Speckled lentiginous nevus in Infant/Neonate
Nevus spilus can be associated with a number of other conditions and findings, including but not limited to corneal dystrophy, scleral pigmentation, hearing loss, Ebstein anomaly, ichthyosis, neurofibromatosis type 1, epidermal nevi, and a nevus sebaceus. Nevus spilus is a component of certain subtypes of phakomatosis pigmentovascularis. The speckled lentiginous nevus syndrome is the rarely occurring association of a nevus spilus (usually nevus spilus papulosis) and neuromuscular abnormalities, including a sensory or motor neuropathy and hyperhidrosis. Onset of the neurologic abnormalities is typically in adulthood, but childhood onset has been reported.
Nevus spilus shows no predilection for either sex but is slightly more common in individuals of Northern European descent than in other ethnicities. Most lesions are present at birth or become evident during the first year of life. They are benign lesions with a rare incidence of malignant transformation. Risk of melanoma development appears to correlate with lesions greater than 4 cm, zosteriform or segmental subtypes, and lesions present at birth. In addition, a particular mosaic mutation of PTPN11 was found to be correlated with the development of melanoma within a large segmental nevus spilus.
D22.9 – Melanocytic nevi, unspecified
I78.1 – Nevus, non-neoplastic
398660000 – Nevus spilus
Differential Diagnosis & Pitfalls