British Association of Dermatologists National Clinical Audit on the Management of Hidradenitis Suppurativa in the UK.

BACKGROUND: The first UK guidelines for the management of hidradenitis suppurativa (HS) were published by the British Association of Dermatologists (BAD) in 2018. The guidelines contained a set of audit criteria. AIM: To evaluate current HS management against the audit standards in the BAD guidelines. METHODS: BAD members were invited to complete audit questionnaires between January and May 2020 for five consecutive patients with HS per department. RESULTS: In total, 88 centres participated, providing data for 406 patients. Disease staging using the Hurley system and disease severity using a validated tool during follow-ups was documented in 75% and 56% of cases, respectively, while quality of life and pain were documented in 49% and 50% of cases, respectively. Screening for cardiovascular disease risk factors was as follows: smoking 75%, body mass index 27% and others such as lipids and diabetes 57%. Screening for depression and anxiety was performed in 40% and 25% of cases, respectively. Support for smokers or obese patients was documented in 35% and 23% of cases. In total, 182 patients were on adalimumab, of whom 68% had documentation of baseline disease severity, and 76% were reported as having inadequate response or contraindications to systemic treatments; 44% of patients continued on adalimumab despite having < 25% improvement in lesion count. CONCLUSION: UK dermatologists performed well against several audit standards, including documenting disease staging at baseline and smoking status. However, improvements are needed, particularly with regard to screening and management of comorbidities that could reduce the long-term complications associated with HS. A re-audit is required to evaluate changes in practice in the future.

Inflammatory nail conditions. Part 1: nail changes in psoriasis.

Nail changes are visible in a variety of inflammatory dermatoses. The commonest dermatological condition with nail manifestations is chronic plaque psoriasis. This two-part article reviews the nail signs in psoriasis in Part 1, and the nail changes in cutaneous lichen planus and alopecia areata in Part 2. It provides a brief summary of the salient points in the clinical features, management and prognosis of these entities, with practical recommendations that may be beneficial to all dermatologists.

Inflammatory nail conditions. Part 2: nail changes in lichen planus and alopecia areata.

Nail changes are frequently seen in patients with cutaneous lichen planus and alopecia areata. This manuscript provides an updated overview on the clinical features, management and prognosis of both conditions. Searches of electronic databases PubMed and EMBASE were conducted and eligible articles were accessed. Practical management principles relevant to these two conditions are also included.

British Association of Dermatologists (BAD) National Audit on Non-Melanoma Skin Cancer Excision 2016 in collaboration with the Royal College of Pathologists.

BACKGROUND: We conducted a re-audit of the surgical practice of UK dermatologists for the treatment of nonmelanoma skin cancer and examined changes with reference to our previous audit in 2014. The audit was supplemented by a detailed assessment of completeness of the histopathology reports for each tumour. METHODS: UK dermatologists collected data on 10 consecutive nonmicrographic excisions for basal cell carcinoma (BCC) and 5 for squamous cell carcinoma (SCC). Data were collected on site, preoperative diagnosis, histological diagnosis, proximity to previous scars, and histological deep and peripheral margins. RESULTS: In total, 222 responses were received from 135 centres, reporting on 3290 excisions. Excisions from the head and neck accounted for 56.7% of cases. Tumour diameter (mean ± SD) was 11.4 ± SD 7.1 mm (maximum size 100 mm) and 97% of cases were primary excisions. BCCs and SCCs respectively accounted for 65.7% and 26.8% of total cases. Of the suspected BCCs and SCCs, 95.8% and 80.4%, respectively, were confirmed histologically. All margins for any tumour were clear in 97.0% of cases, and complication rate in the audit was < 1%. Of the 2864 histology reports evaluated, only 706 (24.6%) contained all core data items; 95% of these were structure (synoptic) reports. Commonly omitted items were level of invasion, risk and T stage, which were absent from 35.7%, 64.2% and 44.1% of reports, respectively. CONCLUSIONS: Diagnostic accuracy and complete excision rates remain high. Complication rates may be under-reported owing to lack of follow-up. Histopathology reporting has a greater chance of being complete if reports are generated on a field-based platform (synoptic reporting).

National audit on the management of bullous pemphigoid.

