Familial amyloidosis cutis dyschromica in three siblings: Report from Indonesia

Melyawati Hermawan, Rahadi Rihatmadja, Sondang Marisi Holong Aemilia Pandjaitan Sirait

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Amyloidosis cutis dyschromica (ACD) is an extremely rare type of primary cutaneous amyloidosis. To date there are fewer than 40 published cases worldwide; some were reported affecting several family members. Its resemblance to other common pigmentation disorders makes it rarely recognized at first sight. Our patient, the 12-year-old firstborn son of non-consanguineous parents presented with generalized mottled pigmentation starting from lower extremities. His siblings suffered from similar condition. The clue for diagnosis is the amyloid deposition in the papillary der-mis. The etiology of ACD is still unknown, but genetic factors and ultraviolet radiation are implicated. It is proposed that disturbance of ker-atinocyte repair following ultraviolet radiation results in amyloid deposition. The treatment remains a challenge. Oral acitretin treatment, thought to repair keratinization defect, gave a slight improvement in our case. Our is the first case of ACD reported in Indonesia.

Original languageEnglish
Article number5375
Pages (from-to)19-20
Number of pages2
JournalDermatology Reports
Volume6
Issue number1
DOIs
Publication statusPublished - 1 Jan 2014

Keywords

  • Acitretin
  • Amyloidosis cutis dyschromica
  • Familial
  • Histopathology

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