This Article  • References  • Full text PDF  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

Associated With Alopecia, Aminoacidura, and Myasthenia Gravis

Algie C. Brown, MD; Robert G. Crounse, MD; R. K. Winkelmann, MD, PhD

Arch Dermatol. 1969;99(4):478-493.

Abstract
An unusual syndrome of progressive baldness resulting from a distinctive hamartoma of the hair follicle is presented. The patient also had myasthenia gravis, an enlarged sella turcica, and an abnormal urinary amino acid pattern and was suspected of having lupus erythematosus. The generalized hair loss was diagnosed as alopecia universalis until multiple skin biopsies revealed a basal cell hamartoma of each hair follicle. Histochemically and by electron microscopic studies the tumor had characteristics of epithelial cells. A wax reconstruction showed the normal anatomic relationship of sebaceous glands and the arrectores pilorum muscles to this tumor. This study emphasizes the importance of skin biopsy in alopecia of unknown cause and suggests that further study of abnormal ectodermal tissues might provide insight into hair keratin defects.

Author Affiliations
Rochester, Minn

From the Mayo Clinic and Mayo Foundation: Section of Dermatology (Dr Winkelmann), and Mayo Graduate School of Medicine (University of Minnesota) (Dr. Brown), Rochester, Minn. Dr. Crounse is at the Depatment of Dermatology, Medical College of Georgia, Augusta, Ga.

Footnotes
Accepted for publication Nov 14, 1968.

Read before the 117th Annual Convention of the American Medical Association, San Francisco, June 1968.

Reprint requests to Mayo Clinic, 200 First St SW, Rochester, Minn 55901 (Dr. Winkelmann).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Genetics of skin appendage neoplasms and related syndromes
Lee et al.
J. Med. Genet. 2005;42:811-819.
ABSTRACT | FULL TEXT  

Basaloid follicular hamartoma, total body hair loss and SLE
Morton et al.
Lupus 1998;7:207-209.
ABSTRACT  

Congenital Generalized Follicular Hamartoma Associated With Alopecia and Cystic Fibrosis in Three Siblings
Mascaro et al.
Arch Dermatol 1995;131:454-458.
ABSTRACT  

Basaloid Follicular Hamartoma: A Histologic Diagnosis With Diverse Clinical Presentations
Nelson et al.
Arch Dermatol 1993;129:915-917.
ABSTRACT  

Folliculocentric Basaloid Proliferation: The Bulge (der Wulst) Revisited
Leshin and White
Arch Dermatol 1990;126:900-906.
ABSTRACT  

Reticulate Pigmented Anomaly of the Flexures: Case Reports and Genetic Investigation
Brown
Arch Dermatol 1982;118:490-493.
ABSTRACT  

Multiple Trichoepithelioma and Breast Cancer
Brown
Arch Dermatol 1979;115:637-637.
ABSTRACT  

Reticulate Pigmented Anomaly of the Flexures: Dowling Degos Disease, A New Genodermatosis
Jones and Grice
Arch Dermatol 1978;114:1150-1157.
ABSTRACT  

Congenital Disseminate Organoid Tumors
Solomon and Eng
Arch Dermatol 1977;113:851-851.
ABSTRACT  

Generalized Follicular Hamartoma: Complicated by Multiple Proliferating Trichilemmal Cysts and Palmar Pits
Mehregan and Hardin
Arch Dermatol 1973;107:435-438.
ABSTRACT  

Multiple Hamartoma Syndrome (Cowden's Disease)
Weary et al.
Arch Dermatol 1972;106:682-690.
ABSTRACT