Impact of a see-and-treat melanoma clinic on patient experience.

BACKGROUND: See-and-treat ('one stop') clinics for cutaneous and noncutaneous tumor streams have been shown to enhance patient experience, amongst other benefits, such as reduced waiting time to surgery if required. To date, there have been no studies assessing patient perception of one-stop clinics dedicated to the diagnosis and treatment of melanomas and pigmented lesions. OBJECTIVE: To perform a prospective survey study examining patient's perception of a see-and-treat pigmented lesion clinic. METHODS AND MATERIALS: Consenting patients were contacted by phone to complete a survey comprising 10 statements relating to different facets of the clinic, four weeks after their initial assessment. Reponses were documented on a 5-point Likert scale. RESULTS: A total of 107/142 (75.4%) patients consented to partake in the study. Compared to overall mean response, patients who underwent same-day biopsy reported higher satisfaction (4.9 vs. 4.5, p < .01) and perceived convenience (4.8 vs. 4.4, p < .01). Of those who received same-day procedures, no patient reported being given insufficient time to consider surgical treatment. CONCLUSION: A see-and-treat model for pigmented lesions, incorporating same-day excisional biopsy for lesions suspicious for melanoma, is viewed upon favorably by patients.

Multidisciplinary Pigmented Lesion Clinic at Auckland District Health Board: impacts on melanoma diagnosis and treatment outcomes.

AIM: To investigate the outcomes and effect of a multidisciplinary 'see and treat' pigmented lesion clinic, run jointly by dermatology and general surgery, on the diagnosis and treatment of melanoma at Auckland District Health Board (DHB). METHOD: All patients attending the newly established Pigmented Lesion Clinic (PLC) between 1 March 2019 and 31 August 2019 were included in the study. They were compared against a retrospective cohort of patients seen for suspected or biopsy-proven melanomas during the same corresponding period in 2016. RESULTS: 251 new patients attended the PLC, compared to 148 new patients seen at Auckland DHB in 2016. There was a significant reduction in proportion of pigmented lesions requiring biopsy (35.2% vs 64.3%, p<0.001), with a benign-to-malignant ratio of 2.4:1. Fifty-three melanomas were treated through the PLC, with a significant reduction in mean waiting time from referral to first specialist assessment (22.6 vs 35.1 days, p=0.038), and from referral to wide local excision (50.6 vs 99.1 days, p<0.001). 86.5% of patients received full skin check, from which additional skin malignancies were detected in 1-per-5.3 patients. CONCLUSION: The novel PLC model has led to reduction in unnecessary excisional biopsies of benign pigmented lesions, while streamlining and improving timely access to specialist review and surgical treatment for patients with melanomas.

Managing atopic dermatitis with systemic therapies in adults and adolescents: An Australian/New Zealand narrative.

With the rapid development of new, targeted therapies for the treatment of moderate/severe atopic dermatitis, it is opportune to review the available conventional systemic agents. We assess the published evidence for systemic therapies for atopic dermatitis and amalgamate this with real-world experience. Discussions are centred on when systemic therapy should be considered, which drug(s), what dose, how to sequence or combine these therapies, how long they should be continued for and what is considered success.

Long-term effect of methotrexate for childhood atopic dermatitis.

AIM: To evaluate methotrexate (MTX) for paediatric atopic dermatitis (AD) while on and post-treatment. METHODS: Medical records of children prescribed MTX for AD between 2011 and 2016 at Starship Children's Hospital, Auckland, New Zealand, were reviewed for demographics, dose and duration of MTX and hospitalisations for AD. In the follow-up by telephone in 2017, parents of the patients reported response on MTX, AD relapses and use of additional systemic treatment and completed a patient-oriented eczema measure (POEM). RESULTS: Forty-three patients aged 2-16 years were included. Four (9%) had previous systemic treatment, and 14 (33%) were hospitalised (28 admissions). MTX was given at median dose of 0.33 mg/kg (interquartile range (IQR) 0.26-0.40) for a median of 17 months (IQR 7.5-20). After initiating MTX, only six (14%) were hospitalised (nine admissions). Thirty (70%) parents of patients were followed up for a median of 29 months (IQR 14-45) after discontinuing MTX. Five (17%) reported 'no change', 2 (7%) 'slightly better' and 23 (77%) 'a lot better' AD on MTX. Of the 25 who responded to MTX, AD relapsed in 10 (40%) at a median of 24 months post-MTX; only four (16%) restarted MTX. Median POEM at follow-up was 6 (IQR 1-17). Eleven (37%) were clear (POEM 0-2), 11 (37%) had mild to moderate AD (POEM 3-16), and 8 (27%) had severe AD (POEM ≥17). CONCLUSIONS: Although a natural resolution cannot be excluded, MTX for severe AD was effective and well tolerated. Improvement was reported by 83%, and AD hospitalisation reduced by half. At a median of 2 years after discontinuing MTX, one third were clear, and one third had mild to moderate AD, suggesting persistence of benefit post-MTX.

