Frontal fibrosing alopecia: survey of severity assessment methods in routine clinical practice and validation of the International Frontal Fibrosing Alopecia Cooperative Group measurement guidance.

BACKGROUND: The lack of validated and responsive outcome measures in the management of frontal fibrosing alopecia (FFA) significantly limits assessment of disease progression and treatment response over time. AIM: To understand how FFA extent and progression is currently assessed in UK specialist centres, to validate components of the International FFA Cooperative Group (IFFACG) statement on FFA assessment, and to identify pragmatic advice to improve FFA management in clinic. METHODS: Consultant dermatologists with a specialist interest in hair loss (n = 17) were invited to take part. Preferred FFA assessment methods were explored using questionnaires and clinical scenarios. Participants were asked to identify and mark the current hairline in 10 frontal and 10 temporal hairline images (Questionnaire 1), with assessment repeated 3 months later to assess intraindividual variability (Questionnaire 2) and 12 months later to test whether interindividual accuracy could be improved with simple instruction (Questionnaire 3). RESULTS: All 17 clinicians (100%) completed the questionnaire at each time interval. We identified a wide variation in assessment techniques used by our experts. Measurements were perceived as the most accurate method of assessing frontal recession whereas photography was preferred for temporal recession. Inter-rater reliability between clinicians measuring the frontal hairline scenarios indicated a moderate strength of agreement [intraclass coefficient (ICC) = 0.61; 95% CI 0.40-0.85], yet intrarater reliability was found to be poor with wide limits of agreement (-8.71 mm to 9.92 mm) on follow-up. Importantly, when clear guidance was provided on how the hairline should be identified (Questionnaire 3), inter-rater reliability improved significantly, with ICC = 0.70, suggesting moderate agreement (95% CI 0.51-0.89; P < 0.001). A similar pattern was seen with temporal hairline measurements, which again improved in accuracy with instruction. CONCLUSION: We found that accuracy of measurements in FFA can be improved with simple instruction and we have validated components of the IFFACG measurement recommendations.

Establishing and prioritizing research questions for the prevention, diagnosis and treatment of hair loss (excluding alopecia areata): the Hair Loss Priority Setting Partnership.

BACKGROUND: Hair and scalp problems are common. Unfortunately, many uncertainties exist around the most effective management and treatment strategies for these disorders. OBJECTIVES: To identify uncertainties in hair-loss management, prevention, diagnosis and treatment that are important to both people with hair loss and healthcare professionals. METHODS: A Hair Loss Priority Setting Partnership was established between patients, their carers and relatives, and healthcare professionals to identify the most important uncertainties in hair loss. The methodology of the James Lind Alliance was followed to ensure a balanced, inclusive and transparent process. RESULTS: In total, 2747 treatment uncertainties were submitted by 912 participants; following exclusions 884 uncertainties relating to hair loss (excluding alopecia areata) were analysed. Questions were combined into 'indicative uncertainties' following a structured format. A series of ranking exercises further reduced this list to a top 25 that was taken to a final prioritization workshop where the top 10 priorities were agreed. CONCLUSIONS: We present the top 10 research priorities for hair loss (excluding alopecia areata) to guide researchers and funding bodies to support studies important to both patients and clinicians.

Establishing and prioritizing research questions for the treatment of alopecia areata: the Alopecia Areata Priority Setting Partnership.

BACKGROUND: Alopecia areata (AA) is a common hair loss disorder that results in patchy to complete hair loss. Many uncertainties exist around the most effective treatments for this condition. OBJECTIVES: To identify uncertainties in AA management and treatment that are important to both service users (people with hair loss, carers and relatives) and healthcare professionals. METHODS: An AA priority setting partnership was established between patients, their carers and relatives, and healthcare professionals to identify the most important uncertainties in AA. The methodology of the James Lind Alliance was followed to ensure a balanced, inclusive and transparent process. RESULTS: In total, 2747 treatment uncertainties were submitted by 912 participants, of which 1012 uncertainties relating to AA (and variants) were analysed. Questions were combined into 'indicative uncertainties' following a structured format. A series of ranking exercises further reduced this list to a top 25 that were taken to a final prioritization workshop where the top 10 priorities were agreed. CONCLUSIONS: We present the top 10 research priorities for AA to guide researchers and funding bodies to support studies important to both patients and clinicians.

Psoriatic alopecia.

Alopecia and other hair abnormalities occurring in patients with psoriasis were first recognized over four decades ago, yet psoriatic alopecia is not a well-known concept among clinicians. Alopecia may be directly related to the psoriasis itself, and can affect both the scalp and other parts of the body. On the scalp, psoriatic alopecia most commonly affects lesional skin, but may present as a generalized telogen effluvium. In most cases, there is regrowth of hair, but in rare cases it can cause scarring alopecia. Histological findings include features of psoriasis in the interfollicular epithelium, along with perifollicular inflammation and atrophy or loss of the sebaceous glands. Late changes include destruction of the hair follicle, with perifollicular fibrosis and 'naked' hair shafts lying free in the dermis. In addition to the hair loss caused by the psoriasis itself, data from population and genetic studies reveal that patients with psoriasis are at greater risk of developing alopecia areata. Psoriasis treatments may also contribute to hair loss. Application of topical preparations may cause hair loss through friction, and many of the systemic treatments used for psoriasis can also cause hair problems. Treatment with anti-tumour necrosis factor-α agents can precipitate de novo psoriasis and subsequent psoriatic alopecia.

Predictive value of bile duct dimensions measured by ultrasound in neonates presenting with cholestasis.

