Hyperpigmented example of segmental pigmentation disorder in a child.

Hypopigmented example of segmental pigmentation disorder in an adult. Note the sharper border medially than laterally.

SEGMENTAL PIGMENTATION DISORDER

Segmental Pigmenation Disorder (SPD) is a term used to describe children with segmental and block-like hypo or hyperpigmentation with midline demarcation.

• In one study, the mean age of onset was 3-4 months (median age range 0-25 months).
• The finding is benign and usually isolated--without systemic association.
  
• Sometimes there is clinical overlap with nevus depigmentosus and segmental vitiligo.
  

Clinical

Onset is at birth or soon thereafter. About 3/4 of cases of SPD are hyperpigmented and about 1/4 are hypopigmented. The most common location is the torso, anteriorly. Lesions are segmental, but not dermatomal and do not follow the lines of Blaschko. Somatic mosaicism is thus postulated as the cause. Lesions may cross the midline by one or two centimeters. The lateral border is often less distinct than the medial.

Associations

The risk of systemic association is quite low and in fact, no reproducible pattern has as of yet been identified.

Treatment

SPD usually fades over time, but may persist into adulthood. For hypopigmented lesions, any of the modalities employed for nevus depigmentosus or segmental vitiligo may be tried, e.g. the 308-nm laser.

RegionalDerm

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