Lipoid proteinosis in photosensitivity E80.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Light-induced lipoid proteinosis; Lipoid proteinosis light-induced

History
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Urbach and Wiethe, 1929

Definition
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Today no longer commonly used term for skin symptoms in extremely light-sensitive patients with protoporphyria erythropoetica, with a clinical aspect reminiscent of hyalinosis cutis et mucosae.

Manifestation
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Especially in spring.

Localization
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Skin areas exposed to light.

Clinical features
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Patchy papuloverrucous changes on the sides and edges of the fingers, on the elbows, in the nose area. No mucous membrane changes, no involvement of internal organs.

Diagnosis
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Differential diagnosis
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Therapy
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Literature
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  1. Findlay GH (1987) Lipoid proteinosis. In: Fitzpatrick TB, Eisen AZ, Wolff K, Freedberg IM, Austen KF (eds) Dermatology in General Medicine. McGraw-Hill New York St. Louis San Francisco, pp 1760-1763
  2. Hamada T et al (2003) Extracellular matrix protein 1 gene (ECM1) mutations in lipoid proteinosis and genotype-phenotype correlation. J Invest Dermatol 120: 345-350
  3. Hamada T (2002) Lipoid proteinosis. Clin Exp Dermatol 27: 624-629
  4. Ko C et al (2003) Vesicular lesions in a patient with lipoid proteinosis: a probable acantholytic dermatosis. Am J Dermatopathol 25: 335-337
  5. Nanda A et al (2001) Lipoid proteinosis: report of four siblings and brief review of the literature. Pediatric Dermatol 18: 21-26
  6. Oyama N et al (2003) Autoantibodies to extracellular matrix protein 1 in lichen sclerosus. Lancet 362: 118-123
  7. Sethuraman G et al (2003) Lipoid proteinosis in two siblings: a report from India. J Dermatol 30: 562-365
  8. Urbach E, Wiethe C (1929) Lipoidosis cutis et mucosae. Virchows Arch A Pathol Pathol Anat 273: 285-319

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Last updated on: 29.10.2020