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Generalized essential telangiectasia (GET) is a rare, clinically benign condition but a source of cosmetic concern for affected patients. There is a dearth of publications and known treatment options for GET. This case report reviews the clinical course of a 54-year-old woman who presented with a long-standing history of telangiectatic patches on her dorsal feet and ankles with progressive spread to the lower extremities consistent with GET. The patient proceeded with two pulsed dye laser (PDL) treatments and had complete resolution of her skin findings maintained at her 1.5-year follow-up appointment.

Cutaneous collagenous vasculopathy (CCV) is a rare idiopathic microangiopathy affecting superficial dermal blood vessels. It is characterized by asymptomatic, disseminated, telangiectatic macules that typically involve the extremities. We report the case of a 58-year-old woman who presented with widespread telangiectasias of three years' duration on the upper extremities and abdomen. Cutaneous collagenous vasculopathy may be underdiagnosed as it is often mistaken for disorders that are morphologically very similar, including generalized essential telangiectasia, hereditary hemorrhagic telangiectasia, and pigmented purpuric dermatosis. The diagnosis of CCV is made histologically and findings feature dilated superficial dermal vessels with thickened walls containing periodic acid-Schiff-positive eosinophilic hyaline material. Effective treatment options for CCV have yet to be established and given its benign disease course, treatment may not be necessary. Pulsed dye laser can be offered to patients for cosmetic improvement but the extent of the disease makes complete lesion clearance challenging in most cases. Given the lesions were asymptomatic our patient declined all offered treatment modalities.

The late development of symmetrical, ascending telangiectasias over an extensive area of the skin with no associated systemic manifestations is a common presentation of generalized essential telangiectasia (GET). It was recently suggested that cutaneous collagenous vasculopathy (CCV) is clinically identical to GET but that the 2 conditions can be distinguished by their distinctive histopathologic findings. We present 2 patients, both women, with multiple telangiectasias and describe the histopathologic findings that led to the diagnoses of GET and CCV. Dermoscopic findings in both cases were similar, except that the older telangiectasias in the patient with CCV were violaceous and distributed in a tortuous, serpentine pattern. During follow-up 12 years for the woman with GET and 42 years for the woman with CCV we saw that in GET the lesions remained stable in appearance whereas in CCV there was progressive darkening and morphological changes eventually resulting in superficial varicose veins.

A 14-year-old boy presented with progressive incoordination and cognitive issues. Family history was negative for any neurological condition. Examination revealed cerebellar ataxia and cognitive impairment as well as prominent ocular conjunctival telangiectasia (figure), which made ataxia-telangiectasia, a recessive disorder, a plausible diagnostic hypothesis. Serum alpha-fetoprotein levels were however normal. At follow-up, he was accompanied by his father, who was found to have significant gait ataxia – warranting genetic testing to assess for an autosomal dominant spinocerebellar ataxias. Abnormal CAG repeats in the ATXN3 gene were identified in the pathogenic range(31/52 CAG repeats) consistent with spinocerebellar ataxia type 3/ Machado-Joseph disease (SCA3). Ocular conjunctival telangiectasia can be found in different diseases, including ataxia-telangiectasia (ATM syndrome), generalized essential telangiectasia, with conjunctival involvement, and hemorrhagic telangiectasia of Rendu-Osler, among others1,2. Ocular conjunctival telangiectasia can be found in association with cerebellar ataxia, even though they may not share a common cause.

Cutaneous collagenous vasculopathy (CCV) is a rare benign microangiopathy of the superficial dermal vessels. Clinically, it is characterized by widespread, asymptomatic development of cutaneous telangiectasia in the absence of systemic symptoms. Morphologically, it most resembles generalized essential telangiectasia and other telangiectatic syndromes such as telangiectasia macularis eruptiva perstans (TMEP), ataxia telangiectasia, and hereditary hemorrhagic telangiectasia. It is distinctive in its histology, showing characteristic dilated thick-walled blood vessels in the superficial dermis. The thickened walls of these superficial dermal blood vessels demonstrate reduplication of the basement membrane on PAS staining. We report a 63-year-old man with CCV with this condition for 20 years, starting in 1996. He was diagnosed in the past as having essential telangiectasia. The development of the telangectasias occurred after coronary artery bypass grafting, also performed in 1996. This case not only demonstrates the characteristic clinical and histologic findings, but also suggests a possible mechanism. Moreover, it illustrates that cases of generalized essential telangiectasia may in fact be CCV that are misclassified.

