Guidelines for the Management of Atopic Dermatitis in Singapore.

INTRODUCTION: Atopic dermatitis is a common, chronic pruritic condition affecting both children and adults, which has a negative impact on the quality of life. These guidelines were developed by an expert workgroup appointed by the Dermatological Society of Singapore, to provide doctors with information to assist in the management of their patients with atopic dermatitis. The workgroup members are experienced dermatologists with interest and expertise in eczemas. MATERIALS AND METHODS: Workgroup members arrived at a consensus on the topics to be included. Relevant studies from the literature were assessed for best evidence, supplemented by the collective experience of the workgroup. RESULTS: For mild atopic dermatitis, emollients, mild potency topical steroids and topical calcineurin inhibitors are recommended. For moderate-to-severe atopic dermatitis, the use of emollients, moderate-to-potent topical steroids, topical calcineurin inhibitors, wet dressings, antimicrobials for secondary skin infection, phototherapy, and systemic therapy (e.g. prednisolone, cyclosporine, azathioprine or methotrexate) may be warranted. Patients with moderate-to-severe atopic dermatitis should be managed in conjunction with a dermatologist. CONCLUSION: Good outcomes can be achieved with an individualised therapeutic approach combined with adequate patient and parental education.

Prevention of glucocorticoid-induced osteoporosis in immunobullous diseases with alendronate: a randomized, double-blind, placebo-controlled study.

OBJECTIVE: To evaluate the efficacy and safety of oral alendronate sodium therapy once daily in preventing glucocorticoid-induced bone loss in patients with immunobullous skin diseases treated with long-term glucocorticoid therapy. DESIGN: A 12-month randomized, double-blind, placebo-controlled trial. SETTING: A tertiary referral dermatology center in Singapore. PARTICIPANTS: Patients newly diagnosed as having an immunobullous disease and deemed to require at least 6 months of systemic glucocorticoid therapy. INTERVENTIONS: The patients were randomized to receive either oral alendronate sodium (10 mg/d) or a matching placebo for 12 months. All patients also received concurrent calcium with vitamin D, 2 tablets daily. MAIN OUTCOME MEASURES: Percent change in bone mineral density (BMD) at the lumbar spine and the femoral neck at 12 months. RESULTS: A total of 29 patients (alendronate [n = 15], placebo [n = 14]) were evaluated. The percent change in BMD in the alendronate group was +3.7% and +3.5% at the lumbar spine and the femoral neck, respectively, whereas in the placebo group, it was -1.4% and -0.7% at the lumbar spine and the femoral neck, respectively. The increase in BMD observed in the alendronate group compared with the placebo group was statistically significant at both the lumbar spine (P = .01) and the femoral neck (P = .01). There was also a statistically significant decrease in serum heat-labile alkaline phosphatase levels after 12 months (-32.6%, P < .01) in the alendronate group but not in the placebo group. Adverse events were generally minor, and the frequency of occurrence did not differ significantly between both treatment groups (P = .59). CONCLUSIONS: There were statistically significant increases in BMD at both the lumbar spine (P = .01) and the femoral neck (P = .01) with alendronate therapy. It is imperative to use bisphophonate therapy in patients with immunobullous disorders who are receiving oral corticosteroids because it largely prevents the morbidity associated with low BMD.

Meta-analysis of randomized, controlled trials comparing griseofulvin and terbinafine in the treatment of tinea capitis.

