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Sneddon syndrome (SNS) is characterizedby the associationof ischaemic cerebrovascularevents and widespread livedo racemosa. Its pathophysiology is still controversial. The aim of this study was to evaluate the prevalence of factor V Leiden mutation in consecutive patients referred for SNS according to antiphospholipid antibodies (aPL) status. Fifty-three Caucasian patients were enrolled from 1996 to 2001. Diagnosis of SNS was based on the presence of a widespread livedo racemosa and at least one clinical neurologic ischaemic event. The following investigations were performed: detection of antithrombin III, protein C and protein S deficiency, lupus anticoagulant, anticardiolipin and anti-b2 glycoprotein I antibodies, biologic false-positive test for syphilis, and factor V Leiden mutation by direct genomic analysis. Fisher’s test and t-test were used for statistics. Detection of aPL on multiple determinationswas negativein 31 patients (group 1) and positivein 22 patients (group 2). Factor V Leiden mutation was detected in six patients (11.3%), heterozygousin all. The frequency of this mutation was statistically higher in group 1 (6/31, 19.3%) than in group 2 (0/22; P 0.035). Within aPL-negative SNS, the comparison of patients with versus without factor V Leiden mutation showed no difference for clinical data or familial history of thrombosis. A high prevalence of heterozygous factor V mutation was found in aPL-negative patients with SNS. This finding adds further arguments to consider SNS as a heterogeneous entity.

Sir, Sneddon’s Syndrome is a rare and poorly understood syndrome characterized by the association of ischemic cerebrovascular events and livedo racemosa [1]. We report a case of a 35-year-old woman with Sneddon’s Syndrome and digital ulcers successfully treated with Bosentan. Patient was admitted to our center with pain and trophic ulcers in both hands and feet, Raynaud’s phenomenon, livedo racemosa on the extremities, headache and memory loss. Her past medical history was notable for Cushing syndrome treated with total hypophysectomy and bilateral adrenalectomy, a history of recurrent miscarriage and hypertension under treatment. Physical examination was normal except for the skin lesions. The search for antiphospholipid antibodies [anticardiolipin (aCL), anti-beta2GPI and lupus anticoagulant (LA)], antinuclear autoantibodies (ANA), anti-extractable nuclear antigens (ENA) and anti-double-stranded DNA (anti-dsDNA) was negative. Cerebral MRI showed multiple subcortical and periventricular lesions suggestive of previous ischemic events. In the light of these findings, a carotid ultrasonography and an echocardiography were performed to rule out micro embolism. Echocardiography showed a partial thickening of the mitral valve. Livedo racemosa was confirmed by a skin biopsy showing a light leukocyte infiltrate and capillary dilatation. The diagnosis of Sneddon’s Syndrome was made. Due to the lack of ulcer’s healing with conventional therapies we decided to replace iloprost pulse therapy with Bosentan treatment. Bosentan is a dual endothelin-1 (ET-1) receptor antagonist with affinity for both ET-1 A and ET-1 B receptors. ET-1 is one of the most powerful vasoconstrictor agents [2], able to induce fibrosis and cellular proliferation. These effects were mediated by the ET-1 binding on both receptors in vascular smooth muscle cells. Bosentan has been approved by the European Medicines Agency (EMA) for the prevention of new digital ulcer formation in Systemic Sclerosis (SS) [3]. A recent study on patients with SS demonstrated a 30 % reduction of new digital ulcers during treatment with Bosentan but no healing of active ulcers was observed [4]. In our patient, a few weeks after the start of Bosentan administration, ulcers rapidly healed, with an important reduction of pain and livedo (Fig. 1). Clinical conditions remained good and no new ulcers developed over the next 6 months. Fig. 1 Patient’s livedo and trophic ulcers before (left) and after (right) Bosentan treatment To our knowledge, this is the first case of Sneddon’s Syndrome treated with Bosentan; in contrast to that reported in SS, Bosentan led to a complete healing of pre-existing cutaneous ulcers. The success of this anti-endothelial proliferative drug supports the hypothesis [5] that an endothelial proliferation could be the main pathophysiological mechanism of this obliterative vasculopathy.

