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September 2007

Verrucous Papules and Plaques in a Pediatric Patient—Diagnosis

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Arch Dermatol. 2007;143(9):1201-1206. doi:10.1001/archderm.143.9.1201-f

Histopathologic examination of the left arm lesion showed a hyperplastic epidermis with vascular ectasia and a mononuclear cell infiltrate in the dermis. The findings were consistent with verrucous acanthoma (papilloma). The patient was referred to the plastic surgery department to discuss treatment options for the facial papillomas.

Approximately 150 cases of Costello syndrome have been identified since the condition was described in 1977 by Costello1 and later named by Der Kaloustian et al2 in 1991. The major features include growth and mental retardation, with a sociable personality; coarse facies (macrocephaly, thick lips, big tongue, depressed nasal bridge, and low-set ears with thick lobes); short neck; musculoskeletal abnormalities (tight Achilles tendon, hyperextensible fingers, and limited joint mobility), cardiac defects (hypertrophic cardiomyopathy, cardiac malformations, and supraventricular arrhythmias); increased risk for malignant tumors (rhabdomyosarcoma, ganglioneuroblastoma, neuroblastoma, and bladder carcinoma); and distinctive cutaneous findings.3 Cutaneous papillomas are a unique skin manifestation of Costello syndrome, although they are present in only about50% of patients.4 Papillomas occur on the face, anus, axillae, elbows, knees, vocal cords, and abdomen and usually develop between the 2nd and 15th years of life. The pathogenesis is unknown, but mechanical stress and human papillomavirus have been implicated.5 Clinically, the papillomas resemble verrucae, but histologically, verrucae can be distinguished by the presence of koilocytes, vertical columns of parakeratosis, and foci of clumped keratohyaline granules. In addition to verrucae, the differential diagnosis includes epidermal nevus, verrucous carcinoma, keratoacanthoma, actinic keratosis, squamous cell carcinoma, lipoid proteinosis, deep fungal infections, and epidermodysplasia verruciformis. Treatment options are limited to destruction and excision of the lesions, both of which may result in profound scarring. Patients with Costello syndrome also invariably have loose, redundant skin, especially of the neck, hands, and feet, which results in the appearance of deep creases on the palms and soles, as seen in our patient. Acanthosis nigricans, pigmented acral nevi, vascular birthmarks, hyperkeratosis, hyperpigmentation of the skin, thin deep-set nails, thick eyebrows, and sparse curly scalp hair also frequently occur.4

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