Review article: Emerging issues in pediatric skin of color, Part 2.

Dermatology for pediatric skin of color is the application of dermatology to the genetically diverse and distinctive segment of the pediatric population that includes children of non-White racial and ethnic groups with increased pigmentation including individuals of Asian, Hispanic/LatinX, African, Native American, Pacific Island descent, indigenous people among others with overlap in particular individuals, and mixtures thereof. The discipline of pediatric skin of color can be challenging with difficulty in diagnosis of common conditions due to underlying pigmentation, variations in common hair styling practices, and differences in demographics of cutaneous disease. Whereas some conditions are more common in children of color, other conditions have nuances in clinical appearance and therapeutics with regard to skin color. This article, the second of the series, focuses on inflammatory skin disease nuances, melanocytic disorders, and hypopigmented mycosis fungoides.

Review article: Emerging issues in pediatric skin of color, part 1.

Dermatology for the pediatric skin of color population is the application of dermatology to the genetically diverse and distinctive segment of the pediatric population that includes children of non-White racial and ethnic groups with increased pigmentation including individuals of Asian, LatinX, African, Native American, Pacific Island descent, Indigenous Peoples, among others, with overlap in particular individuals, and mixtures thereof. Treating children of color is a unique skill set within the field of pediatric dermatology, requiring knowledge and sensitivity. The discipline of pediatric skin of color can be challenging. Difficulty in diagnosis of common conditions stems from underlying pigmentation, variations in common hairstyling practices, and differences in demographics of cutaneous disease, whereas some conditions are more common in children of color, other conditions have nuances in clinical appearance and/or therapeutics with regard to skin color. This article is the first in a series of two articles looking at recently published skin-related issues of high concern in children of color. Conditions reviewed in Part 1 include (1) hairstyling hair-related concerns (traction alopecia, central centrifugal cicatricial alopecia, endocrine disruption), (2) autoimmune concerns (cutaneous lupus, vitiligo), and (3) infections (tinea capitis, progressive macular hypomelanosis).

Comparison of Two Formulations of Topical Timolol for the Treatment of Infantile Hemangiomas.

This report compares the efficacy of timolol maleate 0.5% eyedrops (TM) with that of timolol maleate 0.5% ophthalmic gel-forming solution in the treatment of infantile hemangiomas (IHs). Sixty-six patients undergoing treatment with topical timolol were retrospectively reviewed; our results revealed similar therapeutic efficacies for both types of timolol formulations. Early treatment initiation (<3 mos) and superficial lesions correlated with better treatment response. TM eyedrops may be a more cost effective, equally efficacious modality of treatment for IHs.

Inflammatory morphea mimicking an acquired port-wine stain initially treated with pulsed-dye laser.

The early inflammatory lesions of morphea may present with erythema or violaceous patches and plaques before evolving into areas of sclerosis. They have been misdiagnosed as acquired port-wine stains (PWSs). We report a previously well 7-year-old Chinese girl presenting with early facial morphea mimicking an acquired PWS with unusual histologic features of perineural inflammation. The presence of cutaneous perineural inflammation may be seen in a small percentage of cases of morphea and appears to be a feature of early inflammatory morphea. We report this case to highlight the importance in recognizing this entity and summarize the reported cases of inflammatory morphea mimicking acquired PWSs.

Pediatric Cutaneous Nontuberculous Mycobacterium Infections in Singapore.