ulcerations. Cutaneous nodules (100.0%) were the most common skin manifestations and were observed in all of our patients with CPN. Forty (88.9%) of the patients with CPN had livedo racemosa, and 33 (73.3%) had purpuric lesions on their lower extremities. Twenty-seven (60.0%) of the patients with CPN were positive for serum CRP, and LAC activity was observed in 27 of them (60.0%). Nineteen (42.2%) of the patients with CPN had elevated serum IL-6 levels and 29 (66.4%) had elevated serum IL-8 levels, while only 4 (8.9%) had elevated serum TNF-α levels. Seven (15.6%) were positive for serum IgG anti-PS/PT antibody, 33 (73.3%) for IgM anti-PS/ PT antibody, and 6 (13.3%) for IgG aCL antibody. DIF studies were performed on skin biopsy samples from 29 of the patients with CPN, and revealed complement 3 expression within affected vessels of the lesions in 21 (72.4%) of them. In addition, 18 (62.1%) of those 29 patients with CPN showed IgM deposition within affected vessels. Thirty-four (75.6%) of the patients with CPN were treated with warfarin, and prednisolone therapy was administered to 27 (60.0%) of them. We divided the 45 patients with CPN into two groups; the elevated IL-6 group and the normal IL-6 group (Table I). The number of men in the elevated IL-6 group was significantly higher than in the normal IL-6 group. Patients with elevated IL-6 had a significantly higher frequency of arthralgia compared with patients without elevated IL-6. Skin ulcerations were also significantly more prevalent among patients with elevated IL-6 compared with patients without elevated IL-6. Elevated IL-6 patients had significantly higher CRP serum levels than normal IL-6 patients. LAC in the elevated IL-6 group was significantly more prevalent than in the normal IL-6 group. Serum IgG anti-PS/PT antibody levels differed significantly between the elevated IL-6 group and the normal IL-6 group. Similarly, serum IgG aCL antibody levels were significantly higher in the elevated IL-6 group compared with the normal IL-6 group. In contrast, IgM anti-PS/PT antibody levels in patients with CPN with elevated IL-6 were significantly lower than in normal IL-6 level patients with CPN. The selected prevalence of prednisolone in the elevated IL-6 group was significantly higher than in the normal IL-6 level group.

A 55-year-old woman presented with a 5-year history of livedo racemosa on her limbs. Histology showed vasculitis of medium-sized arteries with a circumferential, hyalinised, intraluminal fibrin ring. Her laboratory investigations did not indicate any underlying systemic disease. The findings were consistent with lymphocytic thrombophilic arteritis (LTA), alias macular arteritis, which is a recently described entity. The importance of LTA lies in the fact that it is a close clinical and microscopic mimic of polyarteritis nodosa (PAN). LTA is believed to be a distinct entity by some and as a form of PAN by others. We have discussed this case in our report.

We report a multimorbid patient with end-stage renal failure showing a large necrosis and livedo racemosa on the right thigh. Histology revealed medial calcification of the small arteries typical of calciphylaxis. We found the typical features of the disease with different risk factors like elevated calcium-phosphate product, diabetes mellitus and oral anticoagulation. On account of the location of the skin lesions, a bad prognosis was expected. In spite of therapeutical measures with lowering of the calcium and phosphate levels, the patient died 1 month after the diagnosis had been made.

