Primary localized cutaneous amyloidosis associated with autoimmune cholangitis

A 48-year-old woman had suffered from gradual progression of skin pigmentat ion since 1987 and liver dysfunction since 1994, without having been treate d 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 rh eumatoid arthritis, referred her to the Departments of Orthopedics, Rheumat ology, and Internal Medicine of our hospital. Laboratory findings revealed elevation of titers of antinuclear antibody (x 320, homogeneous pattern), a nti-smooth-muscle antibody (x 320), levels of immunoglobulin G (2.58 g/L; n ormal, 8.70-1.70 g/L), anti-DNA antibody (16 IU/mL; normal, 0-7 IU/mL), asp artate aminotransferase (97 U/L; normal, 0-35 U/L), and alanine aminotransf erase (88 U/L; normal, 0-35 U/L). Results of tests for rheumatoid factor im munoglobulin M and G, hepatitis B surface antigen, hepatitis C virus antibo dy, lupus erythematosus cells, lupus erythematosus test, antimitochondrial antibody, anticentromere antibody, and anti-Sjogren syndrome A and B antibo dies were all negative. Other laboratory findings were within normal ranges . Autoimmune hepatitis was suspected and liver biopsy was performed. A live r biopsy specimen revealed chronic nonsuppurative destructive cholangitis w ith dense lymphocytic infiltration around the portal veins, piecemeal/spott y necrosis, and periductal fibrosis. On the basis of these clinicopathologi c 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 pigmen tation was found on the nape of the neck, upper back (Fig. 1), and the exte nsor aspects of both arms (Fig. 2). There was no Raynaud's phenomenon. No s clerodactylia 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 papill ary dermis and upper dermis. These homogeneous masses were positive to Dylo n and Congo red staining (Fig. 3). In the liver biopsy specimen, no materia ls positive to Dylon or Congo red staining were found. In addition, results of electrophoresis of urinary and blood protein were normal. The patient w as therefore diagnosed with macular-type primary localized cutaneous amyloi dosis. Administration of prednisolone 30 mg daily improved liver function and arth ralgia. The dosage of prednisolone was gradually decreased to 10 mg daily w ithout exacerbation. Although no progression of skin lesions has occurred, skin pigmentation has persisted as of November 1999.