Nevus depigmentosus

Last updated

Nevus depigmentosus
Other namesNevus achromicus [1]
Nevus Depigmentosus.jpg
Man's chest with nevus depigmentosus.
Notice the affected (white) area to the left of the nipple.
Specialty Dermatology

Nevus depigmentosus is a loss of pigment in the skin which can be easily differentiated from vitiligo. Although age factor has not much involvement in the nevus depigmentosus but in about 19% of the cases these are noted at birth. Their size may however grow in proportion to growth of the body. The distribution is also fairly stable and are nonprogressive hypopigmented patches. [2] The exact cause of nevus depigmentosus is still not clearly understood. A sporadic defect in the embryonic development has been suggested to be a causative factor. [3] It has been described[ by whom? ] as "localised albinism", though this is incorrect.

Contents

Those with nevus depigmentosus may be prone to sunburn due to the lack of pigment, and the patient should use good sun protection. [4] Sunscreen should be applied to all exposed skin, since reduced tanning of normal skin will decrease the contrast with hypopigmented skin. [4] Most patients with nevus depigmentosus do not pursue treatment for their lesion. [5] There is no way to repigment the skin. [5] If, however, the lesion is of cosmetic concern, camouflage makeup is effective. [5] If the lesion is small one could also consider excision. [5]

Signs and symptoms

These are localized white spots on skin which may affect any area of the body, but these white spots are quite stable lesions. [2] In the majority of patients, the lesions are not completely achromic, but are hypopigmented and resemble splashed paint. [6] The individual lesions are permanent and there are no effective therapies for re-pigmenting this nevus. [6] If there is hair in an affected area, it is usually colourless or white.

Causes

All 3 components that is melanocytes, melanosomes, and melanogenesis is normal but the defect lies in the transfer of melanosomes.

Diagnosis

Initial assessment can be made based on the presence of depigmented patches, and confirmed with a skin biopsy.[ citation needed ]

Treatment

Different therapeutic modalities have been attempted to repigment the lesions of nevus depigmentosus such as PUVA, excimer laser, and different grafting techniques. PUVA therapy has not been shown to be beneficial. [7] Successful repigmentation was reported in a single case with 14 sessions of excimer laser treatment. [8] Though the repigmentation of nevus depigmentosus is possible by grafting techniques, the results are inconsistent and recurrence is possible. In consideration of the experience of other authors and us, the quality and retention of pigment are unpredictable. These factors need to be considered while consulting and offering any treatment to the patient of nevus depigmentosus. [9]

See also

Related Research Articles

<span class="mw-page-title-main">Melanocytic nevus</span> Medical condition

A melanocytic nevus is usually a noncancerous condition of pigment-producing skin cells. It is a type of melanocytic tumor that contains nevus cells. Some sources equate the term mole with "melanocytic nevus", but there are also sources that equate the term mole with any nevus form.

<span class="mw-page-title-main">Melanocyte</span> Melanin-producing cells of the skin

Melanocytes are melanin-producing neural crest-derived cells located in the bottom layer of the skin's epidermis, the middle layer of the eye, the inner ear, vaginal epithelium, meninges, bones, and heart. Melanin is a dark pigment primarily responsible for skin color. Once synthesized, melanin is contained in special organelles called melanosomes which can be transported to nearby keratinocytes to induce pigmentation. Thus darker skin tones have more melanosomes present than lighter skin tones. Functionally, melanin serves as protection against UV radiation. Melanocytes also have a role in the immune system.

<span class="mw-page-title-main">Vitiligo</span> Skin condition where patches lose pigment

Vitiligo is a chronic autoimmune disorder that causes patches of skin to lose pigment or color. The cause of vitiligo is unknown, but it may be related to immune system changes, genetic factors, stress, or sun exposure. Treatment options include topical medications, light therapy, surgery and cosmetics.

<span class="mw-page-title-main">Nevus</span> Mole or birthmark; visible, circumscribed, chronic skin lesion

Nevus is a nonspecific medical term for a visible, circumscribed, chronic lesion of the skin or mucosa. The term originates from nævus, which is Latin for "birthmark"; however, a nevus can be either congenital or acquired. Common terms, including mole, birthmark, and beauty mark, are used to describe nevi, but these terms do not distinguish specific types of nevi from one another.

PUVA is an ultraviolet light therapy treatment for skin diseases: vitiligo, eczema, psoriasis, graft-versus-host disease, mycosis fungoides, large plaque parapsoriasis, and cutaneous T-cell lymphoma, using the sensitizing effects of the drug psoralen. The psoralen is applied or taken orally to sensitize the skin, then the skin is exposed to UVA.

<span class="mw-page-title-main">Leser–Trélat sign</span> Onset of multiple seborrheic keratoses

The Leser–Trélat sign is the explosive onset of multiple seborrheic keratoses, often with an inflammatory base. This can be a sign of internal malignancy as part of a paraneoplastic syndrome. In addition to the development of new lesions, preexisting ones frequently increase in size and become symptomatic.

<span class="mw-page-title-main">Lentigo maligna</span> Medical condition

Lentigo maligna is where melanocyte cells have become malignant and grow continuously along the stratum basale of the skin, but have not invaded below the epidermis. Lentigo maligna is not the same as lentigo maligna melanoma, as detailed below. It typically progresses very slowly and can remain in a non-invasive form for years.

<span class="mw-page-title-main">Congenital melanocytic nevus</span> Congenital mole caused by genetic mutations

The congenital melanocytic nevus is a type of melanocytic nevus found in infants at birth. This type of birthmark occurs in an estimated 1% of infants worldwide; it is located in the area of the head and neck 15% of the time.

