Primary localized cutaneous amyloidosis associated with autoimmune cholangitis

A 48-year-old woman had suffered from gradual progression of skin pigmentation since 1987 and liver dysfunction since 1994, without having been treated by specialists. In September 1996, she suffered from acute development of morning stiffness of the hands and multiple arthralgias, especially of the fingers. In November 1996, her regular physician, who suspected she had rheumatoid arthritis, referred her to the Departments of Orthopedics, Rheumatology, and Internal Medicine of our hospital. Laboratory findings revealed elevation of titers of antinuclear antibody (× 320, homogeneous pattern), anti-smooth-muscle antibody (× 320), levels of immunoglobulin G (2.58 g/L; normal, 8.70–1.70 g/L), anti-DNA antibody (16 IU/mL; normal, 0–7 IU/mL), aspartate aminotransferase (97 U/L; normal, 0–35 U/L), and alanine aminotransferase (88 U/L; normal, 0–35 U/L). Results of tests for rheumatoid factor immunoglobulin M and G, hepatitis B surface antigen, hepatitis C virus antibody, lupus erythematosus cells, lupus erythematosus test, antimitochondrial antibody, anticentromere antibody, and anti-Sjogren syndrome A and B antibodies were all negative. Other laboratory findings were within normal ranges. Autoimmune hepatitis was suspected and liver biopsy was performed. A liver biopsy specimen revealed chronic nonsuppurative destructive cholangitis with dense lymphocytic infiltration around the portal veins, piecemeal/spotty necrosis, and periductal fibrosis. On the basis of these clinicopathologic findings, autoimmune cholangitis was diagnosed. Her skin pigmentation was examined at the Department of Dermatology of our hospital. On initial examination, reticular or ‘‘rippled,’' gray–brown pigmentation was found on the nape of the neck, upper back ( Fig. 1), and the extensor aspects of both arms ( Fig. 2). There was no Raynaud's phenomenon. No sclerodactylia or proximal scleroderma was found. She had no history of pre-existing dermatoses, such as atopic dermatitis, chronic eczema, or contact dermatitis. She denied the occurrence of excessive friction of the skin. A skin biopsy specimen revealed eosinophilic homogeneous masses in the papillary dermis and upper dermis. These homogeneous masses were positive to Dylon and Congo red staining ( Fig. 3). In the liver biopsy specimen, no materials positive to Dylon or Congo red staining were found. In addition, results of electrophoresis of urinary and blood protein were normal. The patient was therefore diagnosed with macular-type primary localized cutaneous amyloidosis. Figure 1. Reticular or ‘‘rippled’' gray–brown pigmentation on the upper back Download figure to PowerPoint Figure 2. Skin pigmentation on the extensor aspects of both arms Download figure to PowerPoint Figure 3. Homogeneous masses, positive to Dylon staining, in the papillary dermis (Dylon staining; original magnification, × 300) Download figure to PowerPoint Administration of prednisolone 30 mg daily improved liver function and arthralgia. The dosage of prednisolone was gradually decreased to 10 mg daily without exacerbation. Although no progression of skin lesions has occurred, skin pigmentation has persisted as of November 1999.