Incontinentia pigmenti – a description of three cases

Incontinentia pigmenti (IP) or the Bloch-Sulzberger syndrome

is a rare genetic dermatosis that occurs in females, which is

classified as an ectodermal dysplasia. Papulovesicular eruptions

appear on the skin in the first days of a patient’s life and then,

with the lapse of time they become verrucous and

hyperpigmentation lesions evolving into incontinentia pigmenti

achromians. Apart from such characteristic cutaneous lesions,

female patients suffering from incontinentia pigmenti may

also present other cutaneous symptoms, as well as motor

system and ophthalmological dysfunctions. In 54 per cent of

patients dental symptoms are observed, those including mainly

hypodontia, pegged teeth and delayed tooth eruption. Aim.

The aim of this study was to describe three cases of female

patients diagnosed with the IP syndrome who were being treated in the Department of Orthodontics of the Medical

University of Warsaw. Case descriptions. Descriptions of

three cases of female patients with diagnosed IP were

presented. The patients had cutaneous lesions typical for IP.

Medical interviews and physical examinations were conducted

with them all; radiographs were performed and diagnostic

casts were made. Class III malocclusions were diagnosed as

well as dental abnormalities characteristic for IP (hypodontia,

malformation of teeth). Conclusions. An early diagnosis allows

for a child to receive a multispecialist medical and dental care.

An interdisciplinary approach makes it possible to reach,

functionally and esthetically, satisfactory results in the

treatment and maintain patients’ teeth in good health until

the period of adultness thus ensuring that the physical and

mental state of female patients affected by the syndrome

improves. (Laskowska M, Dąbkowska M, Szpinda-

Barczyńska A, Witanowska J, Zadurska M. Incontinentia

pigmenti – a description of three cases. Orthod Forum

2018; 14: 321-32).