British Association of Dermatologists national clinical audit on the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults.

BACKGROUND: UK guidelines for managing adults with Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN), published by the British Association of Dermatologists (BAD) in 2016, outline a set of audit standards. OBJECTIVES: To audit current management of SJS/TEN in adults against standards in the BAD guidelines. METHODS: BAD members were invited to submit data on five consecutive adults with SJS/TEN per department over an 8-week period in 2022. RESULTS: Thirty-nine dermatology centres in the UK (29%) participated, and data for 147 adults with SJS/TEN were collected. Within 24 h of the diagnosis being made or suspected, the following were documented, per 147 submitted cases: Severity-of-Illness Score for Toxic Epidermal Necrolysis (SCORTEN) for 76 (52%), list of medications for 113 (77%) and timelines for commencement/alterations of medications for 104 (71%). The initial assessment was documented of the eyes by an ophthalmologist in 71 (48%), of the mouth in 130 (88%), of the genital skin in 103 (70%) and of the urinary tract in 93 (63%). During the first 10 days after a suspected or confirmed diagnosis of SJS/TEN, daily assessments of the mouth were documented in 26 of 147 cases (18%), of the eyes in 12 (8%), and of the urinary tract and genital skin in 14 (10%). At discharge, a drug was declared to be the cause of SJS/TEN for 130 of 147 cases (88%), while 9 (6%) were thought to be secondary to infection. Eleven of 147 (8%) had no response to this question. Documentation regarding advice was present on avoidance of the culprit drug in 76 of 130 declared SJS/TEN cases (58%), and on requesting a MedicAlert® bracelet/amulet in 9 of the 147 cases (6%). CONCLUSIONS: This audit suggests that a clinical review checklist might be needed to enable colleagues to maintain standards outlined in the guidelines, including documentation of SCORTEN, daily assessments of mucosal areas, and advice to avoid culprit drug(s) and to request a MedicAlert® bracelet/amulet.

WHO Surgical Checklist in Dermatology: Compliance, Barriers, and Attitudes.

BACKGROUND: The World Health Organization (WHO) surgical checklist is associated with reduced morbidity and mortality. Efficacy correlates with compliance. OBJECTIVE: This study aims to (1) establish completion rate and (2) identify and address barriers to use. METHODS: Records of patients undergoing dermatological surgery were studied. Staff completed attitude and barriers questionnaires. Checklist process was modified, and use was reassessed twice. RESULTS: Cycle 1 involved 217 subjects; 72% had excisions. Thirteen percent had surgery to multiple sites. Five percent of checklists were fully completed, with an average of 76% of available points per checklist marked as checked. The lowest single field use included "patient identity" (76%) and "surgical site" (72%). Questionnaire responses from 25 staff showed the checklist to be "important" and "relevant" in dermatology; key barrier to completion was lack of time. Checklist modifications and educational sessions were undertaken; checklist use was reassessed twice more with 103 and 134 patients. Average use increased to 96% and 98%; full completion increased to 71% and 70%; "surgical site" and "identity" completion increased to 100%. CONCLUSION: The WHO checklist is relevant and important in dermatology. Introduction must be supported by repeated training sessions. Adequate time and training can significantly improve checklist completion and patient safety.

An international survey about nail histology processing techniques.

BACKGROUND: There are limited data on nail histopathology techniques. The objective of this study was to examine nail histopathology techniques currently in use internationally. METHODS: An online survey was sent to the European Nail Society and Council for Nail Disorders during 2015-2016. RESULTS: There were 57 respondents, from twenty countries comprising dermatologists, podiatrists and pathologists. Specimens were unmarked or marked using ink or a suture and fixed in 10% formalin, from 6 to 48 hours before embedding in paraffin wax (90% [17/19]), liquid nitrogen (frozen section, 1/19) and 2-hydroxyethylmethacrylate (plastic, 1/19). Nail softening was undertaken by 71% (17/24) of respondents for 6 to 48 hours using Mollifex Gurr (12.5%, 3/24), 10% potassium hydroxide solution (12.5%, 3/24) or 10% potassium thioglycolate cream (12.5%, 3/24). Section thickness was 4 to 9 µm (62.5%), using a steel microtome (92%,12/13) on glass slides (91.6%, 11/12). Hematoxylin and eosin (H&E) was routine for all biopsies and Periodic acid Schiff (PAS) for fungus. The favored stain for differentiating melanin and hemoglobin was Fontana-Masson (60%, 6/10). For pigmented lesions, Melan-A was always employed by all respondents (9/9). CONCLUSION: Nail histopathology processing has some small variations from normal skin processing.

