Acrocyanosis Research Articles

Case Report: Hydranencephaly in a female newborn with congenital cytomegalovirus infection

Background: Hydranencephaly is a congenital abnormality of the central nervous system characterized by massive hemispheric necrosis and ventricular dilatation with most of the cerebral hemispheres being replaced by membranous sacs filled with cerebrospinal fluid (CSF). Cytomegalovirus (CMV) infection can be one of the causes of hydranencephaly. The choice of therapy for hydranencephaly is a challenge because of poor prognosis, but now neural stem cell (NSC) transplantation may give new hope. Case report: We report a case of a female newborn born at term by cesarean section from a gravida 5, para 3, abortus 1 (G5P3A1) mother with suspected hydrocephalus. Head circumference was 35 cm at birth. The baby was born crying immediately, looked active, and did not appear cyanotic, but on the second day, the baby looked less active, moaned, showed acral cyanosis, and had a large fontanelle that was dilated and soft. Signs of hydrocephalus such as Macewen’s sign, setting sun phenomenon, and transillumination were found to be positive. Echocardiographic examination showed floppy interatrial septum and mild mitral regurgitation. Non-contrast computerized tomography (CT) scan of the head showed hydranencephaly. The toxoplasmosis, rubella, CMV, and herpes simplex virus (TORCH) screening showed positive anti-CMV immunoglobulin G (IgG). The baby underwent a ventriculoperitoneal (VP) shunt procedure with a head circumference of 36 cm before surgery. VP shunt was performed with an opening pressure of 15 cmH2O. Conclusions: After the VP shunt procedure, the baby's general condition improved with the head circumference within normal limits. The baby was discharged from the hospital after 16 days of treatment.

Pulmonary arterial hypertension due to ventriculoatrial shunts: A case report and literature review

Cardiopulmonary complications are infrequent in patients with ventriculoatrial shunts (VAS). We report the case of an 18-year-old man with congenital hydrocephalus who developed pulmonary arterial hypertension (PAH) in the long-term follow-up after a VAS procedure. He presented with progressive exertional dyspnea, stabbing chest pain and acral cyanosis. Echocardiography showed severe PAH and a digitiform mass adhered to the distal catheter. A ventilation/perfusion scan suggested chronic pulmonary embolisms. Anticoagulation with intravenous heparin was started and thrombus resolution was achieved, but PAH remained. It is necessary to bear in mind complications linked to VAS when treating a patient with this device.

Case Report: Hydranencephaly in a female newborn with congenital cytomegalovirus infection

Background: Hydranencephaly is a congenital abnormality of the central nervous system characterized by massive hemispheric necrosis and ventricular dilatation with most of the cerebral hemispheres being replaced by membranous sacs filled with cerebrospinal fluid (CSF). Cytomegalovirus (CMV) infection can be one of the causes of hydranencephaly. The choice of therapy for hydranencephaly is a challenge because of poor prognosis, but now neural stem cell (NSC) transplantation may give new hope. Case report: We report a case of a female newborn born at term by cesarean section from a gravida 5, para 3, abortus 1 (G5P3A1) mother with suspected hydrocephalus. Head circumference was 35 cm at birth. The baby was born crying immediately, looked active, and did not appear cyanotic, but on the second day, the baby looked less active, moaned, showed acral cyanosis, and had a large fontanelle that was dilated and soft. Signs of hydrocephalus such as Macewen’s sign, setting sun phenomenon, and transillumination were found to be positive. Echocardiographic examination showed floppy interatrial septum and mild mitral regurgitation. Non-contrast computerized tomography (CT) scan of the head showed hydranencephaly. The toxoplasmosis, rubella, CMV, and herpes simplex virus (TORCH) screening showed positive anti-CMV immunoglobulin G (IgG). The baby underwent a ventriculoperitoneal (VP) shunt procedure with a head circumference of 36 cm before surgery. VP shunt was performed with an opening pressure of 15 cmH2O. Conclusions: After the VP shunt procedure, the baby's general condition improved with the head circumference within normal limits. The baby was discharged from the hospital after 16 days of treatment.

