Criteria of Landy and Donnai for the Diagnosis of Sporadic Incontinentia Pigmenti

 

Patient selection: no first degree female relative with incontinentia pigmenti

 

Major criteria:

(1) typical neonatal rash (erythema, vesicles, eosinophilia)

(2) typical hyperpigmentation (mainly trunk, Blaschko's lines, fading in adolescence)

(3) skin lesions that are linear, atrophic, hairless

 

Minor criteria:

(1) anomalous dentition (delayed eruption, missing or abnormal teeth)

(2) wooly hair and abnormal nails

(3) retinal disease

(4) alopecia

 

The diagnosis of IP requires at least 1 major criteria.

 

The absence of all minor criteria makes the diagnosis uncertain.