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Papular Eruption on a Patient With Down Syndrome—Diagnosis

Papular Eruption on a Patient With Down Syndrome—Diagnosis Diagnosis: Multiple syringomas. Microscopic findings The skin biopsy specimen showed dermal fibrosis. In the upper dermis, there was a proliferation of eccrine-type ducts, many of which were dilated and contained eosinophilic material. The ducts were lined by 2 or more layers of cuboidal cells, often with luminal clear cell change. There was also a mild perivascular lymphohistiocytic inflammatory cell response. The epidermis displayed mild overlying hyperkeratosis. Discussion Syringomas are benign tumors that originate in the intraepidermal eccrine ducts.1 Clinically, they are asymptomatic, small, yellow or red-brown, firm papules. They are seen more frequently in females and commonly occur in the periorbital area, especially in patients with Down syndrome. Other sites that are affected less commonly include the neck, chest, and abdomen. The incidence of syringomas in patients with Down syndrome has been reported to be approximately 30 times greater than that in the general population.2 Friedman and Butler3 classified syringomas into the following 4 groups according to their clinical features and associations: (1) a localized form, (2) a generalized form, including multiple and eruptive variants, (3) a form associated with Down syndrome, and (4) a familial form. Although the history of onset was not available in our patient, his case may represent an example of eruptive syringomas. Eruptive syringomas typically occur on the anterior body surfaces as successive crops.1 There have been previous case reports of eruptive syringomas occurring in patients with Down syndrome,4-6 but in contrast with the periorbital syringomas, there is no evidence that the incidence of eruptive syringomas is greater in patients with Down syndrome than in the general population. Clinically, syringomas can resemble other papular eruptions, and diagnosis is often only made through histologic examination, which typically shows multiple small ducts lined by rows of flattened epithelial cells,7 with epithelial strands within the dermis. Syringomas are asymptomatic but pose a cosmetic problem. They can be treated with electrocoagulation or cryotherapy. Eruptive syringomas are difficult to treat mainly because of the sheer number of lesions that are involved; however, topical retinoic acid1 and atropine8 have been successfully used to treat them. Article Submissions Clinicians, local and regional societies, and residents and fellows in dermatology are invited to submit quiz cases to this section. Cases should follow the established pattern and be submitted double-spaced. Photomicrographs and illustrations must be clear and submitted as 3 positive color transparencies and as 3 color prints. Material should be accompanied by the required copyright transfer statement, as noted in "Instructions for Authors." Material for this section should be submitted to Michael E. Ming, MD, Department of Dermatology, University of Pennsylvania Health System, 2 Maloney Bldg, 3600 Spruce St, Philadelphia, PA 19104-4283. Reprints are not available from the authors. References 1. Gómez MIPérez BAzaña JMNúñez MLedo A Eruptive syringoma: treatment with topical tretinoin Dermatology. 1994;189105- 106PubMedGoogle Scholar 2. Butterworth TStean LBeerman HGraywood M Syringoma and mongolism Arch Dermatol. 1964;90483- 487Google ScholarCrossref 3. Friedman SJButler DF Syringoma presenting as milia J Am Acad Dermatol. 1987;16310- 314PubMedGoogle ScholarCrossref 4. Schepis CTorre VSiragusa M et al. Eruptive syringomas with calcium deposits in a young woman with Down's syndrome Dermatology. 2001;203345- 347PubMedGoogle ScholarCrossref 5. Urban CDCannon JRCole RD Eruptive syringomas in Down's syndrome Arch Dermatol. 1981;117374- 375PubMedGoogle ScholarCrossref 6. Carey ABPark HKBurk WA Multiple eruptive syringomas associated with Down's syndrome J Am Acad Dermatol. 1988;19759- 760PubMedGoogle ScholarCrossref 7. Soler-Carrillo JEstrach TMascaró JM Eruptive syringoma: 27 new cases and review of the literature J Eur Acad Dermatol Venereol. 2001;15242- 246PubMedGoogle ScholarCrossref 8. Sánchez TSDaunden ECasas APGarcia-Diez A Eruptive pruritic syringomas: treatment with topical atropine J Am Acad Dermatol. 2001;44148- 149PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Papular Eruption on a Patient With Down Syndrome—Diagnosis

