The Diagnosis: Unilateral Nevoid Telangiectasia
Unilateral nevoid telangiectasia (UNT) is an uncommon, or perhaps underreported, cutaneous condition involving telangiectatic patches in a unilateral dermatomal or blaschkoid pattern.1 The condition has been described as either congenital or acquired. Congenital UNT is thought to be a result of somatic mosaicism, whereby a mutation during embryogenesis leads to a distinct population of cells expressing the vascular malformation.1 Congenital UNT has been associated with Becker nevus, which also is thought to be a result of somatic mosaicism, further providing evidence for this theory, though it is unclear whether this finding is incidental.2 The acquired form often is associated with fluctuation of hormones, such as in pregnancy or with oral contraceptive initiation, as well as with hepatic disease as seen in our patient. However, there are many cases of acquired UNT with no implicated underlying disease, alcohol abuse, or hormonal changes, which calls into question if UNT is definitively an estrogen-related condition.3 One study demonstrated an increased level of estrogen and progesterone receptors in affected skin, which may have led to expression of the cutaneous changes at that site.4 More research is needed to elucidate this point, as other studies have not reproduced similar findings.
Congenital UNT occurs more commonly in males, whereas the acquired variant is seen more frequently in females. The third and fourth cervical dermatomes most often are involved.5 Most lesions persist without spontaneous resolution. Treatment options are limited and include pulsed dye laser treatment and makeup application to cover the telangiectatic patches. The main side effect seen with pulsed dye laser treatment is reversible pigmentary changes, with 1 report of textural skin change.6
A biopsy was deemed unnecessary for the clinical diagnosis in our patient because there was a clear explanation for the physical examination findings due to long-standing underlying liver disease. When biopsied, UNT characteristically demonstrates dilated dermal capillaries.5 Our patient elected not to pursue laser therapy but expressed interest in using makeup to camouflage the lesion.
The differential diagnosis includes acquired nevus flammeus, which typically is present on the face and often appears following mechanical or thermal trauma. Angioma serpiginosum most often occurs on the buttocks and legs as small red papules or puncta coalescing into a serpiginous linear arrangement. It often appears in childhood. Angiosarcoma is an aggressive malignancy that often occurs on the head and neck in elderly patients. It is associated with areas of long-standing lymphedema and often appears as a bruiselike lesion. Rosacea typically is not fixed in its clinical appearance and presents as transitory flushing of the head and neck with or without a history of acneform eruptions on the face. It typically is not unilateral.