Sneddon syndrome is characterized by the association of livedo reticularis and cerebral ischemic arterial events (stroke or transient ischemic attack). Reported prevalence of antiphospholipid antibodies is highly variable. We conducted this study to compare the clinical and pathologic features of patients with Sneddon syndrome according to the presence or absence of antiphospholipid antibodies. Forty-six consecutive patients with Sneddon syndrome were analyzed. All were examined by the same dermatologist who classified the livedo of the trunk according to the regularity of the fishnet reticular pattern and according to the thickness of the fishnet reticular pattern (> or = 10 mm = large; < 10 mm = fine). Skin biopsies were systematically performed, from both the center and the violaceous netlike pattern in 38 patients. Antiphospholipid antibodies-positive Sneddon syndrome was defined by the presence of lupus anticoagulant or abnormal titers of anticardiolipin antibodies on repeated determinations. Group I consisted of 27 antiphospholipid antibodies-negative patients and Group II, of 19 antiphospholipid antibodies-positive patients. All patients except I in Group II had irregular livedo reticularis. Large livedo racemosa was more frequently observed in Group I (89%) than in Group II (21%, p < 0.001). On skin biopsy, arteriolar obstruction was detected in only 8 patients (4 in each group). The following parameters were not statistically different between the 2 groups: gender, mean age at detection of livedo, mean age at first clinical cerebral event, hypertension, Raynaud phenomenon, patients with extracerebral and extracutaneous arterial or arteriolar thrombosis or stenosis, patients with venous thrombosis, and women with 2 fetal losses or more. In contrast, seizures (11% in Group I versus 37% in Group II, p < 0.05), mitral regurgitation on echocardiogram (19% versus 53%, p = 0.02), and thrombocytopenia < 150,000/muL (0% versus 42%, p < 0.005) were more frequently observed in Group II. The number of events per year of follow-up was lower with antiplatelet therapy (0.08 versus 0.5) in Group I, but was not different with anticoagulation (0.056 versus 0.06). Antiphospholipid antibodies-negative and -positive patients with Sneddon syndrome belong to close but different subsets of Sneddon syndrome.

Sirs: A 46-year-old woman was referred to our hospital because of suspected cerebral ischemia. Two days earlier the patient had recognized a left-sided weakness and clumsiness. On neurological examination we found a mild left-sided hemiparesis and hemiataxia. There was a generalized shrinking violaceous netlike pattering of the skin especially on both legs and arms but also on the trunk and buttocks (Fig. 1). The patient reported the skin changing to be more prominent on cold exposure. The patient’s family remembered this skin finding to be evident since the age of five years. A diagnosis of livedo racemosa had been made 5 years ago. The neuropsychological assessment of this highly educated civil servant revealed a slight cognitive decline. MRI showed a right-sided cerebral ischemia in the middle cerebral artery (MCA) territory. Her medical history was significant for migraine-like headache for many years, a miscarriage 18 years before and a deep vein thrombosis of the left leg six years ago. She had no history of smoking or other cerebrovascular risk factors including no estrogen-containing oral contraceptives. The patient underwent intensive examinations including duplex sonography of extraand intracranial arteries, transesophageal echocardiography, 24-h ECG, 24-h blood pressure monitoring, multimodal evoked potentials, electroencephalography, lumbar puncture and sonography of abdomen. All these tests were negative. Extensive laboratory examinations revealed a heterozygote prothrombin 20210 mutation, which is associated with a slightly increased risk for thrombosis. Antiphospholipid antibodies (aplAB) and other laboratory examinations to exclude vasculitis, toxic metabolic disturbances and other causes for livedo racemosa were negative. Skin biopsy showed vasculopathy with intimal proliferation and an occluding thrombus. The patient was diagnosed as having antiphospholipid-antibodynegative Sneddon’s syndrome (SS) based on cerebral ischemia combined with wide-spread livedo racemosa associated with a history of miscarriage, deep vein thrombosis, migraine like headaches and mild cognitive decline. We started long-term prophylactic pharmacological therapy with captopril as a myocyte proliferation agent and with aspirin as an antiplatelet therapy. Furthermore we recommended thrombosis prophylaxis in case of immobilization. One month later the patient experienced vein thrombosis of her right forearm and suffered from dyspnea. Antiphospholipid antibody testing again was negative. EBT and CT of thorax showed an aneurysmatic dilatation of aorta ascendens up to 4.5 cm. After careful consideration of the possible disadvantages we nevertheless decided to start long-term anticoagulation instead of antiplatelet therapy because of the second thrombotic event. The elucidating and interesting issue of this case is the association of miscarriage and two vein thromboses in aplAB-negative SS. Little is known about this phenomenon and there are only a few reports about these symptoms in aplABLETTER TO THE EDITORS

A 24-year-old man with livedo racemosa and psychiatric disturbances, manifesting as low intelligence (IQ 80) and delusions, had anti-cardiolipin antibody and showed shortening of the fingers and toes. A skin biopsy of the livedo lesion revealed endoarteritis obliterans, being compatible with Sneddon's syndrome. MRI of the brain demonstrated multiple infarction and moderate cortical atrophy. A single photon emission tomography of the brain showed a marked reduction of the blood flow in the front-temporal lobe. These findings might relate to the psychiatric disturbance. After intravenous administration of cyclophosphamide and the start of oral prednisolone, the anti-cardiolipin antibody level decreased and his physical condition improved. However, a low dose of haloperidol is still necessary to maintain his mental condition.

