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Clinical and histopathologic characteristics of nevus depigmentosus

https://doi.org/10.1016/j.jaad.2006.04.053Get rights and content

Background

Nevus depigmentosus (ND) is known to be a rare congenital, nonprogressive disorder characterized by a hypopigmented lesion that remains stable over time. There have been only few studies of clinical and histopathologic characteristics of ND, and the etiopathogenesis is not fully established.

Objective

The purpose of this study was to investigate the clinical and histopathologic characteristics of ND.

Methods

A clinical survey was carried out with 60 patients given the diagnosis of ND. Punch biopsies (2 mm) from lesional and perilesional normal skin were performed. The sections were stained with hematoxylin-eosin, Fontana-Masson, antibodies to S-100 protein, MART-1, NKI/beteb, CD1a, CD3, CD20, and CD68.

Results

The lesions were usually present before the age of 3 years (68.3%), but some lesions appeared later in childhood (31.7%). In all, 27 patients (45%) had one lesion, but there were 14 patients (23.3%) who had more than 10 lesions. Fontana-Masson stain showed that the amount of melanin was significantly decreased in ND skin compared with perilesional normal skin. Melanocyte counts were significantly decreased in ND skin when stained with antibodies to GP-100 and MART-1. However, there were no significant differences in the number of melanocytes identified as S-100 protein–positive cells. There were no significant differences in histologic findings or dermal inflammatory infiltrates between ND skin and perilesional normal skin.

Limitations

Only 29 patients (48.3%) were followed up, and the average follow-up period after initial diagnosis was relatively short (68 months); therefore, these overall results may not be representative of the clinical course of the patients.

Conclusion

Only 18 patients (30.0%) presented with ND since birth and only 27 patients (45.0%) had one lesion. Both the amount of melanin and the number of melanocytes in ND skin were decreased in patients with ND. Therefore, both clinical and histologic findings should be considered together to make a diagnosis of ND.

Section snippets

Patients

We examined 60 Korean patients with ND who attended our institution between January 2001 and December 2004. In each patient, the diagnosis of ND was determined by clinical diagnostic criteria proposed by Coupe4 and Wood's light examination. The clinical data compiled included sex, age, age of onset, number of lesions, involved sites, shape and pattern of lesions, and associated systemic disease.

Stains and immunohistochemistry

Punch biopsies (2 mm) from lesional and perilesional normal-appearing (usually ≥6 cm distant) skin

Clinical findings

As shown in Table I, of the 60 patients with ND, 33 (55%) were male and 27 (45%) were female. Their ages ranged from 3 months to 35 years (mean 9.4 years, median 1.0 year) and onset age of ND ranged from immediately after birth to 13 years (mean 2.1 years). The earliest onset was immediately after birth, whereas in 19 patients (31.7%) it appeared after 3 years of age. In all, 27 patients (45%) had only one lesion, whereas 9 patients (15.0%) had more than 20 lesions. The trunk was the most

Discussion

ND is generally known to be a congenital, nonfamilial disorder characterized by hypopigmented lesion that remains stable over time. Although the clinical features of ND may appear similar to those of vitiligo, the clinical courses of ND are different from those of vitiligo.8 We investigated the clinicopathologic features of ND that are essential not only to diagnosis but also to understanding the pathogenesis of ND.

The development of ND usually takes place at an early age. However, sporadic

References (18)

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Supported by a 2004 grant from the Department of Medical Sciences, The Graduate School, Ajou University.

Conflicts of interest: None identified.

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