Acute-Onset Multifocal Hand Dysfunction Due to Alkaptonuria.

Alkaptonuria is a rare metabolic disorder characterized by the accumulation of homogentisic acid. Its effects on the central nervous system are well-recognized; however, cases of pathologic homogentisic acid deposition in the peripheral nervous system are less well-described. We report the case of a 72-year-old man with a prior history of alkaptonuria presenting with bilateral carpal tunnel and left-sided cubital tunnel symptoms. This case is of note because the patient demonstrated a rapid onset of symptoms due to pathology at multiple foci.

A Consensus on Minimizing the Risk of Hyaluronic Acid Embolic Visual Loss and Suggestions for Immediate Bedside Management.

BACKGROUND: Hyaluronic acid fillers have a satisfactory safety profile. However, adverse reactions do occur, and rarely intravascular injection may lead to blindness. Currently there is no internationally recognized consensus on the prevention or management of blindness from hyaluronic acid filler. OBJECTIVES: The authors sought to give guidance on how to minimize the risk and optimize the management of this rare but catastrophic adverse reaction. METHODS: A multinational group of experts in cosmetic injectables from multiple disciplines convened to review current best practice and develop updated consensus recommendations for prevention and bedside intervention if visual loss occurs after cosmetic injection of hyaluronic acid filler. RESULTS: The consensus group provided specific recommendations focusing on the consenting process, prevention, and early management of visual impairment related to intravascular hyaluronic acid filler injection. CONCLUSIONS: Although visual loss due to filler injections is rare, it is important that both patient and physician be aware of this risk. In this paper the authors describe methods and techniques available to reduce the risk and also document suggested initial management should a clinician find themselves in this situation.

High Patient Satisfaction with Daylight-Activated Methyl Aminolevulinate Cream in the Treatment of Multiple Actinic Keratoses: Results of an Observational Study in Australia.

INTRODUCTION: Actinic keratoses (AK) are treated to reduce the risk of progression to squamous cell carcinoma and for symptomatic and cosmetic benefits. The objective of this observational study was to generate real-life data on the use of daylight photodynamic therapy with methyl aminolevulinate cream (MAL DL-PDT) in treating mild to moderate facial/scalp AK. METHODS: A multicenter, prospective, observational study was conducted in Australia in patients receiving a single treatment of MAL DL-PDT for mild to moderate AK. Efficacy was assessed 3 months after treatment by investigator-assessed improvement and patient- and physician-completed satisfaction questionnaires. Adverse events were recorded throughout the study. RESULTS: Overall, 81 patients were enrolled of mean age 62.7 years, mostly men (76.5%) with skin phototype I (64.2%) or II (35.8%) and a long history of AK (mean duration 16.8 years). Most had multiple lesions (82.7% had >10 lesions) of predominantly grade I (75.3%). At 3 months after treatment, almost half the patients (46.8%) required no further treatment. The proportions of patients and physicians satisfied to very satisfied with the MAL DL-PDT treatment were 79.7% and 83.3%, respectively. After receiving the treatment, 74.1% of patients indicated via the questionnaire that they were not bothered at all by the pain. Related AEs were reported in 48.1% of patients, mainly mild erythema (44.4%). CONCLUSIONS: In clinical practice in Australia, the use of MAL DL-PDT in treating multiple mild to moderate non-hyperkeratotic AK of the face and/or scalp results in high levels of patient and physician satisfaction reflecting the good efficacy and tolerability of this almost painless, convenient procedure. TRIAL REGISTRATION: ClinicalTrials.gov identifier, NCT02674048. FUNDING: Galderma R&D.

Isotretinoin and mental health in adolescents: Australian consensus.

Acne is a common condition among adolescents and has the potential to negatively impact on the psychological well-being of those who suffer from it. In particular, depression and suicidal ideation are more common in adolescents with acne. Successful treatment of acne can improve the quality of life and reduce levels of anxiety and depression in these individuals. The current treatment of choice for severe or refractive acne is isotretinoin, a retinoid. While the possible causal association between isotretinoin and mental illness remains a controversial topic, a recent systematic review has presented evidence to support this relationship. In light of this evidence, a group of dermatologists and psychiatrists have collaborated to develop these recommendations to aid the safe prescribing of isotretinoin in adolescents. These clinical suggestions are aimed at practitioners in both disciplines to increase awareness of the current evidence in support of the association between isotretinoin and adolescent depression.

