Iranian guideline for rituximab therapy in pemphigus patients.

Pemphigus vulgaris (PV) is an autoimmune blistering disease affecting the skin and/or mucosa. Rituximab (RTX) has been approved recently by US FDA as an effective and safe treatment of PV. The high incidence of PV in Iran encouraged our team to prepare a consensus guideline for RTX administration based on literature review and a decade experience of an expert panel. RTX is recommended for the treatment of new cases of PV as well as patients not responding to conventional therapy. Contraindications include history of anaphylaxis or IgE-mediated hypersensitivity to murine proteins of RTX, severe active infections, pregnancy, breastfeeding, severe heart failure, and arrhythmia. Prophylactic antiviral therapy is recommended in patients at risk of reactivation of HBV and isoniazid for those at risk of reactivation of tuberculosis. Concomitant use of systemic corticosteroids is recommended as a rule. Except for methotrexate, the combination with other immunosuppressive drugs is discouraged. Intravenous immunoglobulin is recommended for those at risk of infections or with extensive disease. The recommended dosage of RTX for the first cycle is 2 g either 500 mg weekly or 1 g biweekly. There is no general consensus whether the next doses of RTX be administered upon relapse or as maintenance therapy. We strongly recommend RTX sooner in the course of pemphigus.

Behcet's disease in Iran: Analysis of 7641 cases.

Objective: To analyze Behcet's Disease (BD) in Iran, from 1975 to 2018, and compare to 35 large/small reports from other countries.Methods: Patients from all over Iran, when suspected, were sent to the BD Unit. The diagnosis was done by expert opinion. All data were recorded in the BD registry (updated in each follow-up). The data are given in percentage with 95% confidence Intervals.Results: The mean age at onset was 25.6 years. Standard deviation (SD) was 9.8. The mean disease duration was 11.7 years (SD: 8.9). Males were 55.8% (54.7-56.9), Females 44.2% (43.1-45.3), Oral Aphthosis (OA) 97.5% (97.1-97.9), genital aphthosis (GA) 64.4% (63.3-65.5), skin lesions 62.2% (61.1-63.3), ocular lesions 55.6% (54.5-56.7), Joint Manifestations 38.1% (37.0-39.2), Gastrointestinal 6.8% (6.2-7.4), Vascular 8.9% (8.3-9.5), neurological (central-peripheral) 3.9% (3.5-4.3), epididymitis 4.6% (4.1-5.1). Lab tests were positive pathergy test 50.4% (49.3-51.5), elevated ESR 51.1% (50.0-52.2), abnormal urinalysis 13.4% (12.6-14.2). The International Study Group (ISG, 1990) criteria and the International Criteria for Behcet's Disease (ICBD, 2014) had respectively a sensitivity of 76.2% (75.2-77.2) and 96.6% (96.2-97.0). The specificity was 99.3% (99.1-99.5) and 97.3% (96.9-97.7). The accuracy was 86.4% (85.8-87.0) and 96.9% (96.6-97.2).Conclusion: The most frequent manifestations were OA, GA, skin manifestations, and ocular manifestations.

Paediatric Behçet's disease in Iran: report of 204 cases.

OBJECTIVES: This study proposed to report the characteristics of paediatric Behçet's disease (PED-BD) in a cohort of patients from Iran's registry and compare them with different reports throughout the world. METHODS: From a cohort of 7504 Iranian patients with Behçet's disease those diagnosed before the age of 16 years were included in this study. Data were collected on a standard protocol comprising 105 items, including demographic features, type of presentation, and different clinical and laboratory findings. RESULTS: PED-BD was seen in 2.7% of patients. The male/female ratio was 1.02/1, and the mean age at onset was 10.5±3.4. Positive familial history was present in 9.9%. As a first manifestation, oral aphthosis was the most frequent (75%) followed by ocular lesions in 19.1%. The prevalence rates of various manifestations were as follows: oral aphthosis: 91.7%; genital ulcer: 42.2%; skin: 51.5% (pseudofolliculitis: 43.1%, erythema nodosum: 10.3%); ocular lesions: 66.2% (anterior uveitis 52%, posterior uveitis 58.3%, retinal vasculitis 39.7%); articular manifestations: 30.9%; neurological involvement: 4.9%; vascular involvement: 6.4% (venous 4.9%, arterial 2.5%); gastrointestinal manifestations: 5.9%; epididymo-orchitis: 8.7% (boys); high ESR (≥20): 50.8%; abnormal urine: 14.1%; positive pathergy test: 57%; HLA-B5/51: 48.7%. ICBD criteria have the highest sensitivity for the classification of PED-BD patients in Iran (91.7%). CONCLUSIONS: The clinical spectrum of PED-BD in Iran in this study was similar to that of other reports; however, genital ulcers, skin lesions (notably erythema nodosum), and gastrointestinal involvement were noticed to occur less frequently, while ocular lesions were more frequent and more severe compared to other reports.

