A. Affleck

Seven years' experience of Mohs micrographic surgery in a UK centre, and development of a UK minimum dataset and audit standards.

Mohs micrographic surgery (MMS) is the gold‐standard treatment for high‐risk basal cell carcinomas and a variety of other cutaneous tumours, including dermatofibromasarcoma protuberans and microcystic adnexal carcinoma. Previous large‐scale case series, audits and reviews have allowed evaluation of MMS outcomes, such as BCC recurrence rates. However, to date there has been no systematic UK MMS audit, and certain important aspects of care, such as postoperative functional outcomes, have not yet been subject to scrutiny.

Dermatoscopic features of extrafacial lentigo maligna.

The dermatoscopic features of classic lentigo maligna (LM) are well described; however, there is little literature available on extrafacial LM, which is a less frequently reported condition. The skin architecture is especially rich in adnexae on sun‐exposed areas such as the face, relative to other parts of the body, thus it is possible that the dermatoscopic findings of extrafacial LM will differ from the usual findings of LM. We carried out a dermatoscopic study on three patients with extrafacial LM. The dermatoscopic patterns reflected the underlying histological features of the disease, with increased melanocytes seen along the rete ridges and around follicular ostia, which result in a unique pigment network architecture.

Diagnosis and management of lentigo maligna: an observational study comparing 2005 with 2014 data in one institution

The incidence, clinical characteristics and management of lentigo maligna (LM) were assessed in a university hospital setting in 2005 and 2014. Multiple clinical variables were compared, and 28 and 43 cases, respectively were identified during the two time periods. The most common site of presentation was the cheek (50% vs. 44%), and an accurate clinical diagnosis of LM was made in 60% vs. 72%) of cases. Most of the patients received surgical treatment (75% in 2005 vs. 97% in 2014), with 47% and 33% of excisions, respectively, remaining involved at the peripheral surgical margin. During the 10‐year follow‐up for the 2005 cohort, 7 of the 28 patients had recurrence (3 of whom already had previously involved margins following surgery). This study shows that making an accurate clinical diagnosis of LM remains a significant challenge. Although surgery has become the preferred management option, achieving clear excision remains difficult, with involved margins increasing the risk of local recurrence and need for further intervention.

Body dysmorphic disorder in dermatology: beware of diagnostic overlabelling

G. J. Parkins, K. M. Brennan and G. Wylie Department of Dermatology, Dermatology Out-patients, Southern General Hospital, Alan Lyell Centre for Dermatology, Glasgow, UK; and Department of Neurology, Carver College of Medicine, University of Iowa, Iowa, IA, USA E-mail: parkins38@hotmail.co.uk Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 2 January 2015

Aggressive cutaneous squamous cell carcinoma arising from a human papillomavirus-infected epidermoid cyst of the conchal bowl

to move from their region of training after obtaining their CCT, with comparable results in 2011 and 2016; the percentages prepared to work only in their own region or an adjacent region were 86% in 2011 and 87.5% in 2016, while the percentages of those prepared to move anywhere in the UK were 14% and 13%, respectively. There was a high level of awareness of the current volume of unfilled Consultant posts in the UK, probably due to the impact that staff shortages have on the working environment. Not one respondent believed there to be no Consultant shortages in the UK, compared with 12% in 2011, and this was accompanied by a 130% increase in respondents who were aware of current shortages. In summary, the results of the BAD 2016 trainee survey suggest that trainees plan to work on average for 7.6 years in the 10 years following CCT. There was a 50% decrease in trainees who were interested in academic work. This data collection enables the association to consult with the Department of Health, Public Health England and Health Education England, who provide intelligence to the health and care systems to inform workforce planning decisions at a national and local level. This information on careers choices will help predict consultant vacancies and regional workforce distribution and thereby projected workforce demand. Further investigation is required to understand why trainees are turning from academic careers, which, if confirmed, threatens the future of academic dermatology. Unless national workforce planning takes into account the career plans of trainees that will affect their availability to fill NHS roles, there will be no resolution of the current workforce crisis. N. Goad and N. J. Levell British Association of Dermatologists, London, UK; and Dermatology Department, Norfolk and Norwich University Hospital, Norwich, UK E-mail: nina@bad.org.uk Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 1 July 2017

