Nalanda Medical College and Hospital, Patna, India
Pooja Nupur
Keywords
Nevus depigmentosusNevus achromicusMelanosomesHypomelanosis of Ito
A 6-year-old girl was brought to the outpatient department with complaints of an asymptomatic white coloured irregular lesion of the left cheek (Fig. 15.1). Mother observed the lesion 3 years back and gave the history of treatment for vitiligo for three months from a general practitioner with no relief. Mother gave the history of slight increase in size of lesion with age. There was no history of any skin disease or any injury prior to development of lesion. Rest of the history was non-contributory. On examination, patient had solitary hypopigmented patch with irregular, but well-defined, border on cheek. On diascopy, the margin of the lesion did not fade (Fig. 15.2). Based on the case description and photograph, what is your diagnosis?
1.
Nevus depigmentosus
2.
Hypomelanosis of Ito
3.
Nevus Anaemicus
4.
Piebaldism
Figure 15.1
Well demarcated hypopigmented patch on cheek. Note serrated margin
Figure 15.2
The margins are not fading or disappearing on diascopy
Diagnosis
Nevus depigmentosus
Discussion
Nevus depigmentosus (ND), also known as nevus achromicus, is a rare congenital non-progressive hypopigmented nevus. It can affect any race or gender. Exact cause of nevus depigmentosus is still not clearly understood. It is believed to be a cutaneous pigmentary mosaic disorder wherein functional defect of melanocytes with morphological abnormalities of melanosomes are major factors resulting in ND. Melanocytes have reduced ability to produce melanin and to transfer melanosomes to keratinocytes. Melanosomes can be diminished in number, heteromorphic, aggregated in melanocytes, or located in membrane bound aggregates [1]. It presents with solitary well circumscribed hypo- to depigmented patch, with an irregular serrated but well defined border, which do not cross midline. Many times smaller lesions may appear distributed over edge giving it a splashed paint appearance. It is commonly visible at birth and may appear later in adulthood. Commonly affected sites are trunk, neck, face and proximal part of extremities but it may occur at anywhere on the body. It may increases in size with the growth of the body and after reaching its maximum dimensions it usually remains unchanged throughout life. In contrast to what its name suggests, the nevus depigmentosus presents itself as a hypopigmentation rather than a depigmentation [2]. Three clinical subtypes have been described:
1.
Isolated or localised form (solitary and well defined lesion)—It is most common pattern.
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