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Hidrotic Ectodermal Dysplasia With Diffuse Eccrine Syringofibroadenomatosis

Hidrotic Ectodermal Dysplasia With Diffuse Eccrine Syringofibroadenomatosis Abstract To the Editor.— Hidrotic ectodermal dysplasia (HED) is an uncommon autosomal-dominant disorder characterized by palmoplantar hyperkeratosis, dystrophy of the nails, and defects of the hair. It is sometimes associated with a large constellation of other abnormalities.1 In this report, we describe a case of HED in a patient with numerous papules on the palmoplantar surfaces, which revealed the histologic pattern of the eccrine syringofibroadenoma. Report of a Case.— A 53-year-old woman presented in our clinic with a thickening of palms and soles, hypotrichosis, and defects of the nails that had been present since infancy. For at least 2 years she had noticed papular lesions on the soles and palms. She was the fourth of five siblings of nonconsanguineous parents. Only her elder brother, 63 years of age, exhibited a similar condition, but he refused to undergo the skin biopsies. She told us that her teeth were conical, small, and References 1. Freire-Maia N, Pinheiro M. Fisher-Jacobsen-Clouston's syndrome . In: Freire-Maia N, Pinheiro M, eds. Ectodermal Dysplasia: A Clinical and Genetic Study . New York, NY: Alan R Liss; 1984:67-70. 2. Mascaro JM. Considerations sur les tumeurs fibroépithéliales: le syringofibroadénome eccrine . Ann Dermatol Venereol . 1963;90:143-153. 3. Weedon D, Lewis J. Acrosyringeal nevus . J Cutan Pathol. 1977;4:166-168.Crossref 4. Ogino A. Linear eccrine poroma . Arch Dermatol. 1976;112:841-844.Crossref 5. Wilkinson RD, Schopflocher P, Rozenfeld M. Hidrotic ectodermal dysplasia with diffuse eccrine poromatosis . Arch Dermatol. 1977;113:472-476.Crossref 6. Nordin H, Mansson T, Svensson A. Familial occurrence of eccrine tumours in a family with ectodermal dysplasia . Acta Derm Venereol. 1988;3:27-31. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Hidrotic Ectodermal Dysplasia With Diffuse Eccrine Syringofibroadenomatosis

Archives of Dermatology , Volume 125 (12) – Dec 1, 1989

Hidrotic Ectodermal Dysplasia With Diffuse Eccrine Syringofibroadenomatosis

Abstract

Abstract To the Editor.— Hidrotic ectodermal dysplasia (HED) is an uncommon autosomal-dominant disorder characterized by palmoplantar hyperkeratosis, dystrophy of the nails, and defects of the hair. It is sometimes associated with a large constellation of other abnormalities.1 In this report, we describe a case of HED in a patient with numerous papules on the palmoplantar surfaces, which revealed the histologic pattern of the eccrine syringofibroadenoma. Report of a Case.— A...
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Publisher
American Medical Association
Copyright
Copyright © 1989 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1989.01670240117032
Publisher site
See Article on Publisher Site

Abstract

Abstract To the Editor.— Hidrotic ectodermal dysplasia (HED) is an uncommon autosomal-dominant disorder characterized by palmoplantar hyperkeratosis, dystrophy of the nails, and defects of the hair. It is sometimes associated with a large constellation of other abnormalities.1 In this report, we describe a case of HED in a patient with numerous papules on the palmoplantar surfaces, which revealed the histologic pattern of the eccrine syringofibroadenoma. Report of a Case.— A 53-year-old woman presented in our clinic with a thickening of palms and soles, hypotrichosis, and defects of the nails that had been present since infancy. For at least 2 years she had noticed papular lesions on the soles and palms. She was the fourth of five siblings of nonconsanguineous parents. Only her elder brother, 63 years of age, exhibited a similar condition, but he refused to undergo the skin biopsies. She told us that her teeth were conical, small, and References 1. Freire-Maia N, Pinheiro M. Fisher-Jacobsen-Clouston's syndrome . In: Freire-Maia N, Pinheiro M, eds. Ectodermal Dysplasia: A Clinical and Genetic Study . New York, NY: Alan R Liss; 1984:67-70. 2. Mascaro JM. Considerations sur les tumeurs fibroépithéliales: le syringofibroadénome eccrine . Ann Dermatol Venereol . 1963;90:143-153. 3. Weedon D, Lewis J. Acrosyringeal nevus . J Cutan Pathol. 1977;4:166-168.Crossref 4. Ogino A. Linear eccrine poroma . Arch Dermatol. 1976;112:841-844.Crossref 5. Wilkinson RD, Schopflocher P, Rozenfeld M. Hidrotic ectodermal dysplasia with diffuse eccrine poromatosis . Arch Dermatol. 1977;113:472-476.Crossref 6. Nordin H, Mansson T, Svensson A. Familial occurrence of eccrine tumours in a family with ectodermal dysplasia . Acta Derm Venereol. 1988;3:27-31.

Journal

Archives of DermatologyAmerican Medical Association

Published: Dec 1, 1989

References