Audit of a state-wide store and forward teledermatology service in Australia.

In 2008, the Skin Emergency Telemedicine Service was established at the Princess Alexandra Hospital (PAH) in Brisbane. We conducted an audit by evaluating all email communication during 2012, and administering a clinician questionnaire. A total of 167 cases were discussed via 685 email communications (46 being in-house PAH referrals). The highest number of external referrals came from Mt Isa (27%), located 1200 km from the nearest dermatology clinic, with a further 25% sent from centres located 50-600 km from a clinic. The main referring condition was rash (65%), followed by skin lesions (13%). The most commonly provided telemedicine diagnoses were dermatitis/eczema (23%), infection (20%) and drug eruption (17%). Most external referrals received a reply within 3 hours of the enquiry. Junior doctors (2nd-4th postgraduate year) represented the majority of referring clinicians (62% of questionnaire respondents). There were 111 potential questionnaire recipients. Responses were received from 34 clinicians, a response rate of 31%. Overall 100% of respondents stated that the service was useful to them and 97% said they would use it again in the future with one respondent stating 'possibly'. It seems likely that teledermatology will serve an important role in the provision of healthcare to Queensland, and other remote Australian communities in the future.

Successful treatment of type I adult-onset pityriasis rubra pilaris with infliximab.

A 59-year-old woman presented with a painful, pruritic eruption that had commenced as an erythematous, dry patch on the upper back but progressed to erythroderma. Examination revealed orange-tinged erythroderma, scalp scaling, ectropion, palmoplantar keratoderma and nail changes. A diagnosis of type I adult-onset pityriasis rubra pilaris was made, and a subsequent skin biopsy was consistent with this. She was treated with a number of topical and systemic agents with minimal improvement or major side-effects. The patient was then treated with intravenous infliximab 5 mg/kg. She improved dramatically within 2 weeks and was no longer erythrodermic. Five further infusions resulted in additional improvement. Methotrexate was briefly added to the regime, but was ceased owing to nausea. Topical tar and keratolytics were used on the scalp. The patient was left with minimal disease activity and was maintained on emollients.

Gianotti-Crosti syndrome in an adult following recent Mycoplasma pneumoniae infection.

A 44-year-old insulin-dependant diabetic woman presented with a pruritic papular eruption involving her hands, forearms and elbows. One week prior to the eruption, the patient had an upper respiratory tract infection and had taken oral ibuprofen 400 mg q.i.d. p.r.n. Skin biopsy revealed histological features consistent with Gianotti-Crosti syndrome. Serology was consistent with recent Mycoplasma pneumoniae infection and past Epstein-Barr viral infection. Her liver function tests were deranged and serum protein electrophoresis showed two sharp discrete monoclonal immunoglobulin bands. The eruption resolved completely 15 days after onset. Her serum protein studies and liver function tests subsequently normalized and she had no recurrences of her cutaneous eruption. It was concluded that the patient had Gianotti-Crosti syndrome associated with Mycoplasma pneumoniae infection.

Gallbladder vasculitis associated with cutaneous leucocytoclastic vasculitis.

A 72-year-old woman presented with a 1-week history of a painful, purpuric, pruritic rash on her legs, buttocks and arms. Skin biopsy revealed histological features typical of leucocytoclastic vasculitis. She was admitted, her usual medications were withheld, and she was commenced on ibuprofen and loratadine. The patient had undergone a laparoscopic cholecystectomy 2 months prior to her rash appearing. She had been having upper abdominal pain for 2 years and, following a more severe acute episode, an abdominal ultrasound scan had revealed a solitary cholelithiasis. Histology of the gallbladder revealed acute fibrinoid vasculitis in two small arteries, on a background of chronic cholecystitis. A mild postoperative wound infection was treated with a short course of cephalexin and no other investigations were conducted. As an inpatient, the patient's rash improved, but she progressed to develop systemic vasculitis and acute renal failure. Renal biopsy showed focal necrotizing glomerulonephritis, consistent with vasculitis. She was subsequently commenced on cyclophosphamide 100 mg daily, prednisolone 50 mg daily and one prophylactic trimethoprim/sulphamethoxazole (160 mg/800 mg) tablet 3 days/week. Following discharge, the patient's cutaneous vasculitis eventually resolved and renal function gradually improved. Her prednisolone was gradually reduced to 7 mg daily and cyclophosphamide was weaned, then substituted with azathioprine 100 mg daily.