Juvenile dermatomyositis in Thai children: Retrospective review of 30 cases from a tertiary care center

Background: Juvenile dermatomyositis is a rare condition, but it is the most common idiopathic inflammatory myopathy in pediatric patients. Aim: To study the clinical manifestations, investigations, treatment, clinical course, and outcomes of juvenile dermatomyositis in Thai children. Method: This retrospective study included juvenile dermatomyositis patients treated at Siriraj Hospital, a 2,300-bed national tertiary referral center in Bangkok, Thailand, from 1994 to 2019. Results: Thirty patients (22 females and 8 males) were included with a female to male ratio of 2.7:1. Median age at diagnosis was 5.1 years (range, 2.6-14.8 years). Median duration of illness before diagnosis was 6.5 months (range, 0.3-84.0 months). Acute and subacute onset occurred in the majority of patients. Presenting symptoms included muscle weakness in 27/30 (90%), skin rash in 26/30 (86.7%), muscle pain in 17/26 (65.4%), and arthralgia in 4/18 (22.2%) of patients. Dermatologic examination revealed Gottron’s rash, heliotrope rash, and periungual telangiectasia in 25/30 (83.3%), 21/30 (70.0%), and 15/24 (62.5%) of patients, respectively. Interestingly, scalp dermatitis was found in 8/21 (38.1%) of patients. The most commonly used treatment regimen in this series was a combination of prednisolone and methotrexate. During the median follow-up of 3.1 years (range, 0.0-18.5 years), only one-third of patients were seen to have monocyclic disease. Extraskeletal osteosarcoma at a previous lesion of calcinosis cutis was observed in one patient at 12 years after juvenile dermatomyositis onset. Limitations: This was a retrospective single-center study, and our results may not be generalizable to other healthcare settings. Prospective multicenter studies are needed to confirm the findings of this study. Conclusion: juvenile dermatomyositis usually poses a diagnostic and therapeutic challenge, which can be compounded by the ethnic variations in the clinical presentation, as observed in this study. Asian patients tend to present with acute or subacute onset of disease, and arthralgia and/or arthritis are less common than in Caucasian patients. Scalp dermatitis is not uncommon in pediatric juvenile dermatomyositis patients. An association between juvenile dermatomyositis and malignancy, though rare, can occur

Comparative identification of protein profiles and major allergens of saliva, salivary gland and whole body extracts of mosquito species in Thailand.

Allergic reactions to mosquito bites, such as generalized urticaria or severe local reactions are common problems worldwide. The diverse sources of allergen prepared from different mosquito body parts usage are a major obstacle to obtaining safe and effective tests and immunotherapy for mosquito bite allergy. Thus, the reactions are often not recognized and allergen immunotherapy is seldom used for severe reaction to mosquito bites. In a search for appropriate allergen sources, the protein profiles of saliva, salivary glands and whole body extracts were comparatively analyzed from 4 common mosquito species of Thailand and/or South East Asia; viz. Culex quinquefasciatus, Aedes aegypti, Aedes albopictus and a zoophilic strain, Anopheles minimus. The major allergens in the extracts which elicited specific IgE responses in the pooled sera of subjects allergic to mosquito bites were identified. It was concluded that mosquito saliva was the best source of allergens. Additionally, both species-specific and species-shared allergens of the 4 mosquito species were identified. The major saliva allergens having MWs of 36, 32 and 22 kDa were identified. The identificstion of major allergens should facilitate the production of specific recombinant allergens and contribute to improvement in the diagnosis and specific immunotherapy of Thai mosquito bite allergy patients.

Comparison of topical methylprednisolone 1% versus placebo in stevens – johnson syndrome and/or toxic epidermal necrolysis.

Objective: To study the efficacy of topical methylprednisolone compared with placebo in patients with Stevens-Johnson syndrome (SJS) and/or toxic epidermal necrolysis (TEN). Methods: Thirty-six patients were prospectively studied at Siriraj Hospital. The clinical severity score for ocular involvement was classified as mild, moderate and severe. In 12 patients with moderate severity, 1% methylprednisolone eye drop was added four times daily in one eye and 0.5% normal saline as placebo in another eye for one week. In 2 severe cases, these medications were added every 2 hours till bedtime for one week and four times daily for another week. Results: The average age of 14 patients was 50 ± 16.2 years (± SD), with female preponderance (12). Common precipitating factors were carbamazepine, nevirapine, phenytoin and dimenhydrinate. The common associated diseases were HIV. Five patients were not given systemic corticosteroid due to infection, and minimal skin involvement. The total severity score change in each group was the same at one month. In an average of 7.6 months follow up, most patients recovered normal appearance except for two eyes of the placebo group and one eye of the methylprednisolone group. The percentage of dry eye in the Schirmer test and the rose bengal score in the topical methylprednisolone eyes were not significantly less than in the control eyes. However, less tear production was statistically significant in patients with systemic corticosteroid. Conclusion: Topical corticosteroid might be used cautiously in early stages of SJS or TEN.

