Nevus spilus L81.4

Burkley, 1842

A sharply defined, irregularly shaped, usually lentil- to palm-sized, milk-coffee-brown spot with small brown or also black-brown injections (also Kiebitzei nevus), which is already present at birth. The latter often develop only in the course of the first years of life.

Trunk, extremities. 1 case of oral nevus spilus has been reported (Torres KG et al. 2017).

Already present at birth (often overlooked), sharply defined, irregularly shaped, usually 0.4-10.0 cm large, milky coffee-brown patch with small dark brown, splatter-like spots, which often only develop during the first years of life.

In the course of life, the initially rather discrete splashes can transform into clearly defined black-brown papules, giving this nevus variant its typical aspect.

In rare cases, the development of malignant melanomas is observed in a nevus spilus. In this respect, the nevus spilus requires regular clinical monitoring.

Speckled lentiginous nevus syndrome is a cutaneous phenotype with nevus spilus (speckled lentiginous nevus) with hyperhidrosis, muscle weakness, dysesthesia or other neurological abnormalities.

Phacomatosis spilorosea (Happle 2005) is characterized by the combination of a naevus spilus with a naevus roseus, a capillary hamartoma, which was previously referred to as naevus flammeus lateralis.

Phacomatosis pigmentokeratotica (Happle 1996) is characterized by the combination of a nevus spilus with a nevus sebaceus.

The nevus spilus resembles the cafe-au-lait stain, but differs from it in the dark indentations in the underlying brown stain. The term "lapwing egg naevus "describes this aspect very well.

Becker ne vus: This nevus is not congenital, usually develops postpubertally (androgen dependence?) and shows hypertrichosis in addition to a broken reticulated border.

Annual inspection. Excise conspicuous, injected nevi early, as the danger of degeneration cannot be completely negated. If necessary, cosmetic cover with e.g. Dermacolor.

"spilus" = speckled

The lapwing is a bird whose eggs have a conspicuous mottle. The name is most likely derived from these.

1. Breitkopf CB et al (1996) Neoplasms on nevus spilus. Dermatologist 47: 759-762
2. Cramer SF (2001) Speckled lentiginous nevus (nevus spilus): the "roots" of the "melanocytic garden". Arch Dermatol 137: 1654-1655
3. Happle R (2002) Speckled lentiginous nevus syndrome: delineation of a new distinct neurocutaneous phenotype. Eur J Dermatol 12: 133-135.
4. Mang R et al (2003) Unusual clinical presentation of melanocytic nevi. Dermatologist 54: 370-372
5. Ramolia P et al (2009) Speckled lentiginous nevus syndrome associated with musculoskeletal abnormalities. Pediatr Dermatol 26: 298-301
6. Sarin KY et al (2014) Activating HRAS mutation in nevus spilus. J Invest Dermatol 134:1766-1768.
7. Schaffer JV et al (2001) Speckled lentiginous nevus: within the spectrum of congenital melanocytic nevi. Arch Dermatol 137: 172-178
8. Tavoloni Braga JC et al (2014) Early detection of melanoma arising within nevus spilus. J Am Acad Dermatol 70:e31-e32
9. Torres KG et al (2017) Nevus spilus (Speckled Lentiginous Nevus) in the Oral Cavity: Report of a Case and Review of the Literature. Am J Dermatopathol 39:e8-e12.