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Congenital erosive and vesicular dermatosis
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Congenital erosive and vesicular dermatosis

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Contributors: Mari M. Batta DO, Lowell A. Goldsmith MD, MPH
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Synopsis

Congenital erosive and vesicular dermatosis is an extremely rare congenital disorder of unknown etiology. All cases thus far have been sporadic and with a slight male predominance. This entity presents at birth, typically in the premature neonate, with extensive vesicles, superficial erosions, and crusting, often covering 75% of the cutaneous surface. The trunk and extremities are most severely involved with relative sparing of the face, palms, and soles. New lesions generally do not develop after the neonatal period, and existing lesions heal within 10 days to 3 months, leaving behind characteristic supple, reticulated scars. Of note, joints are mobile and functional despite the extensive scarring.

A significant associated clinical feature of affected children is heat intolerance with an increased risk of hyperthermia. Scarred areas are hypohidrotic while uninvolved skin has compensatory hyperhidrosis. Some children also continue to have skin fragility in areas of scarring and in sites of trauma, which may lead to recurrent blisters and erosions; however, these lesions heal without scarring.

Associated findings are variable, with reported cases involving:
  • Tongue – atrophy of lingual papillae; however, buccal mucosa and dentition are normal
  • Scalp – sparse hair with resultant cicatricial alopecia, which may improve with time
  • Nail – anonychia or hypoplasia of some or all nails
  • Ocular – scarring of the cornea, fundus, or nasolacrimal duct
  • Neurologic – cerebral palsy, hemiparesis, hearing loss, intellectual disability, mild developmental delays; it is unclear whether these are complications of prematurity or part of the cutaneous disease
  • Other associated findings observed include erosive lichen planus, digital tip gangrene, aplasia cutis congenita, and hydronephrosis
  • There may be a history of maternal chorioamnionitis
With time, the reticulated scarring becomes less conspicuous. Most children have normal cognitive and motor development and have unremarkable skeletal films and ophthalmologic examinations. The prognosis is generally good.

Codes

ICD10CM:
Q82.8 – Other specified congenital malformations of skin

SNOMEDCT:
7392002 – Dermatitis of the newborn

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Last Updated: 10/12/2018
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Congenital erosive and vesicular dermatosis
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Congenital erosive and vesicular dermatosis : Crust, Heat intolerance, Present at birth, Skin erosion, Vesicle, Widespread
Clinical image of Congenital erosive and vesicular dermatosis
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