Prurigo actinic L56.4

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 21.04.2021

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Synonym(s)

actinic prurigo; Actinic Prurigo; Familial actinic prurigo; Hereditary polymorphic light dermatosis; Hutchinson's prurigo; Hutchinson's summer eruption; hydroa aestivale; solar prurigo

History
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Hutchinson 1878; Haxthausen 1918; Fox 1939; Escalona 1959; Lopez-Gonzales 1961;

Definition
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.

Classification
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Two forms are distinguished by some authors:

Occurrence/Epidemiology
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Common among Indians (prevalence among Mexicans is up to 5% of the population), mestizos and Eskimos; sporadically also occurring in Europe.

Etiopathogenesis
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The disease is regarded as a variant of polymorphic light dermatosis and is therefore considered a delayed-type hypersensitivity reaction to a photoinduced antigen. Depending on ethnicity, different HLA-restrictions (e.g. HLA-A28, HLA-B39, HLA-DR4) were observed. The action spectrum for provocation of pathological skin reactions lies in both the UVB and UVA range with a predominance of UVA.

Manifestation
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Predominantly occurring in childhood or adolescence (average age of first manifestation: 10-18 years), less frequently in adults. 5-7 times more frequent in women than in men.

Localization
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Areas that are particularly chronically exposed to the sun: face (centrofacial, especially lips and conjunctiva), ears, neck, forearms and back of the hands.

Clinical features
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Immediately after radiation exposure, development of an early urticarial phase. This slowly changes into a persistent, lichenified dermatitis. Subsequently or overlapping, the typical pruriginous skin changes in the light-exposed areas as well as scattering reactions develop. Mostly a year-round course with worsening in summer.

Histology
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Papular efflorescence: acanthosis, focal spongiosis; epidermotropic lympho-histiocytic perivascular infiltrate. Eosinophilia is possible.

Diagnosis
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Clinic. Photoprovocation test: Triggering of the typical morphs especially after provocation with UVA light. The MED may be lowered.

Complication(s)
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Pseudopterygium

External therapy
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Physical, especially textile light protection. See also light protection agent.

Radiation therapy
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Photo(chemo)therapy is only effective in individual cases.

Internal therapy
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In severe, therapy-resistant cases, immunosuppressive treatment with azathioprine 100 mg/day or with glucocorticoids such as prednisolone (e.g. Solu-Decortin H) 40-60 mg/day Thalidomide 200 mg/day ( off-label use) seems to be effective.

Progression/forecast
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Chronic course with persistent exacerbations, initially seasonal, later perennial. In adulthood spontaneous improvement in 25% of cases.

Literature
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  1. Arrese JE et al (2001) Effectors of inflammation in actinic prurigo. J Am Acad Dermatol 44: 957-961
  2. Escalona PE, Magana LM (1959) Dermatologfaia: Io esencial para el estudiante, 2nd ed., Mexico, pp. 174-178
  3. Fox H (1939) Diseases of the skin in Oklahoma Indians. Arch Dermatol 40: 544-546
  4. Hutchinson J (1878) Summer Prurigo, prurigo aestivalis, seu prurigo adolescentium, seu acne-prurigo.Medical Times and Gazette (London) 1: 161
  5. Hojyo-Tomoka MT et al (2003) Diagnosis and treatment of actinic prurigo. Dermatol Ther 16: 40-44
  6. Kuno Y et al (2003) Actinic prurigo: a 10 years follow-up study by questionnaire. Br J Dermatol 149(Suppl 64): 48-49
  7. Lane PR et al (1992) Actinic prurigo: Clinical features and prognosis. J Am Acad Dermatol 26: 683-692
  8. Millard TP et al (2001) A candidate gene analysis of three related photosensitivity disorders: cutaneous lupus erythematosus, polymorphic light eruption and actinic prurigo. Br J Dermatol 145: 229-236
  9. Neumann NJ et al (2004) Actinic prurigo. STDG 2: 373-375
  10. Umana A et al (2002) Lymphocyte subtypes and adhesion molecules in actinic prurigo: observations with cyclosporin A. Int J Dermatol 41: 139-145

Disclaimer

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

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Last updated on: 21.04.2021