Polymorphous light eruption (PLE) is a non-life-threatening and potentially distressing skin condition that presents with itchy red small bumps on sun-exposed skin, particularly the face, neck, forearms and legs. It generally appears 30 minutes to a few hours after sun exposure and may last between one and 14 days. The bumps may become small blisters or plaques and may appear bloody, often healing with minimal scarring. It is triggered by sunlight and artificial UV exposure in a genetically susceptible person, particularly in temperate climates during the spring and early summer. The resulting itch can cause significant suffering. PLE is also defined as an idiopathic primary photodermatosis in which the photosensitizer is unknown. For its varying clinical appearances, it is interchangeably named polymorphic or polymorphous. Treatments include prevention with sun avoidance and supervised light therapy, and symptom control with topical steroids.
Signs and symptoms
Typically, the first episode develops in the spring following the first exposure to intense sun. Further episodes of the irritable rash occur several hours to days following subsequent sun exposure. PLE appears on areas of the skin newly exposed to sunlight such as the visible part of the neckline, backs of hands, arms and legs, and feet, but less commonly the face. At these areas, there may be feelings of burning and severe itching. Smooth red-topped small papules which merge into plaques, small fluid-filled blisters (papulovesicles) and less commonly target-shaped lesions which look like erythema multiforme may be visible. In addition, it may occur in other parts of the body in some people treated for inflammatory skin diseases with phototherapy. The rash is usually quite symmetrical and characteristic for each individual, appearing similar with each recurrence, but can look dissimilar in different people. Fever, fatigue and headaches have been previously associated with the eruption, but are rare. The rash may persist for many days to a couple of weeks, resolving spontaneously without scarring as long as further sunlight exposure is avoided. Recurring yearly, the eruption can sometimes last longer than a few days if persistent and repeated sun exposure occurs. However, the "hardening" effect, with respite during the later summer, frequently occurs with gradual exposure of sunlight, eventually leading to significant improvement.
Causes The cause of PLE is not yet understood, but several factors may be involved. It is thought to be due to a type IV delayed-type hypersensitivity to an allergen produced in the body following sunlight exposure, in a genetically susceptible person. It is also thought that skin microbiome or microbial elements could be involved in pathogenesis of the disease
UV exposure PLE can be provoked by UVA or UVB (chief cause of sunburn) rays, meaning it can be triggered even by sunlight through glass. UV-A is a major constituent of sunlight, can pass through glass, is relatively resistant to sunscreen and can cause light eruption without sunburn. Artificial UV light sources from tanning units and phototherapy treatment units can also trigger PLE. About three-quarters of patients acquire PLE after UV-A exposure only, one-tenth after UV-B exposure only, and the rest after a combination of UV-A and UV-B exposure. People vary in the amount of sun exposure needed to trigger the rash.
Oxidative stress Oxidative stress and the modification of the redox status of the skin has been implicated in the expression of PLE.
Photosensitizer It has been suggested that an undefined endogenous or exogenous photo-allergen may trigger a delayed immune reaction resulting in PLE.
Genetics Half of patients have a family history of PLE, demonstrating a clear genetic influence.
Oestrogen effect The preponderance in women with a decline in severity following menopause has been thought to be associated with oestrogen effects. A natural fall in oestrogens may account for the tendency to remit after the menopause.
Diagnosis The diagnosis of PLE is typically made by assessing the history and clinical observations. Any investigations are usually to exclude other conditions, particularly lupus and porphyria. Blood tests are usually normal. However, positive antinuclear antibody and extractable nuclear antigen (anti-Ro/La) in low titre may be found, even in the absence of other criteria to suggest a diagnosis of lupus erythematosus. If clinical findings suggest a possibility of porphyria, urinary and red cell porphyrin screening may be performed and are negative in PLE. Photoprovocation tests are usually not required but may be undertaken by specialised centres in winter. When a decision to undertake this is made, a small area of the frequently affected skin is exposed to varying doses of UVA and minimal erythema dose (MED) (amount of UV radiation that will produce minimal redness of skin within a few hours following exposure) of broadband UVB for three consecutive days. An examination of the skin to detect the rash is made, however, up to 40% have false negative responses.
Biopsy findings Depending on the clinical signs, histology of a skin biopsy may vary. There may be oedema in the epidermis with a dense superficial and deep lymphocytic infiltrate without vasculitis. Recently appearing lesions may show neutrophils. Spongiosis and vesicle formation may also be present. Direct immunofluorescence testing is negative.
Differential diagnosis The photosensitivity connected with lupus erythematosus is the main condition that may appear like PLE. However, the rash of lupus is inclined to be more persistent. PLE does not increase the risk of lupus. Other similar appearing conditions are solar urticaria, which has a shorter duration, the eczema-like condition, photosensitive dermatitis, and photosensitivity drug reaction. Prickly heat, which is caused by warm weather or heat is not the same as PLE. Photosensitivity is also found in some of the porphyrias. Nearly all cases of porphyria cutanea tarda exhibit blister formation on the skin within 2–4 days of light exposure. Variegate porphyria and hereditary coproporphyria can also exhibit symptoms of light-induced blisters.
Classification Sunlight has been documented to trigger numerous skin conditions and the confusing terminology and categorisation previously has made the correct diagnosis and subsequent treatment difficult. Variants of PLE have been described:
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