Background : Sneddon's syndrome consists of widespread livedo reticularis and ischemic cerebral manifestations. Its pathogenesis remains unclear. Endothelial cells could be the primary target tissue. Objective : Our aim was to determine the prevalence of anti-endothelial cell antibodies (AECA) in a large series of patients with Sneddon's syndrome. The results were compared with those of three groups of control subjects: 39 patients with active periarteritis nodosa, 20 patients hospitalized for stroke without livedo, and 28 healthy persons. Methods : AECA were detected with enzyme-linked immunosorbent assay with hybrid cells (EA.hy926) before and after absorption on epithelial cells (A 5498) to avoid false positivity from antibodies reacting with membranous epithelial antigens. Results : Twenty-two patients with Sneddon's syndrome had AECA (35%). Of the control subjects, 11 patients with active periarteritis nodosa (28%), 1 of 20 patients with a recent stroke without livedo, and no healthy persons had AECA. Conclusion : AECA were frequently found in patients with Sneddon's syndrome, in contrast to the patients with stroke without livedo. The clinical significance and involvement of these antibodies in the pathogenesis of endothelial lesions in Sneddon's syndrome remain to be ascertained.

Sneddon syndrome (SS) is rare, arterio-occlusive disorder characterized by generalized livedo racemosa of the skin and various central nervous symptoms due to occlusion of medium-sized arteries of unknown. Seizure, cognitive impairment, hypertension, and history of repetitive miscarriages are the other symptoms seen in this disease. Livedo racemosa involves persisting irreversible skin lesions red or blue in color with irregular margins. Usually, SS occurs in women of childbearing age. Protein S deficiency is an inherited or acquired disorder associated with an increased risk of thrombosis. We present a 33-year-old woman with SS with diffuse livedo racemosa, recurrent cerebrovascular diseases, migraine-type headache, sinus vein thrombosis, and protein S deficiency. Protein S deficiency and with Sneddon syndrome rarely encountered in the literature.

Sneddon's syndrome, characterized by generalized livedo racemosa and cerebrovascular lesions, is an underdiagnosed disease. We evaluated clinical, laboratory, histological, and neuroradiological findings in a series of 17 patients to improve diagnostic criteria for Sneddon's syndrome. Patients with generalized livedo racemosa and cerebrovascular events were included in the study. All underwent neurological and dermatological examination, skin biopsy, computed tomographic scan, magnetic resonance imaging as well as magnetic resonance angiography, sonography of the extracranial arteries, and a comprehensive laboratory protocol. Completed stroke was present in eight patients, and 15 reported transient neurological deficits. Magnetic resonance imaging yielded cerebral abnormalities in 16 of 17, whereas computed tomographic scans were abnormal in only 12 of 16 patients. Magnetic resonance imaging revealed more lesions in individual patients than did computed tomography. Magnetic resonance angiography demonstrated patent intracranial vessels in 16 of 17 patients. Skin biopsy showed distinct histopathological findings in all patients. The involved vessels were small to medium-sized arteries at the border between dermis and subcutis. Early inflammatory reactions were followed by subendothelial proliferation and a late fibrotic stage. Laboratory examinations showed impaired creatinine clearance in eight patients, whereas all other laboratory tests, including antiphospholipid antibodies, were normal. In this series, magnetic resonance imaging and skin biopsy were useful for confirmation of the diagnosis of Sneddon's syndrome. Magnetic resonance findings were not specific, but the high sensitivity for detection of asymptomatic brain lesions helped to confirm the diagnosis in patients with transient symptoms. Histological features of skin biopsies were characteristic if appropriate techniques were employed.

