Acrodermatitis continua of hallopeau successfully treated with adalimumab

Acrodermatitis continua of Hallopeau, first described by Hallopeau in 1890, is an uncommon variant of pustular psoriasis. Acrodermatitis continua of Hallopeau presents as sterile pustules on the hands and feet. It has a relapsing course. Although acrodermatitis continua of Hallopeau is a pustular psoriasis variant, the condition is not easily treated with antipsoriatic medications. In the following case, the authors report a case of acrodermatitis continua of Hallopeau successfully treated with adalimumab. In addition, the cases of acrodermatitis continua of Hallopeau treated with tumor necrosis-alpha inhibitors are summarized.     

Pustular psoriasis after renal transplantation

Cutaneous manifestations in renal transplant recipients are frequently represented by infections and cancerous lesions. However, dermatologic lesions secondary to autoimmune diseases are rare. We report a case of pustular psoriasis occurring after renal transplantation in a 31-year-old woman with a history of vitiligo. The patient was on hemodialysis for 2 years for undetermined chronic nephropathy. She received an HLA identical live related transplant from her brother. She was maintained on an immunosuppressive regimen of corticosteroids, azathioprine, and cyclosporine, which was replaced with mycophenolate mofetil because of neurotoxicity and azathioprine was stopped. Thirty-one months after renal transplantation, she developed pustular psoriasis which was treated with retinoids; she experienced a relapse and resistance to treatment despite the reintroduction of cyclosporine.     

A Case of Palmoplantar Pustulosis Induced by Certolizumab Pegol: New Anti-TNF-alpha Demonstrates the Same Class Effect

The development of de novo psoriasis in patients treated with tumor necrosis factor-alpha antagonists is well recognized. The authors hereby report a case of palmplantar pustular psoriasis in a patient with rheumatoid arthritis treated with etanercept. The condition responded to topical steroids but re-occurred upon treating the patient with certolizumab pegol. This strongly suggests that the development of de novo psoriasis is a class effect.     

Anaesthesia for an adult with Angelman syndrome

Angelman syndrome is a complex genetic condition involving abnormalities of chromosome 15 in the majority of cases. These defects involve a gene encoding an ubiquitin protein ligase and may be associated with abnormal γ-aminobutyric acid (GABA)_A receptor subunits. Angelman syndrome may have profound implications for anaesthesia: potential exists for airway difficulties; refractory bradyarrythmias; and pharmacodynamic unpredictability. A case of an adult with Angelman syndrome undergoing dental work under general anaesthesia is presented. Induction and maintenance of anaesthesia was unremarkable but emergence was complicated by generalised muscular hypertonia and temporary respiratory embarrassment which resolved spontaneously.     

Cervical spinal cord injury in sapho syndrome.

Cervical spinal fracture and pseudarthrosis are previously described causes of spinal cord injury (SCI) in patients with spondylarthropathy. SAPHO (Synovitis Acne Pustulosis Hyperostosis Osteitis) syndrome is a recently recognized rheumatic condition characterized by hyperostosis and arthro-osteitis of the upper anterior chest wall, spinal involvement similar to spondylarthropathies and skin manifestations including palmoplantar pustulosis and pustular psoriasis. We report the first case of SAPHO syndrome disclosed by SCI related to cervical spine ankylosis.     

Status epilepticus following enflurane anaesthesia

Two patients had generalised convulsions which occurred several hours after anaesthesia supplemented with enflurane. One patient had to be treated for life threatening status epilepticus 7 hours after anaesthesia and the other had a myoclonic convulsion 17 hours after recovery from anaesthesia. Neither had any relevant pre-operative history of epilepsy or convulsions. Possible synergistic roles of the metabolite of atracurium, laudanosine, as well as of cephazolin and photic stimulation, are discussed.     