Excision of nonmelanoma skin cancer overlying arteriovenous fistulae

Surgical excisions of skin cancers overlying an arteriovenous fistula (AVF) are rarely reported, but they pose challenges in excision and reconstruction. We present two such patients and discuss the potential clinical issues. Patient 1 was a 45-year-old male renal transplant recipient, who presented with a 6-month history of a persistent, pink, indurated plaque 6 mm in diameter on his right wrist, directly overlying an AVF created 20 years previously to facilitate haemodialysis (Fig. 1a). Initially, curettage and electrodissection were attempted. Histopathology confirmed a basal cell carcinoma of multiple histological subtypes with deep margin involvement. There was clinical persistence at the 2-month review. Patient 2 was an 84-year-old man, who developed a raised erythematous firm lesion 10 mm in diameter, directly overlying the anastomosis of his left brachiocephalic fistula used for haemodialysis for end-stage hypertensive renal disease (Fig. 1b) Based on the clinical findings, a squamous cell carcinoma (SCC) was suspected. In both cases, the lesions were excised and the defects repaired in a linear bilayer subcutaneous manner with a vascular surgeon assisting. Careful slow dissection above the fistula avoided inadvertent trauma, and no surgical complications were encountered (Fig. 1c). In both cases, histology revealed clear deep and peripheral margins. An AVF is a vital high-pressure vascular flow structure, which allows vascular access for patients with chronic renal failure requiring haemodialysis, and therefore its preservation is paramount. The presentation of a nonmelanoma skin cancer overlying an AVF poses the question of whether an artificially augmented circulation can be considered as a preferential site for tumour formation. Theoretically, this could be possible, although the exact underlying mechanism is unknown; however, it has has been postulated to result from the combined effects of an altered endothelial milieu caused by aberrant flow dynamics and a long-term local immunosuppression. One large study identified 68/980 transplant recipients who developed a skin cancer on the same side as their AVF, but no significant difference was found in the distribution of skin cancers, total malignancies or malignancy subgroups between the limbs with AVF and those without. Tumour development at a fistula site may lead to AVF dysfunction due to multiple reasons such as direct invasion of the tumour into the wall of the fistula, altered haemodynamics and access difficulty secondary to extrinsic compression of the fistula due to mass effect. Nonsurgical treatment may be tempting, but it risks incomplete tumour clearance and local recurrence. (a)

Periodic facial erythema in a patient with postural orthostatic tachycardia syndrome

A 27-year-old woman had been attending our Dermatology department for the ongoing management of longstanding periodical facial erythema. These episodes were associated with a burning sensation and, were typically brought on by heat or exercise and were relieved only with cooling measures such as sitting in front of a fan. The patient had noted that the flares were becoming more frequent (occurring almost daily) and more severe. Initially, the episodes were unilateral but over time, they became bilateral in distribution. On physical examination, a well-demarcated patch of erythema was seen, which was warm on palpation (Fig. 1a,b). The patient had also noticed similar burning red skin on the dorsum of her foot (Fig. 1c). Routine blood tests, including plasma viscosity, serum tryptase, antinuclear antibodies and antineutrophil cytoplasmic antibodies, were negative. The patient had been treated initially with various different oral antihistamines and montelukast because the diagnosis was thought to be urticaria, and later with topical bromonidine cream [MIRVASO; Galderma (UK) Ltd, Watford, Hertfordshire, UK], which had offered minimal relief. It was at this stage the patient’s longstanding background of difficult-to-control postural orthostatic tachycardia syndrome (POTS) was considered to be possibly contributing to her dermatological presentation. POTS was first described in 1993 as an abnormal response by the autonomic nervous system to standing up, and is associated with multiple symptoms, including dizziness, and resolution of symptoms with recumbence. Various different subtypes of the syndrome are recognized, such as neuropathic, hyperadrenergic, volume dysregulation and physical deconditioning, but ultimately the aetiology is that of underlying autonomic dysfunction. Clinically, POTS can manifest as fatigue, exercise intolerance, presyncope, syncope, headaches, brain fog, anxiety, nausea or sleep disturbance. Our patient had seven of these nine symptoms. She had been diagnosed 3 years earlier by a consultant cardiologist when an active stand test showed an increased heart rate from 60 bpm when supine to a maximum of 131 bpm after 10 min standing upright, with only a slight fall in systolic blood pressure of 10 mmHg. Several dermatological manifestations of POTS appear to be common, including livedo reticularis, acrocyanosis, Raynaud phenomenon, flushing and evanescent hyperaemia. We believe our patient’s painful facial and acral flushing episodes are likely to be a variation of erythromelalgia secondary to POTS. Erythromelalgia is a rare clinical entity that involves an intense burning sensation or discomfort associated with heat and erythema. Typically, this involves gravitydependent acral surfaces, but a few cases have reported involvement of the ear, scrotum and face. The aetiology remains controversial and poorly understood, but many authors agree that although the symptoms are heterogeneous, the primary abnormality is of dysfunctional neurovascular dynamics and a small-fibre neuropathy. Although the diagnosis of POTS is clinical, it is important to exclude other causes of secondary erythromelalgia such as haematological disorders (e.g. polycythemia) and connective tissue disease. Evidence for successful treatment of erythromelalgia is limited to individual case reports or small case series. Cohen summarized treatment responses of Correspondence: Dr Sidra Khan, Department of Dermatology, Ninewells Hospital, Ninewells Drive, Dundee, DD1 9SY, UK E-mail: sidra.khan2@nhs.net

