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Les acrosyndromes vasculaires paroxystiques sont liés à un trouble vasomoteur et se manifestent par des modifications paroxystiques de couleur des doigts. Ils incluent le phénomène de Raynaud (PR) primitif, le plus fréquent, les PR secondaires et les érythermalgies. Ils sont à distinguer des acrosyndromes non paroxystiques, comme l'acrocyanose et les engelures, très fréquentes et souvent associées à un PR, les ischémies et nécroses digitales, les hématomes digitaux spontanés et les acrocholoses. L'enjeu d'une consultation pour acrosyndrome vasculaire est, d'une part, d'en faire le diagnostic positif grâce à l'interrogatoire et à l'examen clinique et, d'autre part, de préciser sa nature, permettant de prescrire des examens complémentaires ciblés. Chez tout patient consultant pour un PR, les examens complémentaires incluent au minimum une recherche d'anticorps antinucléaires et une capillaroscopie. Pour une érythermalgie, un hémogramme est indispensable, voire une recherche de mutation de JAK2. Un dosage de la TSH, une recherche d'anticorps antinucléaires et une recherche de neuropathie des petites fibres sont également réalisés. Le traitement du PR est essentiellement documenté pour les PR secondaires et repose en 1re intention sur les inhibiteurs calciques et l'iloprost dans les cas sévères. Le traitement des érythermalgies repose sur les inhibiteurs des canaux sodiques, comme la mexilétine ou les perfusions de lidocaine, et sur les médicaments efficaces sur les douleurs neuropathiques, comme la gabapentine ou l'amitryptiline, en cas d'association à une neuropathie des petites fibres. Le traitement des érythermalgies associées aux syndromes myéloprolifératifs repose sur le traitement étiologique et l'aspirine. Paroxysmal vascular acrosyndromes are related to a peripheral vasomotor disorder and presented as paroxysmal color changes of the fingers. They include primary Raynaud's phenomenon (RP), which is the most common, secondary RP and erythermalgia. They are to be distinguished from non-paroxysmal acrosyndromes such as acrocyanosis and chilblains, which are very frequent and often associated with RP, digital ischemia and necrosis, spontaneous digital hematoma and acrocholosis. The challenge of a consultation for a vascular acrosyndrome is to make positive diagnosis through history and clinical examination, and to specify its nature, to prescribe complementary exams. In any patient consulting for RP, assessment includes at least an antinuclear antibody test and capillaroscopy. For erythermalgia, a blood count and even a search for JAK2 mutation are required. A thryoid-stimulating hormon assay, a test for antinuclear antibodies, and a search for small fiber neuropathy are also performed. The treatment of RP is essentially documented for secondary RP where calcium channel blockers are indicated in first line, and iloprost in severe cases. The treatment of primitive erythermalgia is based on sodium channel blockers such as mexiletine or lidocaine infusions, and on drugs effective on neuropathic pain, such as gabapentin or amitryptiline, in case of erythermalgia associated with small fiber neuropathy. The treatment of erythermalgia associated with myeloproliferative syndromes is based on etiological treatment and aspirin. [ABSTRACT FROM AUTHOR]

Abstract We present the case of a 50‐year‐old woman with febrile myalgia, chilblains‐like lesions, and cough. Antinuclear antibodies and anti‐PL‐12 antisynthetase autoantibodies were found in complementary examinations. Interstitial lung disease was found on chest computed tomography. Nailfold capillaroscopy showed microangiopathic lesions. Antisynthetase syndrome is a recently described entity in inflammatory myopathies, with specific clinical criteria. Interstitial lung disease is very common, especially in anti‐PL‐12 associated antisynthetase syndrome. Raynaud's phenomenon is another well‐defined criterion. However, microangiopathic damage is probably underestimated and the role of nailfold capillaroscopy in the diagnosis has not been established yet.

IntroductionBier anemic spots, cyanosis, and urticaria-like eruption (BASCULE) syndrome is an underreported pediatric vascular disorder from the group of acrosyndromes. In children, these include paroxysmal acrosyndromes (Raynaud’s phenomenon and chilblain-like lesions), permanent acrosyndromes (acrocyanosis), and transient acrosyndromes, in which their pathogeneses are associated with virus infections, Epstein-Barr virus, and, more recently, SARS-CoV-2, respectively.MethodsWe reported a case of BASCULE syndrome associated with postural orthostatic tachycardia syndrome (POTS) and provided a narrative review of case reports describing the BASCULE syndrome in children. Moreover, we presented the results of a prospective practice survey that we performed in the French medical community.ResultsA 14-years-old boy reported pruritic erythrocyanic lesions on the lower limbs, which occurred whenever he was in a standing position and fully resolved when he laid down. He reported asthenia and cramps. He presented a typical BASCULE syndrome associated with POTS confirmed by a tilt-test. Physical and vascular examinations were within the normal range. We identified 12 case reports, describing 21 pediatric cases since 2016. Most patients were adolescents between 12 and 19 years of age or were newborns. Furthermore, 20% of cases in the literature have presented POTS or orthostatic intolerance. Our survey among 95 French physicians confirmed that BASCULE syndrome is an underdiagnosed and under recognized disease in the general pediatric practice, at least in France. Among these physicians, 65% had already encountered patients with similar symptoms, but only 30% declared that they had knowledge of the BASCULE syndrome.ConclusionThe under-recognition of the clinical manifestations leads the patients to consult emergency rooms, with multiple unnecessary investigations performed. Therefore, we suggest that the diagnosis of BASCULE syndrome is based on clinical observations, without the need for laboratory tests, to avoid unnecessary health costs. We suggest physicians to perform a tilt-test when POTS is suspected.