The diagnostic and therapeutic problems posed by Sneddon's syndrome are discussed with reference to the case of a 31-year-old pregnant women. This patient developed episodes of cerebral ischemia with multiple neurological deficits in the 24th week of pregnancy. The symptoms were associated with the dermatological signs of livedo racemosa. Delivery by Cesarean section in the 36th week of pregnancy resulted in marked improvement of the neurological signs and symptoms.

SUMMARYWe report a case of Sneddon’s syndrome in a 39-year-old woman who developed recurrent Cerebral ischaemic events associated with a livedo racemosa. We describe the clinical and radiologini features of this rare vasculopathy. We also c°mrnent on the nosological place of the disorder and its possible association with antiphospholipid Antibodies.

We report the case of a patient who had 2 rare diseases, annular atrophic lichen planus (AALP) and Sneddon's syndrome (SNS). This patient had also digital nodules with histological abnormalities suggestive of SNS vasculopathy, which have not been reported so far. AALP is the most rare of all varieties of lichen planus since this case is the third reported to date. The association of livedo racemosa and cerebrovascular disease is the hallmark of SNS, the incidence of which is estimated to be 4 cases per year per million inhabitants. In both diseases, an abnormal production of elastic-tissue-degrading enzymes or a constitutional abnormality of the elastic tissue can be postulated, since SNS is characterized by arteriolar changes with deterioration of the internal elastic lamina and AALP by destruction of the dermal elastic tissue.

It is with great interest that we read the article in the July 1995 issue of theArchivesby Somach et al 1 concerning fatal cutaneous necrosis mimicking calciphylaxis in a patient with type 1 primary hyperoxaluria. This case is remarkable for several reasons. First, this case shows that no clear distinction is often made between livedo reticularis and livedo racemosa. Livedo reticularis is a fine, regular netlike patterning of the skin, usually arising after the extremities have been exposed to cold and clearing after they have been warmed. The classic example is seen in cutis marmorata, but numerous neurohumoral and blood viscosity disturbances such as polyglobulia, cold agglutinin disease, and paraproteinemia may also display this symptom. 2 In contrast, the skin in livedo racemosa displays an irregular netlike pattern with broken circular segments resulting in a seemingly larger pattern that persists when the skin is warmed. The classic example

Fifteen patients with Sneddon's syndrome presenting since 1979 were re-examined. After a neurological examination, an orienting test of mental ability as well as an electroencephalogram were performed in all patients. In 10 of the 15 patients computed tomography of the brain was performed, too. Preceding reports and the results of the present study have been used to discuss the characteristics and prognosis of Sneddon's syndrome.

The authors describe three young female patients with generalized livedo racemosa and cerebral infarction. The radiologic and biopsy findings point to a widespread vasculopathy, involving small and mid-sized arteries. The histologic examination of the temporal artery biopsy in two patients gives additional evidence for a chronic endarteritis as possible underlying cause of this disease entity.This paper presents 3 cases of generalized livedo racemosa and cerebral infarction in female patients ages 27, 39, and 42 years. Livedo racemosa is characterized by a broken, irregular pattern on the skin. It is probably caused by patchy impairment of cutaneous arteriolar circulation, resulting in reflectory venous dilation and stasis of blood. Livedo may accompany diseases such as atherosclerosis, diseases with intravascular occlusion, and collagen disorders, indicating a need for a careful search for an underlying condition. These 3 patients demonstrated several risk factors for atherosclerosis: hypertension (1 patient), oral contraceptive use (2 patients), and smoking (2 patients). The clinical findings in these 3 cases provide support to the theory that a chronic endarteritis obliterans of the small and medium-sized arteries is the underlying cause for the skin and neurologic manifestations in livedo racemosa associated with stroke.

Diagnostic and therapeutic problems of Sneddon's syndrome are reviewed on the basis of the observation of a pregnant 36-year-old female. She had had Hodgkin's disease stage I, curatively treated when she was 23 years old. She developed cerebral ischemic events, initially ascribed to isolated cerebral angiitis, associated with progressive dermatological lesions (generalised livedo racemosa). A temporal artery biopsy did not reveal giant cell angiitis, while the cutaneous arterioles in a biopsy showed marked intimal proliferation without inflammatory cell infiltration. The literature on Sneddon's syndrome is reviewed.