BACKGROUND: Bullous pemphigoid (BP) is an autoimmune, subepidermal, blistering condition that typically affects elderly people. AIM: To undertake a national clinical audit based on standards derived from the British Association of Dermatologists (BAD) clinical guidelines on the management of BP. METHODS: In 2018, BAD members were invited to submit data for five consecutive adults with BP per centre, who had been under hospital supervision for at least 12 months, in a national audit over an 11-week period. RESULTS: In total, 123 responders from 120 hospitals provided data for 524 cases. Diagnosis was made either clinically (10.7%; 56 of 524) or through histology with direct immunofluorescence (41.6%; 218 of 524), indirect immunofluorescence (10.3%; 54 of 524) or both (37.4%; 196 of 524). Most patients had very mild baseline disease (63.9%; 225 of 352) with 21.9% (77 of 352) considered mild, 9.8% (31 of 352) moderate and 5.4% (19 of 352) severe. Documentation of diabetes, glycated haemoglobin (HbA1c), blood pressure and hypertension was available for 54.1% (283 of 523), 51% (267 of 524), 44.2% (231 of 522) and 61.5% (321 of 522) of cases, respectively. Oral corticosteroids were commenced in 85.5% (448 of 524) of patients, with 38.4% (172 of 448) of these having documented risk of osteoporosis; data regarding prescription of bone-protection therapies were available for 99.7% (447 of 448) of cases, with 75.6% (338 of 447) of these having a bone-protection prescription. Patient satisfaction was documented in 59.3% (310 of 523) of cases. Systemic treatment was commenced in 95.9% (502 of 524) of cases during the 12-month assessment period, with baseline blood test and follow-up data available for 96.6% (485 of 502) and 95.6% (480 of 502), respectively. Documentation of baseline blood tests was available for 87.4% (424 of 485) of cases, with follow-up tests recorded in 69.8% (335 of 480). CONCLUSION: Overall, compliance with elements of documentation was moderate or low, whereas standards pertaining to direct care were high.

Compliance with national guidelines on isotretinoin: where are we 2 years since the last audit? Results of the National Isotretinoin Re-Audit 2014.

BACKGROUND: In 2010, the British Association of Dermatologists (BAD) published clinical guidelines for the safe introduction and continued use of isotretinoin in patients with acne in the UK. The BAD provides UK dermatologists with a facility for national audit, and it undertook an audit on compliance with these guidelines in 2012. AIM: To determine current clinical practices relating to use of isotretinoin among dermatologists in the UK (including geographical variations) as measured against BAD standards, and to ascertain any improvement since the 2012 audit. METHODS: The 2012 isotretinoin audit proforma was used, with additional questions on clinical setting, complaints and litigation. A web-based survey tool was used for data entry and submission, with email invitation to working, UK-based BAD members (n = 1226) in December 2013 and weekly reminders during the 8.5-week data collection period. Responders were requested to enter data for the three most recent consecutive patients (including one male and one female patient) who had completed treatment within the previous 6 months. RESULTS: In total, 338 (27.6%) respondents provided data on 1013 patients. Serum lipids were checked in 93.4% of patients and documentation of mental health and/or mood state was recorded in 82.1%. Regarding the Pregnancy Prevention Programme (PPP), 91.6% of female patients of childbearing potential had signed the PPP information form, while 93.3% who had followed the PPP had taken pregnancy tests both before and during treatment, and 54.7% had taken a pregnancy test 5 weeks post-treatment. CONCLUSION: Overall, there is currently good compliance with standards. Certain aspects of care that are less frequently preformed, such as pregnancy testing post-treatment, are highlighted.

British Association of Dermatologists' national audit on nonmelanoma skin cancer excision, 2014.

BACKGROUND: Diagnosis and management of nonmelanoma skin cancer (NMSC) represents a large part of the dermatology workload, and complete excision is a required surgical standard for treatment. AIM: To conduct an audit of the surgical practice for the treatment of NMSC by dermatologists in the UK. METHODS: Data on 10 consecutive nonmicrographic excisions of nonmelanoma skin cancer by UK dermatologists. Data collected included site, preoperative diagnosis, histological diagnosis, proximity to previous scars, and histological deep and peripheral margins. RESULTS: A total of 227 responses from 135 centres reported 2739 excisions. Excisions on the head and neck accounted for 58.3% of cases. Tumour diameter (mean ± SD) was 10.61 ± 6.9 mm (maximum 130 mm), and 96.7% of cases were primary excisions, with 3.3% being re-excisions. Basal cell carcinomas (BCCs) accounted for 79.1% (n = 2167) of the total cases and squamous cell carcinomas (SCCs) for 17.9% n = 491). Of the suspected BCCs and SCCS, 94.4% (n = 2045) and 66.8% (328), respectively, were confirmed histologically to be the respective carcinomas. Similar proportions of BCC and SCC cases were within 10 mm of a previous excision. Lateral and deep margins were clear in 98.3% and 99.2% of BCC cases, respectively, and in 98.4% and 97.1% of SCC cases, respectively. Reported surgical complication rate in the audit was 3.4%. CONCLUSIONS: The majority of excisions for NMSC are for BCC and SCC. Our figures for diagnostic accuracy are at the upper range of previously published figures. Most patients were not followed up in secondary care, hence complication rates may be under-reported.

