Polymorphic light dermatosis L56.4

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Benign summer light eruption; dermatopathy photoelectrica; Eccema solar; Eczema solar; hydroa aestivale; juvenile spring eruption; Light deflection polymorphic; Light dermatosis lupus erythematosus-like; Light eczema; Light exanthema polymorphic; Light sensitive eruption; Lupus erythematosus-like light dermatosis; PLD; PMLE; Polymorphic light deflection; Polymorphic light eruption; Polymorphic light exanthema; Prurigo Summer Prurigo; Solar Allergy; summer eruption; Summer Prurigo; Vegetable Prurigo aestivalis

History
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Bateman 1817; Hutchinson 1878; Rasch 1900

Definition
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Most frequent light dermatosis induced by UV rays, occurring after a latency period of hours or a few days, which, under various clinical manifestations always accompanied by considerable itching (papules, vesicles, plaques, coccardiac lesions, etc.), occurs exclusively in sun-exposed areas. The disease affects all ethnic groups. People with lighter skin type are more frequently affected.

Classification
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Clinically, 3 main types and further subtypes are distinguished:

  • Papular type:
    • Hemorrhagic type.
  • Plaque type:
    • Erythema-exudativum-multiforme type.
  • Papulo-vesicular type:
    • Ictus type
    • Vesiculo-bullous type
  • Lichenoid type (described in the coloured population)

Occurrence/Epidemiology
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Prevalence data vary between 10-20% of the Central European population, depending on the source (similar prevalence figures are given for the USA and Scandinavia).

Etiopathogenesis
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Currently unknown. The disease is probably based on a genetically dispositioned hypersensitivity reaction of the delayed type, which is set in motion by one or more (so far unknown) photo-induced antigens. In addition, a disorder of UV-induced immunosuppression also appears to lead to a cellular immune response by photo-induced antigens. There is an increased sensitivity, especially to UVA, but some patients also respond to UVB and visible light.

Manifestation
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In 60% of the Pat. first manifestation between the ages of 10 and 30. In at least 20% of cases there is a positive family history.

Women are affected about 10 times more frequently than men (Note: in California a gender distribution of 1:1 is reported!)

Seasonal accumulation: In spring to early summer (March to June), in light-weathered persons also occurring later.

Mostly patients with lighter skin type are affected.

Localization
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Uncovered areas of skin, especially lateral parts of the face, neckline, lateral upper arm, extensor sides of the forearms, back of the hands. Rare are scattering reactions. An essential characteristic of the disease is the preference for certain, individually different but recurring predilection sites.

Clinical features
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Very different from patient to patient (hence the name "polymorphic light dermatosis"), also in the individual patient various skin manifestations with mostly pronounced itching.

Occurrence of clinical symptoms a few hours to days after (single and first-time) intensive sun exposure. Occurrence is also possible after less intensive sun exposure on several consecutive days (probably exceeding a critical cumulative total dose).

Decay (without further UV provocation) within a few days without leaving residuals. Clinically, the predilection sites usually contain small spots, more rarely large red erythema, disseminated papules or plaques, but also papulo vesicles and erythema exudativum multiforme-like lesions. In the further course of the sunny period, a habituation effect (hardening) occurs in most cases despite further exposure.

Histology
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Sparsely to dense cuff-shaped, superficial (in a later stage also deep), perivascular lymphohistiocytic cell infiltrate. Subepidermal edema, possibly spongiosis and vacuole degeneration of the basal epithelia. Mostly normal stratum corneum. In case of more severe epidermal changes also parakeratosis.

Different histopathological patterns may occur in the different clinical variants, although the basic pattern is preserved.

Diagnosis
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Photoprovocation test in a non-appearance interval in skin not exposed to UV; MED is usually normal. Triggering of isomorphic skin phenomena on larger test fields (5 x 8 cm) by multiple UVA exposure (at least 3 times; 50-100 J/cm2) in 50-70% of cases In general, irradiation is carried out on 3-5 consecutive days in non-irradiated skin areas. Readings are taken up to 72 hours after the last irradiation at 24-hour intervals. A late reading after 2-3 weeks can be performed to exclude lupus erythematosus. In most studies it has been shown that testing with UVA or solar simulated radiation is more effective than UVB photoprovocation.

Depending on the degree of severity, a distinction is made between photoprovocation:

  • Grade 0: pigmentation, redness, no papulovesicles.
  • Grade I: erythema and pruritus.
  • Grade II: > 20 papulovesicles in the provocation field, or formation of a plaque > 50% of the provocation field.
  • Grade III: Like grade II or blisters and hemorrhages in the provocation field.

Differential diagnosis
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Light dermatosis of the papular or papulovesicular type

Clinical:

  • Photoallergic Dermatitis: Characteristic and thus diagnostically groundbreaking heliotropic macro-pattern, the micro-pattern showing the image of eczema of varying acuteity and severity (erythema, papules, papulo-vesicles, extensive scaling and itching). In contrast to phototoxic dermatitis, the "contact pattern" is not sharp, but, as in contact allergic eczema, blurred with "eczematous scattering foci" beyond UV exposure.
  • Ictus: No anamnestic connection with UV exposure. Rarely localized to UV-exposed sites.
  • REM syndrome: Chronic persistent clinical picture with reticular to planar, sharply defined, irregularly configured, bright red papules. Only slight itching.
  • Lupus erythematodes tumidus: Pronounced photosensitivity. Confluence may lead to the formation of annular or gyrated borderline plaques. No scarred healing. Characteristic and therefore diagnostic is the chronic course with persistence of the efflorescences for months.
  • Airborne Contact Dermatitis: Proof of the type of sensitization; there is less of a clinical connection with the sun than with the best known sun exposure. pollen. Clinically and histologically clear "eczema picture".

