A skin disturbance usually localized to the neck; less frequently to upper extremities, face, lower extremities, trunk. Asymptomatic or slight pruritus. Slight erythematous keratotic papules (2 to 5 mm in diameter), with small central scaling arranged in diffuse, serpiginous, or circular pattern; satellite lesions usually appear. Frequently, symmetric distribution on both sides of neck, both forearms. It may also be associated with van der Hoeve’s syndrome, Grönblad-Strandberg syndrome, Marfan’s syndrome, Rothmund-Thomson syndrome, Ehlers-Danlos syndrome, and Down’s syndrome.
Prevalent in males; onset before 30 years of age in 90% of cases (youngest patient 5 years old; oldest patient, 84); all races. Etiology unknown. Familial incidence has been reported. May be autosomal dominant.
- W. Lutz:
Keratosis follicularis serpiginosa.
Dermatologica, Basel, 1953, 106: 318-320.
- G. Miescher:
Ëlastoma intrapapillare perforans verruciforme.
Dermatologica, Basel, 1955, 110: 254-266.