Clinical Review & Education

JAMA Dermatology Clinicopathological Challenge

Progressive Widespread Cutaneous Telangiectasias Katherine M. Hunt, BS; Rebecca Kissel, MD; Lucia Seminario-Vidal, MD, PhD

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B

Figure. A, Multiple telangiectasias coalescing to form large, retiform patches on the bilateral lower extremities. B, Dilated, ectatic vessels in the superficial dermis with no associated inflammation or fibrinoid necrosis (hematoxylin-eosin, original magnification ×200).

A woman in her 50s presented with “speckled” red lesions on her bilateral arms, lower legs, and feet. They had first appeared on her lower legs 15 years ago and were slowly increasing in number and spreading to involve her feet and bilateral upper extremities. The patient denied tenderness, pruritus, and edema. She reported no family history of this probQuiz at jamadermatology.com lem and denied any personal history of anemia, epistaxis, hematemesis, melena, hematochezia, or hemoptysis. She had a medical history of hypertension, hyperlipidemia, and tobacco abuse, requiring treatment with losartan and atorvastatin. Physical examination revealed punctate, blanchable telangiectasias coalescing to form large, retiform patches on her bilateral lower extremities, feet, and upper extremities (Figure, A). There was no conjunctival or mucosal involvement. The patient reported no dermatographism or purpura. Histologic findings from a biopsy specimen are shown in the Figure, B.

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WHAT IS THE DIAGNOSIS?

A. Hereditary hemorrhagic telangiectasia B. Cutaneous collagenous vasculopathy C. Generalized essential telangiectasia D. Systemic lupus erythematosus

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Clinical Review & Education JAMA Dermatology Clinicopathological Challenge

Diagnosis C. Generalized essential telangiectasia

Microscopic Findings and Clinical Course Punch biopsy specimens of the leg and elbow revealed dilated, ectatic vessels in the superficial dermis with no associated inflammation or fibrinoid necrosis. The epidermis was normal with typical basket weave appearance of the stratum corneum, and no spongiosis was present. Results from laboratory tests, including complete cell blood count, complete metabolic panel, anti-nuclear antibodies and anti-SSA/Ro and anti-SSB/La antibodies, were within normal limits. The patient’s clinical picture and histopathologic and laboratory findings were consistent with a diagnosis of generalized essential telangiectasia (GET). The patient was initially prescribed doxycycline, 100 mg twice per day, with no clinical improvement. Subsequently, she was treated once with pulsed-dye laser therapy with slight improvement. She declined further treatments.

Discussion Generalized essential telangiectasia is rare disease characterized by the development of ectatic vessels beginning in dependent skin surfaces with a typical “upward march” progression. It is more common among women, with onset usually between the third to fifth decades of life.1 The incidence and prevalence of GET are unknown. Diagnosis is made by clinical findings of progressive telangiectasias that are more prominent in dependent areas and a family history that is negative for the disease in the setting of no other known cause of telangiectasias such as dermatomyositis, systemic lupus erythematosus, systemic sclerosis, medications, radiation therapy, cirrhosis, syphilis, ataxia telangiectasia syndrome, and telangiectasia macularis eruptiva perstans.2 Rarely, patients may also present with conjunctival3 and ocular4 involvement. The lesions are typically asymptomatic, although symptoms of mild pruritus, burning, or tingling have been reported.2 The disease is ARTICLE INFORMATION Author Affiliations: University of Alabama School of Medicine, Birmingham (Hunt); Department of Dermatology, University of Alabama at Birmingham, Birmingham (Kissel, Seminario-Vidal). Corresponding Author: Katherine M. Hunt, BS, University of Alabama School of Medicine, 3536 William and Mary Rd, Hoover, AL 35216 (mmarchio @uab.edu). Section Editor: Molly A. Hinshaw, MD; Assistant Section Editors: Soon Bahrami, MD; Nicole Fett, MD, MSCE; Anna K. Haemel, MD; Arni K. Kristjansson, MD; Lori D. Prok, MD. Published Online: August 20, 2014. doi:10.1001/jamadermatol.2014.1183. Conflict of Interest Disclosures: None reported. REFERENCES 1. McGrae JD Jr, Winkelmann RK. Generalized essential telangiectasia: report of a clinical and histochemical study of 13 patients with acquired cutaneous lesions. JAMA. 1963;185:909-913.