The report concerns a neurological post-traumatic syndrome described by Herman (1937) and consisting of livedo racemosa universalis, pyramidal and extrapyramidal symptoms and speech disorders, combined with epileptic seizures and progressive dementia. The clinical course of the illness in 7 cases of this syndrome is reported with a neuropathological study of 1 of the cases. It is assumed that the neuropathological changes found in this case, in the form of a progressive angio-encephalopathy with the formation of dilated vascular shunts, represents a specific morphological correlate of the clinical syndrome. The pathogenesis of the syndrome is discussed, considering the role of haemodynamic disorders which, it is suggested, may be due to primary traumatic damage to the cerebral vasomotor centres.

Eight patients with diffuse livedo reticularis and cerebrovascular lesions (Sneddon's syndrome) are reported. The disorder was inherited by autosomal dominant transmission in 3 cases. Multiple occlusions in medium-sized arteries were demonstrated by cerebral and hand arteriograms. Digital artery biopsies showed intimal hyperplasia in 7 cases and recanalized thrombosis in one case. Our findings are compared with an extensive review of the literature. Differential diagnosis with other vascular disorders, especially cerebral thromboangiitis obliterans and the corticomeningeal angiomatosis of Divry and Van Bogaert is considered. We conclude that Sneddon's syndrome is a new genetic and progressive arteriopathy, occlusive and noninflammatory, involving medium-sized vessels. The pathogenesis has yet to be elucidated.

We describe two cases of livedo racemosa generalisata with cerebrovascular defects (Sneddon syndrome). The histology is characterized by a proliferation and migration of medial smooth muscle cells in ascending arterioles of the upper subcutis and deep dermis. Migrating smooth muscle cells with a high content of intermediate filaments colonize the sub-endothelial intimal space, with subsequent narrowing of the vessel lumen. Since the discoloration of the skin is provoked by a reactive dilatation of venules, the biopsy should be performed in the adjacent normal-looking skin, taking in the upper subcutis.

Two patients on quinidine Durettes medication developed a photosensitive reaction 48 h after sun exposure. The reaction started with a pronounced oedema, followed by a reticulate daik blue discolouration; purpura developed in the discoloured areas. Patch tests and photo patch tests with quinidine sulphate and with quinidine Durettes were negative. As yet it has not been decided whether the quinidine or another constituent of the quinidine Durettes has caused the reaction.

Idiopathic livedo reticularis*may occasionally be associated with ulcerations, especially of the lower extremities, occurring during the winter months.† It has been assumed that in idiopathic livedo reticularis the effect of cold on the blood vessels of the skin accounted for the predominance of the symptoms and the occurrence of ulcerations during the fall and winter months. The purpose of this article is to define a new syndrome associated with idiopathic livedo reticularis in which the ulcerations occur only or predominantly during the warmer months of the year. DEFINITIONS We would define "livedo reticularis" as a condition of the skin characterized by a reddish-blue, mottled, reticular or blotchy discoloration. It persists with a variation of degree regardless of the skin temperature, and has also been described as a marbling or a fish-net discoloration. Synonyms in the medical literature for this condition have been "generalized telangiectasia," ‡ "livedo racemosa," "livedo

Susac’s Syndrome (SS) consists of the triad of encephalopathy, branch retinal artery occlusions (BRAO) and hearing loss (HL). Histopathologically, SS is characterised by a microangiopathy, and some observations suggest that an immune-mediated damage of endothelial cells might play a role. These findings also implicate a similarity between SS and other autoimmune diseases, most notably juvenile dermatomyositis (JDM). However, SS and JDM are commonly thought to affect distinct and non-overlapping sets of organs, and it is currently not clear how these specificities arise. Moreover, in the absence of clinical trials, some authors suggest that therapeutic approaches in SS should rely on the model of other autoimmune diseases such as JDM. Here, we report a case of SS in a 32-year-old pregnant woman. She initially was admitted to the hospital with subacute severe encephalopathy and multifocal neurologic signs. As cranial magnetic resonance imaging (MRI) revealed multifocal white matter lesions including the corpus callosum, erroneously a diagnosis of multiple sclerosis (MS) was made, and intravenous methylprednisolone (IVMP) therapy was initiated. A few days later, an exanthema appeared on the trunk and extremities, which was diagnosed as livedo racemosa (LR). Several weeks later, the patient was readmitted to the clinic with an obscuration of her left visual hemifield and a bilateral HL. Ophthalmologic examination revealed extensive ischemic damage to both retinae. Now the correct diagnosis of SS was established, based on the above triad of clinical symptoms in conjunction with typical MRI and fundoscopic findings. When SS was diagnosed, the standard therapy with intravenous cyclophosphamide (IVCTX) was not instituted because of a significant risk of permanent infertility. Instead, sustained control of disease activity could be achieved with a therapeutic regime combining prednisolone, intravenous immunoglobulins (IVIG), mycophenylate mofetil (MM), and methotrexate (MTX). An association with LR has only been described in very few cases of SS before and further underlines the pathogenetic relationship between SS and other autoimmune diseases such as JDM. In young women with SS and the desire for a child the combination of MM and MTX may represent a reasonable alternative to IVCTX.

