Neurologic Manifestations of Incontinentia Pigmenti Differential Diagnoses

Updated: Dec 11, 2018
  • Author: Celia H Chang, MD; Chief Editor: Amy Kao, MD  more...
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Diagnostic Considerations

Differential for multiorgan involvement

  • MIDAS syndrome - X-linked dominant dermato-ocular syndrome with similar skin changes but different eye changes

  • Hypomelanosis of Ito (incontinentia pigmenti achromians) - Swirls or streaks of hypopigmentation and depigmentation; not inherited; no stage 1 or 2; 33-50% with multisystem involvement—eye, skeletal, neurological abnormalities; Xp11

  • Focal dermal hypoplasia of Goltz - X-linked dominant disorder with similar findings, Xp22

  • Naegeli syndrome - Autosomal dominant dermato-ocular syndrome with symptoms starting at age 2, mostly on the hands and feet, and similar ocular changes

  • X-linked chondrodysplasia punctata - Has more skeletal dysplasia, congenital cataracts, and alopecia, as well as follicular pitting, which is not seen in incontinentia pigmenti

  • Dyskeratosis congenita - Skin findings similar to stages 3 and 4 but no inflammatory changes. It is also associated with progressive pancytopenia with bone marrow failure being a frequent cause of death. This has been mapped to the DKC1 gene, which is proximal to the factor VIII gene on the X chromosome.

Differential for stage 1 lesions

  • Arthropod reaction

  • Bullous congenital ichthyosiform erythroderma (epidermolytic hyperkeratosis)

  • Bullous dermatosis

  • Bullous impetigo

  • Bullous mastocytosis

  • Bullous pemphigoid

  • Drug reaction

  • Epidermolysis bullosa, especially Dowling Meara type

  • Eosinophilic cellulitis (Wells syndrome)

  • Eosinophilic pustular folliculitis (Ofuji disease)

  • Erythema toxicum neonatorum

  • Hypereosinophilic syndrome

  • Herpes simplex and varicella zoster

  • Milker nodule

  • Pemphigus

  • Polycythemia rubra vera

  • Porokeratosis of Mibelli (benzyl hydrochlorothiazide induced)

  • Scabies

  • Subcorneal pustular dermatosis (Sneddon Wilkinson disease)

Differential for stage 2 lesions

  • Linear epidermal nevus

  • Lichen striatus

Differential for stage 3 lesions

  • Linear and whorled nevoid hypermelanosis

  • Dermatopathia pigmentosa reticularis