<span class="mw-page-title-main">Becker's nevus</span> Medical condition

Becker's nevus is a benign skin disorder predominantly affecting males. The nevus can be present at birth, but more often shows up around puberty. It generally first appears as an irregular pigmentation on the torso or upper arm, and gradually enlarges irregularly, becoming thickened and often hairy (hypertrichosis). The nevus is due to an overgrowth of the epidermis, pigment cells (melanocytes), and hair follicles. This form of nevus was first documented in 1948 by American dermatologist Samuel William Becker (1894–1964).

<span class="mw-page-title-main">Nevus of Ota</span> Human facial birthmark

Nevus of Ota is a hyperpigmentation that occurs on the face, most often appearing on the white of the eye. It also occurs on the forehead, nose, cheek, periorbital region, and temple.

<span class="mw-page-title-main">Transient acantholytic dermatosis</span> Medical condition

Grover's disease (GD) is a polymorphic, pruritic, papulovesicular dermatosis characterized histologically by acantholysis with or without dyskeratosis. Once confirmed, most cases of Grover's disease last six to twelve months, which is why it was originally called "transient". However it may last much longer. Nevertheless, it is not to be confused with relapsing linear acantholytic dermatosis.

<span class="mw-page-title-main">Halo nevus</span> Medical condition

Halo nevus is a mole that is surrounded by a depigmented ring or 'halo'.

<span class="mw-page-title-main">Nevus anemicus</span> Medical condition

Nevus anemicus is a congenital disorder characterized by macules of varying size and shape that are paler than the surrounding skin and cannot be made red by trauma, cold, or heat. The paler area is due to the blood vessels within the area which are more sensitive to the body’s normal vasoconstricting chemicals.

Large plaque parapsoriasis are skin lesions that may be included in the modern scheme of cutaneous conditions described as parapsoriasis. These lesions, called plaques, may be irregularly round-shaped to oval and are 10 cm (4 in) or larger in diameter. They can be very thin plaques that are asymptomatic or mildly pruritic. Large-plaque parapsoriasis is a common associate of retiform parapsoriasis, can be accompanied by poikiloderma vasculare atrophicans, and can in rare occasions be a precursor to cutaneous T-cell lymphoma.

Cutaneous amoebiasis, refers to a form of amoebiasis that presents primarily in the skin. It can be caused by Acanthamoeba or Entamoeba histolytica. When associated with Acanthamoeba, it is also known as "cutaneous acanthamoebiasis". Balamuthia mandrillaris can also cause cutaneous amoebiasis, but can prove fatal if the amoeba enters the bloodstream It is characterized by ulcers. Diagnosis of amebiasis cutis calls for high degree of clinical suspicion. This needs to be backed with demonstration of trophozoites from lesions. Unless an early diagnosis can be made such patients can develop significant morbidity.

Ectodermal dysplasia with corkscrew hairs is a skin condition with salient features including exaggerated pili torti, scalp keloids, follicular plugging, keratosis pilaris, xerosis, eczema, palmoplantar keratoderma, syndactyly, onychodysplasia and conjunctival neovascularization.

<span class="mw-page-title-main">Inflammatory linear verrucous epidermal nevus</span> Medical condition

Inflammatory Linear Verrucous Epidermal Nevus is a rare disease of the skin that presents as multiple, discrete, red papules that tend to coalesce into linear plaques that follow the Lines of Blaschko. The plaques can be slightly warty (psoriaform) or scaly (eczema-like). ILVEN is caused by somatic mutations that result in genetic mosaicism. There is no cure, but different medical treatments can alleviate the symptoms.

Pseudomelanoma is a cutaneous condition in which melanotic skin lesions clinically resemble a superficial spreading melanoma at the site of a recent shave removal of a melanocytic nevus.

Oral pigmentation is asymptomatic and does not usually cause any alteration to the texture or thickness of the affected area. The colour can be uniform or speckled and can appear solitary or as multiple lesions. Depending on the site, depth, and quantity of pigment, the appearance can vary considerably.

References

  1. Lee HS, Chun YS, Hann SK (January 1999). "Nevus depigmentosus: clinical features and histopathologic characteristics in 67 patients". J. Am. Acad. Dermatol. 40 (1): 21–6. doi:10.1016/S0190-9622(99)70524-4. PMID   9922008.
  2. 1 2 Lee, H S; Chun, Y S & Hann, S K (January 1999). "Nevus depigmentosus: clinical features and histopathologic characteristics in 67 patients". J Am Acad Dermatol . 40 (1): 21–26. doi:10.1016/S0190-9622(99)70524-4. PMID   9922008.
  3. Pinto, FJ; Bolognia, JL (1991). "Disorders of hypopigmentation in children". Pediatric Clinics of North America. U.S. National Library of Medicine. 38 (4): 991–1017. doi: 10.1016/S0031-3955(16)38164-0 . PMID   1870914.
  4. 1 2 J. Kwiatkowski, David (8 June 2010). Tuberous Sclerosis Complex: Genes, Clinical Features and Therapeutics. Wiley. p. 286. ISBN   978-3-527-32201-5.
  5. 1 2 3 4 J. Nordlund, James (1998). The pigmentary system: physiology and pathphysiology. Oxford University Press. p. 651. ISBN   978-0-19-509861-7.
  6. 1 2 Thappa (10 November 2010). Clinical Pediatric Dermatology. Elsevier India. p. 92. ISBN   978-81-312-1489-3.
  7. Berg M, Tarnowski W. Nevus pigmentosus. Arch Dermatol 1974;109:920.
  8. 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.
  9. Mulekar, S.; Al Issa, A.; Al Eisa, A. (2011). "Nevus depigmentosus treated by melanocyte-keratinocyte transplantation". Journal of Cutaneous and Aesthetic Surgery. 4 (1): 29–32. doi: 10.4103/0974-2077.79185 . PMC   3081481 . PMID   21572678.