We present the case of a 50‐year‐old woman with febrile myalgia, chilblains‐like lesions, and cough. Antinuclear antibodies and anti‐PL‐12 antisynthetase autoantibodies were found in complementary examinations. Interstitial lung disease was found on chest computed tomography. Nailfold capillaroscopy showed microangiopathic lesions. Antisynthetase syndrome is a recently described entity in inflammatory myopathies, with specific clinical criteria. Interstitial lung disease is very common, especially in anti‐PL‐12 associated antisynthetase syndrome. Raynaud's phenomenon is another well‐defined criterion. However, microangiopathic damage is probably underestimated and the role of nailfold capillaroscopy in the diagnosis has not been established yet. [ABSTRACT FROM AUTHOR]

This is a clinical case illustrating a diagnosis of an IgG4 related-disease (IgG4-RD) diagnosed in a vascular context. A 47-year-old man with no past medical history consulted for a recent and disabling Raynaud phenomenon without trophic disorder. Vascular examinations revealed multiple arterial thromboses with no abnormal finger and toe pressures. Secondly, weight loss and submandibular glands enlargement appeared, leading to the diagnosis of IgG4-RD without a link being able to be established with vascular involvement. This is the second observation of this association. A French translation of the new classification criteria for IgG4-RD published in 2019 by the American College of Rheumatology and European Ligue Against Rhumatism (ACR/EULAR) is offered with direct application to the clinical case. A Raynaud phenomenon with distal arterial thrombosis is rarely observed in the IgG4-RD. [ABSTRACT FROM AUTHOR]

Erythromelalgia is a rare syndrome with a generally unknown etiology. Whether primary or secondary, this condition is characterized by paroxysmal episodes of erythema, pain, and heat in the extremities. We report two cases of erythromelalgia occurring after the initiation of treatment with infliximab. The first case involves a 38-year-old patient who had been followed since August 2022 for ileocolonic Crohn's disease classified as A2L3B3 according to the Montreal classification, which was resistant to treatment and required infliximab therapy. Two months after the first infusion of infliximab, the patient developed symptoms of erythromelalgia. After ruling out other potential causes through an etiological assessment and conducting a pharmacological investigation, infliximab was considered the most likely cause. Infliximab was discontinued, and symptomatic treatment was initiated, including vascular laser sessions. The patient showed significant clinical improvement. In the second case, a 16-year-old patient with ileocolonic Crohn's disease classified as A1L3B3 according to the Montreal classification was treated with ileocecal resection and received an infusion of infliximab. Sixteen days after the second infusion, she developed clinical symptoms of erythromelalgia. The etiological assessment was inconclusive. Due to a strong suspicion of erythromelalgia secondary to tumor necrosis factor (TNF) alpha inhibitor therapy, infliximab was replaced with ustekinumab. The patient also received symptomatic treatment, and her clinical condition improved, marked by the disappearance of pain., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, El Rhaoussi et al.)

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Background: Anti-cancer drugs have many adverse effects including vascular side effects. Herein we present the case of a patient presenting digital ischaemia with high imputability of ipilimumab., Observation: A 47-year-old male patient was treated for popliteal melanoma, initially stage IIIA but which subsequently became metastatic (stage IV), and for which ipilimumab was given after the failure of two lines of chemotherapy. During the 4th course of ipilimumab, the patient developed autoimmune hepatitis. Ipilimumab was suspended. Three months later, he developed a drug-like neuropathy followed one month later by ulceration of the right index finger. Causes of embolic, autoimmune and occupational origin (thrombotic microangiopathy, thrombosed aneurysm) were rapidly ruled out. Although a paraneoplastic origin could not be formally excluded, drug-induced immune disorder remained the most plausible origin., Discussion: This is the first reported case of digital ulceration under ipilimumab., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Background: Digital necrosis is rarer than lower limb necrosis and constitutes a medical or surgical emergency. Etiological evaluation is required. Cold agglutinin disease is a cause of digital necrosis but diagnosis is difficult., Patients and Methods: Herein we report the case of a 57-year-old man presenting recent paroxysmal acrosyndrome of the left hand subsequently complicated by digital necrosis following occupational exposure to cold in his work as a forklift driver. After etiological evaluation, a diagnosis of primary cold agglutinin disease was made. Intravenous rituximab and topical treatment resulted in complete healing., Discussion: Cold agglutinin disease is a rare type of auto-immune hemolytic anemia. Following exposure to cold, paroxysmal cutaneous signs are frequent. The disease may be either primary or secondary with B-cell lymphoproliferative disorder, auto-immune disease or infection. A thorough workup is required. To date, the treatment combining the best positive response rate and good safety is rituximab in weekly perfusions over a 1-month period., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Background: Peripheral nerve system (PNS) involvement is common in Fabry's disease (FD), predominantly affecting the small nerve fibers that are difficult to investigate with conventional electrophysiological methods., Patients and Methods: Eighteen patients followed for Fabry's disease underwent a prospective series of electroneurophysiological explorations, including a study of the cardiac parasympathetic autonomic nervous system (ANS) and electrochemical skin conductance (ESC) tests. Data were compared with those obtained in 18 matched healthy controls., Results: All patients had at least one clinical sign suggestive of neuropathy: 16 reported an acrosyndrome and 12 had dyshidrosis. Cold hypoesthesia was found in 15 patients and heat hypoesthesia in 13. Electroneurophysiological investigations and study of the cardiac parasympathetic ANS were normal in all patients. The ESC was significantly lower in FD patients compared with controls., Conclusion: PNS involvement is common in FD and should be suspected in patients exhibiting an acrosyndrome, dyshidrosis and/or cold hypoesthesia. Conventional electrophysiological investigations are normal. New techniques, such as ESC, provide early diagnosis of small fiber involvement that currently requires more sophisticated tests difficult to apply in routine practice., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)