BACKGROUND: Nontuberculous mycobacterium (NTM) infections are rare in children, with limited published studies. The course of the disease can be variable and there are no accepted treatment guidelines for the management of NTM infections in children. OBJECTIVE: To review a cohort of pediatric patients admitted to a tertiary pediatric hospital in Singapore for cutaneous NTM infections. METHODS: A retrospective review was performed of all children admitted to KK Women's and Children's Hospital with cutaneous NTM infections from 2002 to 2012. RESULTS: Sixty-seven patients with positive NTM cultures from various body sites were identified. Eight of the 67 patients (11.9%) presented with cutaneous NTM without evidence of systemic involvement. The mean age at diagnosis for these eight patients was 10 years (range 5-21 yrs). Mycobacterium abscessus was the most common NTM isolated (five patients), followed by Mycobacterium hemophilium (two patients) and Mycobacterium kansasii (one patient). Most patients presented with isolated skin abscesses. Two patients were immunocompromised. Six patients required multidrug antibiotic treatment for a median duration of 5.5 months (range 3-17 mos). The median follow-up duration was 8.5 months (range 2 wks-29 mos). CONCLUSION: Although the incidence of cutaneous NTM is rare, the diagnosis should be considered in patients presenting with chronic wounds. Most patients require treatment with multidrug antibiotic therapy, although uncomplicated abscesses can be treated with surgical incision and drainage alone.

Cutaneous Langerhans cell histiocytosis: study of Asian children shows good overall prognosis.

AIM: To describe an Asian population with isolated cutaneous Langerhans cell histiocytosis (LCH), presenting a review on previous studies on this subgroup of patients and comparing our cohort with other studies of cutaneous LCH with systemic involvement. METHODS: All patients diagnosed as LCH with cutaneous involvement presenting to a tertiary paediatric hospital (KK Women's & Children's Hospital) between January 2001 and December 2011 were reviewed. Information recorded included clinical presentation, investigation results, treatment and outcome. RESULTS: We identified 10 patients with cutaneous manifestations of LCH. Six had isolated cutaneous LCH with no visceral or bony involvement, while four had cutaneous lesions with involvement of other organ systems. Of the patients with isolated skin involvement, three were treated with surgical excision, one with topical corticosteroids and two with multi-agent chemotherapy. The four patients with concomitant systemic involvement were treated with multi-agent chemotherapy. None of our patients with isolated cutaneous LCH progressed to multisystem disease during the follow-up period. CONCLUSION: Cutaneous LCH, with or without other organ involvement at diagnosis has a good overall prognosis. However, long-term follow-up is recommended in view of possible recurrence.

Retrospective analysis of earlobe keloids treated with the carbon dioxide laser ablation or cold steel debulking surgery.

BACKGROUND: Treatment of keloids is a therapeutic challenge. OBJECTIVES: To determine the outcome and the risk of recurrence after debulking cold steel surgery or carbon dioxide (CO2) laser ablation of earlobe keloids. MATERIAL AND METHODS: The case records of 16 patients with earlobe keloids managed at Changi General Hospital, Singapore, from 2003 to 2009 were reviewed retrospectively. RESULTS: Fourteen patients were females, and the mean age at presentation was 20 years. Eight patients underwent CO2 laser ablation, six patients underwent cold steel surgery, one patient underwent both surgery and CO2 laser ablation, and one patient received only 40 mg/ml of intralesional triamcinolone acetonide. Fourteen patients were followed up for 1-24 months post procedure, and two patients defaulted. Both modalities were equally effective in debulking the earlobe keloids. All 13 patients who had either CO2 laser ablation or cold steel surgery had recurrence of keloid growth at 2-18 weeks post procedure. The patient who received intralesional triamcinolone acetonide therapy alone had only partial response to the therapy. CONCLUSIONS: Both the CO2 laser ablation and cold steel surgery were equally useful in reducing the size of the earlobe keloids, but were not effective in preventing regrowth of the keloids, even with adjunctive intralesional steroids. Patients should be clearly counselled regarding this.

