Background: Cutaneous amyloidosis has been classified into primary cutaneous amyloidosis (PCA, OMIM #105250),\r\nsecondary cutaneous amyloidosis and systemic cutaneous amyloidosis. PCA is the deposition of amyloid in\r\npreviously apparent normal skin without systemic involvement. Amyloidosis cutis dyschromica (ACD) is a rare\r\ndistinct type of PCA. Here, the unique clinical and histological findings of two Chinese female siblings with ACD\r\nwere described.\r\nCases presentations: Patient 1 was a 34-year-old female, presented with mildly pruritic, diffuse mottled\r\nhyperpigmentation and hypopigmentation. The lesions involved all over the body since she was 10 years old.\r\nThere were a few itchy blisters appearing on her arms, lower legs and truck, especially on the sun-exposed areas in\r\nsummer. Some hypopigmented macules presented with slight atrophy. Patient 2 was 39-year-old, the elder sister\r\nof patient 1. She had similar skin lesions since the same age as the former. The atrophy and blisters on the skin of\r\nthe patient with amyloidosis cutis dyschromica have not been described in previous literature. Histological\r\nexaminations of the skin biopsies taken from both patients revealed amyloid deposits in the whole papillary\r\ndermis. Depending on the histological assessment, the two cases were diagnosed as amyloidosis cutis\r\ndyschromica.\r\nConclusion: The two cases suggest that the atrophy and blisters may be the uncommon manifestations of\r\namyloidosis cutis dyschromica. It alerts clinicians to consider the possibility of ACD when meeting patients with\r\ncutaneous dyschromia. Skin biopsy is essential and family consultation of genetic investigation is very important in\r\nsuch cases.
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