Mohs Surgery for Periocular Basal Cell Carcinoma Without a Mohs Surgeon: The First Series in Hong Kong.

Purpose To report the first series of Mohs micrographic surgery (MMS) in Hong Kong, where the roles of a Mohs surgeon were shared and coordinated by a "mobile" surgeon. METHODS DESIGN: Prospective non-comparative interventional case series. SUBJECTS: 20 consecutive Chinese patients (10 male, age 78.5+10.4 years, range 55-91 years) with primary periocular basal cell carcinoma (pBCC) referred to the university oculoplastic unit between October 2007 and August 2013. INTERVENTION: MMS were conducted according to a streamlined standard operating procedure emphasizing surgeon-driven mapping, specimen orientation, and on-site clinico-histological correlation with the dermatopathologist at the frozen-section laboratory. MAIN OUTCOME MEASURES: Clinical and histological characteristics of tumors, layers of Mohs procedures, complications, and biopsy-confirmed recurrence at the same location. Results All 20 patients received MMS as planned. Sixteen pBCCs (80%) were diffusely pigmented, and three (15%) were focally pigmented. Sixteen were also nodular. The average tumor diameter was 7+3 (3-15) mm. Seven (35%) were within 2 mm of the punctum. Histologically, 11 (55%) were nodules, and four (20%) were superficial. An average of 1.8+0.8 Mohs levels were performed. Apart from the initial two patients, who required four and three levels, respectively, seven (35%) patients were cleared after the first level of MMS using a 1mm clinical margin. The remaining 11 patients required two levels with an additional 1-2mm margin, but only focally as guided histologically. Defects in 16 patients (80%) were reconstructed by local flaps, two by direct closure, and two with pentagon closure. Among the seven patients with pericanalicular BCC, three patients had their remaining canaliculi successfully intubated, while two developed stenotic upper and two lower punctae postoperatively. One patient had prolonged wound healing. Three patients had lid margin notching, two had medial ectropion, one had medial canthal rounding, and two had lateral canthal dystopia. No recurrence was detected at a mean follow-up of 80+23 months (43 to 113 months) in all patients. Conclusions MMS was successfully introduced in Hong Kong without a Mohs surgeon. Providing complete microscopic margin control and preserving tissues, it was proven to be a valuable treatment option for pBCC. Our multidisciplinary protocol demonstrated that these merits are possible and warrant validation in other resource-limited healthcare settings.

Hand eczema among Hong Kong nurses: a self-report questionnaire survey conducted in a regional hospital.

BACKGROUND: Hand eczema is common in the nursing profession, and has been reported widely in various parts of the world. OBJECTIVES: The aim of this study was to determine the prevalence and severity of hand eczema among nurses working in a regional hospital in Hong Kong, as well as its psychosocial impact and any possible associated risk factors. PATIENTS/MATERIALS/METHODS: The study took the form of a self-report questionnaire survey; 1240 nurses in a regional hospital were asked to participate in the survey by completing the questionnaire and returning it anonymously within 2 weeks. RESULTS: Seven hundred and twenty-four nurses returned the questionnaire (a response rate of 59%). The prevalence of hand eczema among the respondents was 22.1% (160/724). More than 90% had moderate to severe hand eczema. Itchiness and dryness were the most common symptoms. Occupational work, housework, mood, social activities and sleep were particularly affected. Multinomial logistic regressions showed that a personal or family history of atopy and a hand washing frequency of >20 times per day were independent risk factors for hand eczema. CONCLUSIONS: Hand eczema is common and severe among Hong Kong nurses. The results of this study suggest that hand eczema is an important problem for nurses and that preventive measures should be emphasized.

Epidermolysis bullosa in three Chinese neonates.

Epidermolysis bullosa (EB) is one of the rare blistering diseases that may present in the neonatal period. Diagnosis is based on clinical symptomatology, histopathology, electron microscopy and genetic studies. Age of onset, symptomatology and prognosis of the various subtypes are varied. We describe three cases of EB dystrophica (EBD) followed at the paediatric dermatology clinic. All of them were symptomatic and diagnosed in the neonatal period. One patient had very severe disease and was confirmed to have the Hallopeau-Siemens subtype with both copies of his entire chromosome three from his mother. This extremely rare mode of inheritance is known as uniparental isodisomy. The clinical course of the other two neonates was milder and suggested that they had the dominant form of EBD. Many issues associated with the disease may be encountered. Specifically, details in skin care, counselling and advice were provided and reinforced to the parents. A multidisciplinary approach with inputs from the neonatologists, paediatricians, dermatologists, plastic surgeons, dietitians, physiotherapists, occupational therapists, and special nurses are required to assure optimal outcome.

Efficacy and problems associated with using a wet-wrap garment for children with severe atopic dermatitis.