The pathophysiology of generalized essential telangiectasia is not well understood. Generalized essential telangiectasia is an uncommon disorder in which widespread telangiectasias of unknown cause develop without associated systemic or antecedent dermatologic disease. We report a case of generalized essential telangiectasia in an otherwise healthy 49-year-old man.

Cutaneous collagenous vasculopathy (CCV) is a distinct, rare, and underdiagnosed condition. We report a case of CCV in a 50-year-old woman presenting as asymptomatic, erythematous to hyperpigmented nonblanchable macules over both the lower extremities. The clinical differential diagnosis of the lesions was pigmented purpuric dermatoses (Schamberg's purpura) and cutaneous small vessel vasculitis. Histology of the lesions revealed dilated superficial dermal vessels with abundant pink hyaline material in the vessel wall, which stained with periodic acid Schiff stain. The patient was diagnosed as CCV. This condition remains largely underdiagnosed and is commonly mistaken for pigmented purpuric dermatosis or generalized essential telangiectasia. Emphasis on the differentiation of CCV from its clinical and histological mimicks is made.

Cutaneous collagenous vasculopathy (CCV) is a rare cutaneous microangiopathy that clinically resembles generalized essential telangiectasia with only 12 cases reported to date. The perivascular fibrosis is thought to be due to production of abnormal collagen by veil cells in the outer vessel walls as a result of unknown factors. This report is of an 84-year-old male with progressive telangiectasia. Biopsies showed characteristic features of CCV. In addition, there were multiple intravascular fibrin thrombi, some organizing and associated with endothelial cell hyperplasia with recanalization reminiscent of glomeruloid bodies and simulating reactive angioendotheliomatosis (RAE). Histochemically and ultrastructurally fibrin was noted within the vessel walls integrating into the fibrous tissue around the vessels; however, the patient had no evidence of coagulation disorder, cryoglobulinemia or cold agglutinemia. Immunofluorescence showed fibrinogen within the vessel walls but no immunoglobulins or C3. As well, there were minimal inflammatory cells. This suggests pauci-inflammatory injury to the endothelial cells by unknown angiogenic factors causing local intravascular fibrin thrombi with fibrin leaking and incorporating into the vessel walls, eventually leading to reparative perivascular fibrosis. This case suggests that some cases of CCV are related to a primary local intravascular occlusive thrombotic microangiopathy. However, the primary triggering factor causing the endothelial cell damage has yet to be elucidated.

Cutaneous collagenous vasculopathy is a rare microangiopathy of superficial dermal blood vessels. Patients present with telangiectatic macules, predominantly on the extremities. A skin biopsy specimen is necessary to distinguish cutaneous collagenous vasculopathy from generalized essential telangiectasia. Microscopically, cutaneous collagenous vasculopathy resembles the superficial telangiectasias of generalized essential telangiectasia but additionally shows hyaline material in thickened vessel walls. The amorphous pink material is periodic acid-Schiff-positive and resistant to diastase. We describe a series of four patients with cutaneous collagenous vasculopathy and highlight its clinical and histopathologic features.

Cutaneous collagenous vasculopathy (CCV) is an entity first described in 2000 by Salama and Rosenthal [1]. Because of its rarity, we felt it opportune to report the present case.

Cutaneous collagenous vasculopathy (CCV) is an idiopathic microangiopathy with characteristic histological findings. It was described in 2000, and 9 cases have since been described. Two women of 83 and 74 years consulted for long-standing telangiectasias. In case 1, they affected the limbs and trunk and in case 2 were located on the legs. Biopsies of these lesions showed dilated vascular structures whose walls were thickened due to deposition of eosinophilic hyaline material. The affected vessels were located in the superficial dermis in case 1, and in case 2 the reticular dermis was also affected. CCV is a microangiopathy of unknown etiology. Clinically it is indistinguishable from generalized essential telangiectasia and differs in its histology. CCV may be underdiagnosed, and some nonbiopsied cases of generalized essential telangiectasia may really be CCV. We contribute 2 new cases of this entity to help establish its clinical and epidemiological characteristics and make its etiology better known.

