNA repair deficient disorders, some disorders of cornification, the Smith-Lemli-Opitz syndrome and porphyria. Examples of photoaggravated diseases include lupus erythematosus, erythema multiforme, atopic eczema, psoriasis, viral exanthemata, pemphigus, dermatitis herpetiformis and rosacea. Drugs and chemicals may interact with UV to induce photosensitivity. In many of these diseases the action spectrum is known or may be determined by phototesting. Recognition of the reaction patterns associated with the photodermatoses greatly assists clinical classification of the photodermatoses.

Bloom Syndrome↗

Evaluation of the photosensitive patient.

During the last few decades, a number of centers around the world have contributed to our knowledge of photosensitivity disorders. However, diagnostic methods, particularly with respect to phototesting and photopatch testing, have often differed in important details between centers. The potential advantages of using relatively standardized techniques are now being recognized. This article discusses the following areas of evaluation of the photosensitive patient: history, examination, phototesting, photopatch testing, and histopathologic and other laboratory studies.

4-Aminobenzoic Acid↗

Photodynamic DNA-breaking activity of serum from patients with various photosensitivity dermatoses.

Various drugs and chemicals break the DNA strand under ultraviolet irradiation. This study aimed to clarify the DNA-breaking activity (DBA) of serum from 39 patients with various photosensitivity disorders and that from eight normal subjects. A mixture of serum and circular plasmid DNA was exposed to longwave ultraviolet radiation, and the photoinduced cleavage of plasmid DNA was examined by electrophoretic analysis. DBA was found in serum from patients with erythropoietic protoporphyria (2 of 2), drug-induced photosensitivity (3 of 5), chronic actinic dermatitis (1 of 12) and hydroa vacciniforme (1 of 1). DBA was not found in serum from patients with porphyria cutanea tarda, collagen diseases with photosensitivity, papulovesicular light eruption or pellagra. The inhibition profile of DBA by active oxygen scavengers was different between afloqualone- and tetracycline-induced photosensitivity and chronic actinic dermatitis. The present method was useful for the detection of serum phototoxicity and the investigation of the pathomechanisms of photosensitivity.

Adult↗

Analysis of patients with suspected photosensitivity referred for investigation to an Australian photodermatology clinic.

BACKGROUND: Australia's first dedicated photodermatology clinic was established at St Vincent's Hospital Melbourne in 1993. OBJECTIVE: We sought to review clinical diagnoses and results of investigations performed on patients seen at this institution. METHODS: A database was created to enable a retrospective and prospective analysis of all patients attending for evaluation of suspected photosensitivity from April 1993 to October 2000. RESULTS: A total of 513 patients were seen, 289 (56.3%) female and 224 (43.7%) male, with a mean age of 45.2 years (range: 2.6-85.9). A photosensitive disorder was diagnosed in 397 patients (77.4%), with the acquired idiopathic photodermatoses accounting for diagnoses in 215 (41.9%) of all patients seen. The most common diagnosis was polymorphous light eruption. Reduced minimal erythema doses were seen in 25.3% of all patients light tested. In those photopatch tested, 23.3% had at least 1 photocontact reaction. Allergic contact dermatitis in a photosensitive distribution was diagnosed in 7.4% of the clinic population. CONCLUSION: A large proportion of referrals to a photodermatology clinic comprise people with acquired idiopathic photodermatoses, with other common diagnoses that may mimic photosensitivity including allergic contact dermatitis, atopic dermatitis, and rosacea.

Adolescent↗

Phototesting and photoprotection in LE.

Photosensitivity and induction of skin lesions following UV radiation is a common problem of patients with cutaneous and systemic forms of lupus erythematosus. The detrimental effect of UV radiation to patients with lupus erythematosus was already recognized in the last century. Skin lesions can now be provoked under standardized conditions allowing the diagnosis and classification of patients with photosensitive disorders. The aim of this review is to give an overview on the history, test procedure and test results in patients with lupus erythematosus.

History, 19th Century↗

UV hardening therapy: a novel intervention in patients with photosensitive cutaneous lupus erythematosus.

