Ann Dermatol.  2012 May;24(2):206-208.

Unilateral Nevoid Telangiectasia Associated with Ipsilateral Melorheostosis

• Kim J
• Cho SB
• Cho S
• Bang D

Abstract

Melorheostosis is a rare disorder characterized by irregular, flowing hyperostosis in long bones, commonly described on radiographs as wax flowing down a candle. In addition to bony sclerosis, cutaneous manifestations overlying the involved bones have been reported including linear scleroderma, neurofibromatosis, and vascular and lymphatic malformations. Unilateral nevoid telangiectasia (UNT) is a rare primarily cutaneous condition characterized by linearly arranged small dilated blood vessels in dermatomal or Blaschkoid patterns on the skin. Here, we present the case of a nine-year-old Korean male with UNT associated with ipsilateral melorheostosis.

Keyword

Melorheostosis; Nevoid; Telangiectasia; Unilateral

MeSH Terms

Blood Vessels
Humans
Hyperostosis
Male
Melorheostosis
Neurofibromatoses
Scleroderma, Localized
Sclerosis
Skin
Telangiectasis

Figure

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  Fig. 1 (A) A nine-year old boy with dermatomal arranged linear telangiectasias on the left chest wall and left arm. (B) Magnification of the chest lesion shows linearly arranged erythematous telangiectasias (white arrowheads) on the left chest.

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  Fig. 2 (A, B) Roentgenogaraphic examination of the left arm shows diffuse bony sclerotic changes on the distal humerus, ulna, carpal, and second to fourth metacarpal and phalangeal bones. (C) Bone scintigraphy shows abnormal uptake in the left humerus, radius, and hand bones.

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  Fig. 3 Histopathology shows numerous dilated thin-walled, small-sized blood vessels without endothelial proliferation and vasculitis in the papillary dermis (H&E, ×100).

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