HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text (PDF) Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Rubinow, A. Articles by Cohen, A. S. Search for Related Content PubMed PubMed Citation Articles by Rubinow, A. Articles by Cohen, A. S.

Skin involvement in familial amyloidotic polyneuropathy

Thorndike Memorial Laboratory and the Department of Medicine, Boston City Hospital, the Evans Department of Clinical Research, University Hospital, and the Arthritis Center of Boston University School of Medicine, Boston, MA.

A 3-mm punch biopsy of clinically normal skin was obtained from the forearm of 11 patients from five kinships with familial amyloid polyneuropathy. Seven of the 11 patients had sensory polyneuropathy in the arms as well as the legs. Abnormalities of the autonomic nervous system (nocturnal diarrhea and sphincter abnormalities) were observed in all patients. Four patients had multiple atrophic scars and poorly healed ulcers on the limb; two had petechiae after gentle' stroking of the skin. Histopathology revealed amyloid deposits in all 11 skin biopsies (100%). Amyloid infiltration was noted in blood vessels, sweat glands, dermis, and arrector pili muscles. Intra-cutaneous neural deposits were not found. Skin biopsies from family members at risk may be an effective method of early diagnosis.

Address correspondence and reprint requests to Dr. Rubinow, Department of Medicine, Thorndike 303, Boston City Hospital, 818 Harrison Avenue, Boston, MA 02118.

Accepted for publication January 27, 1981.

Grants in support were received from the U.S. Public Health Service, National Institute of Arthritis, Metabolism and Digestive Diseases (Nos. AM-04599 and AM 07014), the General Clinical Research Center Branch, Division of Research Resources, NIH (No. RR-533); the National Institutes of Health Multipurpose Arthritis Center (No. AM 20613); the Massachusetts Chapter of the Arthritis Foundation; and the Arthritis Foundation.

This article has been cited by other articles:

				L. Hamidi Asl, J. J. Liepnieks, K. Hamidi Asl, T. Uemichi, G. Moulin, E. Desjoyaux, R. Loire, M. Delpech, G. Grateau, and M. D. Benson Hereditary Amyloid Cardiomyopathy Caused by a Variant Apolipoprotein A1 Am. J. Pathol., January 1, 1999; 154(1): 221 - 227. [Abstract] [Full Text] [PDF]