Progressive systemic sclerosis associated with cutaneous amyloidosis

A 43-year-old woman presented with a 3-year history of Raynaud's phenomenon and a six-month history of numbness in both arms. Sclerosis was noted on the entire body surface. The skin of the face was smooth and the lips were constricted (Fig. 1). The fingers and hands were atrophic and sclerotic, and full extension of the fingers and metacarpal joints was impossible (Fig. 2). There was pigmentation on the dorsal aspect of the hands. From the nape to the upper back, pruritic wavy, rippled or reticular pigmented macules in addition to sclerosis were noted (Fig. 3). Other parts of her skin did not show such a wavy pigmentation. Physical examination revealed no specific findings in the lung, heart, and abdomen. Neurologic examination was unremarkable. Motor function including muscle tonus was normal. Laboratory studies disclosed that the complete blood count and tests of hepatic and renal function were within normal limits. Antinuclear antibody was I:80 and showed a speckled pattern, antitopoisomerase 1 antibody, anticen-tromere antibody, anti-Sm antibody and anti-RNP antibody were all negative, and the CH50 was 31.5 units/ml; C3 was 59.4 mg/dL; and C4, 17 mg/dL. Radiologically the chest and esophagus were normal. Pulmonary function and electro-cardiogram were also normal. Histologic examination of a skin biopsy obtained from the upper back revealed that the collagen bundles throughout the dermis were thickened, homogenous, and closely packed. In the upper dermis, a small number of inflammatory cells around blood vessels was observed. Eosinophilic homogeneous masses were seen in the papillary dermis and upper dermis (Fig. 4). These homogeneous masses were positive to Dylan' (Fig. 5), Congo red, and thioflavin T staining. Therefore, the diagnosis of cutaneous macular amyloidosis was made.