Clinical features and course of pemphigus in Thai patients.

Background: Pemphigus is a rare, organ-specific autoimmune disease. The epidemiology and clinical course vary between reports from different countries. Objective: To evaluate clinical manifestations, investigation and clinical course of Thai patients with pemphigus. Methods: Demographic data, clinical presentations, laboratory investigations and treatment outcomes in 124 pemphigus patients who had attended the specialized autoimmune skin clinic at Siriraj Hospital during the period from January 1991 to December 2009 were retrospectively studied. Results: Of the 124 pemphigus patients, 79% were diagnosed with pemphigus vulgaris (PV) and 15.3% with pemphigus foliaceus (PF). The male to female ratio was approximately 1:2 in both groups. The mean age of onset was 45.4 years in PV patients and 57.4 years in PF patients. Oral mucosal involvement at the onset of disease was presented in 37.8% of PV patients. The sensitivity and specificity of DIF in the diagnosis of pemphigus was 97.8% and 98.3% while that of IIF was 94.7% and 98.4%. Disease control was achieved in 93.9% of PV patients and 94.7% of PF patients. Remission (off therapy) was achieved in 31.6% of patients in both groups. Conclusions: PV is the most common subtype of pemphigus in Thailand and usually affects females more than males. The disease usually occurs in the fifth decade of life and mucosal involvement is common. Immunofluorescence studies yields very high sensitivity and specificity. Corticosteroids are the mainstay of treatment. The majority of patients attain disease control and one-third of them achieve remission (off therapy).

Prevalence and clinical features of Thai patients with bullous pemphigoid.

Background: Bullous pemphigoid (BP) is a rare, subepidermal autoimmune blistering disease. Studies from different regions show discrepancies in clinical features and courses. Objectives: To reveal clinical characteristics, investigations and clinical outcomes of Thai patients with BP and to evaluate the association of BP with malignancy, diabetes mellitus and neurologic diseases. Methods: Patients diagnosed as BP who had visited the autoimmune skin clinic at Siriraj Hospital between 1991 and 2009 were retrospectively studied. Results: Fifty-eight patients were enrolled. Mean age of onset was 69.3 years. The female to male ratio was 2.7:1. Fifteen percent of the patients had mucosal involvement and 38.9% showed peripheral blood eosinophilia. The sensitivity of the direct and indirect immunofluorescence test in the diagnosis of BP was 95.7% and 73.5%, respectively. The frequency of diabetes mellitus in BP patients was significantly higher than that in the general population (p < 0.001). BP patients had a significantly higher chance of having neurologic diseases compared with other autoimmune vesiculobullous disease patients (adjusted odd ratios 4, 95% confidence interval 1.2-13.3). Disease control was achieved in 89.7% of the patients. One-year and three-year remission rate was 6.4% and 66.3%, respectively. Conclusions: BP usually occurred in the seventh and eighth decade of life and affected females more than males. BP is associated with diabetes mellitus and neurologic diseases. Corticosteroids are the mainstay of the treatment. Two-thirds of patients are likely to be in remission within three years.

Diagnostic significance of colloid body deposition in direct immunofluorescence.

Background: Colloid bodies (CB) in direct immunofluorescence (DIF) studies are usually found in interface dermatitis. Furthermore, CB can be found in various skin diseases and even in normal skin. Aim: To evaluate the diagnostic value of CB deposits in DIF studies. Methods: From 1996-2007, data from 502 patients where DIF studies showed immunoreactants at CB were enrolled. The definite diagnoses of these patients were based on clinical, histopathological and immunofluorescent findings. The results of DIF studies were analyzed. Results: Immunoreactants at CB were detected in 44.4%, 43.8%, 4.2%, 3.8%, and 2.2% of interface dermatitis, vasculitis, autoimmune vesiculobullous disease, panniculitis, and scleroderma/morphea, respectively. The most common immunoreactant deposit of all diseases was Immunoglobulin M (IgM). Brighter intensity and higher quantity of CB was detected frequently in the group with interface dermatitis. Conclusions: Immunoreactant deposits at CB alone can be found in various diseases but a strong intensity and high quantity favor the diagnosis of interface dermatitis. CB plus dermoepidermal junction (DEJ) deposits are more common in interface dermatitis than any other disease. Between lichen planus (LP) and discoid lupus erythematosus (DLE), CB alone is more common in LP; whereas, CB plus DEJ and superficial blood vessel (SBV) is more common in DLE. The most common pattern in both diseases is CB plus DEJ. The quantity and intensity of CB in LP is higher than in DLE.

Role of omalizumab in a patient with hyper-IgE syndrome and review dermatologic manifestations.

Hyper-IgE syndrome (HIES) is a rare idiopathic primary immunodeficiency. It is characterized by a triad of findings, including high levels of serum IgE, recurrent skin abscesses and pneumonia and leads to pneuma-tocele formation. The diagnosis of HIES is complicated by a diversity of clinical and immunological spectrums and a heterogeneous set of genetic defects. The National Institute of Health (NIH) developed a scoring system for HIES in which a score greater than 14 indicates a probable diagnosis. Our patient presented with recurrent multiple ab-scesses on her scalp, recalcitrant eczema, candida onychomycosis, alopecia universalis, and highly elevated levels of serum IgE. Using the NIH scoring system, a 30 total-point score in this patient indicated the likelihood of carrying the HIES genotype. To our knowledge, there are no specific treatments of HIES. The humanized recombinant mo-noclonal antibody against IgE, subcutaneous omalizumab, was successfully used in this patient.

Adult-onset atopic dermatitis: a cross-sectional study of natural history and clinical manifestation.

