Abstract

Cutis marmorata telangiectatica congenita is characterized by the presence of a bluish-purple reticulated cutaneous vascular network on the skin intermixed with telangiectasia and occasionally prominent veins at birth. Areas of the skin within the reticulated cutaneous vascular network may be normal, erythematous, atrophic, and, at times, ulcerated. Areas of ulcerations and focal cutaneous and subcutaneous atrophy occasionally occur resulting in body asymmetry. On the other hand, cutaneous and subcutaneous atrophy, extensive and severe enough leading to hemiatrophy, of the entire limb is rare. A search of the English literature revealed only eight documented cases to which we are adding two more cases.

Highlights

  • Hemiatrophy refers to wasting or loss of tissue on one side of the body. e condition may result from congenital hemiatrophy due to intrauterine insult, contralateral cerebral insult, amyotrophic lateral sclerosis, syringobulbia, ipsilateral peripheral neuropathy, trauma to the muscles on one side of the body, prolonged immobilization on one side of the body, congenital or acquired lipodystrophy, unilateral morphea, Duchenne muscular dystrophy, facioscapulohumeral muscular dystrophy, or cutis marmorata telangiectatica congenita [1,2,3,4]

  • Cutis marmorata telangiectatica congenita is characterized by the presence of a reticulated cutaneous vascular network of bluish-purple color on the skin intermixed with telangiectasia and occasionally prominent veins at birth [7, 8]

  • Clinical improvement of the cutaneous lesions occurs within the first two years of life, [22] the lesions occasionally persist [22]. e associated hemiatrophy, on the other hand, tends to persist. e present cases suggest that hemiatrophy associated with cutis marmorata telangiectatica congenita is more common than being presently appreciated. Physicians should bear this in mind when encountering a child with cutis marmorata telangiectatica congenita

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Summary

Introduction

Hemiatrophy refers to wasting or loss of tissue on one side of the body. e condition may result from congenital hemiatrophy due to intrauterine insult, contralateral cerebral insult, amyotrophic lateral sclerosis, syringobulbia, ipsilateral peripheral neuropathy, trauma to the muscles on one side of the body, prolonged immobilization on one side of the body (disuse atrophy), congenital or acquired lipodystrophy, unilateral morphea, Duchenne muscular dystrophy, facioscapulohumeral muscular dystrophy, or cutis marmorata telangiectatica congenita [1,2,3,4]. Hemiatrophy refers to wasting or loss of tissue on one side of the body. E condition may result from congenital hemiatrophy due to intrauterine insult, contralateral cerebral insult, amyotrophic lateral sclerosis, syringobulbia, ipsilateral peripheral neuropathy, trauma to the muscles on one side of the body, prolonged immobilization on one side of the body (disuse atrophy), congenital or acquired lipodystrophy, unilateral morphea, Duchenne muscular dystrophy, facioscapulohumeral muscular dystrophy, or cutis marmorata telangiectatica congenita [1,2,3,4]. Documented cutis marmorata telangiectatica congenita associated with hemiatrophy is rare. A PubMed search of the English literature using the key terms “cutis marmorata telangiectatica congenita” AND “hemiatrophy” revealed only eight cases, to which we are adding two cases to alert readers of such association

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