Light dermatosis of the plaque type

  • Drug exanthema, maculo-papular: Connection with drug intake must be established. When taking photosensitizing substances, sunburn-like images are usually encountered(phototoxic dermatitis).
  • Light urticaria: Very rare light dermatosis characterized by erythema and wheals on the exposed skin areas within minutes of light exposure (this is not the case with polymorphic light dermatosis). Subjectively, patients suffer from extremely severe itching. Typical is an extensive erythema formation.
  • Erythema exsudativum multiforme: Analogous images of the clinical morphology can be obtained with the erythema exsudativum multiforme type. The spreading pattern with strict emphasis on the sun-exposed areas speaks against the diagnosis "erythema exsudativum multiforme". The histological pattern can cause delimitation difficulties!

Histological:

General therapy
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Light protection as well as symptomatic external treatment.

External therapy
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In the acute stage glucocorticoids such as 0.1% betamethasone lotio(e.g. Betagalen Lotio, R030 ) or 0.1% triamcinolone acetonide cream.

Internal therapy
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Antihistamines such as desloratadine (e.g. Aerius) 5 mg/day to stop itching In severe cases, systemic glucocorticoids such as prednisolone (e.g. Decortin H) in high doses, e.g. 100 mg/day p.o. Balance out quickly.

Progression/forecast
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Overall chronic recurrent course from season to season. Effective sun protection and photo(chemo)therapy ("light-hardening") can have a very positive influence on the disease.

Prophylaxis
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Externally applied antioxidants (flavonoids such as alpha-glycosylrutin, tocopherol acetate) 1 week before exposure to the sun, starting twice a day and before current tanning, rub in thinly.

Inform the patient about the action spectrum and the character of the disease.

Consistent sun protection with textiles (only for light forms is sun protection alone with covering measures such as clothing, hats, make-up sufficient) and physical sunscreens (e.g. Anthelios; Eucerin Phase 1 Pre-Sun Gel combined with Eucerin Phase 2 Protection Gel 15). Always use light protection products with UVA and UVB filters.

If necessary, get used to light slowly by light-hardening in spring. The majority of patients remain completely symptom-free during the summer season after a pre-seasonal treatment cycle. Conventional UVB-hardening is inferior to PUVA-therapy hardening. Narrowband UVB-hardening (311 nm) is said to have an effect similar to photochemotherapy.

Naturopathy
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Successes with light protection and skin care products containing the algae enzyme photolyase (e.g. Ladival med Active Sun Protection Fluid and Ladival med Active Care Fluid) have been described: Apply Active Sun Protection Fluid immediately before sun exposure and renew after heavy sweating or bathing.

Note(s)
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Remember! Chronic forms with an eczematous picture do not belong to the spectrum of polymorphic light dermatosis, but point to the group of forms of chronic actinic dermatitis (see below Dermatitis, chronic actinic).

Literature
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  1. Bateman T (1817) Delineations of cutaneous diseases: exhibiting the characteristic appearances of the principal genera and species comprised in the classification of the late Dr. Willan; and completing the series of engravings begun by that author. Longman, Hurst, Rees, Orme and Brown (eds.), Paternoster-Row, London
  2. Boonstra HE et al (2000) Polymorphous light eruption: A clinical, photobiologic, and follow-up study of 110 patients. J Am Acad Dermatol 42(2 Pt 1): 199-207
  3. Fesq H et al (2003) Management of polymorphous light eruption: clinical course, pathogenesis, diagnosis and intervention. At J Clin Dermatol 4: 399-406
  4. Hutchinson J (1878) Summer Prurigo, prurigo aestivalis, seu prurigo adolescentium, seu acne-prurigo. Medical Times and Gazette (London) 1: 161
  5. Lindmaier A et al (1991) The PLD patient. Skin type, hardening and other light-associated features. Dermatologist 42: 430-433
  6. Lindmaier A et al (1992) The polymorphic light dermatosis. Dermatologist 43: 621-624
  7. Lim HW, Hönigsmann H, Hawk JLM (eds.) (2007) Photodermatology. Informa Healthcare USA, Inc. New York
  8. Mang R, Krutmann J (2003) Sun protection during holidays. dermatologist 54: 498-505
  9. Neumann NJ et al (2004) Polymorphic light dermatosis. JDDG 2: 220-226
  10. Stratigos AJ et al (2002) Polymorphous light eruption. J Eur Acad Dermatol Venereol 16: 193-206
  11. Tanew A et al (1996) The polymorphic light dermatosis. Act Dermatol 22: 43-46
  12. Watanabe M et al (1999) Polymorphous light eruption. A case report and consideration of the hardening mechanism. Dermatology 199: 158-161

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020