Henoch-Schoenlein Purpura Following Severe Acute Respiratory Syndrome Coronavirus-2 Infection: A Case Report

Introduction: As the novel coronavirus pandemic continues to affect people worldwide, immune-mediated inflammatory syndromes related to this virus have been reported. SARS Coronavirus-2 infection disease (COVID-19) may accompany various cutaneous symptoms, such as viral exanthems or wheels. These symptoms may occur at the early course of the disease or after that. Cutaneous symptoms of COVID-19 usually have a favorable outcome. Case Presentation: We presented a rare case of inpatient COVID-19 pneumonia, who developed a purpuric rash, abdominal pain, and hematuria. His dermal histopathological study revealed small-vessel vasculitis. We diagnosed Henoch–Schoenlein purpura based on clinical and histopathological findings and treated him with intravenous dexamethasone followed by oral prednisolone. The purpuric rashes disappeared in two weeks. Conclusions: Cutaneous manifestation of COVID-19 is usually diffuse maculopapular rashes. Other presentations include vesicular rashes and acral cyanosis. Dermal histopathological study in most patients with COVID-19 who developed cutaneous symptoms revealed perivascular inflammation and microthrombus in some cases. Thus, COVID-19 should be considered in any patient with a new onset of cutaneous symptoms.

Treating Cold Agglutinin Disease with Sutimlimab

Clinical complications of cold agglutinin disease (CAD) include hemolytic anemia and acrocyanosis. The disease is driven by IgM autoantibodies that

Sudden convulsion with multiple pulmonary nodules in a girl aged 15 years

A girl, aged 15 years, was admitted due to sudden convulsion once and multiple pulmonary nodules on lung CT. Acrocyanosis or acropachy/toe deformity was not observed. Laboratory examinations showed an increase in hemoglobin (162 g/L) and a reduction in arterial partial pressure of oxygen (61.5 mm Hg). Lung CT showed irregular slightly high-density nodules in the middle lobe of the right lung, and contrast-enhanced CT scan showed obvious enhancement with thick vascular shadow locally. An investigation of medical history revealed that the girl's mother had a history of epistaxis and resection of pulmonary mass and the girl presented with tongue telangiectasia. The girl was diagnosed with hereditary hemorrhagic telangiectasia and pulmonary arteriovenous malformation. she was given interventional embolization therapy. Transcutaneous oxygen saturation reached 98% without oxygen inhalation on the day after surgery. Pulmonary angiography at 3 months after surgery showed the recurrence of pulmonary vascular malformation, and embolization of pulmonary arterial fistula was not performed since the guide wire could not enter the branch artery. There was still a need for long-term follow-up.

Thrombotic Microangiopathy in COVID-19 and its Association with Complement Classical Pathway

COVID-19 pandemic has affected the whole world in last 1 year with catastrophic effects. This virus has been commonly associated with thromboembolic tendency, resulting in pulmonary embolism, Cerebrovascular accidents, Myocardial infarctions and Acral Cyanosis. Acute kidney injury is also very common amongst patients with COVID-19 infection, mostly due to pre-renal AKI, Acute tubular necrosis or collapsing glomerulopathy. Renal injury due to thrombotic microangiopathy and cortical necrosis is not a common finding in COVID-19 infections. Here we are reporting a case of young female with Critical COVID Pneumonitis and Cytokine release syndrome, who developed AKI due severe TMA and cortical necrosis.

Infrared thermography in children: a reliable tool for differential diagnosis of peripheral microvascular dysfunction and Raynaud\u2019s phenomenon?