BACKGROUND: Griseofulvin has been the standard treatment for tinea capitis but newer antifungal agents, particularly terbinafine, are increasingly being used because of their shorter duration of treatment and more consistent absorption rates. OBJECTIVE: We sought to compare the efficacy of oral griseofulvin and oral terbinafine in the treatment of tinea capitis. METHODS: A search of MEDLINE, EMBASE, Cochrane Central Register of Clinical Trials, and the Cochrane Skin Group Ongoing Skin Trials Register was performed up to January 2010 for randomized controlled trials comparing griseofulvin and terbinafine in the treatment of tinea capitis in immunocompetent patients. The primary outcome measure was the complete cure rate. The mycological and clinical cure rates and adverse effects were secondary outcome measures. Pooling of treatment effect was accomplished using a random effects model and the I(2) test was used to check for heterogeneity among the studies. RESULTS: Seven studies involving 2163 subjects were included. There was no significant difference in efficacy between griseofulvin (mean duration of treatment 8 weeks, range 6-12 weeks) and terbinafine (mean duration of treatment 4 weeks, range 2-6 weeks); odds ratio = 1.22 favoring terbinafine (95% confidence interval [CI] = 0.785-1.919; P = .37). In the pooled analysis of 5 studies in which Trichophyton species were the predominant (≥65%) pathogenic dermatophyte, terbinafine showed a trend toward greater efficacy (odds ratio 1.49; 95% CI = 0.975-2.277; P = .065). Subgroup analysis revealed that terbinafine was more efficacious than griseofulvin in treating Trichophyton species (1.616; 95% CI = 1.274-2.051; P < .001) and griseofulvin was more efficacious than terbinafine in treating Microsporum species (0.408; 95% CI = 0.254-0.656; P < .001). Both griseofulvin and terbinafine demonstrated good safety profiles in the studies. LIMITATIONS: Data on efficacy of griseofulvin and terbinafine for separate groups of Trichophyton and Microsporum species were not available from every study. In the subgroup analysis of Microsporum species, data from only 3 studies were available. CONCLUSION: This meta-analysis suggests that terbinafine is more efficacious than griseofulvin in treating tinea capitis caused by Trichophyton species, whereas griseofulvin is more efficacious than terbinafine in treating tinea capitis caused by Microsporum species.

Aggressive subcutaneous panniculitis-like T-cell lymphoma with hemophagocytosis in two children (subcutaneous panniculitis-like T-cell lymphoma).

Subcutaneous panniculitis-like T-cell lymphoma is an uncommon form of cutaneous lymphoma in the pediatric population. It is characterized histologically by subcutaneous infiltration of pleomorphic cytotoxic T cells, mimicking a lobular panniculitis. Although usually described as having an indolent clinical course, the condition may be complicated by systemic involvement and hemophagocytic syndrome, resulting in a poorer prognosis. We present two pediatric patients with aggressive forms of subcutaneous panniculitis-like T-cell lymphoma complicated by hemophagocytic syndrome, and discuss the current literature.

Unique and recurrent mutations in the filaggrin gene in Singaporean Chinese patients with ichthyosis vulgaris.

Filaggrin is an abundant protein of the outer epidermis that is essential for terminal differentiation of keratinocytes and formation of an effective barrier against water loss and pathogen/allergen/irritant invasion. Recent investigations in Europe and Japan have revealed null mutations in the filaggrin gene (FLG) as the underlying cause of ichthyosis vulgaris (IV), a common skin disorder characterised by dry skin, palmar hyperlinearity and keratosis pilaris. Following the development of a strategy for the comprehensive analysis of FLG, we have identified five unique mutations and one recurrent mutation in Singaporean Chinese IV patients. Mutation 441delA is located in the profilaggrin S100 domain, whereas two additional frameshift mutations, 1249insG and 7945delA, occur in the first partial filaggrin repeat ("repeat 0") and in filaggrin repeat 7, respectively. Both nonsense mutations Q2147X and E2422X are found in filaggrin repeat 6, whereas R4307X was found on one of the longer size variant alleles of FLG, within duplicated repeat 10.2. Mutation E2422X, previously found in a single Dutch patient, was found in one Singaporean IV patient and at a low frequency in Asian population controls. Our study confirms the presence of population-specific as well as recurrent FLG mutations in Singapore.

Skin surgery in children: local anaesthesia and sedation techniques.