Penicillin to prevent recurrent leg cellulitis.

BACKGROUND: Cellulitis of the leg is a common bacterial infection of the skin and underlying tissue. We compared prophylactic low-dose penicillin with placebo for the prevention of recurrent cellulitis. METHODS: We conducted a double-blind, randomized, controlled trial involving patients with two or more episodes of cellulitis of the leg who were recruited in 28 hospitals in the United Kingdom and Ireland. Randomization was performed according to a computer-generated code, and study medications (penicillin [250 mg twice a day] or placebo for 12 months) were dispensed by a central pharmacy. The primary outcome was the time to a first recurrence. Participants were followed for up to 3 years. Because the risk of recurrence was not constant over the 3-year period, the primary hypothesis was tested during prophylaxis only. RESULTS: A total of 274 patients were recruited. Baseline characteristics were similar in the two groups. The median time to a first recurrence of cellulitis was 626 days in the penicillin group and 532 days in the placebo group. During the prophylaxis phase, 30 of 136 participants in the penicillin group (22%) had a recurrence, as compared with 51 of 138 participants in the placebo group (37%) (hazard ratio, 0.55; 95% confidence interval [CI], 0.35 to 0.86; P=0.01), yielding a number needed to treat to prevent one recurrent cellulitis episode of 5 (95% CI, 4 to 9). During the no-intervention follow-up period, there was no difference between groups in the rate of a first recurrence (27% in both groups). Overall, participants in the penicillin group had fewer repeat episodes than those in the placebo group (119 vs. 164, P=0.02 for trend). There was no significant between-group difference in the number of participants with adverse events (37 in the penicillin group and 48 in the placebo group, P=0.50). CONCLUSIONS: In patients with recurrent cellulitis of the leg, penicillin was effective in preventing subsequent attacks during prophylaxis, but the protective effect diminished progressively once drug therapy was stopped. (Funded by Action Medical Research; PATCH I Controlled-Trials.com number, ISRCTN34716921.).

Trends in Pediatric Laboratory-Diagnosed Onychomycosis Between 2006 and 2014 in the Southwest of England.

This is the largest study of laboratory-diagnosed onychomycosis in England for children younger than 17 years. The most common (91.5%) cultured organism in this population was Trichophyton rubrum. Candida species were isolated only from fingernails, and the majority were from children younger than 5 years. Continued analysis of fungal pathogens is vital to identify changing trends.

Full-thickness skin grafts for lower leg defects: an effective repair option.

BACKGROUND: Repair of lower leg defects after excision of skin lesions that are not amenable to primary closure can be challenging. OBJECTIVE: To evaluate the outcome of full-thickness skin grafts (FTSG) to repair lower leg defects after excision of cutaneous lesions. To assess graft take at Days 7 and 30 and the number of visits to secondary care after procedure. MATERIALS AND METHODS: Retrospective review of 50 consecutive patients who underwent FTSG to cover defects below the knee between January 2009 and February 2014. Graft take was defined as good (90% healing and pink/purple), moderate (50% healing and pink/purple and >50% graft take), or poor (>50% graft failure). RESULTS: Mean age was 75 years (range, 49-96 years). The mean area of the defect was 52.4 cm. The mean maximum and minimum diameters of the defect were 2.8 and 2.3 cm. Graft take was good in 44 patients (88%), moderate in 5 patients (10%), and poor in 1 patient (2%) at Day 30. Complications were infrequent and included infection and ulceration. There was no significant association between the graft size and graft take. CONCLUSION: Full-thickness skin graft is an effective method of repairing defects on the lower leg after removal of cutaneous lesions. The aftercare of FTSG was acceptable with 86% of patients requiring 5 or fewer visits to secondary care.

The gene encoding R-spondin 4 (RSPO4), a secreted protein implicated in Wnt signaling, is mutated in inherited anonychia.

Anonychia and hyponychia congenita (OMIM 206800) are rare autosomal recessive conditions in which the only presenting phenotype is the absence or severe hypoplasia of all fingernails and toenails. After determining linkage to chromosome 20p13, we identified homozygous or compound heterozygous mutations in the gene encoding R-spondin 4 (RSPO4), a secreted protein implicated in Wnt signaling, in eight affected families. Rspo4 expression was specifically localized to developing mouse nail mesenchyme at embryonic day 15.5, suggesting a crucial role in nail morphogenesis.

