Clinical practice guidelines for the diagnosis and management of atopic dermatitis.

Atopic dermatitis (AD), a chronic, relapsing dermatitis, is characterized by dry and pruritus skin in patients with a personal or family history of atopy. It affects up to 20% of children and 1-3% of adults in most countries worldwide, and leads to significant treatment costs and morbidity. These guidelines are developed in accordance with evidence-based publications and expert opinions. Following simple algorithms, the guidelines aim to assist adult and pediatric physicians in the better care of patients with AD. As with other diseases, there have been several diagnosis criteria proposed over time. Nonetheless, the classical Hanifin and Rajka criterion with no pathognomonic laboratory biomarkers is still the most widely used worldwide for the diagnosis of AD. The management of AD must be considered case by case to provide suitable care for each patient. Basic therapy is focused on avoiding specific/unspecific provoking factors and hydrating skin. Topical anti-inflammatory treatments such as glucocorticoids and calcineurin inhibitors are suggested for disease flare, and proactive therapy is best for long-term control. Other therapies, including antimicrobial agents, systemic antihistamines, systemic anti-inflammatory agents, immunotherapy, phototherapy, and psychotherapy, are reviewed in these guidelines. Crisaborole, a new topical phosphodiesterase 4 inhibitor, can be used twice daily in AD patients over three months old. Dupilumab, a biological drug for patients with moderate-to-severe AD, may be considered in patients with no improvement from other systemic treatments.

Clinical practice guideline for diagnosis and management of urticaria.

Urticaria is a common skin condition that can compromise quality of life and may affect individual performance at work or school. Remission is common in majority of patients with acute spontaneous urticaria (ASU); however, in chronic cases, less than 50% had remission. Angioedema either alone or with urticaria is associated with a much lower remission rate. Proper investigation and treatment is thus required. This guideline, a joint development of the Dermatological Society of Thailand, the Allergy, Asthma, and Immunology Association of Thailand and the Pediatric Dermatological Society of Thailand, is graded and recommended based on published evidence and expert opinion. With simple algorithms, it is aimed to help guiding both adult and pediatric physicians to better managing patients who have urticaria with/without angioedema. Like other recent guideline, urticaria is classified into spontaneous versus inducible types. Patients present with angioedema or angioedema alone, drug association should be excluded, acetyl esterase inhibitors (ACEIs) and non-steroidal anti-inflammatory drugs (NSAIDs) in particular. Routine laboratory investigation is not cost-effective in chronic spontaneous urticaria (CSU), unless patients have clinical suggesting autoimmune diseases. Non-sedating H1-antihistamine is the first-line treatment for 2-4 weeks; if urticaria was not controlled, increasing the dose up to 4 times is recommended. Sedating first-generation antihistamines have not been proven more advantage than non-sedating antihistamines. The only strong evidence-based alternative regimen for CSU is an anti-IgE: omalizumab; due to very high cost it however might not be accessible in low-middle income countries. Non-pharmacotherapeutic means to minimize hyper-responsive skin are also important and recommended, such as prevention skin from drying, avoidance of hot shower, scrubbing, and excessive sun exposure.

SLC39A4 mutation in zinc deficiency patients.