In the past, procedural pain control in young children was undertreated as it was incorrectly alleged that their neuronal pain pathways were undeveloped. However, it is now recognised that even neonates are able to experience pain. Moreover, intensely painful physical experiences in childhood can have persisting physiological and psychological consequences. Therefore, the management of acute pain is essential. In this paper, the authors provide an in-depth discussion regarding the anaesthetic options for paediatric patients undergoing dermatological surgery.

A study on the knowledge, attitudes and practices of Southeast Asian dermatologists in the management of atopic dermatitis.

INTRODUCTION: This study evaluated the knowledge, attitudes and practices of Southeast Asian dermatologists in the management of atopic dermatitis (AD). MATERIALS AND METHODS: A questionnaire survey of 255 dermatologists in Indonesia, Malaysia, the Philippines, Singapore, Thailand and Vietnam. RESULTS: Familiarity with diagnostic criteria varied considerably. The usage of moisturisers by the respondents from Vietnam and Indonesia was significantly less frequent than the other countries. Most respondents (91% to 100%) used topical corticosteroids in children with mild-to-moderately severe dermatitis. Some respondents in the Philippines (17% to 19%) and Vietnam (11% to 25%) only used topical corticosteroids for severe disease. For infected eczema, most respondents would prescribe systemic antibiotics for mild-to-moderate infection. A minority in the Philippines (14%) and Vietnam (11%) did so only for severe infection. The top 4 systemic antibiotics prescribed most frequently were: erythromycin, cloxacillin, cephalosporin and amoxicillin/clavulanic acid. In Indonesia, a large proportion of the respondents (47%) prescribed amoxicillin most frequently. The majority of respondents (60% to 100%) prescribed both sedating and non-sedating oral antihistamines. Most respondents used oral corticosteroids to treat severe AD. Some in Malaysia, Singapore and Vietnam used cyclosporin (7% to 58%), azathioprine (5% to 31%) and methotrexate (5% to 14%). With the exception of those in Singapore, the majority of respondents (71% to 97%) did not use phototherapy. CONCLUSION: Familiarity with diagnostic criteria, the early and judicious use of moisturisers and topical corticosteroids, as well as the treatment of Staphylococcus aureus superinfection with penicillinase-stable antibiotics should be emphasised in this region.

A retrospective cohort study of Southeast Asian patients with large congenital melanocytic nevi and the risk of melanoma development.

BACKGROUND: The lifetime risk of developing melanoma in Caucasian patients with large congenital melanocytic nevi (LCMN) is estimated to be between 4.5% and 10%. Cohort studies of LCMN and the risk of melanoma development in an Asian population are not available. OBJECTIVE: We sought to determine the risk of melanoma development in a retrospective cohort of patients presenting with LCMN to a dermatology tertiary referral center in Singapore from January 1989 to December 2004. METHODS: Patients with congenital melanocytic nevi (CMN) that covered at least 5% of the body surface area were included in the study. Data were obtained from electronic records and photographic documentation. A search for malignancy was done using the National Cancer Registry database. RESULTS: In all, 39 patients (23 male and 16 female) met the study criteria of LCMN; 15 of 39 patients also met the criteria of having a giant CMN (ie, a CMN that is predicted to be at least 20-cm diameter in adulthood). There were 29 Chinese, 6 Malay, 1 Indian, and 3 Caucasian patients. Their ages ranged from 23 months to 60 years (mean 18.8 years). They presented at a mean age of 26 months and were followed up for an average of 16.9 years. The size of the LCMN ranged from 5% to 40% of body surface area, with a mean of 12.2%. The most common sites were the back (54%), lower limb (28%), and abdomen (26%). Satellite lesions were present in 22 patients. Magnetic resonance imaging of the head or thoracolumbar spine was performed in 7 patients with LCMN on the scalp/face or back, respectively; all produced normal findings. Only one patient was treated: he had carbon-dioxide laser ablation and Q-switched neodymium:yttrium-aluminum-garnet laser treatment of a small part of his LCMN. Skin biopsies were done in 5 patients who had developed nodules; histology showed no evidence of malignancy. No patients had developed any form of malignancy. LIMITATIONS: The addition of 3 adult patients born before the start of the cancer registry may have led to survivor bias. The small sample size did not allow a precise estimate of the risk of melanoma development in our study population. CONCLUSION: The risk of melanoma development in LCMN within a predominantly Southeast Asian cohort appears to be very low. Prophylactic complete excision of LCMN is ideal, but seldom achievable. Hence, patient education, regular melanoma surveillance, and biopsy of suspicious lesions are very important.