Clinical triage of cutaneous squamous cell carcinoma and basal cell carcinoma to avoid treatment delay: value of an electronic booking system.

BACKGROUND: Provisional clinical diagnosis is the first step in planning skin surgery. Different clinical priorities are given to basal cell carcinoma (BCC), squamous cell carcinoma (SCC) and malignant melanoma (MM). Discriminating between SCC and BCC can be difficult. The rate of misdiagnosis of SCC as BCC is reported as 5.7-87.6%, and can cause treatment delay. We have developed a web-based surgery booking system that requires clinical commitment to a putative differential diagnosis category, rather than a single diagnosis, at the time of consultation. This includes a crucial overlap category of 'SCC or SCC/BCC'. AIM: To assess whether our system helped avoid treatment delay to patients with SCC, and to measure the number needed to treat (NNT). METHODS: This was a retrospective analysis from April 2012 to August 2013, comprising all patients undergoing booked excisional surgery in our unit. The clinical triaging category was compared with the histological diagnosis. Sensitivity, specificity, positive predictive value, negative predictive value (NPV), NNT and mean Breslow thickness were calculated. RESULTS: In total, 1455 lesions were excised, identifying 789 (54.2%) invasive malignancies (86 MM, 115 SCC, 583 BCC, 5 other), 100 in situ lesions and 150 dysplastic lesions. The majority (83.2%) of malignancies were designated into the correct malignant category. Misdiagnosis of SCC as BCC was 5.2%. Sensitivity and NPV for SCC were 94.8% and 99.4%, respectively. NNT was 1.26, 4.12 and 3.19 for BCC, SCC and MM respectively, and 1.73 for all malignancies. Mean invasive Breslow thickness was 1.29 mm [0.78 mm including melanoma in situ (MMIS)], and the MM to MMIS was 1.6. CONCLUSION: An overlap triage category of 'SCC or SCC/BCC' helps to prevent delay in the treatment for patients with SCC.

Subungual corn: a tender pigmented subungual lesion in older people.

BACKGROUND: Heloma durum occurs as a tender mass in the distal nail bed beneath the big toenail in older women. OBJECTIVES: To define and report a variant of heloma durum not referenced in the literature. METHODS: This was a retrospective study whereby records, including photographs, of all cases of subungual corn were reviewed. All patients were seen in an outpatient setting. RESULTS: The records of 16 patients [15 women, one man, mean age 68 years (range 49-87)] were examined; history was between 6 and 30 months, none of the patients had received effective treatment. Mycology was negative. All reported discomfort under the big toe nail; 12 had associated subungual haemorrhage. The right big toenail was involved in 10 of 16 patients. Shared clinical features were of a subungual focus of hyperkeratosis (100%) with haemorrhage admixed in 75% of cases. The lesion was in the midline third of the nail in 11 of 16 patients (69%). The affected distal margin of nail was the uppermost point in the lateral profile of the toe (100%). Hyperextension at the distal interphalangeal joint of the affected toe was demonstrated with the patient standing. Local excision was performed when diagnosis was unclear (eight patients); simple clearance of the keratin plug was performed in the other eight patients. There was no relapse in patients who were followed up for > 6 months (n = 7). CONCLUSIONS: Clinical explanation and paring down should be attempted in order to avoid surgery at this poor healing site in the elderly.

Multiple-digit periungual Bowen's disease: a novel treatment approach with radiotherapy.

Multiple periungual Bowen disease [BD; also known as squamous cell carcinoma (SCC) in situ] is rare. The pathogenesis of the disease is linked to human papilloma virus, and in some instances to chronic immunosuppression. The usual management of periungual BD is by local excision, Mohs micrographic surgery or distal phalanx amputation. Our patient was offered radiotherapy in the hope of maximizing residual function and minimizing morbidity from treatment. A good response was seen at 2 months post-radiotherapy, but this was followed by relapses at 4 and 6 months post-radiotherapy. Persistent anonychia resulted in improved access to the involved skin, making topical therapy possible. Radiotherapy can be a valuable management approach for periungual SCC/BD in locations where amputation could result in substantial disability.

Hair diagnoses and signs: the use of dermatoscopy.