BackgroundInfrared Thermography (IRT) has been used for over 30 years in the assessment of Raynaud Phenomenon (RP) and other peripheral microvascular dysfunctions in adults but, to date, very little experience is available on its use in children for this purpose. The first aim of the study was to assess reproducibility of thermographic examination after cold exposure by comparing inter-observer agreement in thermal imaging interpretation. The secondary aim was to evaluate whether IRT is reliable to diagnose and differentiate peripheral circulation disturbances in children.MethodsChildren with clinical diagnosis of primary Raynaud’s phenomenon (PRP), secondary RP (SRP), acrocyanosis (AC) and age-matched controls underwent sequential measurements of skin temperature at distal interphalangeal (DIP) and metacarpophalangeal (MCP) joints with IRT at baseline and for 10 min after cold challenge test. Intraclass correlation coefficient (ICC) was calculated for inter-rater reliability in IRT interpretation, then temperature variations at MCP and DIP joints and the distal-dorsal difference (DDD) were analysed.ResultsFourteen PRP, 16 SRP, 14 AC and 15 controls entered the study. ICC showed excellent agreement (> 0.93) for DIPs and MCPs in 192 measures for each subject. Patients with PRP, SRP and acrocyanosis showed significantly slower recovery at MCPs (p < 0.05) and at DIPs (p < 0.001) than controls. At baseline, higher temperature at DIPs and lower at MCPs was observed in PRP compared with SRP with significantly lower DDD (p < 0.001). Differently from AC, both PRP and SRP showed gain of temperature at DIPs and less at MCPs after cold challenge. PRP but not SRP patients returned to DIPs basal temperature by the end of re-warming time. Analysis of DDD confirmed that controls and PRP, SRP and AC patients significantly differed in fingers recovery pattern (p < 0.05).ConclusionIRT appears reliable and reproducible in identifying children with peripheral microvascular disturbances. Our results show that IRT examination pointed out that PRP, SRP and AC patients present significant differences in basal extremities temperature and in re-warming pattern after cold challenge therefore IRT can be suggested as an objective tool for diagnosis and monitoring of disease.

Vascular acrosyndromes in young adult population. Definition of clinical symptoms and connections to joint hypermobility.

Clinical recognition of vascular acrosyndromes is often challenging. The term Raynaud's phenomenon (RP) is commonly overused to describe any form of cold-related disorder. This study aims to formally evaluate peripheral vascular symptoms affecting the population, aged ≤ 40years, and identify any correlations to joint hypermobility (JH). Fifty patients (31 males, 19 females) with vasomotor symptoms enrolled in this five-year prospective observational study. Clinical examination by a rheumatologist and a vascular surgeon was performed along with cardiology, echocardiographic and Doppler evaluation. Patients underwent blood cell count, biochemistry, thyroid and selectively immunologic testing. Twenty-four (48%) of them performed nailfold capillaroscopy. The SPSS for Windows, v.17.0, Chicago, USA, was used for the statistical analyses. Twenty-eight patients (56%) presented with erythromelalgia (EM), 6 (12%) with acrocyanosis (AC) and 9 (18%) as a combination of the above disorder. RP diagnosed in five (10%) while two patients (4%) presented as a mix of EM-RP. There was no correlation with abnormal laboratory tests. Increased incidence of JH was found in EM and AC patients. Among those who were tested with nailfold capillaroscopy, 75% had abnormalities ranged from mild to autoimmune-like diseases. Erythromelalgia is the commonest functional vasculopathy in young population followed by acrocyanosis and a combination of these conditions. Joint hypermobility is markedly increased, indicating that dysautonomy may be considered the causative factor following a trigger event. Overall, RP was observed in 14% of patients. Clinical recognition of these disorders avoids unnecessary investigation. Key Points • Vascular acrosyndromes in young adults are commonly functional disorders resembling vascular algodystrophy induced by thermic stress. • Dysautonomy of joint hypermobility is the co-factor influencing the appearance of the vascular disorders. • Raynaud's phenomenon accounts to approximately 14% of vascular acrosyndromes presented in the young adult population.

P019: Cryofibrinogenemia with severe acrocyanosis during pregnancy

P019: Cryofibrinogenemia with severe acrocyanosis during pregnancy

Diversity and severity of adverse reactions to quinine: A systematic review.

Quinine is a common cause of drug-induced thrombocytopenia and the most common cause of drug-induced thrombotic microangiopathy. Other quinine-induced systemic disorders have been described. To understand the complete clinical spectrum of adverse reactions to quinine we searched 11 databases for articles that provided sufficient data to allow evaluation of levels of evidence supporting a causal association with quinine. Three reviewers independently determined the levels of evidence, including both immune-mediated and toxic adverse reactions. The principal focus of this review was on acute, immune-mediated reactions. The source of quinine exposure, the involved organ systems, the severity of the adverse reactions, and patient outcomes were documented. One hundred-fourteen articles described 142 patients with definite or probable evidence for a causal association of quinine with acute, immune-mediated reactions. These reactions included chills, fever, hypotension, painful acral cyanosis, disseminated intravascular coagulation, hemolytic anemia, thrombocytopenia, neutropenia, acute kidney injury, rhabdomyolysis, liver toxicity, cardiac ischemia, respiratory failure, hypoglycemia, blindness, and toxic epidermal necrolysis. One hundred-two (72%) reactions were caused by quinine pills; 28 (20%) by quinine-containing beverages; 12 (8%) by five other types of exposures. Excluding 41 patients who had only dermatologic reactions, 92 (91%) of 101 patients had required hospitalization for severe illness; 30 required renal replacement therapy; three died. Quinine, even with only minute exposure from common beverages, can cause severe adverse reactions involving multiple organ systems. In patients with acute, multi-system disorders of unknown origin, an adverse reaction to quinine should be considered.