Evaluation of Autoimmune Bullous Diseases in Elderly Patients in Iran: A 10-Year Retrospective Study.

Autoimmune bullous diseases (ABDs) are uncommon but significant skin disorders with relatively high morbidity and mortality. Some surveys have been carried out to describe the spectrum of ABDs in a region, but this is the first that has focused on ABDs in elderly patients. This study was conducted to determine the clinicoepidemiologic features of ABDs in elderly patients. Medical records of all ABD patients with disease onset after the age of 60 years who presented to the Autoimmune Bullous Diseases Research Center, Tehran, Iran between April 2003 and March 2013 were reviewed. Patients with dermatitis herpetiformis were not included. During the 10-year period studied, 296 patients with ABD and disease onset after 60 years of age were diagnosed. Bullous pemphigoid (BP) was observed to be the most common ABD (48.3%), followed by pemphigus vulgaris (45.3%), pemphigus foliaceus (3.7%), mucous membrane pemphigoid (1.4%), paraneoplastic pemphigus (0.7%), epidermolysis bullosa acquisita (0.3%), and linear IgA bullous disease (0.3%). A predominance in women was observed for total ABDs, BP, and pemphigus vulgaris. Although Iran is known to have a high prevalence of pemphigus, BP is the most frequent ABD among elderly patients in Iran, highlighting the importance of the clinical diagnosis of BP in elderly patients.

Immunologic prediction of relapse in patients with pemphigus vulgaris (PV) in clinical remission.

BACKGROUND: Pemphigus vulgaris (PV) is characterized by multiple relapses, occurring especially in patients on minimal therapy or off therapy. OBJECTIVE: To identify immunologic predictors (anti-desmoglein [Dsg] 1 and 3 antibodies; direct immunofluorescence [DIF]) for relapse in PV patients. METHODS: Eighty-nine patients in complete clinical remission for at least 6 months and receiving less than or equal to 10 mg prednisolone daily and no immunosuppressive drugs were evaluated using DIF (n=89) and Dsg ELISA (n=46). They were followed until relapse or for at least 18 months. RESULTS: DIF was positive in 44 of 89 patients (49.5%); anti-Dsg 3 antibodies were detected in 18 of 46 patients (39.1%) and anti-Dsg 1 antibodies were detected in 4 of 46 patients (8.7%). Relapse occurred in 38 patients (42.7%). Mean relapse-free time was significantly shorter in anti-Dsg 3-positive patients compared to anti-Dsg 3- negative patients (P = .015) and in DIF-positive patients compared to DIF-negative patients (P = .047), but not in anti-Dsg 1- positive patients compared to anti-Dsg 1-negative patients (P = .501). Sensitivity and predictive values of neither of these tests were high. LIMITATIONS: Small number of anti-Dsg 1-positive patients and use of conventional ELISA. CONCLUSION: Positive anti-Dsg 3 ELISA and, to a lesser degree, positive DIF are predictors of relapse in PV patients in clinical remission. Decision on discontinuing treatment should be based on the results of these tests as well as on clinical findings.

Temporal course of avascular femoral head necrosis in patients with pemphigus vulgaris.

BACKGROUND AND OBJECTIVES: Pemphigus vulgaris (PV) is typically treated with systemic corticosteroids and immunosuppressive agents. Avascular necrosis (AVN) of the femoral head is a well-recognized major complication of corticosteroid therapy. The characteristics of this serious complication in PV remain unknown. PATIENTS AND METHODS: Uncontrolled, retrospective study of all PV-related AVN cases diagnosed at an Iranian autoimmune bullous disease clinic between 1985 and 2013. RESULTS: Of the 2,321 medical records of PV patients reviewed, 45 (1.93 %) cases showed femoral AVN, with 30 (66.7 %) individuals being male. The mean age at diagnosis of AVN was 47.4 ± 14.2 years. The mean interval between the diagnosis of PV and the onset of AVN was 25.3 ± 18.3 months. With the exception of eight cases (17.8 %), the majority of patients developed AVN within three years after the diagnosis of PV. The mean cumulative dose of prednisolone in patients with AVN was 13,115.8 ± 7041.1 mg. There was a strong correlation between the total prednisolone dose and the time of onset of AVN (p = 0.001). In patients with a history of alendronate intake, that interval was significantly shorter (p = 0.01). CONCLUSIONS: Occurring in about 2 % of patients, AVN is a serious complication of corticosteroid treatment in patients with PV, predominantly in the first three years of treatment. In individuals receiving higher doses of prednisolone, AVN tends to occur earlier.