BACKGROUND: Hair-shaft examination is diagnostically useful in a range of adult and paediatric conditions. OBJECTIVE: To evaluate the usefulness of dermatoscopy in hair-shaft microscopy. METHODS: Typical examples of selected conditions from an extensive collection of scalp hair were examined using a dermatoscope and a light microscope with paired cross-polarizing filters. Hair-shaft characteristics were photographed using a digital camera. RESULTS: Dermatoscopy was helpful in detecting tapered hairs, weathering, monilethrix, pediculosis capitis, peripilar casts, 'exclamation-mark' hairs of alopecia areata, bubble hair and pili torti. It was less helpful in pili annulati and unhelpful in detecting 'tiger-tail' banding in trichothiodystrophy. Light microscopy provided greater detail in almost all cases; it was necessary for detection of cuticle changes and added significant information in detecting characteristic features of trichothiodystrophy, pili annulati, bubble hair and pili torti. CONCLUSIONS: Dermatoscopy is most revealing in conditions resulting in gross changes in shaft outline and colour, where reflected light is valuable. It is unhelpful for detection of features within the shaft or at higher levels of resolution. When added to its ability to aid evaluation of scalp surface characteristics, dermatoscopy provides an excellent first-line method of assessment in clinics. In vivo it may aid screening and selection of hairs of greatest diagnostic yield for further assessment. In some instances, it may obviate the need for obtaining hair specimens and have implications for public health screening. Where detailed or cortical hair-shaft features need assessment, transmitted light microscopy remains the standard tool.

Survey of patients' experiences after nail surgery.

Nail surgery is performed to aid diagnosis and treatment of nail disease. A survey was conducted to determine whether patients understood the nature and consequences of nail surgery at the time of consent and to ascertain the most important aspects of morbidity after the procedure. The results identified that most patients understood the nature of their surgery and the immediate postoperative limitations they would face. Pain was short-lived, with no patients requiring analgesics after 6 weeks. The most important finding from this survey was that sensory disturbance was recorded by a large proportion (47%; 29/62) of patients. Of these, 35% (22/62) recorded either complete or partial resolution by 6-12 months after surgery, but 11% (7/62) noted no improvement. This is a point that is not made clear in standard surgical texts. The significance of dysaesthesia of a fingertip must be considered when counselling a patient before surgery.

Pili annulati: refinement of the locus on chromosome 12q24.33 to a 2.9-Mb interval and candidate gene analysis.

BACKGROUND: Pili annulati is an autosomal dominant hair shaft disorder characterized by alternating light and dark bands in hairs of affected individuals. Recently, a locus for pili annulati was mapped to chromosome 12q24.32-24.33 and recombination events defined a critical region of 9.2 cM (3.9 Mb). OBJECTIVES: The aim of the current study was to narrow the size of the candidate region and to identify the pathogenic mutation for pili annulati by analysing the candidate genes. METHODS: In three families with 90 individuals, including 40 affected subjects, linkage analysis was performed with 13 microsatellite markers in the candidate region on chromosome 12. Candidate genes were analysed for their expression in hair follicles and other tissues by reverse transcriptase-polymerase chain reaction (RT-PCR) and mutation analysis. RESULTS: Multipoint LOD score analysis for all three families confirmed the locus on the long arm of chromosome 12 with a maximum LOD score of 12.26 at marker D12S357. In two families, recombinations were identified which narrowed the region to 2.9 Mb containing 36 genes. We analysed the candidate genes in this region by RT-PCR and found that 24 were expressed in human hair follicles. Based on the result of the expression analysis, DNA sequencing of the coding region of the candidate genes was performed; this did not result in the discovery of a causal mutation. CONCLUSION: We reduced the critical interval of pili annulati to 2.9 Mb and excluded mutations in the coding region of all 36 possible candidate genes by sequence analysis.

Immunohistochemical staining for the differentiation of subungual keratoacanthoma from subungual squamous cell carcinoma.

BACKGROUND: Subungual keratotic tumours are rare. The clinical and histological distinctions between subungual keratoacanthomas (SUKAs) and subungual squamous cell carcinomas (SCCs) are important, but often difficult. Adequate methods of differentiation between the two are required, both for the purpose of management and for assessment of prognosis. AIM: To establish the value of immunohistochemical staining patterns of proliferating cells to distinguish between SUKAs and subungual SCCs. METHODS: In total, 20 keratotic tumours from 20 patients were examined with immunohistochemical staining techniques using bcl-2, Ki67 and p53. RESULTS: Of 20 patients, 4 had SUKAs, 5 had cutaneous KAs, 6 had subungual SCCs and 5 had cutaneous SCCs. Our results showed that a high index of staining of p53 favours the diagnosis of subungual SCC over SUKA. CONCLUSION: SUKAs do not express Ki67 strongly whereas some subungual SCCs do. Thus we conclude that immunohistochemistry for p53 and Ki67 may help distinguish between a subungual SCC and a SUKA.

