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.

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.

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.

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.

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.

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.