Livedoid vasculopathy: Clinical course and long-term outcome in Asian patients with a review of the literature.

Livedoid vasculopathy (LV) is an uncommon, chronic, and recurrent thrombo-occlusive vascular disorder. Data specific to LV in Thai population remains scarce. This study aimed to evaluate the clinical course and treatment outcomes of LV in Thai patients, and to perform a literature review for studies that reported on anticoagulant treatment in LV. Seventy-four patients with a mean age of 37.6 ± 14.7 years were included. The female to male ratio was 5.2:1, and the median follow-up was 10.5 months. Most patients had primary LV disorder. Forty-eight patients were improved with treatments, with a median duration of 11.4 months. Combination treatments were commonly used, including anti-inflammatories, antiplatelets, and immunosuppressants. Add-on therapy with anticoagulant or psoralen plus ultraviolet-A (PUVA) led to disease improvement in a majority of the patients treated. Kaplan-Meier analysis demonstrated that 38.5%, 53.7%, and 57.9% would have disease improvement at 1, 2, and 3 years, respectively. Of 39 studies (n = 219) that reported on anticoagulant treatment in LV, anticoagulant drug was used as monotherapy in 104 patients. The mean duration of anticoagulant treatment was 7.2 ± 3.8 months, which led to disease improvement in 97 patients (93.3%). Bleeding side effect was found in 9 patients (8.7%). The highest incidence of LV was found among females aged 30 to 40 years. Combination therapy with anti-inflammatory drugs, antiplatelet drugs, and immunosuppressants led to disease improvement. The observed efficacy of add-on PUVA or anticoagulant is promising and should be further investigated. Further studies are needed to guide the development of an LV management guideline.

Chronic idiopathic urticaria: prevalence and clinical course.

The purpose of our study was to assess the prevalence and clinical course of patients with chronic idiopathic urticaria (CIU), as well as possible causes or associated findings, laboratory findings and the duration of the disease in patients with chronic urticaria (CU). We retrospectively reviewed the 450 case record forms of patients with CU and/or angioedema who attended the Department of Dermatology, Siriraj Hospital, during the period 2000-2004. Of 450 patients with CU, 337 patients (75%) were diagnosed as CIU. Forty-three patients (9.5%) had physical urticaria, while 17 patients (3.8%) had infectious causes. Other possible causes were food, thyroid diseases, atopy, drugs, dyspepsia and collagen vascular diseases. In eighty-nine percent of patients, no abnormalities were detected at the time of physical examination. The most common abnormal laboratory finding was minimal elevation of the erythrocyte sedimentary rate (42%). In 61 patients, autologous serum skin tests had been done. Fifteen patients (24.5%) had positive results i.e. autoimmune urticaria. Anti-thyroglobulin and anti-microsomal antibodies were positive in 16 % and 12% of CIU patients respectively. After 1 year from the onset of the symptoms, 34.5% of CIU patients were free of symptoms and after 1.2 years from the onset of the symptoms, 56.5% of autoimmune urticaria patients were free of symptoms. The median disease duration of CIU and autoimmune urticaria were 390 days and 450 days respectively. Our study provided an overview of CU and CIU in a large series of Thai patients, based on etiological aspects and clinical courses.

Autologous serum skin test in chronic idiopathic urticaria: prevalence, correlation and clinical implications.

Some cases of chronic idiopathic urticaria (CIU) have histamine-releasing IgG autoantibodies in their blood. This disease subgroup is called "autoimmune urticaria". To date, the autologous serum skin test (ASST) is the best in vivo clinical test for the detection of basophil histamine-releasing activity in vitro. This study aimed to find the prevalence of ASST positive cases in Thai patients with CIU, to identify factors related to the positivity of ASST and to find the clinical implications of ASST in CIU. A retrospective study was performed among 85 CIU patients who attended the Urticaria Clinic at the Department of Dermatology, Siriraj Hospital and were willing to perform ASST, from January 2002 to December 2003. Twenty-one (24.7%) patients had a positive ASST. There was no significant difference between patients with positive ASST and negative ASST as to the severity of the disease (wheal numbers, wheal size, itching scores and the extent of body involvement) as well as the duration of the disease.

Direct immunofluorescence study in Thai patients with scleroderma.

OBJECTIVE: Previous reports of direct immunofluorescence (DIF) studies of the skin biopsies in scleroderma were either negative or positive at various percentages and patterns. The present study was designed to evaluate the positive yield and pattern of DIF in Thai patients with scleroderma and its possible clinical correlation. MATERIAL AND METHOD: Twenty-two patients with localized or systemic sclerosis, who attended the Department of Dermatology, Siriraj Hospital, from 1996 to 2002, were enrolled in the present study. Skin biopsy was performed for DIF studies. RESULTS: Nine out of 22 patients were diagnosed with systemic sclerosis (SS), eleven with morphea, and two with overlapping syndrome. Fifteen of 22 patients (68%) had positive DIF findings; seven of nine (78%) patients with SS, six of eleven (55%) patients with morphea and two of two (100%) with overlapping syndrome. The common sites of deposit in SS, morphea and overlapping syndrome were dermo-epidermal junction and epidermal nuclei. The common immunoreactant deposit in all groups was IgM. There was no significant difference in the comparison of DIF findings with duration of biopsy lesion, clinical correlation, and the positive result of serum antinuclear antibody (ANA) in the three groups of patients. CONCLUSION: Positive DIF yield in the present study was higher than previous reports from Western countries. Similar to the study reported from Western country, there was no statistical significant difference in comparison of DIF findings with the duration of lesion, clinical correlation, and the positive result ofserum ANA in our three groups of patients. However; patients with SS had a tendency to give more frequently positive ENS and DEJ deposits than those with morphea.

