Segmental Pigmentation Disorder

M. Hogeling; I.J. Frieden

Disclosures

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

In This Article

Abstract and Introduction

Abstract

Background There is little published information about segmental hypo- and hyperpigmentation pigmentation disorder (SegPD) although it is a relatively common problem in paediatric dermatology.
Objectives To define the spectrum of disease, clinical presentation and associations in cases of SegPD and to clarify further the terminology in defining patterned hypo- and hyperpigmentation in children.
Methods This was a retrospective review of cases in an academic paediatric dermatology practice. Thirty-nine patients referred for dermatological evaluation were diagnosed with SegPD. Demographic and clinical features, and distribution and frequency of extracutaneous abnormalities were measured.
Results Twenty female and 19 male patients were included in the study; 33 out of the 39 were referred specifically for a pigmentation abnormality. The mean age at onset was 3·4 months (median age 0·25 months). Family history was positive in two patients. Most (30/39; 77%) had segmental hyperpigmentation whereas nine of 39 (23%) had hypopigmentation. Patches were more often delineated at the ventral midline (32/39) than on the dorsal midline (7/39). The distribution of lesions was as follows: areas of the torso were most often affected (77%) and when the face, neck, arms and legs were affected pigmentation usually extended onto the torso; six patients had SegPD localized to the face. Only three of the 39 patients had extracutaneous abnormalities – atrial septal defect, strabismus with retinal hypopigmentation and a bronchogenic cyst – but the relationship to SegPD was uncertain and none had neurological abnormalities.
Conclusions SegPD is a relatively common pigmentary anomaly and most affected individuals are otherwise healthy. We propose reviving the term 'segmental pigmentation disorder' coined by Metzker and colleagues to describe children with segmental and block-like hypo-/hyperpigmentation with midline demarcation.

Introduction

In 1983, Metzker et al.[1] introduced the term 'segmental pigmentation disorder' (SegPD) when reporting on a case series of 30 children accrued over a 4-year period. These children had a hypo- or hyperpigmented patch, with 'dermatomal' distribution most often sharply delineated along the midline but less well-delineated at its lateral aspect. The abdomen, chest and back were most often affected, and the condition was noted to be more common in children with darker skin colour. Most cases were noted during infancy and in seven of 30 a gradual decrease in colour was observed during follow-up. No association with systemic disorders, such as neurological involvement, was found. Histopathology in one patient revealed basilar hyperpigmentation.[1]

Many other terms have been used to describe patients with SegPD. These include 'segmental pigmentation anomaly', 'giant café-au-lait macule', 'segmental naevus depigmentosus', 'pigmentary mosaicism', 'patterned dyspigmentation', 'segmental hypermelanosis' and 'naevus depigmentosus'.[2–5] These multiple terms add to nosological confusion and to a lack of clarity regarding clinical features and potential associations. To define the spectrum of this disorder more accurately, we retrospectively reviewed the records of 39 children with SegPD.

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