Document Type : Original Article(s)
Authors
1
Assisstant Professor of Rheumatology, Department of Rheumatology, Al-zahra Hospital, Isfahan University of Medical Sciences, Isfahan, Iran
2
Professor of Pathology, Fellowship of Dermatopathology,Department of Pathology, Al-zahra Hospital, Isfahan University of Medical Sciences, Isfahan, Iran
Abstract
Primary amyloidosis is the most prevalent form of systemic amyloidosis and clinical sign of this disease is in association with the extent of amyloid deposition in organ systems. Most of the organs, except central nervous system, involved in this disease (heart, kidneys, liver, GI system, spleen, thyroid, adrenals, lymph nodes, bone marrow, lung, synovium, skin, skeletal muscle and tongue). Background: We describe a 43 years Iranian female patient with sign of hardness of skin and joints (scleroderma skin like), nonspecific mechanical pain, shoulder bigness, dysphasia, hoarseness, popular skin lesions, and signs of carpal tunnel syndrome, which hospitalized because of dyspnea. In the skin biopsy and with light microscopy there was epidermal atrophy and in papillary dermis deposition of amorphous eosinophilic material. In congo-red staining amyloidosis was proved. Case Presentation: In spite of this point that the primary amyloidosis is the most prevalent form of systemic amyloidosis, in our country this is the very rare disease and the diagnosis especially in early stages is difficult. In early stages, skin manifestations of disease can confuse with other diseases like hypothyroidism and scleroderma in edematous stage and in our patient waxy form papules was similar to familiar hypercholesterolemia. Because of this presentation we fall in mistake in primary evaluation but the proteinuria cause the suspicious of amyloidosis and finally the diagnosis. Conclusion: Shoulder pad sign, tongue bigness, primary amyloidosis Key words:
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