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underrecognized, with many patients mistakenly diagnosed as having hereditary hemorrhagic telangiectasia (HHT); in contrast, HHT presents with epistaxis and arteriovenous malformations in addition to telangiectasias in the setting of an autosomal dominant inheritance pattern.3 The differential diagnosis also includes clinically similar cutaneous collagenous vasculopathy (CCV); CCV’s distinctive histopathological findings of ectatic superficial capillaries surrounded by a thick, hyaline collagenous wall (which stains periodic acid-Schiff positive) help to distinguish it from GET.5 The pathogenesis of GET remains to be elucidated. One case report6 of the condition associated with nodular regenerative hyperplasia proposed endothelial dysfunction as a possible cause. An additional report7 has suggested an association between GET and gastric antral vascular ectasia (GAVE), which may support the possible role of endothelial dysfunction in its pathogenesis. However, GAVE is more commonly caused by hepatic cirrhosis and systemic sclerosis,8 underlying conditions that may have been overlooked in these cases. Generalized essential telangiectasia is difficult to treat and may cause considerable psychological distress. Some authors have proposed that venous stasis plays a role in the disease process and have recommended conservative therapy with compression stockings,1 although we found no evidence of success with this approach in the literature. Historically, medical treatment with minocycline and doxycycline9 improved the lesions in some cases. However, our patient and a patient described in a recent report2 demonstrated no change with tetracycline therapy. Acyclovir and ketoconazole have shown benefit in older reports,9 although these findings have not been replicated recently. One study2 reported moderate improvement in a patient treated with hypertonic saline, 11.2%, sclerotherapy injections; however, the treatment was discontinued owing to pain from the procedure. Other patients with resistant disease have experienced more consistent success with pulsed-dye and Nd:YAG laser therapies.10

2. Gordon Spratt EA, DeFelice T, O’Reilly K, Robinson M, Patel RR, Sanchez M. Generalized essential telangiectasia. Dermatol Online J. 2012;18 (12):13.

7. Tetart F, Lorthioir A, Girszyn N, Lahaxe L, Ducrotté P, Marie I. Watermelon stomach revealing generalized essential telangiectasia. Intern Med J. 2009;39(11):781-783.

3. Swensson B, Swensson O, Häring G. Progressive disseminated essential telangiectasia with conjunctival involvement [in German]. Klin Monbl Augenheilkd. 1998;212(2):116-119.

8. Selinger CP, Ang YS. Gastric antral vascular ectasia (GAVE): an update on clinical presentation, pathophysiology and treatment. Digestion. 2008; 77(2):131-137.

4. Seiter S, Gantenbein C, Ugurel S, Ruprecht KW, Tilgen W, Reinhold U. An oculocutaneous presentation of essential progressive telangiectasia. Br J Dermatol. 1999;140(5):969-971.

9. Shelley WB, Shelley ED. Essential progressive telangiectasia in an autoimmune setting: successful treatment with acyclovir. J Am Acad Dermatol. 1989;21(5 Pt 2):1094-1096.

5. Kanitakis J, Faisant M, Wagschal D, Haftek M, Claudy A. Cutaneous collagenous vasculopathy: ultrastructural and immunohistochemical study of a new case. Am J Clin Dermatol. 2010;11(1):63-66.

10. Buscaglia DA, Conte ET. Successful treatment of generalized essential telangiectasia with the 585-nm flashlamp-pumped pulsed dye laser. Cutis. 2001;67(2):107-108.

6. Rothweiler S, Heim MH, Semela D. Nodular regenerative hyperplasia in a patient with generalized essential telangiectasia: endotheliopathy as causal factor [published online November 22, 2013]. Hepatology. doi:10.1002/hep .26942.

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Progressive widespread cutaneous telangiectasias.

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