HISTORY
In the midst of a hot summer, a 49-year-old man gets a haircut. For the first time in years, his wife can see the back of his neck—and a bright red patch of skin on it that she's never noticed before. The patient, of course, had no idea it was there; nor has he ever experienced any symptoms referable to the lesion. Except for mild hypertension, he is in reasonably good health.
EXAMINATION
The lesion is a bright red, 10 x 4–cm, funnel-shaped patch that runs from just inside the hairline to the C6 area of his midline posterior neck. It lacks any palpable portion and blanches readily on digital palpation.
DISCUSSION
It's a rare medical provider who has never seen one of these extremely common congenital capillary malformations called by various names (including stork bite, salmon patch, and angel kiss) but known officially as nevus flammeus nuchae (NFN). About 25% to 50% of Caucasian newborns are affected by this type of lesion, which can vary a great deal in size.
This particular patient's lesion demonstrated one of the condition's peculiar properties: the tendency to become much more pronounced with extertion, anger, hot weather, or combinations thereof. Many NFNs are effectively concealed by the patient's hair—as in this case, in which the patient had worn his hair long and in a ponytail for many years before getting a significant haircut. On his visit to dermatology, he was shown the photograph above and was so astonished and dismayed that he vowed to grow out his hair again!
NFN is the most common example of congenital vascular malformations; the so-called "port wine stain" is another well-known type. Unlike other lesions in this category, the NFN never resolves on its own, and it grows as the owner grows. Several hypotheses have evolved as to its origin; the leading one asserts that inadequate innervations promote decreased vascular tone.
The diagnosis of NFN is clinical, based on its history, location, and appearance. If performed, a biopsy would show dilated superficial dermal blood vessels, the walls of which can be a bit thinner than normal, but without other significant changes in the endothelial lining.
When desired, the lesion can be covered with makeup. Or ablation can be accomplished by laser treatment.
TAKE-HOME LEARNING POINTS
• Nevus flammeus nuchae (NFN) is extremely common, affecting 25% to 50% of Caucasian newborns.
• NFN is typically permanent and grows as the patient grows.
• NFN is almost always completely flat (macular) and blanches readily with digital pressure.
• NFNs become much more pronounced with exertion, heat, or emotional upset.