Landy and Donnai listed criteria for the diagnosis of incontinentia pigmenti (IP, Bloch-Sulzberger syndrome) in a patient without a first degree female relative with IP. The authors are from St. Mary's Hospital in Manchester, England.


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.


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