Pitfalls and pearls in the diagnosis of monilethrix.

A 4-year-old girl presented with sparse, brittle hair on her entire scalp and keratosis pilaris on the nape of her neck. Subtle microscopic and macroscopic diagnostic features presented a challenge for physicians. Only repeated, optimized light microscopy revealed the diagnosis of monilethrix, a rare genetic hair shaft disorder with a variable phenotypic expression and inheritance pattern. We provide a short overview of methods that maximize the diagnostic yield in a clinical setting and of light microscopy to reach a rapid and accurate diagnosis in difficult cases. We conclude with essential learning points, including a link to assistance with hair microscopy from a tertiary center.

Erythronychia.

Erythronychia is a term that covers a range of pathological patterns of red discoloration of the subungual tissues. The intensity of the red contrasts with the pale pink of the nail bed or the cream color of the lunula. It is typically due to one or more actors that include inflammation, vessel proliferation, and engorgement and focal thinning of the nail plate. This article describes the patterns both within an individual digit and when it extends to multiple digits. These patterns cover diverse inflammatory, infiltrative, and neoplastic diseases, and the correlation between these diagnoses and patterns is discussed alongside means of surgical diagnosis and cure.

Concomitant manifestation of pili annulati and alopecia areata: coincidental rather than true association.

The autosomal dominantly inherited hair disorder pili annulati is characterized by alternating light and dark bands of the hair shaft. Concomitant manifestation of pili annulati with alopecia areata has been reported previously on several occasions. However, no systematic evaluation of patients manifesting both diseases has been performed. We studied the simultaneous or sequential occurrence of pili annulati and alopecia areata in individuals diagnosed in different European academic dermatology units. We included 162 Caucasian individuals from 14 extended families, comprising 76 affected and 86 unaffected family members. Statistical analysis showed that the frequency of alopecia areata among patients with pili annulati was higher than within the general population. Five of our patients with pili annulati have gone through severe episodes of alopecia areata. We cannot rule out that the currently unknown genetic defect underlying pili annulati might also confer an increased risk for the development of a more pronounced manifestation of alopecia areata. Based on the current data, and considering the low number within individual families of patients affected by both diseases, however, a direct association between pili annulati and alopecia areata seems unlikely.

Nails in systemic disease.

A change in colour, size, shape or texture of finger- and toenails can be an indicator of underlying systemic disease. An appreciation of these nail signs, and an ability to interpret them when found, can help guide diagnosis and management of a general medical patient. This article discusses some common, and some more rare, nail changes associated with systemic disease.

'Relaxers' damage hair: evidence from amino acid analysis.

BACKGROUND: 'Relaxers' are used by more than two thirds of African females to straighten hair, with easy grooming and increased length often cited as reasons. A recent study reported relaxed hair lengths much shorter than expected, suggesting increased fragility; the potential for scalp inflammation and scarring alopecia remains unclear. OBJECTIVE: To investigate the biochemical effects of 'relaxers' on hair. METHODS: With informed consent, included participants represented 3 groups: natural hair, asymptomatic relaxed hair, and symptomatic (brittle) relaxed hair. Biochemical analysis was performed by using a Biochrom 30 amino acid analyzer. Differences in amino acid levels were assessed using either Wilcoxon rank sum test or matched-pairs signed-rank test. RESULTS: There was a decrease in cystine, citrulline, and arginine; however, an increase in glutamine was found in all relaxed compared to natural hair. Cystine levels (milligram per gram amino acid nitrogen) were similar in natural proximal and distal hair: 14 mg/g (range, 4-15 mg/g) versus 14 mg/g (range, 12-15 mg/g); P = .139. In asymptomatic relaxed hair, cystine levels were higher in less frequently relaxed samples proximal to scalp: 7.5 mg/g (5.6-12) versus 3.3 mg/g (1.3-9.2); P = .005. Cystine levels in distal asymptomatic relaxed and symptomatic relaxed hair were similar to each other and to those in the genetic hair fragility disease trichothiodystrophy. LIMITATIONS: It was not possible to analyze lye and no-lye 'relaxers' separately. CONCLUSIONS: 'Relaxers' are associated with reduced cystine consistent with fragile damaged hair. A decrease in citrulline and glutamine has been associated with inflammation; prospective studies are needed to investigate whether or how 'relaxers' induce inflammation.

Retronychia: proximal ingrowing of the nail plate.

