Generalized essential telangiectasia
Published Web Locationhttps://doi.org/10.5070/D30hx273p5
Generalized essential telangiectasiaThe Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York
Elizabeth A Gordon Spratt MD, Taylor DeFelice MD MPH, Kathryn O’Reilly MD PhD, Maria Robinson MD, Rishi R Patel MD, Miguel
Dermatology Online Journal 18 (12): 13
Generalized essential telangiectasia, which is a rare condition that is characterized by the progressive development of telangiectases on the skin, is a clinical diagnosis of exclusion. We present a 65-year-old man with a ten-month history of an asymptomatic eruption of the trunk and proximal aspects of the arms and hands that was comprised of macules and patches of telangiectases. The clinical presentation, associated diseases, hypotheses regarding pathogenesis, differential diagnoses, and reports on treatment modalities are reviewed. The relatively new association of this entity with systemic signs that include hemorrhage as well as the occurrence of generalized essential telangiectasia in patients with a history of hepatitis is discussed.
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A 65-year-old man presented to the Dermatology Clinic at Bellevue Hospital Center for evaluation of an asymptomatic eruption that consisted of numerous telangiectases on the chest, back, and proximal aspects of the arms. The lesions had been present for approximately ten months and originally appeared on the dorsal aspects of his hands. They were slowly increasing in number and spreading across the anterior and posterior aspects of the trunk. The lesions were not pruritic or painful. He denied a history of urticaria, mucosal lesions, or systemic symptoms, such as epistaxis, melena, abdominal pain, diarrhea, headaches, difficulty breathing, photosensitivity, or muscle weakness. There was no family history of bleeding, telangiectases, or epistaxis.
Past medical history included substance abuse and he is currently enrolled in a methadone program. Additional conditions were hypertension, depression/anxiety, and hepatitis C virus infection, which was treated with interferon ten years ago. Medications include gabapentin, lexapro, trazodone, lisinopril-hydrochlorothiazide, and methadone. There were no known allergies. The involved areas were not cosmetically bothersome, thus therapy was deferred. Punch biopsies were taken of lesions on the right and left sides of the back.
Erythematous macules and patches, which were comprised of numerous telangiectases that were arranged in web-like sheets and which blanched on compression, were present on the chest, back, and proximal aspects of the arms. There were a few, scattered telangiectases on the dorsal aspects of the hands. There was no urtication, dermatographism, or purpura. There were no conjunctival or mucosal lesions.
A complete blood count showed decreased platelets 144 x 109/L. A basic metabolic profile, lipid profile, thyroid stimulating hormone, erythrocyte sedimentation rate, C-reactive protein, creatinine kinase, and INR/PT were normal. Aspartate transaminase was 66 IU/L (11-39), alanine transaminase 65 IU/L (11-35), and alkaline phosphatase 1 oz U/L (25-100). Vitamin C level was low at less than 0.12 mg/dl (range 0.20 to 1.90). Total protein, albumin, and total bilirubin levels were normal. Human immunodeficiency virus antibodies, syphilis immunoglobulin G, anti-nuclear antibody, anti-centromere antibody, and rheumatoid factor were negative. Abdominal ultrasound showed a suggestion of early cirrhosis without varicies, ascites, or evidence of portal hypertension.
There is a sparse mononuclear cell infiltrate with occasional dilated blood vessels within the superficial dermis. A Leder stain fails to show a proliferation of mast cells.
Generalized essential telangiectasia (GET) is a rare condition that is characterized by the development of widespread telangiectases on the skin with a gradual progression to involve additional areas of the body over time [1, 2, 3, 4]. It is comprised of postcapillary venule dilation in the upper dermis . GET most commonly occurs in Caucasian women, aged late thirties to late forties, and presents first on the lower limbs with slow progression proximally to involve the trunk [1, 3-6]. The eruption usually is asymptomatic. However, mild pruritus, numbness, tingling, or burning may occur [1, 6]. Usually there is no mucosal involvement, but some cases have shown conjunctival telangiectases and/or oral mucosal telangiectases [5, 6]. Importantly, there are no other skin lesions that precede the eruption or follow it [6, 7]. One patient has been reported with involvement of GET in a surgical scar, which suggests that GET may target scars . Classically, there is no bleeding from the lesions, no associated changes in pigment, and no purpura or atrophy; the presence of these features may suggest alternative diagnoses [6, 8].