Profile and pattern of Stevens-Johnson syndrome and toxic epidermal necrolysis in a general hospital in Singapore: treatment outcomes.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, but potentially life-threatening, reactions to medications. Both conditions have significant morbidity and mortality. The aim of this study was to document the epidemiological features, aetiologies, treatment and clinical outcomes of retrospectively reviewed data of all patients with SJS or TEN treated from January 2004 to November 2010 in a general hospital. There were 18 cases of SJS, seven cases of SJS/TEN overlap and three cases of TEN. Mean age was 50.6 years, with a range of 13-85 years. The male/female ratio was 1. Drugs accounted for 26 cases; one case was caused by Neisseria gonorrhoea infection. Anti-convulsants (35.7%) were the most common implicated drugs followed by antibiotics (28.5%), non-steroidal anti-inflammatory drugs (NSAIDS) (14.3%), allopurinol (7.1%) and traditional Chinese medication (7.1%). In seven cases, multiple drugs were implicated. Most SJS cases (88%) were treated with corticosteroids, of which 61% were given high-dose systemic corticosteroids. No infective complications were observed. Six out of the seven SJS/TEN overlap syndrome and all three TEN cases were given intravenous immunoglobulins. One patient with TEN died. In conclusion, anti-convulsants, especially carbamazepine, were the most frequently implicated drugs, followed by antibiotics and NSAIDS. High-dose corticosteroids were effective in SJS, whereas intra-venous immunoglobulin were useful in TEN and SJS/TEN overlap syndrome.

Treatment of infantile hemangiomas with the 595-nm pulsed dye laser using different pulse widths in an Asian population.

INTRODUCTION: The pulsed dye laser (PDL) using varying fluences and pulse durations have been used to treat hemangiomas. This study aims to examine the efficacy and safety of the 595-nm PDL for the treatment of infantile hemangiomas using short (1.5-3 milliseconds) versus long (10 milliseconds) pulse durations and high fluences. MATERIALS AND METHODS: This is a retrospective study of patients with hemangiomas (n = 23) treated with the 595-nm PDL from 2003 to 2007. The parameters used for the short pulse duration group (n = 15) were 7-mm spot size, fluence 10-13.5 J/cm(2) and dynamic cooling device (DCD) spray duration of 50 milliseconds and delay of 30 milliseconds. For the long pulse duration group (n = 8), parameters were 7-mm spot size, fluence 10.5-14.5 J/cm(2) and DCD spray duration of 40 milliseconds and delay of 20 milliseconds. RESULTS: The number of treatments required to achieve complete or near complete resolution of the hemangioma ranged from 3 to 14 for the short pulse duration group (mean: 8) and for the long pulse duration group, 4-14 treatments (mean: 9). For both groups, more treatments were needed to achieve clearance of mixed hemangiomas (n = 13) compared to superficial hemangiomas (n = 10) (on average, 4-5 treatments more). Erythema, edema, and purpura lasted for about a week in the short pulse duration group but only 2 days in the long pulse duration group. There was no ulceration or hypertrophic scarring noted in both groups. CONCLUSION: Both short and long pulse durations using moderately high fluences are equally effective in the treatment of infantile hemangiomas. Shorter pulse durations had a slightly higher incidence of side effects compared to longer pulse duration in our patients with darker phototypes. Hemangiomas are tumors with relatively large diameter blood vessels and this provides the basis for the use of longer pulse durations.

Successful treatment of eosinophilic pustular folliculitis with topical tacrolimus 0.1 percent ointment.

Classic eosinophilic pustular folliculitis (EPF), otherwise known as Ofugi disease, is a rare condition commonly treated with topical glucocorticosteroids. If this fails, oral indomethacin is frequently the next line. Because the condition is recurrent, the use of long term steroids may cause side effects such as skin atrophy, hypertrichosis, and dyspigmentation. Topical tacrolimus is an immunosuppressant that is generally used as a steroid-sparing agent in atopic dermatitis. We report a case of classic EPF, which was recurrent over 5 years that had failed topical glucocorticosteroids but was successfully treated with topical tacrolimus 0.1 percent ointment.

Case Report: Mosaicism of a novel nonsense variant in the neurofibromin gene underlies a mosaic generalized NF1 phenotype.