Efficacy and safety of tacrolimus ointment in pediatric Patients with moderate to severe atopic dermatitis.

BACKGROUND: Atopic dermatitis (AD) is an immunological skin disease. It is common in pediatric populations and often requires topical steroid treatment. Moderate to severe AD may not respond to topical steroids. They often require systemic steroids, which may result in growth retardation. Protopic, a non-steroid, tacrolimus based ointment which is a calcinurin inhibitor has been proved to be effective in caucacian with AD. OBJECTIVE: To evaluate safety and efficacy of 0.03% tacrolimus ointment (Protopic&) in moderate to severe AD in pediatric patients age 2-12 years. MATERIAL AND METHOD: This was a one month multicenter open-label clinical trial using tacrolimus ointment twice daily in 61 subjects with moderate to severe AD from September to December 2004. Efficacy assessments were measured by Physician's Global Evaluation of Clinical Response (PhGECR), Eczema area and Severity Index (EASI), Patient's Global Evaluation of Clinical Response (PaGECR), and Quality of Life (QOL). Safety assessment was measured by incidence rate of adverse events. RESULTS: Fifty-eight patients completed the studies. Twenty-two patients were male; thirty-nine patients were female. Twenty-nine patients had moderate AD. Thirty-two patients had severe AD. Three cases had discontinued treatment at the third week due to increase in severity. Over all PhGECR were significantly increased, 94% showed moderate improvement in PhGECR at week 4 or end of treatment (EOT)and 83% had better improvement in PaGECR at EOT Within 7 days, tacrolimus demonstrated rapid onset in reduction of EASI score and itch in patients. Mean QOL were significantly decreased at the end of the present study. Incidence of adverse events included application site burning (21%), itching (17%), pruritus (9%), infections(3%), and erythema and folliculitis (2%). Burning sensation, erythema, pruritus and itching were resolved after the first week. CONCLUSION: Topical tacrolimus ointment is effective and safe in moderate to severe AD. It significantly improved PhGECR, EASI, PaGECR, and QOL in pediatric patients after the first week of treatment and continued through the end of the study. The major adverse events were burning, itching, and pruritus, which were resolved within the first week of therapy.

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.

Histopathologic aid to diagnosis of sarcoidosis: report of 8 cases.

Sarcoidosis is a multisystemic disease of unknown etiology. The disease is common in blacks and is very rare in Thailand. It presents as one of the most variable manifestations usually affecting the lungs and intrathoracic lymph nodes. Other organs such as liver, spleen, joints and eyes including skin are also involved The common cutaneous lesions are maculopapular, erythematous plaque, subcutaneous nodule, scar and lupus pernio. No reliable indicator is useful for diagnosis except the histopathologic change which is the only way for approaching this disease. Sarcoidosis is the disease of exclusion. Various infections producing granulomas should be excluded histologically. The ultimate diagnosis requires clinical correlation, laboratory investigations, chest X-ray as well as available tissue culture. Herein, the authors reported eight cases of sarcoidosis by retrospective study primarily diagnosed by histopathological findings at Siriraj Hospital from January, 1997 to December, 2004 with many different clinical presentations. Despite the diverse clinical pictures, interestingly, the presented patients almost had the same histopathologic findings as small, uniform, discrete naked granulomas usually without necrosis. These findings act as a hallmark for diagnosis of this disease.

Treatment of tinea capitis caused by Microsporum ferrugineum with itraconazole.

A prospective, non-randomized, open clinical trial was conducted to determine the efficacy of itraconazole for treatment of Microsporum ferrugineum tinea capitis. Itraconazole capsules were given every day in continuous group and every day for 1 week on and 3-week off in pulse therapy group. Concomitant topical therapy with 2% ketoconazole shampoo was used daily. Clinical evaluation consisted of assessing the degree of hair loss, scaling, erythema, pustule, and crust. In both groups, the treatment was stopped when the clinical signs of inflammation had resolved and the mycological examination had become negative or at week 12. There were 81 patients consisted of 49 boys and 32 girls enrolled and average dose of itraconazole was 4.5 mg/kg/day. During the 16-week study period (with 4-week follow-up visit) the overall clinical severity score decreased every visit (p < 0.001). The improvement of the scores showed no statistical difference between both groups. The cumulative cure rate using combined clinical and mycological cure at week 16 in patients treated with continuous and pulse regimen was 54.3% (19/35) and 37.0% (17/46), respectively. The cumulative percentage of all cure rates including clinical cure, mycological cure and combined clinical and mycological cure of the continuous group was significantly higher than in the pulse therapy group (p < 0.001). The superior efficacy of the continuous therapy group was observed after week 8. The cumulative cure rate increased with the longer treatment duration but decreased with the larger infected area involvement (p = 0.001). All patients who were not cured showed improvement. There was no significant adverse effect. The higher dosage or the longer treatment duration of itraconazole may be required for treatment of tinea capitis from M. ferrugineum to achieve more cure rate.