To the Editor.— In the July issue of theArchives, Dr. Dennis F. Marion 1 described a patient with a photosensitive livedo racemosa occurring during treatment with quinidine sulfate. Marion's case is closely similar to a patient's condition described by one of us in 1967 at the meeting of the Netherlands's Society of Dermatologists in Amsterdam 2 and to another patient we have seen recently. In our patients, the affected parts were grossly swollen when the eruption developed. In both, there was a latent period of about 48 hours between the exposure to the sun and the onset of the eruption. Our patients were being treated with a compound that releases the quinidine gradually and we thought that the constituents of this compound might be responsible for the reaction, because, despite the frequent use of quinidine sulfate, this reaction has never, to our knowledge, been described. The compound our patients

LATENT SYPHILIS (POSITIVE WASSERMANN) IN COMBINATION WITH ERYTHEMA INDURATUM BAZIN. Presented by Dr. Sachs. A syphilitic patient developed chestnut sized erythema induratum nodes on both lower extremities. Specific antisyphilitic treatment reduced the erythema lesions to half their size after one "course." The case is interesting because: (1) The positive Wassermann reaction could easily induce one to consider the nodes as syphilitic gummas. (2) It shows the remarkable influence of a therapy which is not specific for tuberculosis. CHEIROPOMPHOLYX WITH A CONSIDERABLE INCREASE IN BLOOD SUGAR. Presented by Dr. Krueger. The skin on the palms and soles was reddened, dry and covered with numerous pinhead sized vesicles filled with a yellowish fluid. Some of the eruptions resembled erythema multiforme. The blood sugar showed a distinct increase. LIVEDO RACEMOSA. Dr. Urbach. The patient, who had never had any medicinal treatment, developed also a black, hairy condition on the tongue. In the discussion

Generalized telangiectasia is one of the rarer dermatoses, and little is known concerning its etiology and treatment. Becker1reviewed the literature thoroughly, and to the fifty-four cases already recorded added four cases of generalized telangiectasia without involvement of the mucous membranes which were seen at the Mayo Clinic. For purposes of classification this entity is to be distinguished from livedo racemosa, cutis marmorata and purpura annularis telangiectoides (Majocchi), although all four conditions may be closely related etiologically. The appearance in generalized telangiectasia consists of a more or less diffuse redness covering all or large portions of the cutaneous surface, with a vague capillary network from which the color can be pressed. At times the redness appears to be accentuated in a punctiform manner ; at other times the reticulated capillaries seem to form indistinct rings of from 1 to 2 cm. in diameter. Becker noticed petechiae in one of

The deficiency of adenosine deaminase type 2 (DADA2) is a recessively inherited condition caused by mutations in CECR1. Patients present with recurrent fevers and evidence of vasculitis/vasculopathy, including livedo racemosa, lacunar strokes, polyarteritis nodosa, endothelialization of the hepatic sinusoids with portal hypertension, and active colitis. There is no recombinant form of ADA2 and thus we attempted a) exogenous replacement of ADA2 via fresh frozen plasma (FFP) and b) suppression of the inflammatory response using anti-tumor necrosis factor (anti-TNF) therapy. This abstract documents 22 months of clinical treatment in the NIH DADA2 cohort.