Neurofibromatosis 1 (NF1) is a neurocutaneous syndrome characterized by multiple café-au-lait macules, cutaneous neurofibromas or plexiform neurofibromas, iris Lisch nodules, axillary and inguinal freckling. Mosaicism in NF1 can either present as a generalized disease, or in a localized (segmental) manner. Mosaic generalized NF1 may have presentations that are similar to generalized NF1 or have a milder phenotype and hence may be under-recognised in clinical practice. We report a nonsense mutation in the NF1 gene in a 55-year old Chinese male with the mosaic generalized phenotype. He reported noticing increasing numbers of skin-colored papules over his face, neck, back and abdomen when he was about 40 years old. From both next-generation and Sanger sequencing data, the variant appeared to be mosaic and present at about 24%. It is in exon 39 and has not been reported in any database or published literature.

Practical Recommendations for the Topical Treatment of Atopic Dermatitis in South and East Asia.

INTRODUCTION: There is some evidence to suggest that the prevalence of atopic dermatitis (AD) in Asia is rising. We have therefore developed an algorithm for the topical treatment of AD throughout South and East Asia for use by primary care physicians, pediatricians and dermatologists. METHODS: Nine AD experts from South and East Asia and one from Europe developed the algorithm based upon treatment guidelines, relevant literature and local treatment practices. The algorithm outlines current best practice for the use of emollients, topical corticosteroids (TCS) and topical calcineurin inhibitors (TCI), with the intention of simplifying the treatment regimen of mild-to-moderate AD in South and East Asia. RESULTS: Patients with AD should bathe and cleanse affected skin to remove crusts and scales daily. Emollients should also be applied daily as a maintenance treatment. When selecting appropriate topical anti-inflammatory treatment for AD flares, several factors should be taken into consideration, including the patient's age, attitude to treatment options and site of AD lesions. Given the concerns regarding the risk of skin atrophy with use of TCS, a TCI should be used to treat AD lesions in sensitive skin areas: pimecrolimus is recommended for mild-to-moderate AD in these locations, while tacrolimus should be considered for moderate and severe cases. Either pimecrolimus or tacrolimus is recommended for flares in other, non-sensitive body locations. A proactive or intermittent maintenance treatment strategy involving regular emollient use and twice-weekly application of a TCI to previously affected areas is encouraged to reduce the risk of flares. CONCLUSIONS: The algorithm proposed here is intended to simplify the topical treatment of mild-to-moderate AD in daily practice in South and East Asian countries.

Stevens-Johnson syndrome and toxic epidermal necrolysis in Asian children.

BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but severe drug reactions. There have been few reviews of SJS and TEN in children. OBJECTIVES: To evaluate the clinical profile and treatment outcomes of 15 pediatric patients with SJS or TEN. METHODS: We retrospectively reviewed the case notes of all patients diagnosed with SJS or TEN admitted to a tertiary care pediatric hospital from 2001 to 2006. RESULTS: We identified 13 cases of SJS, 1 case of SJS/TEN overlap and 1 case of TEN. Four patients were treated with intravenous immunoglobulin (IVIg), 5 patients were treated with systemic corticosteroids, and 6 patients were treated with supportive therapy only. The time to cessation of progression of disease was not significantly different in these 3 groups of patients. The duration of hospital stay was longer for patients treated with IVIG compared with those treated with systemic corticosteroids or supportive therapy. The only death was the patient with TEN treated with IVIG. LIMITATIONS: This was a retrospective study with a very small number of patients. CONCLUSION: The use of intravenous immunoglobulins or systemic corticosteroids did not improve the outcome of SJS and TEN.

Retrospective analysis of drug-induced hypersensitivity syndrome: a study of 27 patients.