Cutaneous collagenous vasculopathy is characterized by generalized cutaneous telangiectasia and unique microscopic and ultrastructural vascular changes, consisting of marked collagen deposition within the vascular walls of the post-capillary venules in the superficial dermis. There are only 4 previous cases described in the medical literature, all in males, mostly middle-aged. We have recently seen two female patients with clinical and histopathologic features diagnostic of cutaneous collagenous vasculopathy, indicating that it is not restricted to males. As cutaneous collagenous vasculopathy can be clinically indistinguishable from generalized essential telangiectasia, and histopathologic studies are rarely performed for this condition, it is likely that cutaneous collagenous vasculopathy frequently passes unrecognized, but it may be more common than previously thought.

Summary Generalized essential telangiectasia (GET) is a rare skin condition of unknown aetiology. We report a case of a 39-year-old man who presented to the ophthalmology department with reduced vision, and was diagnosed with generalized essential telangiectasia by indocyanine green and fluorescein angiography of his eyes. The patient was noted to have corneal neovascularization (which was responsible for his reduced visual acuity) and conjunctival telangiectases seen by angiography. Further examination by the dermatology department identified widespread telangiectases on the patient's legs and trunk. Systemic causes and alternative diagnoses were excluded by further investigations, and the patient was eventually diagnosed with GET. Following discharge from the dermatology department, the patient presented with chest pain and required emergency surgery for a type A thoracic aortic dissection. Previous research has not identified an association between GET, corneal neovascularization and thoracic aortic aneurysm formation.

Cutaneous collagenous vasculopathy (CCV) is a rare distinct idiopathic microangiopathy of the superficial cutaneous vasculature. Seven new cases are reported (6 females and 1 male) ranging in age from 42 to 85 years, with some showing unusual clinical and histopathological findings. All presented with macular telangiectases starting on the lower extremities and spreading progressively in 5 cases and were suspected to have generalized essential telangiectasia. Two cases had a history of over 20 years. One case had lesions in the abdominal striae, and 1 was markedly ecchymotic. All skin biopsies showed the characteristic features of CCV with dilatation and marked thickening of the walls of superficial dermal blood vessels displaying reduplication of the basement membrane on periodic acid-Schiff-diastase stain and deposition of hyaline collagenous material immunostaining as collagen type IV, and showing decreased or absent actin staining. However, the changes were subtle and only seen focally in some biopsies. Few lymphoid cells were present around occasional vessels. Electron microscopy showed increased basement membrane lamellae with marked deposition of normal and some abnormal collagen (Luse-like bodies) and focal endothelial damage, suggesting reparative perivascular fibrosis resulting from repeated endothelial injury. These cases (and all 18 previously reported ones) are of a wide age range and no gender predilection. This disorder is underdiagnosed, and it is likely that some cases clinically suspected to be generalized essential telangiectasia may actually represent CCV. Better recognition by dermatologists may lead to more biopsies from patients with generalized telangiectasia and a further understanding of the pathogenesis of CCV and its relationship to other cutaneous vascular disorders.

We report a 54-year-old male, with a 5-year history of spreading asymptomatic generalized cutaneous telangiectases. The patient had no mucosal or nail involvement, no positive family history and no clinical evidence of systemic disease or bleeding diathesis. Histologically, the superficial small dermal blood vessels were dilated and showed thickened walls with hyaline perivascular material, staining as collagen. The vessel walls were PAS and colloidal iron stain positive, and immuno-histochemically lacked actin staining. Collagen IV, fibronectin and laminin antibodies showed the material deposited around the basement membrane zone. Ultrastructurally, the vessels were post-capillary venules (PCV) and showed marked collagen deposition around the basal lamina. There were many abnormally banded widely spaced fibres with 100-150 nm periodicity (Luse bodies), in addition to regular banded collagen. Pericytes were sparse and lacked intracytoplasmic filaments, and few veil or fibroblastic cells were seen embedded within the collagen. We believe this is a form of cutaneous microangiopathy not previously described, with distinct morphology and unique ultrastructural features. It may be due to a genetic defect with erroneous production of disorganized collagen in the cutaneous microvasculature. Dermatologists and Dermatopathologists should be aware of this unusual cutaneous vasculopathy.

