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Multiple Endocrine Neoplasia Type 2a Associated with Cutaneous Lichen Amyloidosis

Robert F. Gagel, MD; Moise L. Levy, MD; Donald T. Donovan, MD; Bobby R. Alford, MD; Thomas Wheeler, MD; and Jaime A. Tschen, MD
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Grant Support: Supported in part by Merit Review and Career Development awards from the Veterans Administration and grants MO1-RR00350 and R01-DK38146 from the National Institutes of Health. Other support for hospital care was provided by the Baylor College of Medicine from the Department of Otolaryngology, The Methodist Hospital Clinic Fund, and the Dermatopathology Research Fund.

Requests for Reprints: Robert F. Gagel, MD, Laboratory of Molecular and Cellular Endocrinology, Veterans Administration Medical Center (111E), 2002 Holcombe Boulevard, Houston, TX 77030.

Current Author Addresses: Dr. Gagel: Laboratory of Molecular and Cellular Endocrinology, Veterans Administration Medical Center (111E), 2002 Holcombe Boulevard, Houston, TX 77030.

Drs. Levy and Tschen: Department of Dermatology, Baylor College of Medicine, 1 Baylor Plaza, Houston, TX 77030.

Drs. Donovan and Alford: Department of Otolaryngology, Baylor College of Medicine, 1 Baylor Plaza, Houston, TX 77030.

Dr. Wheeler: Department of Pathology, Southwest Memorial Hospital, 7600 Beechnut, Houston, TX 77074.

Ann Intern Med. 1989;111(10):802-806. doi:10.7326/0003-4819-111-10-802
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Purpose: To describe and characterize the association of hereditary cutaneous lichen amyloidosis with multiple endocrine neoplasia type 2a (MEN 2a).

Design: Survey of a family for two diseases.

Setting: Evaluation of patients at a clinical research center.

Patients: Nineteen family members with MEN 2a.

Measurements and Main Results: In this family cutaneous lichen amyloidosis presented as multiple infiltrated papules overlying a well-demarcated plaque in the scapular area of the back (right or left). Immunohistochemical studies showed amyloid that stained for keratin but not calcitonin. Three family members had the characteristic skin lesion and also carried the gene for MEN 2a; two additional members carried the gene for MEN 2a, but did not manifest the observable skin changes associated with lichen amyloidosis.

Conclusions: From the findings in this kindred and in another recently reported but unrelated family with an identical type of pruritic skin rash and MEN 2a, several conclusions can be drawn. First, the syndrome of cutaneous amyloidosis and MEN 2a is a clearly defined autosomal dominant hereditary syndrome. Second, the dermal amyloid appears to be caused by deposition of keratin-like peptides rather than by calcitonin-like peptides. Third, known families with hereditary lichen amyloidosis should be screened to determine the true frequency of this syndrome.





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