OBJECTIVE: To analyze the clinical presentation and SLC39A4 mutations in zinc deficiency patients. MATERIAL AND METHOD: The authors conducted a cross-sectional study on all cases of zinc deficiency treated at Queen Sirikit National Institute of Child Health between January 2004 and December 2012. Demographic data, clinical manifestations, laboratory results, treatment and outcome were analyzed. Genetic, SLC39A4 for acrodermatitis enteropathic (AE), mutation analysis was performed in all cases. RESULTS: There were 15 cases, 10 males and 5 females. The age of onset was between 2 and 10 months (median 3 months). Duration of the disease ranged between 3 days and 17 months (median 2 months). Acral and periorificial dermatitis, diarrhea and alopecia were present in 15 cases (100%), 12 cases (80%) and 8 cases (53%) respectively. The characteristic triad of acral and periorificial dermatitis, diarrhea and alopecia was observed in only 6 patients (40%). Serum zinc level ranged between 10 and 111 mcg/dl (mean 49.69 ± 33.87 mcg/100 ml). Low serum zinc level was observed in 10 cases (67%). All of the patients were treated with zinc sulfate 5 mg/kg/day. All cutaneous lesions and diarrhea had resolved within 7 days of starting therapy. A genetic study of SLC39A4 gene in our 15 patients revealed that 3 patients had homozygous c.1878_1879ins21 (p.G627_T633dup) in exonl2. These three patients have to receive lifelong zinc supplementation to prevent recurrence of the disease. The other twelve patients, who did not carry the gene mutation, did not have symptoms after discontinuance of oral zinc therapy. This is the first report of genetically confirmed acrodermatitis enteropathic in Thailand. CONCLUSION: Acrodermatitis enteropathica is a rare disease, which needs lifelong zinc supplementation. A genetic study of SLC39A4 gene will confirm the diagnosis. Most of patients presenting with characteristic triad of acral and periorificial dermatitis, diarrhea and alopecia in Thailand were acquired zinc deficiency. Early recognition and treatment of the disease will decrease morbidity and mortality.

Neonatal lupus erythematosus: a 20-year retrospective study.

OBJECTIVE: To study clinical manifestations, investigations, treatment and outcomes of neonatal lupus erythematosus (NLE) patients and their mothers. MATERIAL AND METHOD: A retrospective descriptive study was performed to review the neonatal lupus erythematosus patients and their mothers at Queen Sirikit National Institute of Child Health during January 1993 to December 2013. The diagnostic criteria required the presence of clinical symptoms plus positive anti-Ro/SSA or anti-La/SSB or both. RESULTS: There were 34 cases, 12 males and 22 females. Age of onset of clinical manifestations was from birth to 60 days with median age of 21 days. Cutaneous, hepatobiliary, hematological and cardiac abnormalities were found in 88.2%, 61.2%, 50%, 14.7%, respectively. Cutaneous lesions included erythematous rash (70%), annular lesions (75.8%), petechiae (26.6%), raccoon eyes (26.6%), and telangiectasia (20%). Among those with hepatic involvement (n = 18), transaminitis was the most common finding (100%) followed by hepatosplenomegaly (38.8%) and cholestasis (22.2%). Seventeen cases (50%) had hematological problems including anemia (29.4%) and anemia with thrombocytopenia (20.6%). The most severe complication, complete heart block was found in 14.7% (n = 5). Anti-Ro/SSA and anti-La/SSB were positive in 91.1% and 58.8% of cases, respectively. All four babies with complete heart block were treated with pacemaker. Systemic corticosteroids were given to eleven babies due to severe skin lesions and hepatic involvement. There was no mortality during the study. Most neonatal lupus erythematosus mothers (24 cases, 74.2%) were asymptomatic. Ten mothers (25.8%) were diagnosed as autoimmune diseases (systemic lupus erythematosus in 6 cases and other autoimmune diseases in 4 cases). However, 7 of 24 asymptomatic mothers developed SLE within 3 years after delivery. CONCLUSION: NLE should be suspected among neonates or young infants presenting with congenital heart block or skin rash with multi-system involvement despite a lack of concurrent maternal autoimmune diseases. Anti-Ro/SSA and/or anti-La/SSB are the most useful laboratory diagnosis. Most NLE patients without congenital heart block have relatively good prognosis.

Subcutaneous zygomycosis in children: 2 case reports.

Basidiobolus ranarum is an uncommon subcutaneous zygomycosis mostly found in immunocompetent children in tropical countries. Presence of slow growing non-tender, non-inflammatory, subcutaneous swelling that does not spread beyond the subcutaneous tissue are classic clinical features. The authors report two cases of subcutaneous zygomycosis which tissue cultures were positive for Basidiobolus ranarum. The first case was a 10-months-old boy presented with prolonged high fever and a rapidly expanding ulcerated plaque unresponsive to systemic antibiotic. The second case was a 2-years-old girl presented with slow expanding mass at the buttock. Histopathology of both cases showed lobular panniculitis with eosinophilic infiltration and fungal culture revealed Basidiobolus ranarum. Oral itraconazole was given with good clinical response in both cases.

Randomized, double-blind, split-side, comparison study of moisturizer containing licochalcone A and 1% hydrocortisone in the treatment of childhood atopic dermatitis.