Use of a wet wrap for short-term relief of itch in children with severe atopic dermatitis has been advocated but objective determination of its efficacy has been difficult and many issues associated with its use are yet to be defined. We tested a new garment for the wet-wrap procedure in six patients with atopic dermatitis and objectively determined whether a 3-day usage could indeed relieve the distressing symptom of itch using a wrist motion monitor. The garments were effective in the short-term improvement of itching, severity of atopic dermatitis and quality of life in these children. Many issues associated with its use were identified. Clear instructions and individualized regimes (such as the choice of emollient, bathing ointment and topical corticosteroid) are essential for optimal outcome.

DNA-based diagnosis of xeroderma pigmentosum group C by Whole-genome scan using single-nucleotide polymorphism microarray.

In this study, we have established the molecular basis of xeroderma pigmentosum (XP) in two unrelated Chinese families. In the first patient with consanguineous parents, we mapped the disease-causing locus XPC using single-nucleotide polymorphism microarray. Mutational analysis of the XPC gene showed that the patient is homozygous for a nonsense mutation, E149X. After developing DNA-based diagnosis of XPC, we screened another XP patient for XPC mutations. We found that the second patient is a compound heterozygote of 1209delG and Q554X in this gene. These are the first XPC-causing mutations identified in Chinese patients.

Quality of life and psychosocial issues are important outcome measures in eczema treatment.

BACKGROUND: Atopic eczema (AE) is a common relapsing inflammatory skin disease in children associated with chronicity and poor quality of life. Many children also display depressive, anxiety and stress symptoms. AIM: To investigate the prevalence of depressive, anxiety and stress symptoms, and if these symptoms are associated with disease severity, quality of life and skin biophysiology in childhood AE. METHODS: Psychological symptoms, eczema severity, quality of life and biophysical skin condition of consecutive adolescents at the pediatric dermatology clinic of a teaching hospital were evaluated with the validated Chinese versions of Depressive, Anxiety, Stress Scales (DASS-42), Beck Depression Inventory (BDI-13), Nottingham Eczema Severity Score (NESS), Children's Dermatology Life Quality Index (CDLQI), transepidermal water loss (TEWL) and stratum corneum skin hydration (SH), respectively. RESULTS: AE patients (n=120) had lower SH, higher TEWL, worse CDLQI and reported higher overall, depressive and stress symptom scores, personal history of atopy, current topical corticosteroid usage and food avoidance than non-AE patients (n=26). Depressive, anxiety and stress symptoms were reported in 21%, 33% and 23% of AE patients, respectively. Multivariate analyses showed that these symptoms were significantly correlated with a poor quality of life (partial correlations of 0.40-0.49; p<0.001). Male patients had more severe disease (higher NESS, p=0.036) and DASS-depressive symptoms (multivariate OR=3.2, p=0.034) than females. Patients who reported current topical steroid usage generally practiced food avoidance (p=0.047), had poor quality of life (p=0.043) but less DASS-depression (multivariate OR=0.354, p=0.043). Only 6% of the 120 AE patients reported prior psychology consultation. CONCLUSIONS: Quality of life impairments correlate with disease severity, aberrant skin biophysiology, depression, anxiety and stress symptoms in adolescents with AE. Physicians caring for these patients must evaluate the different but inter-correlated medical, biophysiological and pertinent psychosocial domains. These significant correlations imply that a holistic approach should encompass psychotherapy, behavioral therapy and coping strategies in conjunction with dermatologic therapy.

Acceptability and efficacy of an emollient containing ceramide-precursor lipids and moisturizing factors for atopic dermatitis in pediatric patients.

