Incontinentia Pigmenti Differential Diagnoses

Updated: Mar 05, 2019
  • Author: Kara N Shah, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
  • Print

Diagnostic Considerations

For stage 1 (vesicular), also consider the following:

  • Bullous impetigo
  • Herpes simplex
  • Varicella (herpes) zoster
  • Epidermolysis bullosa
  • Bullous mastocytosis
  • Bullous congenital ichthyosiform erythroderma (epidermolytic hyperkeratosis)
  • Congenital bullous pemphigoid
  • Linear IgA bullous disease of childhood
  • Langerhans cell histiocytosis
  • Erythema toxicum
  • Miliaria
  • Acropustulosis of infancy
  • Arthropod assault

For stage 2 (verrucous), also consider the following:

  • Linear epidermal nevus
  • Lichen striatus
  • X-linked dominant chondrodysplasia punctata
  • Verruca vulgaris

For stage 3 (pigmented), also consider the following:

  • Linear and whorled nevoid hypermelanosis
  • Pigmentary mosaicism
  • Dermatopathia pigmentosa reticularis
  • Naegeli-Franceschetti-Jadassohn syndrome
  • X-linked dominant chondrodysplasia punctata

For stage 4 (depigmented), also consider the following:

  • Hypomelanosis of Ito (incontinentia pigmenti achromians)
  • Focal dermal hypoplasia syndrome (Goltz syndrome)
  • X-linked dominant chondrodysplasia punctata

Differential Diagnoses