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Nevus depigmentosus

FieldValue
nameNevus depigmentosus
synonymsNevus achromicus
imageNevus Depigmentosus.jpg
captionMan's chest with nevus depigmentosus
fieldDermatology
symptomsSkin hypopigmentation
differentialVitiligo

Nevus depigmentosus is a congenital disorder characterised by localised loss of skin pigmentation and can be readily distinguished from vitiligo. Although age is not a major factor in its development, approximately 19% of cases are present at birth. The lesions may increase in size proportionally with the growth of the body, but their distribution remains stable, and they are non-progressive hypopigmented patches. The exact cause of nevus depigmentosus is not fully understood; however, a sporadic defect in embryonic development has been proposed as a possible etiological factor.

The condition has sometimes been described as "localised albinism", although this terminology is considered incorrect.

Individuals with nevus depigmentosus may be more susceptible to sunburn due to the reduced melanin content in the affected skin, and appropriate sun protection is therefore recommended. Sunscreen should be applied to all exposed skin, as minimising tanning of the surrounding normal skin can help reduce the contrast with the hypopigmented patches. Most individuals with nevus depigmentosus do not seek treatment for the condition. There is currently no effective method to restore pigmentation. If the lesion is of cosmetic concern, camouflage makeup may be used. In cases where the lesion is small, surgical excision may also be considered.

Signs and symptoms

Nevus depigmentosus presents as localized white patches on the skin that may occur on any area of the body. These legions are typically stable over time. In most patients, the patches are not completely achromic, but rather hypopigmented, often described as having a "splashed paint" appearance. Individual lesions are permanent, and there are currently no effective therapies to restore pigmentation in the affected areas. When hair is present within a lesion, it is usually colourless or white.

Causes

In nevus depigmentosus, melanocytes, melanosomes, and the process of melanogenesis are normal; however, the defect lies in the impaired transfer of melanosomes to keratinocytes.

Diagnosis

An initial diagnosis is typically made based on the characteristic appearance of the hypopigmented patches and their stable distribution. The diagnosis may be confirmed with a skin biopsy, although this is not always required.

Treatment

Various therapeutic modalities have been attempted to repigment the lesions of nevus depigmentosus, including psoralen plus ultraviolet A (PUVA) therapy, excimer laser treatment, and different grafting techniques. PUVA therapy has not been shown to be beneficial. Successful repigmentation has been reported in a single case following 14 sessions of excimer laser treatment.

Though repigmentation of nevus depigmentosus may be achieved through grafting techniques, the outcomes are inconsistent, and recurrence can occur. Based on the experience reported, both the quality of repigmentation and long-term pigment retention are unpredictable.

References

References

  1. (January 1999). "Nevus depigmentosus: clinical features and histopathologic characteristics in 67 patients". J. Am. Acad. Dermatol..
  2. Lee, H S. (January 1999). "Nevus depigmentosus: clinical features and histopathologic characteristics in 67 patients". [[Journal of the American Academy of Dermatology]].
  3. (1991). "Disorders of hypopigmentation in children". U.S. National Library of Medicine.
  4. Hafeez, Dr Filza. (2023-02-06). "How Does Nevus Depigmentosus Occur?".
  5. J. Kwiatkowski, David. (8 June 2010). "Tuberous Sclerosis Complex: Genes, Clinical Features and Therapeutics". Wiley.
  6. J. Nordlund, James. (1998). "The pigmentary system: physiology and pathphysiology". Oxford University Press.
  7. Thappa. (10 November 2010). "Clinical Pediatric Dermatology". Elsevier India.
  8. Themes, U. F. O.. (2017-10-14). "Disorders of Pigmentation".
  9. Berg M, Tarnowski W. Nevus pigmentosus. Arch Dermatol 1974;109:920.
  10. Kim DY, Lee KY, Park YK. Use of the 308-nm excimer laser for nevus depigmentosus: A promising treatment for either nevus depigmentosus or vitiligo. J Dermatol 2007;34:217–8.
  11. (2011). "Nevus depigmentosus treated by melanocyte-keratinocyte transplantation". Journal of Cutaneous and Aesthetic Surgery.
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