Cold Agglutinin Disease Secondary to Multiple Myeloma in a Psoriatic Patient

Cold agglutinin disease (CAD) is a chronic compensated hemolytic anemia. We report a case of cold agglutinin disease secondary to multiple myeloma (MM) in a patient of erythrodermic psoriasis. The patient presented with acral cyanosis, progressive weakness and generalized desquamating scaly lesions. After confirming the diagnosis (80-90% plasma cells in bone marrow, M- spike in serum protein electrophoresis, G-ë restriction pattern in immune electrophoresis) he was given chemotherapy with CTD (cyclophosphamide, thalidomide and dexamethasone) protocol. His clinical symptoms improved and he has now completed 4 cycles of chemotherapy. The interim follow up showed achievement of PR (partial response).DOI: http://dx.doi.org/10.3329/jom.v15i2.20688 J MEDICINE 2014; 15 : 141-143

Joint pain, digital necrosis and acute kidney failure in small-vessel autoimmune disease.

A 61-year-old female suffered from disseminated joint pain (both hands, elbows, knees, ankles) with a prominent Raynaud phenomenon in both hands for 4 months before admission due to nausea. Acute kidney failure of unknown origin was diagnosed and acral cyanosis of fingers II–IV of the left hand with beginning finger tip necrosis became obvious. To relieve pain and improve ischaemia, she received acetylsalicylic acid and non-steroidal anti-inflammatory drugs. She had no past medical history except for a rapid weight loss (∼10 kg within the previous 3 months) and a smoking history of <40 pack years. On examination, painful acral cyanosis was revealed in all extremities with no destruction of joints or clinically apparent macroangiopathy; however, angiography indicated reduced blood flow in fingers I–III, V of the right hand and no blood flow in fingers I–IV of the left. The estimated glomerular filtration rate (eGFR) was reduced to 7 mL/min; further laboratory testing showed accompanying metabolic acidosis, hyperkalaemia, secondary hyperparathyroidism and iron deficiency anaemia. Autoantibody testing revealed positive cANCA (1280; ref. range <5) and PR-3 ANCA antibody titres (1587 U/mL; ref. range <10 U/mL). Cryoglobulins, rheumatoid factor, complements C3, C4 and CH50 as well as antistreptolysin titres were within the normal ranges and serology for chlamydia, borreliosis and viral hepatitis infections were negative. A diagnosis of focal segmental, necrotizing ANCA-positive glomerulonephritis with focal segmental glomerulosclerosis, advanced acute tubular damage and interstitial nephritis was made following a kidney biopsy. Immunosuppressive therapy was initiated with high-dose prednisolone pulses, followed by oral prednisolone treatment (1 mg/kg body weight daily); cyclophosphamide i.v. fortnightly (initial 10 mg/kg, then 12.5 mg/kg body weight) [1]. Furthermore vasodilatator amlodipine and i.v. prostavasine infusions were applied to improve rheology. Kidney function improved considerably within weeks (eGFR: 32 mL/min); however, severe necrosis of the left hand remained. Cotton gloves and wool bandages were applied to shield fingers (Figures 1–3) until auto-amputation took place (Figure 4). Fig. 2. Left hand after 1 week. Fig. 1. Left hand at admission. Fig. 3. Left hand after 2 weeks. Fig. 4. Left hand after 18 months. ANCA-associated necrotizing granulomatosis is a leukocytoclastic vasculitis of predominantly small vessels with necrosis and granulomatous tissue formation [2]. Every organ may be affected, with preponderance of lungs, kidneys, gastrointestinal tract and mucosal membranes. Categorizations of systemic vasculitides adhere to vessel sizes; however, clinical presentations may not be confined to small arteries/capillaries/venules [3]. Differential diagnoses of secondary Raynaud's phenomena include scleroderma, progressive chronic polyarthritis, systemic lupus erythematodes, arteriosclerosis obliterans and thrombangitis obliterans, which were all refuted by work-up of the case. Raynaud's phenomenon resulting in necrosis of the fingers/toes is not commonly described for ANCA-positive autoimmune diseases, given the involvement of medium-sized vessels. A literature search reveals similar case reports, some of which may belong to overlap syndromes with vasculitis and autoimmune post-infectious phenomena [4]. In the index case, affection of all limbs makes an underlying immunological pathomechanism likely. In conclusion, we believe that Raynaud's phenomenon and digital necrosis should include the differential diagnosis of ANCA-associated necrotizing vasculitis.

