Congenital hypopigmented macules: A case of systematized nevus depigmentosus

A 3-month-old male infant presented with multiple asymptomatic hypopigmented patches present since birth, which were stable and non-progressive. Born full term through an uneventful vaginal delivery, the child had no significant prenatal or postnatal complications, and there was no family history of similar skin lesions. On examination, multiple well-demarcated hypopigmented patches with irregular margins were noted on the lower abdomen, groin, inner thighs, proximal lower limbs, left side of the chest, back, medial aspects of the left upper limb, chin, and the philtrum [Figure 1]. These followed a Blaschkoid distribution in a phylloid pattern, although midline demarcation was absent in several areas. The patches were non-scaly and non-atrophic and showed no signs of inflammation. The child underwent a thorough systemic examination, and no neurological or skeletal abnormalities were noted. Given the benign and non-progressive nature of the condition, no active treatment was initiated. The parents were counseled regarding the benign nature of the condition, and the expected increase in size proportionate to the child’s overall growth, and recommended regular follow-up to monitor for any changes. As the child matures, future options like non-cultured melanocyte-keratinocyte transfer may be considered for visible areas, if desired.

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Figure 1:

Multiple well-demarcated hypopigmented patches with irregular margins on the lower abdomen, groin, inner thighs, proximal lower limbs, left side of the chest, back, medial aspects of the left upper limb, chin, and philtrum.

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Nevus depigmentosus (ND) is a congenital, non-progressive hypopigmented macule or patch caused by a defect in melanosome transfer from melanocytes to keratinocytes. ND is classified into three subtypes: Isolated, segmental, and systematized. The systematized type, seen in this patient, is rare and features widespread hypopigmented patches following a dermatomal or Blaschkoid distribution. Systematized ND can sometimes be linked with extracutaneous manifestations, such as neurological or skeletal anomalies, though none were present in this case. The concept of pigmentary mosaicism has been proposed to describe various linear or segmental pigmentary anomalies, and systematized ND can be considered part of this spectrum. Differential diagnoses include hypomelanosis of Ito, segmental vitiligo, and ash-leaf macules of tuberous sclerosis.