Connective Tissue Diseases Codexery

Dyschromic and atrophic variation of scleroderma

A rare scleroderma variant with depressed hyperpigmented patches.

Dyschromic and atrophic variation of scleroderma is a form of morphea, which involves isolated patches of hardened skin without internal organ involvement.[1][2] Among those with morphea, the condition appears more frequently in females, with a 3:1 female-to-male ratio.[3]

Quick Facts

Also known as
  • Atrophoderma of Pasini and Pierini
  • Morphea plana atrophica
  • Sclérodermie atrophique d'emblée
Classification
rare disease

Facts from the source article.

Key facts and history

The cause of morphea is unknown to physicians and scientists.[4] Borrelia burgdorferi infection may be relevant for inducing a distinct autoimmune type called "Borrelia-associated early onset morphea"[5] Lesions in morphea typically appear as macules or plaques measuring a few centimeters across, though band-like or guttate forms are also possible.[6] According to case reports, patients diagnosed with morphea more often have relatives with autoimmune diseases.[4]

Morphea patients exhibit elevated levels of anti-histone and anti-topoisomerase IIa antibodies in laboratory tests.[4] Corticosteroids administered topically, intralesionally, or systemically have been among the therapies attempted for morphea.[7] Hydroxychloroquine and chloroquine, both antimalarial drugs, have been employed as treatments.[7] Psoralens combined with ultraviolet A light, or UVA alone, have also been tested as a therapy.[7]

Morphea is considered rare, with an estimated incidence of 2 to 4 cases per 100,000 individuals.[3]

References

  1. Wikipedia, "Morphea" (CC BY-SA 4.0), which cites: Fitzpatrick, "Fitzpatrick's color atlas and synopsis of clinical dermatology", McGraw-Hill Medical Pub. Division, 2005; Bielsa, "Deep morphea", Seminars in Cutaneous Medicine and Surgery, June 2007, p. 90–95 (we also read that source) en.wikipedia.org
  2. Wikipedia, "Morphea" (CC BY-SA 4.0), which cites: "Classification of morphea (localized scleroderma)", Mayo Clin. Proc., 1995, p. 1068–76 (original not read by us) en.wikipedia.org
  3. Wikipedia, "Morphea" (CC BY-SA 4.0), which cites: "Localized scleroderma", Curr Opin Rheumatol, 2006, p. 606–13; James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. Page 171. .; "The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993", J. Rheumatol., 1997, p. 73–80 (original not read by us) en.wikipedia.org
  4. Wikipedia, "Morphea" (CC BY-SA 4.0), which cites: "Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study", Rheumatology (Oxford), 2006, p. 614–20; "Anti-DNA topoisomerase IIalpha autoantibodies in localized scleroderma", Arthritis Rheum., 2004, p. 227–32; "Coexistent linear scleroderma and juvenile systemic lupus erythematosus", Pediatr Dermatol, 2000, p. 456–9; "Simultaneous occurrence of linear scleroderma and homolateral segmental vitiligo", J Eur Acad Dermatol Venereol, 2006, p. 63–5; "Generalized morphea and primary biliary cirrhosis en.wikipedia.org
  5. Wikipedia, "Morphea" (CC BY-SA 4.0), which cites: ""Borrelia-associated early-onset morphea": a particular type of scleroderma in childhood and adolescence with high titer antinuclear antibodies? Results of a cohort analysis and presentation of three cases.", J Am Acad (original not read by us) en.wikipedia.org
  6. Wikipedia, "Morphea" (CC BY-SA 4.0), which cites: James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. Page 171. . (original not read by us) en.wikipedia.org
  7. Wikipedia, "Morphea" (CC BY-SA 4.0), which cites: Albuquerque, "Interventions for morphea", Cochrane Database of Systematic Reviews, 2019-07-16 (original not read by us) en.wikipedia.org

Each numbered sentence above was checked against the source it cites before publication. Sentences citing Wikipedia adapt text from the English Wikipedia article Morphea (contributors), used under CC BY-SA 4.0; this entry is available under the same licence. Other sources were used for facts only; no text or images are copied from them.

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