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- Title
Primary localized cutaneous amyloidosis associated with autoimmune cholangitis.
- Authors
Fujiwara, Kei; Kono, Takeshi; Ishii, Masamitsu; Goto, Hithoshi; Nishiguchi, Shuhei; Kawada, Norifumi; Sakaguchi, Hiroki
- Abstract
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-Sjögren 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...
- Subjects
JAPAN; OSAKA (Japan); AMYLOIDOSIS; CUTANEOUS manifestations of general diseases; AUTOIMMUNE diseases; BILE duct diseases
- Publication
International Journal of Dermatology, 2000, Vol 39, Issue 10, p768
- ISSN
0011-9059
- Publication type
Article
- DOI
10.1046/j.1365-4362.2000.00047-4.x