BACKGROUND: Patients with cutaneous lupus erythematosus (LE) and a history of disease- related photoaggravation, confirmed by phototesting, may not respond to photoprotection and/or medical intervention. Ultraviolet B-hardening therapy may improve tolerance for environmental ultraviolet radiation (UVR) in photosensitive disorders. OBJECTIVE: We studied the effect of UVB hardening on the cutaneous manifestations of patients with LE and their tolerance for UVR. PATIENTS AND METHODS: A retrospective study of continuous, home-based, UVB-hardening therapy in 44 patients with cutaneous LE (systemic LE: 9 patients; chronic LE: 21 patients; subacute cutaneous LE: 10 patients; cutaneous LE not specified: 4 patients) who had confirmed photosensitivity. Exposure to the UVB source was performed year-round, 3 times weekly, with increasing doses to a maximum of 10 minutes per session. Tolerance for environmental UVR was established through questionnaires, phototesting, and assessment of disease activity by physician and patient. RESULTS: Of 44 patients, 35 were able to gradually increase their monthly UVB doses. Nine patients dropped out because of insufficient efficacy or skin irritation, or were unable to adhere to the hardening regimen. Of the 35 patients who succeeded in hardening their skin with UVB, 28 patients reported an improved tolerance for environmental UVR. This outcome was confirmed by repeat phototesting in a subgroup. In only 5 patients, an improvement of cutaneous LE symptoms was noted by either physician or patient. No serious adverse events were encountered. LIMITATIONS: This was a retrospective study and no control group was used. CONCLUSION: This is the first report that describes UVB hardening as a potential therapy in patients with cutaneous LE and confirmed photosensitivity. This intervention may lead to improved tolerance for environmental UVR and, in a minority of patients, even to decreased cutaneous activity of LE.

Female↗

New sunscreens confer improved protection for photosensitive patients in the blue light region.

BACKGROUND: Some patients with photosensitivity disorders are sensitive to visible radiation. As current commercial sunscreens do not significantly absorb in this region, there is a lack of effective topical photoprotection. To meet this need a new range of sunscreens has been developed incorporating zinc oxide and pigmentary grade titanium dioxide as active ingredients. OBJECTIVES: To determine the effectiveness of the new sunscreens in providing protection for patients with visible radiation photosensitivity. METHODS: In the first part of this study, an in vitro transmission spectrum was obtained. The properties of the new sunscreens, as well as a range of commercial agents, were compared, and a new parameter, photosensitivity protection factor (PPF), was developed. This was used to predict the likely degree of protection the various sunscreens would provide for patients with photosensitivity extending into the visible region. In the second in vivo part of the study, patients with known visible (blue) light photosensitivity were tested using light at 430 +/- 30 nm and the protection factor (PF) at this wavelength was determined. RESULTS: Mean +/- SD PPFs for the new sunscreens were between 5.4 +/- 0.3 and 9.6 +/- 0.3, compared with 4.1 +/- 0.1 for Sun E45 (sun protection factor, SPF 25) and 4.2 +/- 0.1 for RoC Total (SPF 25). The derived in vivo PF for Sun E45 ranged between 1 and 4 (median 2). For the new sunscreens the range was 3 to > 10 (median 8). CONCLUSIONS: This study demonstrates that the new sunscreens do provide protection for patients with sensitivity to visible light (blue light region).

Adult↗

Photosensitivity in childhood.

Out of a total of 370 patients with photosensitivity disorders, the light sensitivity had started at an age of 15 years or earlier in 95 cases (26%). Seventy-eight of these suffered from polymorphous light eruptions (PMLE). In half of the PMLE cases the morphology of the light-induced rash was classified as prurigo-like, and in one-fourth as vesicopapular. Other registered photodermatoses with childhood onset were xeroderma pigmentosum, erythropoietic protoporphyria, systemic lupus erythematosus and pellagra. No childhood cases of photosensitivity from external chemicals or internal medication were encountered.

Child↗

Actinic prurigo: clinical features and HLA associations in a Canadian Inuit population.

BACKGROUND: Actinic prurigo (AP) is an idiopathic familial photodermatitis. AP of the Inuit is rarely reported and poorly characterized. OBJECTIVE: Our purpose was to examine the clinical features and HLA associations of AP in an Inuit population. METHODS: Thirty-seven Inuit subjects with AP were administered a questionnaire and underwent a cutaneous examination. Other causes of photosensitivity were excluded. HLA class I typing was performed by polymerase chain reaction and sequence-specific primers and class II typing by polymerase chain reaction and sequence-specific oligonucleotide probes. RESULTS: Subjects were 81.1% female, 67.6% had a family history of photosensitivity, and all experienced seasonal variation. The average age at onset of photosensitivity was 29 years, and only 27% had a trend toward improvement in photosensitivity. Involvement of eyes and nonexposed skin was reported in 62.2% and 18.9% of subjects, respectively. Physical examination revealed involvement of the face (64.9%), lip (32.4%), ear (13.5%), and dorsal aspect of the hand (24.3%). HLA-DRB1*14 was present in 51.2% of subjects and 26.2% of controls (P =.022, odds ratio = 2.975). This is a previously unreported HLA association. CONCLUSION: AP in the Inuit is a seasonal, pruritic photodermatitis, often commencing in adulthood and worsening over time. A novel association with HLA-DRB1*14 has been discovered. Overall, this novel HLA association, the absence of HLA associations previously reported in non-Inuit populations, and clinical distinguishing features support the concept that AP in the Inuit may have a distinct immunopathogenic basis that translates into a different phenotype. It also raises the question of whether AP in the Inuit is a distinct photosensitivity disorder specific to this group that has been genetically isolated because of geographic and cultural seclusion.