Diagnostic criteria for atopic dermatitis in Thai children.

Atopic dermatitis (AD) is a common skin disease in Thai children. There is no clinical or laboratory gold standard for the diagnosis. It is generally based on the guideline proposed by Hanifin and Rajka. Many studies have shown that some criteria are probably not all that significant in making the diagnosis. This study was designed to evaluate the frequency and diagnostic significance of clinical features of AD in Thai children. The authors studied 108 patients with AD and 103 controls including patients with other skin diseases. The AD group consisted of 60 girls and 48 boys. The mean age was 60.3+/-36.1 months. All previously proposed features were evaluated and the difference infrequency was tested with the chi-square test. History of pruritus, rash on typical distribution, chronically relapsing course, duration more than 6 months, personal or family history of atopy, age of onset before 2 years, recurrent conjunctivitis, itch when sweating, intolerance to rough textile, food and milk intolerance, history of dry skin, seasonal variation, visible dermatitis, dermatitis of a typical distribution, xerosis, ichthyosis vulgaris, foot dermatitis, Dennie-Morgan infraorbital fold, orbital darkening, periorbital dermatitis, pityriasis alba, peri-auricular dermatitis, anterior neck fold, truncal dermatitis, perifollicular accentuation, white dermographism and diffuse scaling of scalp were all significantly more frequent in AD (p < 0.05). A minimum set of diagnostic criteria for AD was derived by using multiple stepwise logistic regression technique. It consisted of history of itchy rash, history of flexural dermatitis, chronicity more than 6 months, and visible xerosis, periorbital dermatitis and perifollicular accentuation.

Management of severe atopic dermatitis with thymostimulin.

Two patients with severe atopic dermatitis unresponsive to conventional therapy were enrolled in a clinical trial on thymostimulin (TP-1). TP-1 was administered by subcutaneous injection 1 mg/kg/day for 14 days and then 1 mg/kg/day on alternate days for 2 months. Clinical and immunological status were evaluated at baseline and at regular intervals during the treatment. Clinical severity scores included eight skin conditions (erythema, edema, vesicle, crust, excoriation, scaling, lichenification, pigmentation), two subjective components (itchiness and loss of sleep), and extent of area affected. There was a statistically significant improvement in the overall assessment of the severity scores. There were no definite changes in immunological parameters including CD4, CD8 T-cell subpopulations and serum IgE, but eosinophil count showed a mark decrease in one case. No serious side effects were observed.

Therapeutic efficacy and safety of loratadine syrup in childhood atopic dermatitis treated with mometasone furoate 0.1 per cent cream.

Atopic dermatitis is a common skin disease in Thai children. The treatment of atopic dermatitis requires topical corticosteroids, emollients, systemic antihistamine as well as avoidance of the precipitating factors. A double blind multicenter placebo controlled study was conducted to assess the therapeutic efficacy of topical mometasone furoate 0.1 per cent cream in combination with loratadine syrup. Forty-eight patients, 23 boys and 25 girls, mean age 73.67 months, with atopic dermatitis were included in the study. The severity of the disease was measured by using the SCORAD index including the degree of erythema, dryness, edema/papulation, oozing/crusting, lichenification, and excoriation. Total area involved was measured and a target area of dermatitis was selected for specific evaluation. The degree of clinical signs and pruritic symptom was graded. The sensation of pruritus, disturbance of sleep due to pruritus, and feeling of sleepiness in the morning were recorded. Mometasone furoate 0.1 per cent cream was applied to all patients once daily. One group received loratadine syrup and another group received placebo syrup. They were followed-up on day 5, 8 and 15. The severity of atopic dermatitis and pruritus significantly decreased after 14 days of treatment in both groups (p < 0.001). There was no difference in therapeutic response between the loratadine and placebo groups (p = 0.99). All signs examined had decreased by the end of the study. The result demonstrated that 0.1 per cent mometasone therapy is very effective for treating childhood atopic dermatitis. Loratadine did not show beneficial effect when combined with good topical corticosteroid but it was safe and had no serious side effect on the children.