A case report of a 12-year-old spayed female Golden Retriever with a 4-month history of persistent diffuse erythema involving the right and left antebrachia is presented. Cutaneous biopsies revealed superficial dermal vascular dilation and proliferation with moderate epidermal acanthosis. Diagnostic tests failed to reveal an underlying aetiology. Telangiectasia is defined as a permanent dilation of pre-existing blood vessels creating small focal lesions. Generalized essential telangiectasia of humans is common in women of middle-age and can involve entire segments of the body. Lesions predominantly affect the extremities and can persist for years without any systemic effects. The cutaneous lesions in this case have remained static for 3 years with no manifestation of systemic signs. A diagnosis of idiopathic telangiectasia was made based on the clinical and histological findings in the dog of this report. The lesions described in this dog resemble generalized essential telangiectasia of humans.

Background Widespread idiopathic telangiectasia (generalized essential telangiectasia) is a rare skin disorder characterized by the development and gradual spreading of telangiectases. The condition tends to affect women in their midthirties. For no apparent reason telangiectases start to appear to the lower extremities and progress steadily to involve the skin of the trunk, the arms, and the face. General health is not affected by the condition and standard laboratory tests consistently yield normal results. Case report In February 1997 a 78-year-old lady was admitted for treatment of cataracta corticonuclearis of her left eye. Complete ophthalmological and dermatological examinations were performed. She presented marked conjunctival telangiectases of both eyes and widespread cutaneous telangiectases involving her face, trunk, arms, and legs. Complete blanching of lesional skin was observed on diascopy. The Rumpel-Leede-test was normal. Cutaneous and conjunctival changes appeared not to be associated with internal disease or bleeding abnormalities. Discussion The patient presented here shows widespread idiopathic telangiectasia with marked conjunctival involvement. Ocular changes rarely have been reported in patients with generalized essential telangiectasia to date. Concomittant conjunctival and cutaneous telangiectases may be seen in other conditions such as hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease) and ataxia telangiectasia (Louis-Bar syndrome). The former shows an associated bleeding abnormality and is transmitted autosomal dominantly. The latter presents associated neurological signs such as cerebellar ataxia, strabism, nystagmus, apraxia, and mental retardation.

Generalized essential telangiectasia was well defined more than 30 years ago. There have been no reported cases of associated gastrointestinal (GI) bleeding. Recurrent hemorrhage in the setting of telangiectases, including GI bleeding, is more typically associated with hereditary hemorrhagic telangiectasia. We report a unique case of a woman with generalized essential telangiectasia and GI bleeding from a watermelon stomach. We include a brief review of the literature of watermelon stomach, generalized essential telangiectasia, and hereditary hemorrhagic telangiectasia.

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.

ejd.2011.1404 Auteur(s) : Aysegul Turan1 draysegulgocer@yahoo.com, Hayriye Saricaoglu1, Emel Bulbul Baskan1, Murat Keskin2, Saduman Balaban Adim3, Hakan Turan4, Sukran Tunali1 1 Uludag University Medical Faculty, Dermatology Department, Bursa, Turkey 2 Uludag University Medical Faculty, Gastroenterology Department, Bursa, Turkey 3 Uludag University Medical Faculty, Pathology Department, Bursa, Turkey 4 Duzce University Medical Faculty, Dermatology Department, Duzce, Turkey Unilateral nevoid telangiectasia [...]

Background. Generalized essential telangiectasia is a rare cutaneous disorder with limited therapeutic options. Objective. To evaluate the treatment with high-energy, high-frequency, long-pulse Nd:YAG laser in a patient with generalized essential telangiectasia. Methods. A 62-year-old woman presented with a 20-year history of generalized essential telangiectasia. She had extensive telangiectasias primarily on the forearms and lower legs. The patient was treated with an Nd:YAG laser using a fluence of 20 J/cm2 and a pulse width of 20 msec. A sapphire water-cooled chill tip was used to cool the skin during laser therapy. The treatment was performed every 4 weeks over a period of 6 months. Results. After six treatment sessions an almost complete clearance of telangiectatic lesions was observed. Side effects such as purpura, scarring, and pain did not occur. After a follow-up of 6 months, small telangiectatic lesions relapsed. Conclusion. Our results suggest that the Nd:YAG laser appears to be an effective and safe treatment option for generalized essential telangiectasia.