Burning red face syndrome: a heterogeneous group of facial erythrodysaesthesias.

We congratulate Parkins et al. for their case report and discussion of so-called ‘neurogenic’ rosacea. It is important to raise awareness amongst dermatologists of this symptom complex, which has been recognised for many years, although under-reported and under-researched. Prior to 2011, dermatologists used terms such as ‘facial erythrodysaesthesia’ or ‘rosacea with functional overlay’. The case series from 2011 is very interesting to scrutinise. The authors found that 12/14 patients had > 2 coexisting diagnoses that may have been contributory or sharing the same aetiology: with 6 having migraine, 4 chronic pain, 5 depression, 1 anxiety, 1 obsessive compulsive disorder, 1 bulimia, 5 Raynaud phenomenon and 4 conditions that could be termed somatoform. Attempting to make this a specific entity appear to be too simplistic. Use of the adjective ‘neurogenic’ implies one cause that does not fit with the multifactorial nature of rosacea (and many other inflammatory dermatoses). Notably, the diagnosis of neurodermatitis has gone out of favour in this regard. As the patient’s skin often appears normal between acute flares, their associated symptoms may trigger referral to a number of different medical specialists. We suggest that the diagnostic label the patient is likely to receive depends on the specialist they attend. Cognitive bias and human factors, as well as the physician’s clinical experience, will influence the chosen diagnosis. Therefore a neurologist may diagnose facial migraine and consider multiple sclerosis, while a psychiatrist may suggest depression with prominent somatic symptoms or social anxiety disorder. In a patient with parkinsonism attending a department of medicine for the elderly, the symptoms may be considered to be part of this disorder. Should the patient be attending rheumatology with systemic lupus erythematosus, a diagnosis of secondary erythromelalgia may be favoured, while if the patient attends specialist pain services, their symptoms are likely to be considered part of the chronic pain syndrome. Patients with Raynaud phenomenon may be considered to have primary vasomotor instability. As the symptom complex is nonspecific, it is impossible to reach a definitive diagnosis in many cases, and differential diagnoses will include all the entities noted above. Other accurate descriptions would be neurological flushing or somatoform burning skin. The use of ‘syndrome’ emphasises that there are likely to be many causes for the same symptom complex. We note the patient’s disproportionate sensory symptoms, distress and internet searching for a possible cure – typical findings in illness anxiety disorder. It is perhaps no coincidence that both pregabalin and duloxetine, reported to help in 7/14 patients, are also used to treat generalized anxiety disorder. We suggest that the symptom complex lies best, and is analagous to, other forms of localized cutaneous erythrodysesthesias e.g. angry red scrotum syndrome and burning red ear syndrome. We agree that a biopsychosocial approach to management is desirable, with a detailed psychiatric/psychological assessment and tailored individual therapy, keeping an open mind as to the underlying cause. The goal of treatment is to reduce pain and distress, improve functioning and well-being, and help the patient achieve a sense of control. In our view, a multifactorial management approach is most likely to succeed, and input from clinical psychology and/or liaison psychiatry are often helpful.

