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Skin Tags Vaginal OpeningImages Incontinentia Pigmenti

Figure 1.189. A close-up of die right lower extremity of an infant with incontinentia pigmenti. Note die presence of vesicles and the typical pattern of swirled hyperpigmentation of the skin that has been compared to that of a marble cake. This is present mainly on the extremities and trunk, and increases in intensity until the 2nd year of life. It may persist many years, then gradually fades.

Figure 1.188. In a later phase of incontinentia pigmenti, between 6 to 12 months of age, red-brown, hyper-pigmented lesions appear in a symmetric distribution on die arms and legs while odier lesions have improved. Note die bizarre distribution of die lesions and die pigmentation. The pigmented zones have very irregular patterns and tend to follow die padi of Blaschko's lines. They have a roughly V-shaped configuration over die back, a wavy S-shaped distribution over die anterior trunk, and a longitudinal orientation over die limbs. These lesions tend to persist.

Figure 1.189. A close-up of die right lower extremity of an infant with incontinentia pigmenti. Note die presence of vesicles and the typical pattern of swirled hyperpigmentation of the skin that has been compared to that of a marble cake. This is present mainly on the extremities and trunk, and increases in intensity until the 2nd year of life. It may persist many years, then gradually fades.

Figure 1.190. A close-up of the lower trunk, buttocks, and extremities showing the pigmentary changes which tend to follow the padi of Blaschko's lines.

Figure 1.191. In incontinentia pigmenti achromians (hypome-lanosis of Ito), a neurocutaneous syndrome, the distribution of hypopigmentation is similar to that of the hyperpigmented areas seen in incontinentia pigmenti. Note that there is a pattern of swirled hypo-pigmentation which looks like a photographic negative of the hyper-pigmented streaks seen in inconti-nentia pigmenti. This disorder is associated with seizures and mental retardation. The whorls may appear without the prior development of vesicles or bullae.

Figure 1.192. The back of the same infant as in Figure 1.191 showing the distribution of the hypopig-mented lesions, again demonstrating these bizarre patterns which look like a photographic negative of the hyperpigmented streaks seen in incontinentia pigmenti and which tend to follow Blaschko's lines. In a few patients, the lesions may be patchy and confined to relatively limited areas of the body. In most cases, however, the hypopigmented areas are extensive, often bilateral, and appear to be more pronounced on the ventral surface of the trunk and the flexor surface of the limbs.

Figure 1.190. A close-up of the lower trunk, buttocks, and extremities showing the pigmentary changes which tend to follow the padi of Blaschko's lines.

Blaschko Lines Newborn
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