Adult↗

Haemorrhagic polymorphic light eruption: two cases of a rare variant.

Polymorphic light eruption is a common photosensitivity disorder of unknown aetiology, that usually presents in the spring or summer months as an intermittent non-scarring, itchy erythematous, papulo-vesicular eruption. We present two cases of haemorrhagic polymorphic light eruption, a rare variant of this condition of which there are no case reports in the literature.

Adult↗

Implication for photosensitive patients of ultraviolet A exposure in vehicles.

BACKGROUND: Photosensitive patients sometimes report disease flares during journeys by car. Window glass blocks all UVB but not all UVA. All car windscreens are made from laminated glass. Side and rear windows are usually made of nonlaminated glass. OBJECTIVES: To determine which types of glass provide most protection from UVA with particular reference to the implications for patients with polymorphic light eruption (PLE). METHODS: The percentage transmission of UVA was determined for a selection of glass, both laminated and nonlaminated, and with differing colour tints. RESULTS: Laminated glass transmits less UVA than nonlaminated glass. Tinted glass transmits less UVA than clear glass. Nonlaminated clear glass transmitted the highest percentage of UVA (62.8%) and grey laminated glass the lowest (0.9%). A dose of 5 J cm(-2) UVA, enough to trigger PLE in some patients, could be transmitted through clear nonlaminated glass in 30 min but would take 50 h through grey laminated glass. CONCLUSION: Patients with severe UVA-induced PLE and other photosensitivity disorders may have disease flares from solar UVA transmission through side-window glass. Protective measures such as wearing long-sleeved clothing, keeping the arm beneath the bottom of the window aperture, or choosing tinted and laminated car windows may be helpful.

Automobiles↗

Solar angioedema: an uncommonly recognized condition?

Solar urticaria is a well defined although uncommon photosensitivity disorder, and is said to be the underlying cause of chronic urticaria in approximately 0.5% cases. In contrast, solar angioedema is seldom reported. We describe two patients with postulated solar angioedema, associated with clinical and/or phototest features of solar urticaria. Recognition of solar provocation of angioedema has important consequences for patient management.

Angioedema↗

Skin response to ultraviolet B light in patients infected with human immunodeficiency virus.

Photosensitivity disorders have been reported in human immunodeficiency virus (HIV)-infected patients, often as the initial manifestation of the disease. The objective of this study was to evaluate whether the HIV-infected population demonstrates increased sensitivity to ultraviolet B (UVB) radiation. Minimal erythema dose values to UVB (MED-B) of 57 consecutive HIV-infected patients were compared to those of a control group of 57 consecutive patients with skin diseases, who were otherwise healthy and had no risk factors for HIV infection. MED-B determinations were performed in all individuals prior to the initiation of phototherapy for treatment of skin disease. None of the patients had a history of photosensitivity. Furthermore, the mean levels of the highest UVB doses received by each group during the treatment courses were compared. The mean age of the HIV-infected cohort was 43 years (range 26-61 years). The mean MED-B for this group was 82.8 +/- 3.8 (SEM) mJ/cm2. The mean age of the control group was 45 years (range 24-77 years), and their mean MED-B was 81.0 +/- 3.8 (SEM) mJ/cm2. After 12 weeks of treatment, one HIV-infected patient developed photosensitivity associated with a decreased MED-B value. The mean level of the highest UVB doses received by the HIV-infected group [427.5 +/- 67.2 (SEM) mJ/cm2] was lower than that received by the control group [640.8 +/- 65.9 (SEM) mJ/cm2], since HIV-infected patients received fewer treatments (mean: 34.7 treatments per patient) than the patients in the control group (mean: 65.6 treatments per patient). These data indicate that the HIV-infected patient population, without history of photosensitivity, does not show increased sensitivity to UVB light as determined by MED-B values.

Adult↗