Psoriasis and infection. A clinical practice narrative.

The Australasian Psoriasis Collaboration has developed a clinical practice narrative with respect to the relationship between psoriasis, its treatment and infection. The cutaneous microbiome of patients with psoriasis is different to those without psoriasis, although the significance of this is unclear. Whilst a wide range of microorganisms has been associated with psoriasis (including ß-haemolytic streptococci, Staphylococcus aureus, Porphyromonas gingivalis, Candida albicans, Chlamydia psittaci, human immunodeficiency virus and hepatitis C virus), there is limited evidence that antimicrobial therapy is of direct benefit in preventing flares of psoriasis. Psoriasis is independently associated with an increased risk of serious infection, but the absolute risk is low. The risk of serious infections is further increased with immune-modulatory treatments. The decision whether to, and when to, stop or resume immune-modulatory treatment after a serious infection has occurred depends on risk assessment for that patient, taking into account the infection being treated, the risk of recurrent infection, any interventions that can modify the risk and the need for psoriasis control. Live vaccines (e.g. MMR, varicella, zoster and yellow fever) are generally contraindicated in patients with psoriasis on immune-modulatory agents, but this depends on the degree of immune suppression and individual risk factors. Wound healing in psoriasis is normal. Treatment with infliximab, adalimumab, etanercept, methotrexate and ciclosporin can safely be continued through low-risk surgical procedures. For moderate- and high-risk surgeries, a case-by-case approach should be taken based on the patient's individual risk factors and comorbidities.

Psoriasis and cancer. An Australian/New Zealand narrative.

Patients with psoriasis have an increased risk of cancer, which may be due to impaired immune surveillance, immune modulatory treatments, chronic inflammation and/or co-risk factors such as obesity. The increase in treatment-independent solid cancers, including urinary/bladder cancers, oropharynx/larynx, liver/gallbladder and colon/rectal cancers, seem to be linked to alcohol and smoking. Lung cancer and nonmelanoma skin cancer are also increased in patients with psoriasis. The risk of nonmelanoma skin cancer increases with age and severity of psoriasis. It is also higher in men, particularly for squamous cell carcinoma, which may reflect previous exposure to PUVA and/or ciclosporin. The risk of cutaneous T-cell lymphoma is substantially higher in patients with moderate-to-severe psoriasis. Biologic therapies are independently associated with a slight increase risk of cancer, but this is less than ciclosporin, with the risk confounded by disease severity and other co-risk factors. The risk of cancer from low-dose methotrexate is likely minimal. In contrast, acitretin is likely protective against a variety of solid and haematological malignancies. The data on small molecule therapies such as apremilast are too immature for comment, although no signal has yet been identified. The decision whether to stop psoriasis immune modulatory treatments following a diagnosis of cancer, and when to resume, needs to be considered in the context of the patients' specific cancer. However, there is no absolute need to stop any treatment other than possibly ciclosporin, unless there is a concern over an increased risk of serious infection or drug-drug interaction with cancer-directed therapies, including radiotherapy.

Psoriasis in those planning a family, pregnant or breast-feeding. The Australasian Psoriasis Collaboration.

The Australasian Psoriasis Collaboration has reviewed the evidence for managing moderate to severe psoriasis in those who are pregnant or are breast-feeding, or planning a family. The severity of the psoriasis, associated comorbidities and specific anti-psoriasis treatment, along with other exposures, can have a deleterious effect on pregnancy outcomes. Psoriasis itself increases the risk of preterm and low birthweight babies, along with spontaneous and induced abortions, but no specific birth defects have been otherwise demonstrated. The baseline risk for a live born baby to have a major birth defect is 3%, and significant neuro-developmental problem is 5%. In Australia, pregnant women with psoriasis are more likely to be overweight or obese, depressed, or smoke in their first trimester, and are also less likely to take prenatal vitamins or supplements. Preconception counselling to improve maternal, pregnancy and baby health is therefore strongly encouraged. The topical and systemic therapies commonly used in psoriasis are each discussed separately, with regards to pregnancy exposure, breast-feeding and effects on male fertility and mutagenicity. The systemic therapies included are acitretin, adalimumab, apremilast, certolizumab, ciclosporin, etanercept, infliximab, ixekizumab, methotrexate, NBUVB, prednisone, PUVA, secukinumab and ustekinumab. The topical therapies include dithranol (anthralin), calcipotriol, coal tar, corticosteroids (weak, potent and super-potent), moisturisers, salicylic acid, tacrolimus, and tazarotene. As a general recommendation, effective drugs that have been widely used for years are preferable to newer alternatives with less foetal safety data. It is equally important to evaluate the risks of not treating, as severe untreated disease may negatively impact both mother and the foetus.

