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Incontinentia Pigmenti

definition

Incontinentia Pigmenti is a multisystem disorder predominantly affecting females and characterized by skin changes in three stages – vesiculobullous, verrucous and hyperpigmented. Not all stages occur necessarily, though. Sixty percent of the patients display abnormalities of the ectodermal tissue: Nail dystrophy, dental abnormalities, hair anomalies are common. The incidence of ocular abnormalities is high and may approach 40%. The disorder is thought to be due to an X-linked dominant gene with lethality in affected males.

synonyms

Incontinentia Pigmenti, Bloch-Sulzberger Syndrome

UMLS

Bloch Sulzberger Syndrome, Bloch-Siemans syndrome, Bloch-Sulzberger Syndrome, Incont pig of Bloch-Sulzberger, Incontinentia Pigmenti, Incontinentia pigmenti of Bloch-Sulzberger, Incontinentia pigmenti syndrome, Syndrome, Bloch-Sulzberger

images

27 images found for this diagnose localisation: legs, diagnosis: Incontinentia Pigmenti localisation: back of the feet, diagnosis: Incontinentia Pigmenti localisation: back of the hands, diagnosis: Incontinentia Pigmenti localisation: lower leg, diagnosis: Incontinentia Pigmenti localisation: axilla, diagnosis: Incontinentia Pigmenti localisation: legs, diagnosis: Incontinentia Pigmenti localisation: finger, diagnosis: Incontinentia Pigmenti localisation: back of the feet, diagnosis: Incontinentia Pigmenti localisation: buttocks, legs, diagnosis: Incontinentia Pigmenti localisation: total body view, diagnosis: Incontinentia Pigmenti localisation: back of neck, upper back, diagnosis: Incontinentia Pigmenti localisation: trunk, diagnosis: Incontinentia Pigmenti localisation: back of the feet, diagnosis: Incontinentia Pigmenti localisation: legs, diagnosis: Incontinentia Pigmenti localisation: neck, axilla, diagnosis: Incontinentia Pigmenti localisation: legs, upper leg, diagnosis: Incontinentia Pigmenti localisation: trunk, diagnosis: Incontinentia Pigmenti localisation: trunk, diagnosis: Incontinentia Pigmenti localisation: neck, trunk, arms, legs, diagnosis: Incontinentia Pigmenti diagnosis: Incontinentia Pigmenti diagnosis: Incontinentia Pigmenti diagnosis: Incontinentia Pigmenti diagnosis: Incontinentia Pigmenti diagnosis: Incontinentia Pigmenti diagnosis: Incontinentia Pigmenti diagnosis: Incontinentia Pigmenti diagnosis: Incontinentia Pigmenti