Current treatment practices in the management of cutaneous haemangioma.

Haemangiomas are common, benign tumours of the vascular endothelium which present within the first few weeks of life. Clinically, they are very heterogeneous, with size, location and rate of proliferation having a significant effect on the risk of complications. Most haemangiomas are uncomplicated, and can be observed for spontaneous involution. However, some may be life- or function-threatening and require treatment. Corticosteroids, which may be used topically, intralesionally or systematically, are the mainstay of therapy. IFN-alpha, vincristine and cyclophosphamide are therapeutic options for complicated haemangiomas which do not respond to corticosteroids. Vascular-specific laser therapy may be considered for superficial haemangiomas, ulcerated haemangiomas or postinvolution sequelae, such as telangiectasia. A periorbital haemangioma that obstructs the visual axis or exerts pressure on the globe is an ocular emergency; systemic corticosteroids and patching of the unaffected eye should be instituted as soon as possible.

Vertically transmitted hypoplasia of the abdominal wall musculature.

The prune belly syndrome (OMIM 100100) is an association of bladder dilation with hypoplasia of the abdominal wall muscles. This malformation sequence is due to early urethral obstruction. We report a family with abdominal wall muscular hypoplasia as an isolated defect, not associated with the urethral obstruction sequence. The proband is a q3-year-old male who presented with abdominal wall laxity and severe constipation. His mother, maternal grandmother and younger brother had varying degrees of abdominal wall muscular deficiency and constipation. His mother's condition was aggravated by her 2 pregnancies. This family shows vertical transmission (compatible with autosomal dominant or mitochondrial inheritance) of the abdominal phenotype of prune belly sequence without any evidence of urinary tract or renal pathology. The expression in the sons may remain incomplete because abdominal distention due to pregnancy will not occur.

Therapeutic options in the treatment of tinea capitis.

Tinea capitis is primarily a disease of pre-adolescent children. In North America and the UK, Trichophyton tonsurans is responsible for > 90% of cases. Microsporum canis is the predominant pathogen in certain parts of Europe. The standard of care for the treatment of tinea capitis is oral griseofulvin and so far, it remains the only medication approved by the US FDA for this condition. The newer oral antifungal agents, such as terbinafine, itraconazole and fluconazole, appear to be effective, safe and have the advantage of a shorter treatment duration. Although a significant number of clinical trials and reports have documented experience with terbinafine and itraconazole for the treatment of tinea capitis, it should be noted that only a few trials have been conducted utilising fluconazole. Both 2% ketoconazole and 1% selenium sulfide shampoos have been shown to reduce surface colony counts of dermatophytes in infected individuals, and these agents are often recommended for adjuvant therapy. This article reviews data currently available on various therapeutic alternatives for the treatment of tinea capitis and summarises all relevant clinical trials that have thus far investigated the use of these drugs for tinea capitis in the paediatric population.

New treatments for tinea capitis.