White and Blue Fingers: A Red Flag in Sickle Cell Disease

A 34-year-old woman with hemoglobin SC disease had intermittently painful and swollen wrists, metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints. She photographed her right hand after going outdoors for 10 min (41 °F air temperature). The photograph demonstrated pallor, cyanosis, and erythema (Fig. 1), which abated after 10 min and were unrelated to pain crisis. Based on history and exam, she was diagnosed with Raynaud’s phenomenon. Evaluation revealed an ANA of 1:320 and negative Smith, RF, and CCP antibodies. Anti-RNP was elevated (25 EU/mL), suggesting possible mixed connective tissue disease and secondary Raynaud’s phenomenon. Figure 1. Raynaud’s phenomenon exhibiting acral cyanosis, pallor, and erythema. Hand pain and swelling in sickle cell disease can be from osteonecrosis, osteomyelitis, or hand-foot syndrome during vaso-occlusive crisis (up to 20 % incidence before age two, but rare in adults).1,2 However, Raynaud’s phenomenon is not known to be associated with sickle cell disease, and warrants further investigation for other possible etiologies to avoid misdiagnosis and treatment delays.3 Secondary Raynaud’s may result from vasculopathy, endocrinopathy, drugs, malignancy, or connective tissue diseases. Evaluation may include ESR, ANA, RF, and other disease-specific antibodies (Scleroderma, SLE, MCTD, etc.), as indicated by history. Close monitoring of patients with negative studies may be warranted, as 1 % per year may eventually develop a connective tissue disease.4

Successful oral desensitization to i.v. para-aminosalicylic acid (PAS) using encapsulated PAS dry substance

para-Aminosalicylic acid (PAS) is commonly used in the treatment of drug-resistant tuberculosis, including multidrug-resistant tuberculosis. Since its first use in the 1940s, hypersensitivity reactions frequently limit its use in clinical practice. Cases of successful desensitization against PAS using orally administered ascending doses are described in the literature. A 25-year-old patient with severe pulmonary multidrug-resistant tuberculosis developed drug fever with rash, acral cyanosis, and shivering immediately after the intravenous application of PAS. Hard gelatine capsules containing PAS dry substance were prepared in order to desensitize this patient. Encapsulated PAS was applied orally in rising doses starting with 10 mg/day and doubling the dose every 2 days until the half-maximal dose of 5,120 mg was reached. Desensitization covers a period of 21 days. Subsequent intravenous application of PAS at the full dose was well tolerated. In a 12-month follow-up period, no more allergic reactions appeared. PAS dry substance encapsulated in hard gelatine capsules and administered orally in rising concentrations may be useful to archive a successful desensitization for subsequent intravenous applications.