The previously reported prevalence of adult-onset atopic dermatitis (AD) varied from 13% to 47%. There were a few reports of clinical features of adult-onset AD. The purpose of this article was to study the clinical features of Thai patients with adult-onset AD. We recruited prospective patients from the outpatient Department of Dermatology, Siriraj hospital, Mahidol University in Bangkok, Thailand, from June, 2006 to May, 2007. The diagnosis of AD was made according to the criteria of Hanifin and Rajka and the severity of AD in each patient was assessed using the Rajka and Langeland score. Fifty nine patients were enrolled. The majority of the patients developed their dermatitis during their third decade. Typical lichenified/exudative lesions were found in all cases. Non-typical morphologic variants were found in 76.3%. The most common were nummular lesions. The main sites of involvement were the flexural area. The common sites of non-flexural involvement were the trunk, extensors and hands. The severity of AD was moderate in 64.4% of cases. A personal history of atopy was found in 84.7% of cases. Skin prick testing showed positive results (mostly to multiple allergens) in 25 of 29 patients (86.2%). Elevated serum total immunoglobulin E was detected in 6 of 10 patients (60%). It is concluded that adult-onset AD is not a rare but under-recognized eczematous condition.

Validation of the diagnostic criteria for atopic dermatitis in the adult Thai population.

Atopic dermatitis is a chronic inflammatory skin disorder, for which it is difficult to obtain epidemiologic findings. In a previous study, we suggested the following diagnostic criteria for atopic dermatitis in the adult Thai population: visible flexural dermatitis, a history of flexural dermatitis, a rash of more than six months duration and visible dry skin. However these criteria were not validated against physicians' diagnoses. In the present study, we validated these diagnostic criteria for atopic dermatitis in the Thai population in a clinical setting. A case-controlled study was performed on a total of 259 patients; 33 subjects with active atopic dermatitis, 26 with inactive atopic dermatitis, 100 controls presenting with an inflammatory skin disorder other than atopic dermatitis and 100 controls without any skin disease. Each patient was examined according to the above criteria. Sensitivity, specificity, relative value, positive predictive value, and negative predictive value were calculated for each individual criterion and for composite criteria. Our data confirmed that in order to achieve satisfactory sensitivity and specificity for diagnosing atopic dermatitis in Thai people older than 13 years, a patient must have a history of flexural dermatitis plus two or more of the other mentioned criteria.

Prevalence of onychomycosis in patients with autoimmune diseases.

BACKGROUND: Onychomycosis is the most common nail disorder in adults. Many studies reported a higher prevalence of onychomycosis among particular patients, such as those with diabetes, poor peripheral circulation or immunosuppression. However, studies of the prevalence of onychomycosis in autoimmune patients who carry many of these predisposing factors have been limited OBJECTIVE: Study the prevalence of onychomycosis in autoimmune compared to non-autoimmune female patients. MATERIAL AND METHOD: A cross-sectional study of the prevalence of onychomycosis in autoimmune patients and non-autoimmune female patients visiting a dermatology clinic over a period of 18 months. One hundred and sixty-five female autoimmune patients were enrolled. RESULTS: The prevalence of onychomycosis in autoimmune patients was 10.2% (95%CI 6.5%, 15.9%) compared to 6.7% (95%CI 3.8%, 11.6%), in non-autoimmune patients (p > 0.05, 2-sided). Of vesiculobullous patients, mainly presenting with pemphigus and who were mostly on immunosuppressive medication, 24% had onychomycosis [p = 0.013; OR 4.39 (95%CI 1.27, 14.89)]. CONCLUSION: Exposure to humid microenvironments was an important factor in the occurrence of onychomycosis (p < 0.05, 2-sided). However, the number of patients with each individual disease was too small to conclude a prevalence of onychomycosis in conjunction with these individual cutaneous autoimmune diseases.

Transepidermal water loss, hydration, pH and elasticity of skin in atopic dermatitis and normal Thai subjects.

In order to obtain objective data on skin functions in patients with atopic dermatitis (AD), we studied, by means of measurement of transepidermal water loss (TEWL), skin surface hydration, pH, and elasticity of dry and normal skin at the dorsum and volar aspects of the forearms in patients with atopic dermatitis compared with normal subjects. Transepidermal water loss (TEWL), skin surface hydration, pH, and elasticity function were measured using Tewameter TM 210, Corneometer CM 820, Skin-pH-meter pH 900, and Cutometer SEM 575, respectively. Forty-one subjects, twenty-five atopic dermatitis patients with dry skin, sixteen atopic dermatitis patients without dry skin, and twenty normal subjects, were recruited in the study. Considering all skin sites together, no significant differences were found between the mean values of TEWL in the same sites, the dorsum and volar forearms, of subjects in each group of patients (p = 0.717, and p = 0.981, respectively). Statistically, there were significant differences between the mean values of skin surface hydration at the dorsum and volar forearms (p = 0.019 and p = 0.019, respectively) and skin pH at the dorsum and volar forearms (p = 0.036 and p = 0.043, respectively). Regarding the elasticity function of the dorsum, immediate recovery and biological elasticity were significant differences in each group of the patients (p = 0.048 and p = 0.019, respectively), meanwhile, the elasticity function of the volar forearms, immediate recovery, elasticity index, elastic recovery index, viscoelastic ratio and relative elastic recovery were significant differences (p = 0.014, p = 0.029, p < 0.001, p < 0.001 and p < 0.001, respectively). Therefore, further well-controlled studies, investigating the skin morphology of patients with atopic dermatitis, should be pursued to provide more targeted therapies and establish an optimal standard of care for all patients with atopic dermatitis.