Lentigo Simplex

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Image of Lentigo Simplex

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L81.4 – Other melanin hyperpigmentation

398900000 – Lentigo simplex


A lentigo simplex is an extremely common hyperpigmented macule located anywhere on the body. They generally occur early in life (may be present at birth) and are not associated with sun exposure. They result from an increased number of normal melanocytes in the epidermis producing increased amounts of melanin.

Clinically, lesions are asymptomatic, well-circumscribed, symmetric, homogeneous light brown to black macules. They are usually smaller than 5 mm in size. They are distributed anywhere on the trunk, extremities, genitals, and mucous membranes. Lentigines found on mucous membranes can appear irregular with increased size, irregular borders, and heterogeneous pigmentation. They occasionally form in cutaneous scars and may be associated with psoralen/ultraviolet light therapy. Lentigo simplex may evolve into junctional nevi but are not thought to evolve into melanoma. They differ from solar lentigines in that they appear earlier in life on non-sun-exposed skin.

Lentigo simplex may occur as single or multiple lesions. Occasionally, multiple lentigines are associated with rare genetic disorders. These include the following:
  • LEOPARD syndrome – lentigines, EKG changes, ocular hypertelorism, pulmonary stenosis, abnormal genitalia, growth retardation, and deafness
  • Carney complex – lentigines, atrial myxoma, mucocutaneous myxoma, and nevi
  • Peutz-Jeghers syndrome – lentigines (perioral and oral), multiple gastrointestinal polyps, and visceral tumors (pancreas, ovary, testes)
  • Xeroderma pigmentosum – lentigines on sun-exposed skin and multiple skin cancers
  • Cronkhite-Canada syndrome – lentigines (buccal mucosa, face, palmoplantar), alopecia, nail dystrophy, and intestinal polyps
Other rare disorders associated with multiple lentigines include generalized lentigines, arterial dissection with lentiginosis, Laugier-Hunziker syndrome, Cantú (hyperkeratosis-hyperpigmentation) syndrome, Cowden disease, centrofacial lentiginosis, and Bannayan-Riley-Ruvalcaba syndrome.

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