Familial juvenile generalized pustular psoriasis: response to methotrexate

Two siblings with generalized pustular psoriasis were admitted to the hospital. Case 1: A 7-year-old girl presented with recurrent episodes of multiple pinhead-sized pustules all over her body since the age of 3. The current episode began a week prior to the onset of the lesions. On cutaneous examination, she had generalized erythema topped with tiny pustules, scales, and crusting (Figure 1A). A Gram stain performed from the pustule and cultures taken from the pus and blood were sterile. Histopathology of the pustule was suggestive of pustular psoriasis (Figure 2). Hemoglobin was 8.8 g%. Other hematologic, microbiologic, and radiologic investigations were normal. Treatment was started with methotrexate, to which she responded dramatically with reduction in appearance of new lesions, erythema, and scaling. After 4 weeks of treatment, there was almost 90% clearance of skin lesions. Case 2: A 3-year-old boy, brother of the patient in case 1, presented with similar complaints for the past week. The episode was preceded by high-grade fever. He had generalized erythema, scaling, and edema and multiple pinhead-sized pustules, some of which were arranged in an annular configuration (Figure 1B). All investigations, as in case 1, were normal except for hypocalcemia (Ca2+ = 8.8 g%) and generalized rarefaction on x-rays of both the ankle joints. Treatment was started with prednisolone (because of poor general condition) and methotrexate. Following therapy, significant improvement was evident with reduction in erythema and cessation of appearance of new pustules. The dose of prednisolone was gradually tapered after 3 weeks and then stopped.     

Naloxone-responsive acute dystonia and parkinsonism following general anaesthesia

Various movement disorders such as dystonia may acutely develop during or at emergence from general anaesthesia in patients with or without pre-existing Parkinson disease. These movements are triggered by a variety of drugs including propofol, sevoflurane, anti-emetics, antipsychotics and opioids. The postulated mechanism involves an imbalance between dopaminergic and cholinergic neurotransmitters in the basal ganglia. We report an acute, severe and generalised dystonic reaction in an otherwise healthy woman at emergence from general anaesthesia, dramatically reversed by the administration of naloxone, pointing to a potential role of the fentanyl and morphine that the patient had received. Recent literature on the mechanisms of abnormal movements induced by opioids are discussed. The severity of the reaction with usual doses of opioids, in a patient with no prior history of parkinsonism, led to further investigation that demonstrated the possibility of an enhanced susceptibility to opioids, involving a genetically determined abnormal function of glycoproteine-P and catechol-O-methyltransferase.     

Death potentially secondary to sub-Tenon's block

An 82-year-old ASA 2 patient underwent routine sub-Tenon's block for cataract surgery. One minute after injection of the local anaesthetic, the patient had a generalised tonic-clonic seizure and developed refractory ventricular fibrillation; subsequent resuscitation was unsuccessful. With no evidence for intravascular injection, the lack of structural brain abnormalities, and the most striking feature on post mortem examination being severe triple vessel coronary artery disease, it was concluded that this was primarily cardiac in origin; however, the possibility of brainstem anaesthesia should also be considered.     

Onset or exacerbation of cutaneous psoriasis during TNFalpha antagonist therapy

The widespread use of TNFα antagonists in recent years has led to the recognition of paradoxical adverse effects, defined as the onset or exacerbation of disorders that are usually improved by TNFα antagonists. Cutaneous psoriasis is an example, of which several cases have been reported.ObjectiveTo identify cases of psoriasis onset or exacerbation during TNFα antagonist therapy and to look for potential predictive factors.MethodsWe retrospectively reviewed cases of psoriasis onset or exacerbation during TNFα antagonist therapy. For each case we recorded the following data: age, sex, underlying disease, nature of the TNFα antagonist, effectiveness in improving the underlying disease, history of psoriasis in the patient or family, time to psoriasis development, type of psoriasis (confirmed by an experienced dermatologist), concomitant treatments, whether the TNFα antagonist was stopped or continued, and the outcome of the psoriasis. These data were compared to those in the literature.ResultsWe identified 12 patients, six men and six women, with a mean age of 45.5 years. The TNFα antagonist was adalimumab in four patients, etanercept in six patients, and infliximab in two patients. The underlying disease was ankylosing spondylitis in six cases, rheumatoid arthritis in four, and psoriatic arthritis in two. Mean time from treatment initiation to psoriasis was 4.1 months (range, 1–15 months). A previous history of psoriasis in the patient was noted in six cases, including four of the six patients taking etanercept. TNFα antagonist therapy was effective on the underlying disease in 11 of the 12 patients. The drug was discontinued in five patients, of whom four experienced resolution of their psoriasis. In the remaining seven patients, the drug was continued and the skin lesions remained unchanged. Most of the patients had psoriasis vulgaris (plaque psoriasis); palmoplantar pustulosis was a feature in five patients.DiscussionOver 40 cases of psoriasis onset or exacerbation during TNFα antagonist therapy have been reported in the literature. The prevalence of this adverse effect has been estimated at 1.5–5% of patients taking TNFα antagonists. The findings from our case series are consistent with data in the literature. Psoriasis is a class effect that has been reported with all the currently available TNFα antagonists. The skin lesions develop within the first few months of therapy. Patients with a wide range of underlying diseases can be affected. Palmoplantar pustulosis is a common feature. A previous history of psoriasis seems more common in patients who experience psoriasis onset or exacerbation during etanercept therapy (four of six patients in our study and 55% in the literature); thus, previous psoriasis may be a risk factor for psoriasis exacerbation during etanercept therapy.     