Recently, there have been reports of systemic symptoms associated with GET, although they have only been associated with hereditary hemorrhagic telangiectasia (HHT) or other systemic disease processes . Watermelon stomach, which is a rare vascular condition of the gastric mucosa that is comprised of gastric vascular ectasia with dilated capillaries within the gastric mucosal folds, has been reported in two patients with GET. These findings may suggest that vasculopathic changes in GET extend beyond purely cutaneous manifestations as was originally thought [2, 6]. Importantly, these cases include gastrointestinal bleeding events, which have typically only been associated with HHT and is a new important association.
The pathogenesis of GET is unknown [7, 9]. One author speculated that a case of GET may have been mediated by a vitamin C deficiency secondary to a restrictive diet for colitis, which produced down-regulation of collagen production . Interestingly, our patient also had a low vitamin C level but was without any other features of vitamin C deficiency syndromes or scurvy. Histopathologic features include dilated postcapillary venules with thin walls in the superficial dermis. There are usually no dermal or epidermal abnormalities and a superficial, perivascular, inflammatory infiltrate of lymphocytes may be present [1, 4, 5, 7]. The diagnosis of GET is a clinical diagnosis after exclusion of systemic causes of telangiectases. The differential diagnosis includes systemic or localized arteriovenous malformations, telangiectases related to autoimmune diseases (dermatomyositis, systemic lupus erythematosus, and systemic sclerosis), medications, prior radiation therapy, cirrhosis, syphilis, HHT, ataxia telangiectasia syndrome, and telangiectasia macularis eruptiva perstans [2, 5]. A clinically similar entity, cutaneous collagenous vasculopathy, is characterized by diffuse, progressive telangiectases that mimic GET. The histopathologic features are distinct with thick, periodic acid-Schiff positive hyaline walls around the ectatic superficial capillaries because of collagen deposition [10, 11, 12]. An important differentiation must be made between GET and HHT with the latter being characterized by a positive family history, autosomal dominant inheritance, cutaneous and mucosal telangiectases, and visceral involvement with recurrent episodes of bleeding, most commonly epistaxis .
Importantly, our patient has a history of chronic hepatitis C virus infection, status post treatment with interferon. Therefore, the possibility of liver disease must be considered as a cause of his telangiectases. The cutaneous vascular signs of liver disease appear as spider angiomas (also known as spider nevi) with central red spots and radiating telangiectases . This lesion is distinct from the web-like sheets of GET. Our patient had no other stigmata of liver disease, such as palmar erythema, jaundice, hyperbilirubinemia, asterixis, or pruritus. Our patient’s transaminases remain mildly elevated and an abdominal ultrasound did not show evidence of portal hypertension. Therefore, we do not believe that our patient’s skin findings are related to his history of hepatitis C virus infection. One additional patient has been described in the literature with a diagnosis of GET and hepatitis C virus infection, who also had one isolated spider angioma of the ear . Our patient has no evidence of portal hypertension, which may result in vascular lesions of the skin that also were not found in the other reported case .
GET is difficult to successfully treat, owing to the generalized distribution of involvement as well as the recurrent or progressive nature of the telangiectases. There have been reports of success with tetracycline, minocycline, and doxycycline. However, a recent report showed no improvement with a 12-week course of tetracycline therapy [1, 4, 5]. In one patient with GET and elevated urinary levels of matrix metalloproteinases (MMPs), a trial of doxycycline was initiated since tetracyclines inhibit MMPs. However, a response to therapy could not be appreciated . Other successful treatment modalities include acyclovir and ketoconazole, although these results have not been replicated in recent reports . Sclerotherapy with hypertonic saline resulted in some improvement in one patient, but therapy was not continued owing to the pain of the injections . Others have speculated that the condition is furthered by hypostasis in the limbs and noted that compression stockings may halt the progressive spread of lesions [1, 8]. Vascular laser therapy also has shown successful cosmetic results [1, 3]. A patient was successfully treated with a frequency-doubled, long-pulse 532 nm Nd:YAG laser with nearly complete resolution of the telangiectases after six treatment sessions; only small, relapsing areas were present at a six-month follow-up examination .
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