Congenital erosive and vesicular dermatosis
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
Q82.8 – Other specified congenital malformations of skin
7392002 – Dermatitis of the newborn
- Aplasia cutis congenita – Sharply marginated ulcerations or bullae with erythematous granulating base on scalp that resolve with sharply demarcated scars.
- Focal dermal hypoplasia (Goltz syndrome) – Atrophic linear lesions; associated with subcutaneous fat herniations. Lesion number and severity increase in childhood.
- Herpes group infections (neonatal herpes simplex virus, neonatal varicella) – Rule out with herpes simplex and varicella zoster virus lesion cultures.
- Incontinentia pigmenti – Inheritance is X-linked dominant; characterized by 4 phases: vesicular, verrucous, hyperpigmented, then hypopigmented.
- Mechanobullous disorders (eg, epidermolysis bullosa [EB] simplex, EB Dowling-Meara, junctional EB, dystrophic EB) – Rule out with skin biopsy to examine histopathology, immunofluorescence, and ultrastructural features.
- Staphylococcal scalded skin syndrome – Fever precedes tense blisters, which then desquamate.
- Transient bullous dermolysis of the newborn – Heals without scarring.
- Pemphigoid gestationis – Up to 10% of neonates born to mothers with pemphigoid gestationis will have bullous lesions.