Acute generalized pustular psoriasis,von Zumbusch type,treated in the burn unit. A review of clinical features and new therapeutics

Generalized pustular psoriasis (GPP) is an immune-mediated dermatologic condition that is characterized by a widespread eruption of sterile, subcorneal pustules. Cases of GPP may present to the burn intensive care unit (ICU), and they may be confused with toxic epidermal necrolysis (TEN) due to the generalized erythema and desquamation. GPP often benefits from admission to an ICU for management of fluid and electrolyte imbalances and for complications such as pneumonitis, renal dysfunction and sepsis. We present the case of a 42 year-old man who was transferred to the burn unit for presumed TEN where he was diagnosed with GPP and successfully treated with intravenous cyclosporine and supportive care. Our objective is to increase awareness of this condition in the critical care community, discuss clinical and laboratory findings, and to review the treatment guidelines published by the National Psoriasis Foundation in August 2012. We also discuss the latest reports utilizing biological response modifying drugs.     

Anaesthesia for a child with Walker-Warburg syndrome

Walker-Warburg syndrome (WWS) is a rare lethal autosomal recessive disorder manifested by characteristic central nervous system and eye malformations. We have not come across reports of general anaesthesia in a child with WWS in the English literature. We report a case of general anaesthesia in a 12-month-old male child with WWS. The child also had bilateral cleft lip, cleft palate, urogenital malformation and hydronephrosis. Despite many potential anaesthesia concerns, anaesthesia was uneventful in this child.     

Transtracheale Oxygenierung bei Atemwegsverlegung durch einen Hypopharynxtumor

A 42-year-old male with a history of chronic alcoholism was admitted to the department of otolaryngology with acute respiratory insufficiency and generalised cyanosis due to a respiratory obstruction by a large tumour of the hypopharynx. Because of the size and location of the tumour and the risk of bleeding, orotracheal intubation by direct laryngoscopy was considered almost impossible. To improve oxygenation cricothyroidal punction and oxygen insufflation was done immediately and SpO2 increased from 56% to 82%. Awake fiberoptic nasotracheal intubation was performed under topical anaesthesia, then general anaesthesia was induced and controlled ventilation was started. After surgical tracheotomy the patient was transferred to an intensive care unit and 12 h later the patient was discharged from the ICU.     

Spinal haematoma following epidural anaesthesia in a patient with eclampsia

A patient with a twin pregnancy required a Caesarean section for severe pre-eclampsia. Her platelet count was 71 x 10(9).l-1. Epidural anaesthesia was performed after platelet transfusion. A spinal epidural haematoma was diagnosed postoperatively. A generalised tonic-clonic seizure sparing the lower limbs enabled early diagnosis to be made. The patient recovered with no permanent neurological damage after laminectomy and clot removal. The risks and benefits of regional techniques require careful consideration, and postoperative monitoring for recovery of neural blockade is essential.     

Diagnosis and classification of mandibular osteomyelitis.

To establish a unified classification system for mandibular osteomyelitis, various diagnostic terms were critically assessed and clinicopathologic findings of the lesions were carefully reviewed. We recommend classifying mandibular osteomyelitis into bacterial osteomyelitis and osteomyelitis associated with the synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. Other diagnostic terms were excluded because they were not appropriate for classification. Diagnostic criteria for bacterial osteomyelitis are suppuration and osteolytic change. The lesions are easily cured by antibiotic treatments. Mandibular osteomyelitis in SAPHO syndrome is characterized by nonsuppuration and a mixed pattern on radiography, with solid type periosteal reaction, external bone resorption, and bone enlargement. The presence of osteomyelitis in other bones, arthritis, or skin diseases (palmoplantar pustulosis, pustular psoriasis, and acne) strongly suggests this syndrome. Antibiotic therapy is usually ineffective and the symptoms of SAPHO syndrome are often persistent.     