OBJECTIVES: : The significance of extrahepatic bile duct dilatation on ultrasound examination in jaundiced infants is often uncertain. We wished to clarify the diagnostic and prognostic significance of the present finding in neonatal conjugated hyperbilirubinaemia. PATIENTS AND METHODS: : We retrospectively enrolled all of the infants younger than 3 months with extrahepatic biliary dilatation > or =1.2 mm (nonfasting ultrasound) who presented during the study period. We reviewed clinical, radiological, and laboratory data to determine mode of presentation, diagnosis, interventions, and long-term outcome. RESULTS: Seventy-six infants (41 male) were identified, all of whom were referred with conjugated hyperbilirubinaemia. Median gestational age was 39 weeks (range 24-42 weeks). Inspissated bile was the most common diagnostic category, whereas congenital choledochal malformation was the diagnosis made in 13% infants. Dilatation was an incidental finding in 9% of the infants. Seventeen percent of infants had required either surgical or radiological intervention by the time of follow-up. Overall, 41% infants had spontaneous resolution of bile duct dilatation, including 8% who had "grown into" an unchanged duct size rather than involution of dilatation. The median size of bile duct at presentation for those who required intervention was 4.7 versus 2 mm for the remainder (P < 0.001). Of those who resolved spontaneously, the median size of duct at presentation was 1.8 mm. CONCLUSIONS: : Bile duct dilatation <3 mm (nonfasting ultrasound) with neonatal cholestasis is unlikely to be of significance whereas >4 mm is likely to be associated with choledochal malformation or need for intervention. The intermediate group is likely to be associated with inspissated bile syndrome following resolution of which innocent biliary dilatation may persist.

Assessing the assessments: U.K. dermatology trainees' views of the workplace assessment tools.

BACKGROUND: The workplace assessments, direct observation of procedural skills (DOPS), mini-clinical evaluation exercise (mini-CEX) and multisource feedback (MSF, formerly known as 360 degrees appraisal), are now mandatory during dermatology specialist training in the U.K. The opinions of those undergoing such assessments in any medical specialty have rarely been sought. OBJECTIVES: To collate the experience and views of U.K. dermatology trainees on the three workplace assessments. METHOD: A questionnaire was circulated in autumn 2006 to all U.K. dermatology specialist registrars (SpRs) registered as members of the British Association of Dermatologists (n = 269). RESULTS: A total of 138 responses were received (51%). Seventeen SpRs had not experienced any of the assessments; 92 had undergone MSF, 95 DOPS and 54 mini-CEX. The total experience of the respondents amounted to a minimum of 251 DOPS, 122 MSF and 142 mini-CEX. Trainees appreciated the formative aspects of the assessments, especially feedback and training opportunities, although not all trainees reported receiving useful feedback. MSF was praised for the insights that it provides. All of the assessments were found to be time-consuming and difficult to organize. DOPS and mini-CEX carried a degree of stress and artificiality. Concerns were raised over the possibility of victimization by MSF raters. Discussion of performance in the assessments was rarely prominent in trainees' annual summative reviews. CONCLUSIONS: Trainees appreciate the formative benefits which derive from the assessments, namely feedback, reassurance of satisfactory performance and, in the case of DOPS and mini-CEX, additional one-to-one training from consultants. Some problems came to light. The issues raised will not be unique to dermatology and other specialties should take note.

Keratolytic winter erythema with facial involvement: a novel presentation.

We present a 23-year-old woman with a diagnosis of keratolytic winter erythema (erythrokeratolysis hiemalis), who developed facial lesions following a traumatic experience. This rare genodermatosis usually affects the palms and soles, and appears as mild erythema and annular scaling. The limbs and trunk can rarely be affected. To our knowledge, this is the first reported case of facial involvement.

Multiple aggressive squamous skin cancers in association with nonbullous congenital ichthyosiform erythroderma.

Nonbullous congenital ichthyosiform erythroderma (NBCIE) is one of the autosomal recessive inherited non-syndromic ichthyoses and is currently diagnosed on clinical grounds alone. Skin cancer is not a recognized complication of NBCIE. We report here two NBCIE patients who have developed multiple aggressive nonmelanoma skin cancers, predominantly cutaneous squamous cell carcinoma. NBCIE may be a risk factor for skin cancer development.

Attributes of an effective trainer: implications of the views of UK dermatology trainees.

BACKGROUND: Trainers have a critical influence on the education of specialist registrars (SpRs) and the quality of training programmes. OBJECTIVES: This study gathered the opinions of U.K. dermatology SpRs on the attributes they felt were important in a trainer. METHODS: The questionnaire was sent to all U.K. dermatology SpRs (n=216). It comprised the stem: 'A good trainer (supervising consultant or educational supervisor)', followed by statements such as '...has realistic expectations of the trainee'. Respondents ranked each statement in importance using a five-point Likert scale. A free-text box was also included for respondents to list the least desirable attributes. RESULTS: Completed questionnaires were received from 146 trainees (68%). Four attributes were identified as most important: having an encouraging and supportive attitude, using feedback to identify specific areas for attention, being approachable, and being willing to answer questions. Least important attributes included openness to criticism of teaching skills, auditing of own clinical practice, sense of humour and place of work (district general vs. teaching hospital). Eighty-seven respondents indicated the least desirable attributes of a trainer. Most comments related to being uninterested in training, being unapproachable or unavailable, or undermining the trainee and using unconstructive criticism. CONCLUSIONS: The results highlight the importance to trainees of constructive feedback, and dialogue with trainers. Some of the stated undesirable characteristics in a trainer appeared to derive from personal experience. This is of serious concern, and ways in which the trainer-trainee relationship can be improved are discussed.