BACKGROUND: Proximal nail fold inflammation can be caused by many diseases and has not previously been recognized as a result of posterior embedding of the nail. We describe a new pattern of ingrowth that we have termed retronychia ("retro"--Latin for backwards; "onychia"--Greek for nail). The term describes a combination of proximal nail plate ingrowth into the proximal nail fold which is associated with multiple generations of nail plate misaligned beneath the proximal nail. OBJECTIVE: To describe a new pattern of nail ingrowth which causes a specific form of proximal nail fold paronychia. METHODS: Collective cases were reported to a European Nail Society expert group. RESULTS: Persistent proximal nail fold inflammation can result from an episode of trauma that disturbs longitudinal nail growth and results in reverse embedding of the nail plate. This can cause pain, inflammation, and granulation tissue formation, and is typically relieved by avulsion and antiinflammatory treatment. LIMITATIONS: We cannot demonstrate causality between the proposed precipitants and the clinical features. CONCLUSION: Retroncyhia represents proximal ingrowth of the nail that occurs when the nail embeds backwards into the proximal nail fold. Nail plate avulsion with supplementary medical management is curative.

Strengths and weaknesses of electronic referral: comparison of data content and clinical value of electronic and paper referrals in dermatology.

One hundred and thirty-one electronic referrals and 129 paper referrals were reviewed and their content analysed. Four items of demographic data were better recorded in the electronic referrals. Three items of clinical data were significantly better recorded in the paper referrals. The mean global clinical score for clinical relevance was greater for paper referrals than electronic referrals. This study illustrates the strength of electronic referrals for communicating demographic data and their weakness when revealing what is wrong with the patient.

Childhood nail diseases.

The diagnosis of pediatric nail populations is complicated because a large proportion of pediatric nail presentations involve parents bringing in their children with questions regarding nail appearance rather than the plain presentation of a disease or functional problem. Parents are concerned whether the nails are normal, if they are uncomfortable for the baby or child, if the nails will affect walking or other future function, or if the changes are of wider significance and suggest more sinister medical problems than are yet apparent. To address these concerns, the clinician needs a good grasp of what is normal.

Subungual neurothekeoma.

Neurothekeomas are benign tumors probably of nerve sheath origin and are also known as dermal nerve sheath myxomas. They are commonly found on the face, arm, or shoulder and less frequently the lower limbs. To our knowledge, this is the first case of a subungual neurothekeoma affecting the big toe. Histology confirmed a well-circumscribed, multilobulated tumor composed of bland stellate and spindle cells in a copious myxoid matrix staining positive with S100 protein.

Bariatric surgery in morbidly obese insulin resistant humans normalises insulin signalling but not insulin-stimulated glucose disposal.

AIMS: Weight-loss after bariatric surgery improves insulin sensitivity, but the underlying molecular mechanism is not clear. To ascertain the effect of bariatric surgery on insulin signalling, we examined glucose disposal and Akt activation in morbidly obese volunteers before and after Roux-en-Y gastric bypass surgery (RYGB), and compared this to lean volunteers. MATERIALS AND METHODS: The hyperinsulinaemic euglycaemic clamp, at five infusion rates, was used to determine glucose disposal rates (GDR) in eight morbidly obese (body mass index, BMI=47.3 ± 2.2 kg/m(2)) patients, before and after RYGB, and in eight lean volunteers (BMI=20.7 ± 0.7 kg/m2). Biopsies of brachioradialis muscle, taken at fasting and insulin concentrations that induced half-maximal (GDR50) and maximal (GDR100) GDR in each subject, were used to examine the phosphorylation of Akt-Thr308, Akt-473, and pras40, in vivo biomarkers for Akt activity. RESULTS: Pre-operatively, insulin-stimulated GDR was lower in the obese compared to the lean individuals (P<0.001). Weight-loss of 29.9 ± 4 kg after surgery significantly improved GDR50 (P=0.004) but not GDR100 (P=0.3). These subjects still remained significantly more insulin resistant than the lean individuals (p<0.001). Weight loss increased insulin-stimulated skeletal muscle Akt-Thr308 and Akt-Ser473 phosphorylation, P=0.02 and P=0.03 respectively (MANCOVA), and Akt activity towards the substrate PRAS40 (P=0.003, MANCOVA), and in contrast to GDR, were fully normalised after the surgery (obese vs lean, P=0.6, P=0.35, P=0.46, respectively). CONCLUSIONS: Our data show that although Akt activity substantially improved after surgery, it did not lead to a full restoration of insulin-stimulated glucose disposal. This suggests that a major defect downstream of, or parallel to, Akt signalling remains after significant weight-loss.