Anetodermia L90.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Anetoderma; Anetoderma maculosa cutis; Anetodermia; Atrophia maculosa cutis; Atrophoderma erythemause en plaques; Atrophoderma maculosa pemphigoides; dermatitis atrophicans maculosa; dermatitis maculosa atrophicans; Elastolysis acquired; erythematous anetodermia; Macular atrophy

History
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Schweninger and Buzzi, 1881; Pellizari, 1884; Jadassohn, 1892

Definition
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Anetodermia (from Greek aneto = flaccid) refers to a group of very rare, acquired, inflammatory or non-inflammatory (idiopathic), trunk-accentuated, chronic connective tissue changes leading to elastolysis, which lead to circumscribed atrophy and characteristic hernia-like protrusion (also depressions) of the skin.

Very rarely is light provocation of the anetodermic foci observed.

Etiopathogenesis
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Unknown; pathogenetically, primary or post-inflammatory processes lead to fragmentation and repair of the elastic fiber network of the skin. Detection of phagocytosis of elastic fragments in macrophages. Attachment of IgM and C3 to the basement membrane, of C3 also to elastic fibres. Biochemical detection of desmosin (elastin) in lesional skin.

The occurrence of anetodermia in connection with HIV infections, syphilis as well as lichen planus and other autoimmune diseases has been described several times.

Notice! Anetodermia is caused by a (predominantly) acquired (irreversible) circumscribed (probably inflammation-induced) elastolysis.

Notice! Several cases of familiarity of the disease have been described (in association with skeletal and ocular anomalies and neurological disorders).

Manifestation
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Adolescents and adults (2nd-4th decade of life), female gender is preferentially affected.

Clinical features
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Single or multiple, 0,2 cm to 1,0 cm large, sharply defined, roundish to oval flocks with often finely folded, thinned skin. Flocks partly sunken, partly hernia-like protrusion of the subcutaneous fatty tissue. The changes do not cause any complaints and are discovered rather by chance.

  • Type Jadassohn: Anetodermia after inflammatory stage with redness and swelling.
  • Type Pellizari: Urticarial preliminary stage.
  • Type Alexander: After bullous initial stage.
  • Type Schweninger-Buzzi: Without preliminary inflammatory stage.

Notice! There are legitimate doubts about the validity of the above-mentioned clinical (historical) classification; in most cases, post-inflammatory (secondary) elastolysis was involved, the causes of which remain mostly unknown!

Also a division into primary (idiopathic) and secondary (postinflammatory) anetoderms is not very satisfying from a clinical point of view, because inflammatory stages are rarely observed.

We understand anetodermia as atrophic-scarring end stage of different inflammatory processes of the skin.

Histology
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Fragmentation, repair and phagocytosis of elastic fibres; collagen fibres diluted or fragmented, with larger spaces between the fibre bundles (assessment in the Elastica-van Gieson stain). Number of fibroblasts reduced. Depending on the acuity, spongy perivascular round cell infiltrates.

Differential diagnosis
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Therapy
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All therapeutic approaches to anetodermia are of doubtful value! In the inflammatory stage penicillin is recommended, in case of existing skin atrophy no therapy is possible.

Literature
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  1. Bilen N et al (2003) Anetoderma associated with antiphospholipid syndrome and systemic lupus erythematosus. Lupus 12: 714-716
  2. Dikarinen AI, Palatsie R, Adomian GE et al (1984) Anetoderma: Biochemical and ultrastructural demonstration of elastin defect in the skin of three patients. J Am Acad Dermatol 11: 66-72
  3. Emer J et al (2013) Generalized Anetoderma after Intravenous Penicillin Therapy for Secondary Syphilis in an HIV patient. J Clin Aesthet Dermatol 6:23-28
  4. Fujioka M et al (2003) Secondary anetoderma overlying pilomatrixomas. Dermatology 207: 316-318
  5. Ghomrasseni S et al (2002) Anetoderma: an altered balance between metalloproteinases and tissue inhibitors of metalloproteinases. At J Dermatopathol 24: 118-129
  6. Hodak E et al (2003) Primary anetoderma: a cutaneous sign of antiphospholipid antibodies. Lupus 12: 564-568
  7. Hunt R (2011) Circumscribed lenticular anetoderma in an HIV-infected man with a history of syphilis and lichen planus.Dermatol Online J 17: 2
  8. Jadassohn J (1892) About a peculiar form of Atrophia maculosa cutis. Arch Dermatol Syphilol (Berlin) 1: 342-358
  9. Kasper RC et al (2001) Anetoderma arising in cutaneous B-cell lymphoproliferative disease. At J Dermatopathol 23: 124-132
  10. Patrizi A et al (2011) Familial anetoderma: a report of two families. Eur J Dermatol 21:680-685
  11. Pellizari C (1884) Eritema orticato atrofizzante atrophia partiale idiopathica della pelle. Gior Ital Mal Ven 19: 230
  12. Schweninger E, Buzzi F (1881) Multiple benign tumor like new growth of the skin. In: International Atlas of Rare Skin Diseases, Part 5, Plate 15, L. Voss, Leipzig

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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