Amyloidosis cutis dyschromica in two siblings and review of the epidemiology, clinical features and management in 48 cases.

Amyloidosis cutis dyschromica (ACD) is a rare form of primary cutaneous amyloidosis (PCA). There is a paucity of information in the dermatology literature to guide its diagnosis, investigation and treatment. We present two siblings with ACD and summarise the epidemiology, clinical features, natural history and treatments in 48 cases of ACD from the literature. Familial cases were more common (37) than sporadic cases. ACD is predominantly reported in those of East and South-East Asian ethnicity (63%). The mean age of onset was 6 years in familial cases, and 23 years in sporadic cases. The clinical features of familial and sporadic ACD do not differ substantially. Pruritus was the only symptom, and was reported in 19% of all cases. There were no reported ACD cases with systemic amyloidosis. Acitretin was reported to result in improvement in seven of 10 patients treated. Routine investigation for systemic involvement is not necessary. Acitretin may be helpful.

Novel mutations mapping to the fourth sodium channel domain of Nav1.7 result in variable clinical manifestations of primary erythromelalgia.

We identified and clinically investigated two patients with primary erythromelalgia mutations (PEM), which are the first reported to map to the fourth domain of Nav1.7 (DIV). The identified mutations (A1746G and W1538R) were cloned and transfected to cell cultures followed by electrophysiological analysis in whole-cell configuration. The investigated patients presented with PEM, while age of onset was very different (3 vs. 61 years of age). Electrophysiological characterization revealed that the early onset A1746G mutation leads to a marked hyperpolarizing shift in voltage dependence of steady-state activation, larger window currents, faster activation kinetics (time-to-peak current) and recovery from steady-state inactivation compared to wild-type Nav1.7, indicating a pronounced gain-of-function. Furthermore, we found a hyperpolarizing shift in voltage dependence of slow inactivation, which is another feature commonly found in Nav1.7 mutations associated with PEM. In silico neuron simulation revealed reduced firing thresholds and increased repetitive firing, both indicating hyperexcitability. The late-onset W1538R mutation also revealed gain-of-function properties, although to a lesser extent. Our findings demonstrate that mutations encoding for DIV of Nav1.7 can not only be linked to congenital insensitivity to pain or paroxysmal extreme pain disorder but can also be causative of PEM, if voltage dependency of channel activation is affected. This supports the view that the degree of biophysical property changes caused by a mutation may have an impact on age of clinical manifestation of PEM. In summary, these findings extent the genotype-phenotype correlation profile for SCN9A and highlight a new region of Nav1.7 that is implicated in PEM.

Subcutaneous granuloma annulare of the penis in 2 adolescents.

Granuloma annulare (GA) is a benign inflammatory disorder of unknown etiology characterized histologically by dermal palisading granulomas with central degeneration of collagen (necrobiosis). There is a rare subcutaneous clinical variant, this occurring more frequently in children than in adults and very rarely involves the penis. We describe 2 cases of penile subcutaneous GA developing in adolescent boys who to our knowledge has not previously been described in literature. Both were initially treated with surgical excision. Circumcision was performed on one of the boys, with subsequent improvement with the resolution of most of the nodules. Granuloma annulare of the penis is very rare, with only 7 cases reported to date [Narouz N, Allan PS, Wade AH. Penile granuloma annulare. Sex Transm Infect 1999;75(3):186-7; Trap R, Wiebe B. Granuloma annulare localised to the shaft of the penis. Scand J Urol Nephrol 1993;27(4):549-51; Laird SM. Granuloma annulare of the penis. Genitourin Med 1992;68(4):277; Hillman RJ, Waldron S, Walker MM, et al. Granuloma annulare of the penis. Genitourin Med 1992;68(1):47-9; Kossard S, Collins AG, Wegman A, et al. Necrobiotic granulomas localised to the penis: a possible variant of subcutaneous granuloma annulare. J Cutan Pathol 1990;17(2):101-4] and no previous reports in children or adolescents to our knowledge. All except one of the cases reported so far were of the subcutaneous (nodular) form of GA.

Two unusual cases of toxic epidermal necrolysis.

Two cases of toxic epidermal necrolysis (TEN) are presented. A 27-year-old woman presented with peripherally located targetoid plaques, papules, blisters and lip erosions which began 9 days after 'recreational' use of 'speed' (dexamphetamine and ephedrine) consistent with erythema multiforme major. Three days later she developed widespread lesions with large areas of blistering affecting 40% of body surface area. The diagnosis was revised to TEN. Intravenous cyclosporin led to rapid prevention of new blister formation. A 71-year-old woman, 3 months after commencing amiodarone, developed extensive erythema, blistering and erosions affecting 50% of body surface area, with a maculopapular rash affecting the limbs and extremities consistent with a diagnosis of TEN. She developed septicaemia following bilateral pneumonia with pleural effusions and died 7 days after admission.