BACKGROUND: Atopic dermatitis (AD) is a common chronic inflammatory skin lesion in children. Topical corticosteroid is the mainstay of treatment. OBJECTIVE: To compare the efficacy of moisturizer containing licochalcone A (LicA) and 1% hydrocortisone for the treatment of mild to moderate childhood AD. MATERIAL AND METHOD: This was a multicenter randomized, prospective, split-side, double-blind study in 55 children between the age of three months and 14 years. Patients with AD were treated twice daily, simultaneously with either Lic A or 1% hydrocortisone on opposite sides of the lesion. The SCORAD and transepidermal water loss (TEWL) were performed at the baseline, 2-week, and 4-week visits. Lic A was used on both sides of the body for another four weeks to see the effects and TEWL. RESULTS: In a randomized period, both products were equally effective in the treatment. SCORAD decreased significantly from baseline for both treatments throughout the first four weeks (p < 0.001). There was no statistically significant difference in SCORAD between both treatments (p = 0.321 and p = 0.146 at week 2 and 4, respectively). Lic A had statistically significant decrease in TEWL (p = 0.027 and p = 0.03 at weeks 2 and 4, respectively). One patient had infection on skin lesions of both sides of the body. Forty-three patients continued to the period of using Lic A on both sides of the body. SCORAD and TEWL were comparable to the end of the randomized period and significantly lower from baseline (p < 0.001). Skin lesions flared up in three patients (7.5%). CONCLUSION: Lic A had a similar result in terms of SCORAD compared to 1% hydrocortisone for the treatment of mild and moderate AD. TEWL was significantly lower than baseline on the side that used Lic A. Continuing use of Lic A for four weeks can maintain clinical and barrier improvement.

Stevens-Johnson syndrome in Thai children: a 29-year study.

BACKGROUND: Stevens-Johnson syndrome (SJS) is a rare and severe life-threatening hypersensitivity syndrome. The etiology is unclear but is associated with drug exposure or infections and frequently high morbidity and mortality. OBJECTIVE: To determine etiologies, treatments and complications of Stevens-Johnson syndrome (SJS) in children. MATERIAL AND METHOD: A retrospective descriptive study was performed at Queen Sirikit National Institute of Child Health during 1979 and 2007 (29-year study). The authors collected and separated data into three phases from 1979 to 1987, 1988 to 1997 and 1998 to 2007. Diagnosis was confirmed by pediatric dermatologists. RESULTS: There were 189 patients, 56 cases between 1979-1987, 72 cases between 1988-1997 and 61 cases between 1998-2007. The ratio of male to female was 1.6: 1. The range of age was from 2 months to 15 years old with a mean age of 5.5 years. One hundred and sixty-five cases (87%) had a history of drug taking before onset of the rash. The most common drugs exposure were antibiotics in 69 cases (42%), anticonvulsant drugs in 58 cases (35%), non-steroids anti-inflammatory drugs in 8 cases (5%), antimalarial drugs in 4 cases (2%) and unknown drugs in 26 cases (16%). Mycoplasma infections were found in 5 cases (3%). One hundred and nine cases (58%) were treated with systemic corticosteroids. The corticosteroid treatment was increasing from 18% in the first phase to 64% and 87% in the second and third phase respectively. The overall complications were found in 38 cases (20%) included bacterial skin infections in 16 cases (8%), eye complications in 12 cases (6%), hepatitis in 4 cases (2%) and other complications in 6 cases (2%). Ten patients (5%) died from sepsis and underlying diseases. The mortality rate declined from 9% in the first phase to 1.5% in the third phase. CONCLUSION: Etiology of SJS in children was associated with drug exposure with the most commonly implicated drug being antibiotics and anticonvulsants. Corticosteroid may have a role in the treatment of SJS.

Predictive factor of severe renal involvement in children with Henoch-Schoenlein purpura.