BACKGROUND: Atopic eczema or dermatitis (AD) is associated with atopy and is characterized by reduced skin hydration and an impaired skin barrier in the epidermis. We investigated the patient acceptability and efficacy of an emollient containing ceramide-precursor lipids and moisturizing factors (LMF) in AD. METHODS: Consecutive AD patients were recruited. Swabs and cultures were obtained from the right antecubital fossa and the worst-affected eczematous area, and disease severity [according to the SCORing Atopic Dermatitis (SCORAD) Index], skin hydration, and transepidermal water loss (TEWL) were measured prior to and after 2 weeks' use of the LMF moisturizer. The general acceptability of treatment was documented as being 'very good', 'good', 'fair', or 'poor'. RESULTS: Twenty-four AD patients [mean age 13.8 (standard deviation 5.7) years] were recruited. Two thirds of the patients reported very good or good acceptability of the LMF moisturizer, whereas one third reported fair or poor acceptability. There were no inter-group differences in the pre-use clinical parameters of age, objective SCORAD score, pruritus score, sleep disturbance score, skin hydration, TEWL, topical corticosteroid use, oral antihistamine use, or acceptability of previously used proprietary emollients. However, patients in the fair/poor acceptability group were more likely to have Staphylococcus aureus colonization and to be female (odds ratio 13, 95 % confidence interval 1.7-99.4; p = 0.021). Following use of the LMF moisturizer, the objective SCORAD score, pruritus score, and sleep disturbance score were lower in the very good/good acceptability group than in the fair/poor acceptability group. The mean objective SCORAD score improved (from 31.5 to 25.7; p = 0.039) and skin hydration improved [from 30.7 arbitrary units (a.u.) to 36.0 a.u.; p = 0.021] in the very good/good acceptability group. When the data were analyzed for the strength of the agreement of the rating of acceptability, the κ values were 0.338 (fair) for use of body wash and 0.118 (poor) for use of emollients before and after the trial. CONCLUSION: The LMF moisturizer was considered acceptable by two thirds of the patients with AD. It seems that patients who found the moisturizer acceptable were less likely to be female or to be colonized by S. aureus before switching to the product, and they had less severe eczema, less pruritus, and less sleep disturbance after its use than patients who did not find the product acceptable. Gender and S. aureus colonization may have influenced the patient acceptability and clinical efficacy of the LMF moisturizer. The lack of agreement with regard to the acceptability of the moisturizer implies that there is room for parent/patient education to improve compliance.

Measurement of pruritus in a Chinese woman with pemphigoid gestationis using a wrist movement detector.

Pemphigoid gestationis (PG) is a rare itching bullous disease of pregnancy and the postpartum period. We describe the use of a new methodology for measuring the nature and intensity of itching in a 21-year-old woman with pemphigoid gestationis. At 19 weeks gestation, she developed an itchy rash over the limbs, which spread to the trunk. Blisters then appeared on the hands which subsequently also involved the feet. Intense nocturnal wrist activities in this patient, measured with a wrist monitor and defined as average acceleration in the early hours of sleep, were even higher than that in patients with severe eczema, with an average value of 181.00 +/- 43.49 (mean +/- standard error) g/min for the first three hours, versus 84.47 +/- 8.53 g/min for the group of 24 eczema subjects. Most wrist activities were slower movements at 0 to 1 Hz. This is in striking contrast to the scratching activities at 0 to 3 Hz in eczema subjects. There have been no therapeutic trials for PG. Topical steroid and oral antihistamines are usually ineffective, but worked in our patient. We also discuss the potential application of the monitor in assessing the nature of various dermatological or systemic itching disorders.

Steroid fears in children with eczema.

BACKGROUND: Topical glucocorticoids (GCs) are the mainstay of treatment for eczema, but GC phobia and fears are very common among the parents of paediatric patients. AIM: To survey the nature and extent of "fears" of GC use, and to evaluate if disease severity is associated with such fears. METHODS: Patients with eczema managed in the paediatric dermatology outpatient clinic of a university hospital were recruited in this survey. Disease severity and various aspects of belief and practices of GC use were assessed with the Nottingham Eczema Severity Score and a questionnaire. RESULTS: GC "fears" were present in two fifths of informants with non-eczematous skin disease and mild eczema, but three fifths in moderate-to-severe disease. Requests for steroid-sparing medications (such as tacrolimus or pimecrolimus) had been made in nearly 50% of cases with moderate-to-severe eczema, and many parents would wait until eczema had worsened or apply GC only as a last resort to avoid potential side effects. "Fears" were predominantly interpersonal and rarely iatrogenic in nature. Skin problems (in particular skin thinning) and adverse effects on growth were the side effects of GC of most concern. However, fewer than half of the informants had discussed their concerns with doctors. CONCLUSION: Our results suggest the importance of understanding the nature and extent to which GC fears, regardless of eczema severity, are prevalent so that sound advice can be offered in a timely manner to parents and patients.

Novel mutations in the PATCHED gene in basal cell nevus syndrome.

Basal cell nevus syndrome (BCNS) is an autosomal dominant disease characterized by the presence of multiple basal cell carcinomas, odontogenic keratocysts, palmoplantar pits, and calcification in the falx cerebri caused by mutational inactivation of the PTCH gene. To investigate the molecular basis of BCNS in Chinese, we did a mutational analysis of the PTCH gene by performing denaturing high-performance liquid chromatography in three BCNS families. In this study, three novel mutations, two 1-bp frameshift insertions, i.e., 1468insA and 2392insC, and one 8-bp deletion, i.e., IVS5 + 1delGTAAGTGT, affecting a donor splice site, were identified. All the mutations cause a shift of the open reading frames and lead to premature termination of PTCH protein translation. Our results showed that mutational inactivation of the PTCH gene causes BCNS in Chinese.