Cutaneous collagenous vasculopathy is an idiopathic microangiopathy first described in 2000 by Salama and Rosenthal.It must not be confused with generalized essential telangiectasia. To date, all patients have been white men over the age of 50 years, most of whom had multiple pathologies, were taking multiple drugs, and had no family history of similar conditions or hemorrhagic disorders. The disease is characterized by the development of various numbers of telangiectases on the limbs, lower abdomen, chest, or back, with no involvement of the mucosas or nail bed. Histopathology shows dilated superficial cutaneous vessels with perivascular deposits of periodic acid-Schiff diastase-positive, eosinophilic hyaline material that exhibits positive immunoreactivity to collagen IV. We report a new case in a 68-year-old man with symmetrically distributed telangiectases on his forearms, lower abdomen, posterior thighs, lower legs, and dorsum of the feet.

Cutaneous collagenous vasculopathy is a very rare entity first described in 2000, manifesting clinically with acquired, progressively diffuse, cutaneous telangiectases with a histologically distinct aspect, characterized by a thick hyaline collagenous wall. Its cause is unknown. Differential diagnosis includes mainly generalized essential telangiectasia and telangiectatic mastocytosis. We report a 65-year-old Caucasian patient who presented with progressive telangiectasia starting over the lower limbs that spread progressively upwards. Histopathologic (light and electron microscopic) examinations were typical of cutaneous collagenous vasculopathy. We review the salient clinicopathologic features of this poorly known condition.

Cutaneous collagenous vasculopathy (CCV) is an idiopathic microangiopathy involving the superficial blood vessels that was initially reported in a 54-year-old male. We recently have identified this rarely reported entity in three Caucasian males. The first patient was a 59-year-old male with diabetes, hypertension and hypercholesterolemia who presented with multiple, red, blanchable, asymptomatic telangiectasias covering the extensor surface of the forearms, the lower abdomen and parts of the chest. The second patient was a 62-year-old male with psoriasis and extensive arthritis who presented with prominent telangiectasias on the left lateral distal thigh with mild overlying epidermal atrophy. The third patient was an 80-year-old male with atrial fibrillation who presented with blanching, telangiectatic areas on the abdomen, thighs and back. Histologically, the skin lesions showed ectatic superficial small blood vessels with laminated, hyalinized concretions around vessels that were highlighted with periodic acid-Schiff staining following diastase digestion and reactive by immunohistochemical staining with an antibody to collagen type IV. CCV is a rare and poorly understood entity with distinct histopathological features that may clinically resemble generalized essential telangiectasia (GET), yet which may affect a different demographic population than GET. Awareness of this uncommon entity may further help to elucidate its etiology.

A 56-year-old woman presented with multiple, erythematous cutaneous vessels that blanched with diascopy and were symmetrically distributed over both lower and upper extremities. The lesions had been present for approximately 6 years and were asymptomatic. Generalized essential telangiectasia (GET) is an idiopathic syndrome of widespread, asymptomatic telangiectases of unknown cause. In our patient, an extensive laboratory evaluation showed low levels of circulating vitamin C as well as the presence of urinary matrix metalloproteinases. The relevance of these abnormal findings is unclear. No uniformly effective treatment exists for GET. Our patient was started on oral doxycycline and was referred for laser therapy.

Generalized essential telangiectasia (GET) is a rare skin disorder characterized by generalized development of dilated venules, which start at the lower extremities and progressively spread to the rest of the body. Mucous and conjunctival involvement is rare. The diagnosis is based on clinical examination, after excluding other primary and secondary causes of telangiectasia.