OBJECTIVE: To find out which of the clinical manifestations or laboratory findings is the predictive factor of severe renal involvement in children with Henoch-Schoenlein purpura (HSP). MATERIAL AND METHOD: Data of clinical manifestations and laboratory findings in children with HSP at Queen Sirikit National Institute of Child Health between January 2003-December 2007 were prospectively collected and analyzed. RESULTS: There were 168 cases, 86 boys and 82 girls (M:F ratio= 1.05:1), the age ranged from 2 to 15 years (mean +/- SD = 6.9 +/- 2.6 years, mode = 6.8 years). Development of severe renal involvement was identified in 11 cases (6.6%). Abnormal urinalysis (microscopic hematuria or proteinuria) on the day of diagnosis was statistically significant (p < 0.001) as a predictive factor of severe renal involvement during follow-up. Early systemic corticosteroid administration due to severe abdominal pain was not statistically significantly different between the patients with or without severe renal involvement. CONCLUSION: Abnormal urinalysis on the day of diagnosis was the only predictive factor of severe renal involvement in children with Henoch-Schoenlein purpura. Early systemic corticosteroid administration due to severe abdominal pain did not prevent severe renal involvement.

Cutaneous mastocytosis in Thai children.

BACKGROUND: Mastocytosis is a disorder of mast cells proliferation within various organs, most commonly in the skin. The disease more commonly appears during infancy than adult. OBJECTIVE: To characterize the clinical features, response to therapy and prognosis of cutaneous mastocytosis in children. MATERIAL AND METHOD: A retrospective study of cutaneous mastocytosis was performed at Queen Sirikit National Institute of Child Health during January 1994 to December 2007.All cases were confirmed by histological diagnosis. RESULTS: There were a total of 50 patients. The male to female ratio was 1:1.2. Age at onset of lesions ranged from birth to 7 years. Forty-seven patients (94%) developed skin lesions within the first year of ife. There were 45 cases (90%) of urticaria pigmentosa, 3 cases (6%) of mastocytoma and 2 cases (4%) of diffuse cutaneous mastocytosis. None of the patient had a family history of cutaneous mastocytosis. Most of the children were healthy, except the one who had germ cell ovarian tumor Skin biopsies were performed in all cases and revealed mast cells infiltrate in the dermis. Treatment included oral antihistamine in all cases. Oral mast cell stabilizers were given in 6 patients (12%) and topical corticosteroids in 15 patients (30%). Four patients (8%) were treated with oral prednisolone. The skin lesions resolved only in 1 patient (2%) at age 7.8 years, the others still had skin lesions without systemic symptoms. CONCLUSION: Cutaneous mastocytosis is a benign disease in children without systemic involvement.

Subcutaneous fat necrosis of the newborn.

Subcutaneous fat necrosis of the newborn (SCFN) is an uncommon disorder in full term infants who have a perinatal condition. The authors reported seven cases of subcutaneous fat necrosis of the newborn at Queen Sirikit National Institute of Child Health from 2002 to 2005. All cases were term babies with four male cases, and three female cases. Five cases (70%) had perinatal asphyxia. The mean age of onset was 14 days (range 3-42 days). The locations of SCFN were back (3 cases), shoulder (2cases), arm (2 cases), buttock (1 case), and neck (1 case). Skin biopsy was performed in three cases and was compatible with subcutaneous fat necrosis. The treatment was supportive with close monitoring of serum calcium. Hypercalcemia was seen in five cases (70%) and three cases were treated with oral prednisolone. Cutaneous lesions of all cases resolved without sequelae. In conclusion, infants with subcutaneous fat necrosis should have serial serum calcium determinations and should be observed closely for signs and symptoms of hypercalcemia such as irritability, anorexia, constipation, and failure to thrive.

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.

Comparison of dexpanthenol and zinc oxide ointment with ointment base in the treatment of irritant diaper dermatitis from diarrhea: a multicenter study.

