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Macular amyloidosis

Published: September 18, 2026Updated: September 18, 2026

Important: Informational content intended for healthcare professionals. It does not replace clinical evaluation or establish a doctor-patient relationship.

Overview

  • Definition and synonyms
    • Macular cutaneous amyloidosis (MCA) is a form of primary localized cutaneous amyloidosis characterized by hyperpigmented macules/plaques (often with a rippled or reticulated pattern) associated with amyloid deposition in the papillary dermis , usually derived from epidermal keratins . Janodia 2026 , Chang 2004
    • It can coexist with lichenoid amyloidosis (papular lesions), constituting a spectrum (sometimes called “biphasic”). Janodia 2026 , Brownstein 1970
  • First description
    • The classic systematization of localized cutaneous forms (including macular and lichenoid variants) is attributed to the series by Brownstein and Helwig (1970) . Brownstein 1970
  • Epidemiology
  • ICD codes (indicative; may vary by country/version)
    • ICD-10 (guideline): usually coded within “cutaneous amyloidosis” or “localized amyloidosis” (e.g., L99.0 or E85.4 depending on local adaptation).
    • ICD-11 (indicative): usually grouped under “primary localized cutaneous amyloidosis / amyloidosis” within dermatology/internal medicine.
  • Diagnostic checklist (quick)
    • Directed history: pruritus, friction (habits), atopy, chronic course. Eswaramoorthy 1999 , Chia 2014
    • Examination: wavy/reticular pattern, typical location (upper back). Bandhlish 2012
    • Dermatoscopy : to support and select the site. Chuang 2012
    • 4 mm punch biopsy including papillary dermis + Congo red (or IHC for keratins if in doubt). Alhawas 2024 , Chang 2004
    • Clinical laboratory testing only if there are red flags or alternative suspicion. Janodia 2026

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