A 43-year-old woman presented with a 1-year history of telangiectatic lesions on her lower legs. The lesions progressively spread to involve her thighs and left arm. She had no family or personal history of recurrent haemorrhage or telangiectases. Her general health was unaffected. The diagnosis of generalized essential telangiectasia was made on the basis of clinical presentation and investigations. Generalized essential telangiectasia is a rare condition that can only be diagnosed if other primary and secondary telangiectasias are excluded. It is probably under-reported, with patients mistakenly diagnosed with atypical hereditary haemorrhagic telangiectasia.

The cutaneous microvasculature is organized into upper and lower horizontal plexuses with the dermal capillary loops arising from the upper plexus. The arteriolar and venular sides of the microvasculature can be identified by the ultrastructure of the mural basement membrane material. Collecting venules present in the lower dermis contain valves. Periadventitial cells (veil cells) are present around all microvessels. Their size and number appear to correlate with the quantity of mural basement membrane material found in cutaneous vessels in diabetes, actinic damage, and chronological aging. The contractile cells of the vascular wall surround the endothelial cell tube in a manner suggesting specific functions. The smooth muscle cells in the arteriolar segment form a sleeve, whereas each pericyte in the postcapillary venular simultaneously makes many contacts with several un-derlying endothelial cells. The common telangiectases can be explained by abnormalities in this organization and ultrastructure rather than by neovascularization or random anastomoses. The macular telangiectases seen in scleroderma, generalized essential telangiectasia, and nevus flammeus are produced by dilatation of the postcapillary venules of the upper horizontal plexus. Cherry angiomas are produced by spherical and tubular dilatations of capillary loops in dermal papillae with tortuous cross-connections between individual loops. Angiokeratomas of Fabry and Fordyce have the ultrastructure of collecting venules that contain valves, and appear to represent the ectopic development or placement of small valve-containing collecting veins. The cutaneous lesions of hereditary hemorrhagic telangiectasia represent arteriovenous communications. J Invest Dermatol 93:2S–9S, 1989

Eight types of telangiectases were studied by light and electron microscopy and by 3-dimensional reconstruction from photomicrographs. Five were macular: mat telangiectasia of scleroderma, generalized essential telangiectasia, nevus flammeus, and 2 macular types not previously described. Three were papular: cherry angioma, angiokeratoma (Fabry), and angiokeratoma (Fordyce). The macular telangiectases were produced by dilatation of postcapillary venules of the upper horizontal plexus. There was no evidence of neovascularization or vascular malformation. The walls of the dilated venules were thickened by the peripheral deposition of basement membrane-like material admixed with reticulin fibers. The ultrastructure and configuration of the papular telangiectases were different. The cherry angioma was produced by spherical and tubular dilatations of capillary loops in dermal papillae. Each abnormally dilated loop was connected to the neighboring loop or loops by tortuous vascular channels. The vessels in the upper horizontal plexus were not involved. Ultrastructurally, the cherry angiomas were composed of both venous capillaries and postcapillary venules whose walls were thickened in a manner identical to that observed in the macular telangiectases. The angiokeratomas of Fabry and Fordyce were also produced by vascular abnormalities predominantly involving the dermal papillae. Ultrastructurally these vessels were similar to the small collecting veins which are normally found at the dermal-subcutaneous interface. Thus, the papular telangiectases also arose by alterations of the existing micro-vasculature rather than by proliferation of new vessels with random anastomoses. Reconstruction of the upper horizontal plexus from normal skin showed an undulating network of arterioles and their accompanying postcapillary venules. A 3-layered plexus arranged as venules, arterioles, and venules was not found.

To the Editor.— Generalized essential telangiectasia is an uncommon disorder.1-4It is seen primarily in women,2is sometimes familial,4and the average age at onset is 38 years.2The telangiectasias slowly progress over years or decades2,3and are not accompanied by associated systemic problems.2The telangiectasias vary in pattern and distribution and may respond to tetracycline hydrochloride.2,3 Unilateral nevoid telangiectasia is a similar but segmental disorder5-8that is seen more commonly. It usually occurs in women at puberty, during pregnancy, or while taking oral contraceptives5,6but has also occurred in men with cirrhosis of the liver,6a finding that is reminiscent of the onset of vascular spiders.7It was therefore hypothesized that estrogenic stimulation was involved in the pathogenesis of the disorder.6,8This theory was given considerable impetus in 1983 when Uhlin and McCarty9found a severalfold