PURPOSE OF REVIEW: Tinea capitis, a dermatophyte infection involving the hair shaft on the scalp, is primarily a disease of preadolescent children. The predominant pathogen varies according to the geographical location. Trichophyton tonsurans and Microsporum canis account for the majority of infections in north America and certain parts of Europe. The current standard of care for the treatment of tinea capitis in the USA is oral griseofulvin, but evidence is accumulating that some of the newer antifungal agents may also be useful. RECENT FINDINGS: The newer oral antifungal agents such as terbinafine, itraconazole and fluconazole seem to be effective, safe, and have the advantage of a shorter treatment duration. Although a significant number of clinical studies and reports have documented experience with terbinafine and itraconazole for the treatment of tinea capitis, it should be noted that only a few trials have been conducted utilizing fluconazole. Both 2% ketoconazole and 1% selenium sulfide shampoos are often recommended as adjuvant topical therapy. SUMMARY: Currently, many experts consider griseofulvin to be the drug of choice for tinea capitis. Short-term terbinafine, itraconazole and fluconazole therapy have been shown to be comparable in efficacy and safety with griseofulvin. Regular epidemiological surveillance of causative fungal organisms in the community and their antifungal susceptibility is an essential component in the management of this condition.

Harlequin ichthyosis in association with hypothyroidism and juvenile rheumatoid arthritis.

Harlequin ichthyosis is a rare and severe congenital erythrodermic ichthyosis characterized at birth by hyperkeratotic plates covering the entire body, ectropion, eclabium, poorly developed ears, and contractures of the hands and feet. Two Chinese children, a 2-year-old boy and an 11-year-old girl, presented with these classic features as well as alopecia and loss of eyebrows and eyelashes. The boy was small for his age and was found to have hypothyroidism at the age of 18 months; he is currently on thyroxine replacement therapy. At 6 years of age, the girl developed symmetrical polyarthritis associated with positive rheumatoid factor and radiologic evidence of erosive arthritis, suggestive of juvenile rheumatoid arthritis. She received prednisolone, nonsteroidal anti-inflammatory drugs (NSAIDs), and subsequently methotrexate for her arthritis, with clinical and radiologic improvement. Early therapy with oral retinoids in both children accelerated shedding of the hyperkeratotic plates as well as improved ectropion and eclabium. There was no major adverse reaction to oral retinoids. The development of juvenile rheumatoid arthritis in survivors with harlequin ichthyosis has not been previously described. The use of prednisolone and NSAIDs in the girl did not affect the skin condition, but the addition of methotrexate led to a decrease in erythema. The association with autoimmune disease is probably coincidental. The psychosocial impact of this severe lifelong disease on the two families was enormous. Early retinoid therapy may improve the disorder and help increase survival rates. A multidisciplinary approach, including psychosocial support of the affected families, is vital in the management of this lifelong disease.

Whorled scarring alopecia: a rare phenomenon in incontinentia pigmenti?

Incontinentia pigmenti is a rare X-linked dominant disease that affects the ectodermal tissues and is usually lethal in males. Two girls, 1 Malay and 1 Chinese, with incontinentia pigmenti of the Bloch-Sulzberger type had a whorled pattern of scarring alopecia. This phenomenon, hitherto unreported in association with this disorder, corresponded to the lines of Blaschko. The mother and maternal grandmother of the Malay girl also had whorled scarring alopecia. This phenomenon is permanent and can be used as a marker to ascertain affected adult women who may no longer have cutaneous manifestations. X chromosome inactivation in females during early embryogenesis results in a mosaic population of cells, which explains the linear and patchy cutaneous manifestations of incontinentia pigmenti.

Suggested guidelines for screening and management of tuberculosis in patients taking oral glucocorticoids--an important but often neglected issue.

One third of the world's population is infected with tuberculosis and its incidence is increasing. Systemic glucocorticoids are the mainstay of immunosuppressive therapy for immunobullous diseases and collagen vascular diseases in dermatology. Patients who are given prolonged glucocorticoid therapy may be at risk for both the acquisition of primary tuberculosis and the reactivation of nonactive tuberculosis. The purpose of this article is to highlight the importance of screening for tuberculosis and its management in this group of patients, as there are no published guidelines in the dermatology literature on this subject. We summarize the methods used for tuberculosis screening, give an account of treatment regimens for the various types of tuberculosis infection, and suggest a practical approach to screening and management of tuberculosis in patients receiving long-term glucocorticoid therapy.