Morteza Moatamedi
University of Glasgow, UK
Title: Primary Localised Cutaneous Amyloidosis: case report and literature review
Biography
Biography: Morteza Moatamedi
Abstract
Clinical Finding:
A 63 years old female presented to clinic with 6 years history of discoloured areas on right palm, right middle and ring fingers. This has been asymptomatic except for one episode of bleeding following minor trauma. Examination revealed dull red, flat, non-pulsatile areas on palm. There was no hyperkeratosis or ulceration.
Investigations:
Skin biopsy was performed with the following findings: Epidermis: thick keratin. Dermis: oesinophilic material, probably fibrin with loose strauma and loose red cells, some ectasia of small blood vessels but no hemosiderin.
Diagnosis: Cutaneous Amyloidosis
Discussion:
Amyloidosis is explained by extra cellular deposition of proteinaceous substance or various insoluble proteins that results in considerable tissue dysfunction. It may affect several body organs. Amyloidosis is usually classified as primary or secondary according to underlying cause. In this case clinical findings suggested Primary Localised Cutaneous Amyloidosis (PLCA); that is deposition of amyloid in previously normal skin with no evidence of deposits in internal organs. PLCA is classified to Macular, Papular or lichen amyloidosis, Nodular or tumefactive and Familial. This case suggests Macular form of PLCA although it differs from some usual clinical and pathological presentations.
Primary cutaneous amyloidosis has been reported in different body areas. Familial PLCA shows autosomal dominant inheritance. Management of PLCA is challenging. In this case, patient was treated with potent topical steroid with no significant improvement. Other options include: Calcipotriol, Phototherapy, DMSO (dimethyl sulfoxide), Etretinate, Acitretin, Cyclophosphamide, Ciclosporin, Cryotherapy, Dermabrasion, CO2 laser and surgical measures.