J Korean Med Sci.  2012 Feb;27(2):225-227. 10.3346/jkms.2012.27.2.225.

Hereditary Sclerosing Poikiloderma

• Lee HJ
• Shin DH
• Choi JS
• Kim KH

• KMID: 1120165
• DOI: http://doi.org/10.3346/jkms.2012.27.2.225

Abstract

Hereditary sclerosing poikiloderma (HSP) is a very rare disease. The clinical features are principally widespread poikiloderma and linear hyperkeratotic and sclerotic bands. We report an 18-yr-old male who presented reticular hyperpigmented lesions on the trunk and extremities since 2-yr-old. Also, linear sclerosing bands appeared on both antecubital and popliteal fossae after yr. Histopathologic finding showed dense sclerotic collagen fibers with telangiectasia in the upper dermis and fragmentations of damaged elastic fibers in the elastic stain, consistent with HSP. We report the first Korean case of HSP.

Keyword

Hereditary Sclerosing Poikiloderma; Korean

MeSH Terms

Abnormalities, Multiple
Adolescent
Elastic Tissue/pathology
Fingers/abnormalities
Humans
Hyperpigmentation/pathology
Male
Micrognathism/pathology
Rothmund-Thomson Syndrome/*diagnosis/pathology
Sclerosis/pathology
Skin Diseases/diagnosis/pathology

Figure

• 

  Fig. 1 Gross findings. (A) Micrognathia, (B) Clubbing fingers, (C) Slim extremities compared with trunk.

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  Fig. 2 Hyperpigmented, telangiectatic, and atrophic changes on the skin. (A) Trunk. (B) both axillae.

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  Fig. 3 Linear brownish hyperkeratotic and sclerosing band on the skin. (A) Popliteal fossa. (B) Antecubital fossa.

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  Fig. 4 Histopathological findings of the skin. (A) Increased melanin pigments in the basal layer and sclerotic changes and telangiectasia in the upper dermis (H&E, × 40). (B) Fragmentations of elastic fibers in the dermis (Elastic stain, × 200).

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