Zeitlicher Verlauf der avaskulären Nekrose des Hüftkopfes bei Patienten mit Pemphigus vulgaris.

HINTERGRUND UND ZIELE: Pemphigus vulgaris (PV) wird in der Regel mit systemischen Corticosteroiden und Immunsuppressiva behandelt. Avaskuläre Nekrose (AVN) des Hüftkopfes ist eine gut bekannte schwerere Komplikation einer Corticosteroid-Therapie. Die Charakteristika dieser schweren Komplikation bei PV sind nach wie vor unbekannt. PATIENTEN UND METHODEN: Nicht kontrollierte, retrospektive Untersuchung aller PV-bedingten AVN-Fälle, die in einer iranischen Klinik für bullöse Autoimmunerkrankungen zwischen 1985 und 2013 diagnostiziert wurden. ERGEBNISSE: Anhand der Krankenakten von 2321 untersuchten PV-Patienten wurden 45 Fälle (1,93 %) von femoraler AVN identifiziert. Dreißig davon waren Männer. Das mittlere Alter bei der Diagnose der AVN betrug 47,4 ± 14,2 Jahre. Der mittlere Zeitraum zwischen der Diagnose des PV und dem Einsetzen der AVN lag bei 25,3 ± 18,3 Monaten. Mit Ausnahme von acht Fällen (17,8 %) setzte die AVN bei der Mehrheit der Patienten innerhalb von drei Jahren nach Diagnose des PV ein. Die mittlere kumulative Dosis von Prednisolon bei Patienten mit AVN betrug 13.115,8 ± 7041,1 mg. Zwischen der Prednisolon-Gesamtdosis und dem Zeitraum bis zum Einsetzen der AVN bestand eine starke Korrelation (p = 0,001). Bei Patienten mit Alendronateinnahme in der Vorgeschichte war dieser Zeitraum signifikant kürzer (p = 0,01). SCHLUSSFOLGERUNGEN: Die AVN ist eine schwere Komplikation einer Corticosteroid-Behandlung bei Patienten mit PV. Sie wird bei 2 % der Patienten beobachtet und tritt vor allem in den ersten drei Behandlungsjahren auf. Bei Patienten, die höhere Dosen von Prednisolon erhalten, setzt die AVN tendenziell früher ein.

Trauma-induced pemphigus: a case series of 36 patients.

BACKGROUND AND OBJECTIVES: Pemphigus is a group of autoimmune diseases characterized by intraepidermal acantholytic blisters. Isomorphic responses, or Koebner phenomenon (KP), defined as the appearance of typical lesions of a disease following trauma are rarely reported in pemphigus. Our aim was to present patients who developed new pemphigus lesions as a result of skin trauma. PATIENTS AND METHODS: The medical files of pemphigus patients from the Autoimmune Bullous Diseases Research Center, who had a history of trauma before the onset or flare of their disease, between 1999 and 2013 were reviewed. RESULTS: Thirty-six pemphigus vulgaris (PV) patients had a history of trauma. Thirteen patients developed new-onset PV and the other 23 had previously been diagnosed with PV. Pemphigus lesions developed most often following major surgeries including abdominal, orthopedic, and chest surgeries as well as dental procedures, blunt physical trauma, and skin surgeries. Moreover, post-cataract laser surgery, burns, radiation therapy, and physiotherapy were also shown to induce pemphigus. Mean time between trauma and lesions was 4.7 weeks for recurrent PV and 15.0 weeks for new-onset PV. CONCLUSIONS: Unnecessary surgery and blunt trauma should be avoided in pemphigus patients. Furthermore, posttraumatic pemphigus should be suspected in poorly healing surgical wounds and confirmatory biopsies are mandatory.

Lipoid proteinosis: phenotypic heterogeneity in Iranian families with c.507delT mutation in ECM1.

Lipoid proteinosis (LP) is a rare autosomal recessive genodermatosis caused by loss-of-function mutations in the ECM1 gene, and previous studies have noted phenotypic variability. In this study, we examined 12 patients representing three Iranian families for clinical manifestations and genotyped them for mutations in ECM1. LP was diagnosed with characteristic mucocutaneous and neurologic manifestations. Five patients were also subjected to magnetic resonance imaging (MRI)/computed tomography (CT) scan of the central nervous system. DNA was isolated from peripheral blood from patients and their clinically unaffected relatives, and mutations in ECM1 were sought by PCR-based amplification of all exons and flanking intronic sequences, followed by bidirectional Sanger sequencing. Significant phenotypic variability in this multisystem disorder, including presence of convulsions and epilepsy in about half of the patients was noted. In most cases, this was associated with calcifications in the brain detected by MRI/CT scans. Genotyping of the affected individuals in three families from the central region of Iran revealed presence of homozygous c.507delT mutation in ECM1, reflecting the observed consanguinity in these families. This large cohort revealed extensive phenotypic variability in individuals with the same mutation in ECM1. This observation suggests a role for genetic and epigenetic as well as environmental modulation of the phenotype. Identification of mutations allows screening of unaffected individuals for presence or absence of this mutation in extended LP families, with implications for genetic counseling.