BACKGROUND: Previous reports regarding the characteristics of patients with drug-induced hypersensitivity syndrome (DIHS) are mostly limited to small case reports and drug-specific case series, mainly involving Caucasian patients. OBJECTIVE: We describe the trends in the clinical presentation and laboratory findings of our patients with DIHS and their response to therapy. METHODS: This is a retrospective case series. Clinical records of patients treated in the Department of Dermatology, Changi General Hospital, Singapore, with a diagnosis of DIHS from January 2003 to January 2008 were retrieved and analyzed. RESULTS: In all, 27 patients were analyzed. The 3 most consistent features in our patients were 1) history of drug exposure (100%); 2) a morbilliform cutaneous eruption in 81.5% of the patients; and 3) systemic involvement with hepatitis (96.3%), hematologic abnormalities (81.5%), and fever (77.8%) being most common. Superficial perivascular dermatitis was the most common skin biopsy specimen findings, with tissue eosinophilia occurring in half the biopsy specimens. Severe complications included renal failure requiring dialysis in two patients and hyperthyroidism and myocarditis occurring in one patient. LIMITATIONS: This is a retrospective study with a small number of patients during a 5-year period. We did not routinely document the presence of lymphadenopathy and hepatosplenomegaly in our patient's records. We do not measure viral serologies routinely as part of the workup for DIHS. CONCLUSION: DIHS is not uncommon. The presentation in an Asian population is similar to published studies. Treatment includes discontinuation of the offending drug, monitoring for organ involvement, and using systemic steroids.

Fractional ablative carbon dioxide laser in the treatment of atrophic acne scarring in Asian patients: a pilot study.

INTRODUCTION: Ablative carbon dioxide resurfacing is the gold standard for treating atrophic acne scarring but is associated with prolonged recovery and many side effects. To address these limitations, newer modalities employing the principle of fractional photothermolysis have emerged. METHODS: We undertook a prospective study whereby five Asian patients of skin phototype IV with moderate to severe atrophic acne scarring received two sessions of a fractional carbon dioxide laser 6-8 weeks apart. Treatment parameters were: fluence, 28 J/cm(2); pulse width, 2.5 ms; spot size, 300 microm; penetration depth, up to 500 microm; degree of skin coverage, 20%; single pass. Photographic evaluation was done on the level of improvement according to a quartile grading scale: 75% (excellent). RESULTS: At 2 months post-treatment, all five subjects showed some clinical improvement (four: mild improvement; one: moderate improvement). The treatment was well tolerated. All patients had erythema, which lasted for a mean of 6 days. No other complications were observed. CONCLUSION: Our study has shown that in Asians the fractional ablative carbon dioxide laser produces mild to moderate improvement in acne scarring with the advantage of a quick recovery period with minimal adverse effects.

Juvenile xanthogranuloma in monozygotic twins.

Juvenile xanthogranuloma is usually a benign condition mainly seen in infants and children. It frequently presents as asymptomatic discrete papules on the head, trunk, and limbs. Extracutaneous manifestations, most commonly ocular, are rare but may be associated with significant morbidity. The etiology of juvenile xanthogranuloma is uncertain, although the occurrence in monozygotic twins may suggest genetic predisposition.

An update on Stevens-Johnson syndrome and toxic epidermal necrolysis in children.

PURPOSE OF REVIEW: This study summarizes current research and understanding of the pathogenesis of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) and provides an update on the treatment of these conditions in children. RECENT FINDINGS: The association of specific human leukocyte antigen subtypes with SJS and TEN occurring in certain racial groups to specific drugs has led to recommendations on pretreatment testing. Several pathways have been postulated to lead to keratinocyte apoptosis in SJS and TEN. These include Fas-Fas ligand interaction, cytotoxic T-cell and natural killer-cell damage via perforin/granzyme B/granulysin and tumor necrosis factor-alpha. The use of intravenous immunoglobulins and systemic corticosteroids in TEN is still controversial, and more trials are needed to prove the efficacy of these agents. Newer agents such as cyclosporin, infliximab and plasmapheresis have shown promise in the treatment of SJS and TEN. SUMMARY: As the pathogenesis of SJS and TEN is further unraveled, the emergence of newer therapeutic agents with more specific mechanisms of action may lead to improved survival in this oftentimes devastating disease.

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.