Archives of Dermatology , Volume 140 (9) – Sep 1, 2004

Papular Eruption on a Patient With Down Syndrome—Diagnosis

Abstract

Diagnosis: Multiple syringomas. Microscopic findings The skin biopsy specimen showed dermal fibrosis. In the upper dermis, there was a proliferation of eccrine-type ducts, many of which were dilated and contained eosinophilic material. The ducts were lined by 2 or more layers of cuboidal cells, often with luminal clear cell change. There was also a mild perivascular lymphohistiocytic inflammatory cell response. The epidermis displayed mild overlying hyperkeratosis. Discussion Syringomas are...
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Publisher
American Medical Association
Copyright
Copyright © 2004 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.140.9.1161-b
Publisher site
See Article on Publisher Site

Abstract

Diagnosis: Multiple syringomas. Microscopic findings The skin biopsy specimen showed dermal fibrosis. In the upper dermis, there was a proliferation of eccrine-type ducts, many of which were dilated and contained eosinophilic material. The ducts were lined by 2 or more layers of cuboidal cells, often with luminal clear cell change. There was also a mild perivascular lymphohistiocytic inflammatory cell response. The epidermis displayed mild overlying hyperkeratosis. Discussion Syringomas are benign tumors that originate in the intraepidermal eccrine ducts.1 Clinically, they are asymptomatic, small, yellow or red-brown, firm papules. They are seen more frequently in females and commonly occur in the periorbital area, especially in patients with Down syndrome. Other sites that are affected less commonly include the neck, chest, and abdomen. The incidence of syringomas in patients with Down syndrome has been reported to be approximately 30 times greater than that in the general population.2 Friedman and Butler3 classified syringomas into the following 4 groups according to their clinical features and associations: (1) a localized form, (2) a generalized form, including multiple and eruptive variants, (3) a form associated with Down syndrome, and (4) a familial form. Although the history of onset was not available in our patient, his case may represent an example of eruptive syringomas. Eruptive syringomas typically occur on the anterior body surfaces as successive crops.1 There have been previous case reports of eruptive syringomas occurring in patients with Down syndrome,4-6 but in contrast with the periorbital syringomas, there is no evidence that the incidence of eruptive syringomas is greater in patients with Down syndrome than in the general population. Clinically, syringomas can resemble other papular eruptions, and diagnosis is often only made through histologic examination, which typically shows multiple small ducts lined by rows of flattened epithelial cells,7 with epithelial strands within the dermis. Syringomas are asymptomatic but pose a cosmetic problem. They can be treated with electrocoagulation or cryotherapy. Eruptive syringomas are difficult to treat mainly because of the sheer number of lesions that are involved; however, topical retinoic acid1 and atropine8 have been successfully used to treat them. Article Submissions Clinicians, local and regional societies, and residents and fellows in dermatology are invited to submit quiz cases to this section. Cases should follow the established pattern and be submitted double-spaced. Photomicrographs and illustrations must be clear and submitted as 3 positive color transparencies and as 3 color prints. Material should be accompanied by the required copyright transfer statement, as noted in "Instructions for Authors." Material for this section should be submitted to Michael E. Ming, MD, Department of Dermatology, University of Pennsylvania Health System, 2 Maloney Bldg, 3600 Spruce St, Philadelphia, PA 19104-4283. Reprints are not available from the authors. References 1. Gómez MIPérez BAzaña JMNúñez MLedo A Eruptive syringoma: treatment with topical tretinoin Dermatology. 1994;189105- 106PubMedGoogle Scholar 2. Butterworth TStean LBeerman HGraywood M Syringoma and mongolism Arch Dermatol. 1964;90483- 487Google ScholarCrossref 3. Friedman SJButler DF Syringoma presenting as milia J Am Acad Dermatol. 1987;16310- 314PubMedGoogle ScholarCrossref 4. Schepis CTorre VSiragusa M et al. Eruptive syringomas with calcium deposits in a young woman with Down's syndrome Dermatology. 2001;203345- 347PubMedGoogle ScholarCrossref 5. Urban CDCannon JRCole RD Eruptive syringomas in Down's syndrome Arch Dermatol. 1981;117374- 375PubMedGoogle ScholarCrossref 6. Carey ABPark HKBurk WA Multiple eruptive syringomas associated with Down's syndrome J Am Acad Dermatol. 1988;19759- 760PubMedGoogle ScholarCrossref 7. Soler-Carrillo JEstrach TMascaró JM Eruptive syringoma: 27 new cases and review of the literature J Eur Acad Dermatol Venereol. 2001;15242- 246PubMedGoogle ScholarCrossref 8. Sánchez TSDaunden ECasas APGarcia-Diez A Eruptive pruritic syringomas: treatment with topical atropine J Am Acad Dermatol. 2001;44148- 149PubMedGoogle ScholarCrossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Sep 1, 2004

Keywords: down syndrome,exanthema,papule,syringoma

References