Human cervix: an alternative substrate for detecting circulating pemphigus antibodies.

Monkey esophagus (ME) is a well-accepted substrate for diagnosing pemphigus vulgaris (PV) by indirect immunofluorescence (IIF). However, its availability is sometimes limited due to ethical concerns. This study aimed to investigate the usefulness of human cervix (HC) as a substrate in the diagnosis of PV by IIF. Initially, serum from 1 PV patient was incubated with tissues from 48 HCs. Median IIF titers on HCs that had different demographic and clinical characteristics were compared. Sera from 5 PV patients were then incubated with ME and 21 HCs. For each serum, the titer of IIF on HC that was not different from ME by more than two-fold dilutions was acceptable. Last, sera from 42 PV, 14 pemphigus foliaceous, and 62 non-pemphigus patients were used to evaluate sensitivity and specificity. The results demonstrate that differences in demographic data among HCs did not affect IIF titers. Titers obtained from ME and HC were comparable (81-100 % acceptable values). Sensitivity of HC for diagnosis of PV was better than for diagnosis of pemphigus foliaceus (90.5 and 71.4 %, respectively). Specificity for PV and PF was 96.2 %. We proposed that HC substrate can be used as an alternative substrate for diagnosis of PV by IIF.

Cutaneous leukocytoclastic vasculitis: the yield of direct immunofluorescence study.

BACKGROUND: Leukocytoclastic vasculitis (LCV) is a clinico-pathological entity. Previous direct immunofluorescence study (DIF) studies of vasculitis showed positive findings mainly in the early stage of the disease. OBJECTIVE: To study the positive yield and patterns of DIF in patients with various stages of LCV. DESIGN: One hundred patients with LCV who attended the Department of Dermatology, Siriraj Hospital from 1997 to 2000 were enrolled in the study. RESULTS: The study showed immunoreactive deposits in blood vessel walls in 76 cases (76%). Forty seven per cent of patients showed immunoreactant deposit only in superficial blood vessel walls, 3% had deposits only in deep blood vessel walls. Superficial and deep blood vessel wall deposits were seen in 26%. Dermo-epidermal deposit in addition to blood vessel wall deposit was found in 39%. The most common immunoreactive deposit was C3 (71%), followed by IgM (35%), IgA (12%) and IgG (8%) respectively. The age of the skin lesions at the time of biopsy ranged from 1 to 7 days. 82% of patients with one day old lesions showed immunoreactive deposits in the blood vessel walls and 74% of the group with lesions aged 2-7 days at the time of biopsy showed immunoreactive deposits in the blood vessel walls. CONCLUSION: The present study showed a 76% positive yield for DIF study in patients with LCV when biopsies were performed within one week of onset. There was a tendency for the percentage of positive DIF results to decline when the biopsy was performed on lesions that were more than 1 day old.

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.

Direct immunofluorescence study in patients with lichen planus.

BACKGROUND: Direct immunofluorescence (DIF) studies in patients with lichen planus (LP) show the deposition of multiple immunoglobulins (Igs) at the cytoid bodies (CBs) and fibrin at the dermoepidermal junction (DEJ). The deposition of Ig at the DEJ, as in patients with lupus erythematosus (LE), is occasionally found. For cases with no specific clinical and histologic characteristics, or with ambiguous features, DIF studies may be helpful in disease differentiation. METHODS: From 1996 to 2004, data from 72 patients with LP, diagnosed on the basis of clinical and histologic criteria at the Department of Dermatology, Siriraj Hospital, Bangkok, Thailand, were collected. The results of DIF studies were analyzed. RESULTS: Deposits at the DEJ and CBs were detected in 53% and 60% of cases, respectively. A combination of DEJ (mostly fibrin) and CB (mostly IgM) deposits was found in 38% of cases. A combination of IgM and other immunoreactant deposits, including fibrin at the CBs, was found in 56% of cases. CONCLUSIONS: This study showed that the positive yield of DIF in LP was 75%. Shaggy fibrin deposition at the DEJ, which is the single best indicator in the diagnosis of LP, was found in 56% of cases. The presence of CBs only, which is a poorer indicator than the shaggy deposition of fibrin along the DEJ, was found in 22% of cases. There were no statistically significant differences in positive DIF yield between specimens derived from glabrous skin and oral lesions (P = 0.67). Forty-four per cent of cases had immunoreactants other than fibrin deposited along the DEJ, which resembled those of LE.