BACKGROUND: Severity of irritant diaper dermatitis (IDD) from diarrhea varies from patient to patient depending on the nature of feces and the number of bowel movements. The purpose of the present study was to compare the effectiveness of dexpanthenol and zinc oxide ointment with ointment base in the treatment of irritant diaper dermatitis from acute diarrhea in children by measuring transepidermal water loss (TEWL). MATERIAL AND METHOD: Forty-six children with diarrhea were prospectively, block randomized, investigator-blinded to receive dexpanthenol and zinc oxide ointment on one side and ointment base on the other side. TEWL was measured before and on days 1, 3, and 7 of treatment together with the assessment of severity score. The efficacy of treatment was defined by complete clearance of the lesion. RESULTS: TEWL in the treated and control side was not different before the application of the topical medication. In the present study, the efficacy of 5% dexpanthenol and zinc oxide ointment on D3 was 39% (18from 46 patients) compared to 32% in the ointment base side. On D7, the efficacy of the treated side was 58.7% and the ointment base side was 56%. The patients who still had skin lesions were those who had prolonged diarrhea. On the treated side, the mean of TEWL was lower than the control side on DI (p = 0.18) and had significant improvement on D3 (p = 0. 002). At the end of the present study, TEWL on the treated side was less than TEWL of the control side but it did not have statistical significance (p = 0.07). There was no rash or sign of abnormality on the treated side at the end of D7. CONCLUSION: In the treatment of lDD from acute diarrhea, 5% dexpanthenol and zinc oxide ointment significantly decreased TEWL in the treated side more than the ointment base on day 3 but the severity score was not significantly different on days 1, 3 and 7.

A comparative study between 10 per cent sulfur ointment and 0.3 per cent gamma benzene hexachloride gel in the treatment of scabies in children.

BACKGROUND: Scabies is a common contagious skin disease in children. Treatment of scabies in infants and children is the subject of worldwide concern because of risk and benefit of the variety of scabicides. OBJECTIVE: To compare the efficacy of 10 per cent sulfur ointment and 0.3 per cent gamma benzene hexachloride gel for the treatment of scabies in children. METHOD: A randomized investigator blind study was conducted to compare the efficacy of 10 per cent sulfur ointment and 0.3 per cent gamma benzene hexachloride (GBH) for the treatment of scabies in children at Queen Sirikit National Institute of Child Health from December 1999 to May 2000. Diagnosis was made by the clinical signs of excoriated papules in the classic distribution with nocturnal pruritus and family history of similar symptoms. Diagnosis for all patients was confirmed by positive skin scrapings for eggs, larva, mites or fecal pellets by light microscopy. Patients were followed-up at intervals of 2 and 4 weeks. RESULTS: One hundred children with an age range from 6 months to 13 years were randomized into 2 groups, 10 per cent sulfur group (50 cases) and 0.3 per cent GBH (50 cases). Age, sex, history of contact cases and clinical manifestations were not statistically different between the two groups. After 4 weeks of treatment, there were no statistical differences between the two groups in patients assessed cured (92% vs 94%), clinical cure (92% vs 91%) and parasitic cure (83% vs 84%). The adverse effect of foul odor in the sulfur group was more common than in the GBH group (p < 0.05). CONCLUSION: 10 per cent sulfur ointment is as safe and efficacious as 0.3 per cent GBH for the treatment of scabies in children.

Immunobullous diseases in Thai children: report of 24 cases.

BACKGROUND: Acquired immunobullous diseases in children are very rare and difficult to distinguish clinically. OBJECTIVE: To study the clinical manifestations, immunopathologic features, treatment and outcome of immunobullous diseases in Thai children. MATERIAL AND METHOD: The authors reviewed 24 cases of immunobullous diseases in children under 18 years at Queen Sirikit National Institute of Child Health from 1983 to 2000. Diagnosis of all cases was made by clinical presentations of chronic blistering diseases and confirmed by histopathology and immunofluorescent studies. RESULTS: There were 18 cases of chronic bullous diseases of childhood (CBDC), 4 cases of bullous pemphigoid (BP) and 2 cases of pemphigus vulgaris (PV). The mean age of onset of CBDC and BP were 4 years and 2 years respectively. There was an equal male to female ratio in both CBDC and BP. Both cases of pemphigus (neonate and 4 years old) were female. Most CBDC patients (18 cases) responded well to dapsone therapy although 2 cases had to be treated with prednisolone simultaneously. All cases with BP were treated successfully with prednisolone and dapsone. Neonatal pemphigus was treated symptomatically without steroid therapy. The second case of oral pemphigus was controlled with low dose prednisolone. CONCLUSION: Immunobullous diseases are very rare in children. All patients improved with corticosteroid and/or dapsone therapy.