Recurrent corneal erosion syndrome associated with oral isotretinoin: a cautionary tale.

Oral isotretinoin is known to cause ocular side effects, most commonly dry eyes and very rarely, corneal opacity and keratitis. We describe what we believe is the first case of recurrent corneal epithelial erosion (RCES) precipitated by the commencement of isotretinoin therapy for acne vulgaris. A 38-year-old woman presented to her optometrist with severe, spontaneous, sudden-onset pain, along with photophobia, watering and redness of her left eye. She had persistent excoriated acne, which had been treated successfully over the previous 18 months with oral isotretinoin 20 mg daily. On physical examination, an area of corneal epithelial loss was detected, and standard treatment with topical antibiotic ointment was commenced. One month later, the patient re-presented to the ophthalmology clinic with the same symptoms in the same eye. Corneal examination revealed epithelial microcysts, typical of recurrent corneal erosion syndrome, as well as a corneal epithelial defect. Long-term prophylactic ocular lubrication was prescribed. On further enquiry, the patient described minor trauma to her left eye 2 years previously, for which she had received treatment at a different centre. It was deemed probable that the isotretinoin was having an exacerbating effect on the ocular surface environment, and thus the dose was reduced. The patient continued to use regular lubrication and had no further episodes of recurrent corneal erosion over a follow-up period of 6 months. Recurrent corneal epithelial erosion is an acutely painful relapsing ocular condition caused by defective attachment of the basal cells of the corneal epithelium to their basement membrane, and is typically associated with a history of previous traumatic corneal abrasion. In the months/years following an abrasion, episodes of corneal epithelial breakdown (erosions) are experienced by an affected individual, usually overnight or on waking, when the ocular surface is less well lubricated by the blink mechanism. Salient clinical features include pain, photophobia and epiphora. Long-term ocular lubrication is the mainstay of prophylaxis in order to maintain tear film stability and ensure corneal protection. A stable tear film is essential for normal vision, ocular comfort and maintenance of a healthy ocular surface environment. The meibomian glands secrete the lipid layer of the tears, and are essential to reduce tear evaporation and maintain tear film stability. Isotretinoin has a direct effect on the function of these glands, causing atrophy and hypoplasia. In a study of 1741 adverse drug reactions related to isotretinoin, there were no reports of RCES. Any cause of tear film instability or meibomian gland dysfunction could precipitate or exacerbate RCES in vulnerable patients, and we believe that isotretinoin should be considered a risk factor for RCES in such cases. We advise that clinicians commencing patients on isotretinoin should ask about previous ocular conditions, including previous trauma and corneal abrasions. For patients with such conditions, the low-risk prophylactic intervention of commencing ocular lubricants should be suggested, as well as making the patient aware of the symptoms of RCES.

Atypical hidradenitis suppurativa

Hidradenitis suppurativa (HS) is a chronic relapsing inflammatory disease resulting in subcutaneous abscesses, sinus tracts and scarring, arising predominantly in apocrine gland‐bearing skin. The sites affected, in order of frequency, are the axillae, groins, perianal and perineal region, mammary and submammary skin, buttocks, and pubic region. Other sites that may be affected more rarely include the chest, eyelids, scalp, retroauricular and preauricular skin, thighs, and abdomen. Research has suggested that mechanical friction and follicular occlusion is the primary cause of HS. HS is one of the conditions of the ‘follicular occlusion tetrad’, which share a similar pathogenesis. HS at atypical sites may mimic other conditions both clinically and pathologically, so misdiagnosis is possible. We present a case series that illustrates a range of atypical sites affected. Our patients all had severe disease. Satisfactory control has not been achieved despite years of medical and surgical interventions.