Management of Psoriasis During the Coronavirus Disease 2019 Pandemic

Coronavirus disease 2019 (COVID-19) was first reported in late December 2019 and, since then, has rapidly taken over the globe, with the scientific world furiously working to gather more data on its effect on people with and without concurrent conditions. The dysregulation of the immune system noted in COVID-19 patients is said to be similar to that seen with psoriasis. The pandemic has affected the management of psoriasis, not only for those under treatment but also those about to begin a new therapy. There has been an increasing number of studies in the current literature focusing on the relationship between psoriasis and COVID-19, offering different perspectives. This is a summary of available data in PubMed supplemented by a manual review of reference lists of included articles. There may be lack of robust evidence to drive approaches to the management of psoriasis during the pandemic; however, we hope that the current literature may provide some clues for safety considerations. The conclusion of this article is that each approach to treatment should be personalized, weighing the benefits and risks in each case separately.     

Anaesthesia for caesarean section in spinal muscular atrophy type III

We describe the conduct of general anaesthesia for a patient with spinal muscular atrophy Type III (Kugelberg-Welander disease) undergoing elective caesarean section. Apart from a delayed return of skeletal muscle power following non-depolarising neuromuscular blockade the procedure was uneventful. We found no previously published reports of general anaesthesia for caesarean section in this condition in the English language literature. We review the available literature and discuss the potential anaesthetic problems in the management of obstetric patients with this degenerative neuromuscular disorder.     

Anticipated difficult airway during obstetric general anaesthesia: narrative literature review and management recommendations.

We reviewed the literature on management of general and regional anaesthesia in pregnant women with anticipated airway difficulty. We identified 138 publications comprising 158 cases; these either described equipment or techniques for the provision of general anaesthesia, or the management of women with regional analgesia or anaesthesia, with the aim of avoiding general anaesthesia. Most of the former group described women requiring caesarean section alone, or in combination with other surgery, which was sometimes airway-related. Management techniques were largely similar to those in non-obstetric patients requiring surgery who have airway difficulties, although suggested differences related to physiological changes of pregnancy and avoidance of nasal intubation. In the reports discussing regional anaesthesia, consideration was often given to the possible requirement for urgent out-of-hours anaesthetic intervention, and the predicted difficulty of management of general anaesthesia should it be required. In a number of reported cases, multidisciplinary planning led to the conclusion that elective caesarean section should be performed in order to avoid emergency airway management. Based on this literature review, we advise antenatal planning that includes: assessment of the patient's clinical characteristics; consideration of the equipment and personnel available to provide safe airway management out-of-hours; and elective caesarean section should these be lacking. If general anaesthesia is required, a risk assessment must be made as to the probability of safe airway management after the induction of anaesthesia, and awake tracheal intubation should be used if this cannot be assured. Decision aids are provided to illustrate these points. Online appendices include a comprehensive compendium of case reports on the management of a number of rare syndromes and airway conditions.     

Anaphylactoid reaction to alcuronium

A 67-year-old woman suffered cardiovascular collapse during induction of anaesthesia. This was later shown to be anaphylactic in origin; the causative agent was alcuronium. In vitro testing showed a highly specific sensitivity to alcuronium with minimal cross-reactivity. Future anaesthesia with decreased risk was thereby assured. The clinical nature of this reaction and a review of the literature implicate the cardiovascular system as the principal target in this type of reaction to alcuronium and suggest that the heart is directly involved.     

Myotonia congenita and regional anaesthesia with peripheral nerve stimulation: a case report

Myotonia congenita is a rare disorder caused by a defect in the skeletal muscle chloride channel function, which may cause sustained membrane depolarisation. We report a case regarding a 52-year-old man affected by this muscular pathology and scheduled for shoulder surgery that was performed under sedation and peripheral nerve block. The international literature does not elucidate clear preference toward a particular anaesthesia technique, but we believe that in this setting, regional anaesthesia and complete monitoring should be preferentially utilized when possible. In any case, the anaesthesia plan should be prudent and avoid exposure to known dangerous anaesthetics.