Severe systemic sepsis due to pyogenic liver abscess

Pyogenic liver abscess is a potentially life-threatening disease with a reported mortality of up to 30–50%. Escherichia coli and Klebsiella pneumoniae (Kp) are the most important pathogens for pyogenic liver abscess. Modern imaging techniques such as ultrasonography (USG) and computed tomography (CT) are used for diagnosis and treatment. Treatment with intravenous (iv) antibiotics and application of catheter drainage (under the guidance of imaging techniques) are the principal therapies. Surgical intervention is indicated if failure of medical treatment and rupture of abscess are noted [1, 2] Herein, we present the case of a pyogenic liver abscess due to Kp in a diabetic man in our intensive care unit (ICU). A 60-year-old man with a history of diabetes mellitus was referred to our ICU with deterioration of his general condition. He was treated for suspected urinary tract infection in another hospital for several days, but the symptoms were not resolved. On admission, he was septic with a clouding of consciousness. On examination, he had tachypnea, tachycardia, hypoxemia, fever and impaired peripheral circulation with acral cyanosis. He was hypotensive with a systolic blood pressure of 70 mmHg and responded to fluid resuscitation, but subsequently required ventilatory support for respiratory deterioration. He had acute renal impairment requiring hemodialysis, and disseminated intravascular coagulopathy (DIC), secondary to sepsis. Laboratory tests also revealed impaired liver and renal function tests, leukocytosis, high erythrocyte sedimentation rate and an elevated C reactive protein (CRP) level. Chest X-ray showed consolidation on bilateral lower zones of the lungs. Antibiotherapy was initiated with iv administration of imipenem and ciprofloxacin after blood, urine, and tracheal aspirate cultures were obtained. A bedside abdominal USG showed a thick walled cystic lesion, 7 cm in diameter, in the right lobe of the liver and it was interpreted as liver abscess. Cranial, thoracal and abdominal CTs were performed subsequently. Cranial CT was normal. Thoracal CT revealed multiple cavitating nodules in lung parenchyma, pneumonic consolidations on bilateral basal segments and bilateral minimal pleural effusions (accepted as spread of infection to the lung via septic emboli). Abdominal CT revealed an abscess (7 cm 9 5 cm 9 5 cm) in the right lobe of the liver (Fig. 1). Percutaneous drainage of the liver abscess with the guidance of abdominal USG was performed on the fourth day after hospitalization. The thick pus was drained with needle aspiration and the drainage catheter was left in situ and attached to an external drainage bag. Cultures of the liver aspirate eventually grew Kp. Kp was also found in one set of blood cultures performed on admission. Intravenous antibiotics were not changed according to antimicrobial sensitivities. Despite continued treatment with appropriate antibiotics and drainage, he worsened and died because of disseminated Kp infection and sepsis on the seventh day after hospitalization. Most pyogenic liver abscesses are secondary to infection originating in the abdomen. Cholangitis due to stones or G. Aygencel (&) Division of Critical Care Medicine, Department of Internal Medicine, Gazi University Faculty of Medicine, Besevler, 06510 Ankara, Turkey e-mail: aygencel@hotmail.com

Phenobarbital-induced Acrocyanosis

Phenobarbital-induced Acrocyanosis

What Part of ‘Do Not’ Don't We Understand?

What Part of ‘Do Not’ Don't We Understand?

ACROCYANOSIS AS A CAUSE OF PSEUDOHYPOGLYCEMIA

ACROCYANOSIS AS A CAUSE OF PSEUDOHYPOGLYCEMIA

Peripheral symmetrical gangrene

Conflict of interest: none declared Peripheral symmetrical gangrene (PSG) is a condition defined by sudden onset of acral, frequently symmetrical purpura and gangrene involving all four limbs, both hands or both feet.1 PSG is a rare but well‐documented condition, which can occur at any age and has no gender predilection. Extreme cold, pallor and pain in the affected areas herald the onset of PSG. These symptoms and findings progress to acrocyanosis and, if not reversed, to frank gangrene. We report the case of a patient with acute lymphatic leukaemia who developed the typical lesions of PSG due to disseminated intravascular coagulation (DIC) secondary to septicaemia induced by Escherichia coli and Enterococcus faecalis. A 47‐year‐old man with acute lymphatic leukaemia started a chemotherapy regimen with cyclophosphamide, vincristine, adryamicin, prednisone and imatinib. After a few days, he developed fever and watery diarrhoea, and his fingers and toes became cold, pale and cyanotic. The acral cyanosis was symmetrical and the tips of the digits were bluish‐black, suggesting impending gangrene (Fig. 1a and b). Within 24 hours, the condition progressed rapidly to gangrene, with formation of haemorrhagic bullae involving the hands, feet, forearms and the distal third of the legs (Fig. 1c). Peripheral pulses were intact.