Nail biology and nail science.

The nail plate is the permanent product of the nail matrix. Its normal appearance and growth depend on the integrity of several components: the surrounding tissues or perionychium and the bony phalanx that are contributing to the nail apparatus or nail unit. The nail is inserted proximally in an invagination practically parallel to the upper surface of the skin and laterally in the lateral nail grooves. This pocket-like invagination has a roof, the proximal nail fold and a floor, the matrix from which the nail is derived. The germinal matrix forms the bulk of the nail plate. The proximal element forms the superficial third of the nail whereas the distal element provides its inferior two-thirds. The ventral surface of the proximal nail fold adheres closely to the nail for a short distance and forms a gradually desquamating tissue, the cuticle, made of the stratum corneum of both the dorsal and the ventral side of the proximal nail fold. The cuticle seals and therefore protects the ungual cul-de-sac. The nail plate is bordered by the proximal nail fold which is continuous with the similarly structured lateral nail fold on each side. The nail bed extends from the lunula to the hyponychium. It presents with parallel longitudinal rete ridges. This area, by contrast to the matrix has a firm attachment to the nail plate and nail avulsion produces a denudation of the nail bed. Colourless, but translucent, the highly vascular connective tissue containing glomus organs transmits a pink colour through the nail. Among its multiple functions, the nail provides counterpressure to the pulp that is essential to the tactile sensation involving the fingers and to the prevention of the hypertrophy of the distal wall tissue, produced after nail loss of the great toe nail.

Multiple myxoid cysts secondary to occupation.

We report the case of a 50-year-old woman who presented with eight digital myxoid cysts (DMCs) involving the fingers of both hands. They developed within 12 months of the patient starting a job that involved pushing a garment into an embroidery mould, thus exerting a downward force on the fingertips. The pressure exerted from this force could have potentially damaged the joint synovial capsule, leading to rupture and loss of synovial gel, thus inducing myxoid cysts. This case suggests that DMCs may be related to occupation, and to our knowledge, this is only the second reported case of occupationally induced DMCs.

Cytokeratin expression in pili annulati hair follicles.

Pili annulati is a rare autosomal inherited hair shaft abnormality of unknown pathogenesis in which clinical examination reveals alternating light and dark bands leading to a shiny appearance of the hair due to cavities within the cortex of the hair shaft. This is the first investigation of the proposed cytokeratin defect in pili annulati hair follicles. Four cryopreserved pili annulati and four control scalp specimens were analysed using immunohistochemistry for different 'hard' trichocytic and 'soft' epithelial cytokeratins including K1, K6, K10, K14, K16, K17, K18, K19, Ha1 and Hb1. There was no difference in staining intensity and quality of staining pattern seen in pili annulati and control scalp specimens. These results suggest that pili annulati is not caused by a defect of the cytokeratins investigated in this study.

Update on detection, morphology and fragility in pili annulati in three kindreds.

BACKGROUND: Pili annulati is an inherited hair shaft abnormality with a wide range of clinical expression. OBJECTIVE: We have examined closely three kindreds to reveal levels and character of expression of the phenotype and supplement current literature on the threshold for detection and aspects of hair shaft fragility. PATIENTS AND METHODS: Eleven cases of pili annulati from three families were included in a clinical and morphological study. All cases were assessed clinically and by light and scanning electron microscopy (SEM) of hair shafts. In addition, transmission electron microscopy (TEM) (four patients) and amino acid analysis (three patients) were undertaken on clinically overt cases. Results Examination by light microscopy with a fluid mountant was more sensitive than clinical examination, increasing the detection rate by 120%. Microscopic examination revealed that the characteristic periodic bands become less frequent distally in the hair shaft. Microscopic features of weathering were found in two cases, adding pili annulati to the list of structural hair shaft dystrophies that may weaken hair and dispose to weathering. Amino acid analysis of the hair of three patients with pili annulati showed elevated lysine and decreased cystine content compared to 12 normal controls, consistent with the reduced threshold for weathering. CONCLUSION: Careful light microscopy with fluid-mounted hair is needed to detect subjects mildly affected by pili annulati. Expression of the phenotype varies widely between individuals, between hairs and within hairs of the same individual, where ageing of the hair diminishes detectable features.