The ocular and systemic changes in incontinentia pigmenti are reviewed here and a case is reported with fundus changes which have not been described previously. Incontinentia pigmenti (IP) is characterised by ectodermal defects, with occasional associated mesodermal abnormalities. The typical skin lesions are blue-grey to chocolate-brown arranged along the naevus lines of Blaschko. These occur ...

Incontinentia Pigmenti, also known as Bloch-Sulzberger syndrome, is a rare X-linked dominant disorder considered to be lethal in males. The syndrome usually presents at birth or shortly thereafter and the skin rash evolves in the classic four stages of vesicular, verrucous, hyperpigmented, and hypopigmented lesions following a blaschko linear distribution. Here we present an unusual case of Inc...

The course and histologic skin signs are described at the stage of inflammation in 2 cases of incontinentia pigmenti (IP) or the Bloch-Sulzberger syndrome. In the authors' opinion, the pigmentary stage of skin lesion in IP does not replace the warty stage, but accompanies it or the inflammatory one as blebs disappear. In IP, the latter contain eosinophils; there is plasma and cell infiltration ...

Incontinentia pigmenti (IP, Bloch-Sulzberger syndrome) is a very rare genodermatosis characterized by typical skin lesions accompanied by dental, central nervous system, bone and ocular abnormalities. Incontinentia pigmenti is usually observed among women, as this X-linked dominantly inherited disorder is lethal in males. The hallmark feature of IP is cutaneous eruption along the lines of Blasc...