INTRODUCTION
Generalized essential telangiectasia (GET) is a rare disease characterized by widespread telangiectasias of the lower limbs that may spread superiorly to involve other parts of the body.1,2 GET is typically not associated with other diseases or complications, but this disease can have profound aesthetic and psychologic morbidity. Currently there are no satisfactory treatments available for this condition.
CASE REPORT
A 55-year-old Caucasian woman presented with diffuse, mat-like telangiectasias over the distal aspects of both lower extremities (Figure 1) and were also present on her knees, thighs, and hands. The lesions began as discrete, bilateral telangiectasias over the dorsal surface of the feet and progressed slowly over the next six years. They were blanchable and lacked pigment, purpura, or atrophy. Her condition was asymptomatic but did cause significant psychological distress.Histopathology revealed papillary and reticular dermal telangiectasias with a sparse, perivascular lymphocytic infiltrate, as well as a haphazard array of thick and thin collagen bundles. Immunostains for CD31, CD34, Collagen IV, and UEA-1 demonstrated a continuous layer of endothelial cells in both pre-existing and telangiectatic vessels, a partial to complete loss of pericytes, and a multilayered basement membrane around many of the ectatic vessels.Given the widespread nature of her lesions, laboratory studies included markers for angiogenesis which revealed elevated urinary levels of fibrinogen (456 mg/dL) and mixed metallo-proteinases (MMP dimer, MMP-2, MMP-9, and MMP-9/NGAL), and diminished Vitamin C (0.3 mg/dL). Autoimmune and coagulopathy studies were negative. An immunoglobulin panel was within normal limits. Urinary levels of fibroblast growth factor (FGF) and vascular endothelial growth factor (VEGF). The clinical and histopathologic findings were consistent with a diagnosis of GET.Over the next two years the patient underwent multiple treatments with Intense Pulsed Light (IPL, 18-20 J/cm2) with minimal and transient efficacy. The patient also regularly used compression stockings and Vitamin C supplements with minimal change. Doxycycline was initiated (100 mg BID) but discontinued after 7 weeks due to nausea and limited efficacy. Several sessions utilizing a 585-nm pulsed-dye laser (6.5 J/cm2,) at sites on the right thigh and foot resulted in moderate cosmetic improvement, but the associated erythema soon returned to baseline levels and telangiectasias recurred.Her condition continued unabated for the next two years until her gastroenterologist started her on 6-mercaptopurine (6-MP) for the treatment of ulcerative colitis. She was started at a dose of 25 mg daily, ultimately alternating daily between 50 and 75 mg daily to reach a 6-thioguanine nucleotide (6-TG) level of 254 pmol/8 x 108 RBC (the level associated with increased frequency of clinical response in ulcerative colitis).4 Within one month there was a dramatic improvement in erythema and virtual clearance of the telangiectasias by two months (Figure 2). To date, the patient remains on 6-MP with minimal cutaneous disease.
DISCUSSION
Generalized essential telangiectasia is a primary disorder characterized by widespread sheets of telangiectasias that typically present symmetrically on the lower extremities and may spread superiorly, rarely with oral or ocular involvement.2 It is
