Segmental Pigmentation Disorder

M. Hogeling; I.J. Frieden


The British Journal of Dermatology. 2010;162(6):1337-1341. 

In This Article


Clinical characteristics including sex, age at onset of SegPD, family history, and type and distribution of pigmentation are summarized in Table 1. When patches of SegPD involved the extremities, buttocks or neck, pigment often extended onto the torso. In six patients pigmentary changes involved only the face. Most children in the study had Fitzpatrick skin types II or III. There was no obvious association of SegPD with any inflammatory skin conditions. Five patients had melanocytic naevi or small hyperpigmented patches within their SegPD; however, these were sparse in number and not sufficient to be diagnosed as speckled lentiginous naevi (naevus spilus). In four of these, melanocytic naevi or hyperpigmented macules were noted within a hyperpigmented patch whereas in one, they were superimposed on a hypopigmented patch of SegPD. Fourteen patients had additional birthmarks such as infantile haemangiomas (three), small isolated café-au-lait macules (four), small congenital melanocytic naevi (two), dermal melanocytosis (two), naevus sebaceous (one), lumbosacral tuft of hair (one) and a sacral pit (one). Nine children had more than one distinct spot of SegPD; however, at times it was difficult to tell if the pigmentation was confluent. Three patients had Blaschkoid pigmentation at a location separate from their SegPD. In 36 of 39 patients (92%) no extracutaneous abnormalities were noted. Three patients had extracutaneous abnormalities: one with segmental hypopigmentation had an atrial septal defect (ASD), one with segmental hyperpigmentation had strabismus with retinal hypopigmentation, and one with hyperpigmentation had a bronchogenic cyst. None had developmental delay, seizures or other neurological abnormalities.


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