Genital aphthosis in Behçet's disease: is it associated with less eye involvement?

Behçet's disease (BD) is a multisystem disease classified among the vasculitides with various clinical features. Genital aphthosis (GA) is one of the major manifestations of BD. The aim of this study was to evaluate the characteristics of BD patients with GA. A cross-sectional sample of BD patients registered in 37 years was selected. We determined clinical and laboratory features of BD patients with GA (GA cases) and compared them with the patients who never developed GA (non-GA cases). The comparisons were performed by the chi-square test and logistic regression analysis. Odds ratios (ORs) with 95 % confidence intervals were calculated to estimate the precision of ORs. Among 6,935 BD patients, 4,489 cases (64.7 %) were ascribed to GA cases. Male to female ratio (1.11:1.00 vs. 1.48:1.00 OR 0.753, P value <0.001) and mean age of disease onset (OR = 0.9, P value <0.001) were lower in GA subset. In GA cases, oral aphthosis (OA) was a more common onset manifestation (OR 2.250, P value <0.001), while uveitis (OR 0.140, P value <0.001) and retinal vasculitis (OR 0.077, P value <0.001) were less common at the disease onset. In the whole course of disease, eye involvement was less common in GA cases (OR 0.215, P value <0.001). On the contrary, OA (OR 19.698, P value <0.001), skin (OR 1.762, P value <0.001), joint (OR 1.257, P value = 0.001), gastrointestinal (OR 1.302, P value = 0.009), neurological (OR 1.624, P value <0.001) and vascular involvements (OR 1.362, P value <0.001), epididymitis (OR 1.596, P value <0.001), positive pathergy test (OR 1.209, P value <0.001) and positive familial history of OA (OR 1.325, P value <0.001) were more common in GA subset. This study showed that GA subset of BD is associated with less eye involvement but higher rates of other BD manifestations.

Impact of the positive pathergy test on the performance of classification/diagnosis criteria for Behcet's disease.

BACKGROUND: The only diagnostic test that currently exists for Behcet's disease (BD) is the pathergy test. A positive pathergy test (PPT) is an important component of many of the 16 sets of classification/diagnosis criteria used to diagnose BD. The aim of this study was to determine the importance of a PTT in the performance of the diagnosis/classification criteria for BD. PATIENTS AND METHODS: All patients listed in the BD registry of the Rheumatology Research Center, Tehran (6,727) and 4,648 BD controls were enrolled in the study. The diagnosis was clinical when no other diagnosis could explain the patient's manifestations. The criteria were tested with and without PPT results. Sensitivity, specificity, and accuracy were calculated. RESULTS: Without PPT, all sets of criteria lost sensitivity, gained specificity, and lost accuracy, with the exception of the Cheng-Zhang criteria. The largest loss in sensitivity was for the Hubault-Hamza (35 %) and Dilsen (17.3 %) criteria; the least was for the Curth (1.9 %) and ICBD (6.5 %) criteria. The largest gain on specificity was for the Dilsen (4.7 %) and Curth (3.1 %) criteria; the least was for the Japan (0.1 %) and Japan revised (0.1 %) criteria. The greatest loss in accuracy was for the Hubault-Hamza (20.4 %) and Dilsen revised (9.3 %) criteria; the least was for the ICBD (3.6 %), while Curth gained 0.3 %. CONCLUSION: Without PPT as a criterion for the diagnosis of BD, the sensitivity and accuracy of the sets of classification/diagnosis criteria decrease, while the specificity improves.

Fatal paraneoplastic pemphigus after removal of Castleman's disease in a child.

Paraneoplastic pemphigus (PNP) is seen most frequently in the setting of Castleman's disease (CD) in childhood. We report herein a 10-year-old girl with PNP appearing a few weeks after resection of a recurrent CD. Despite improvement in skin and mucosal lesions with prednisolone and azathioprine, she had severe bronchiolitis obliterans and died from respiratory failure a few months later.

Direct immunofluorescence of plucked hair for evaluation of immunologic remission in pemphigus vulgaris.

BACKGROUND: Negative direct immunofluorescence (DIF) is a predictor of immunologic remission in pemphigus vulgaris. Recently, it has been shown that plucked hair can be used as substrate for DIF in the diagnosis of pemphigus. OBJECTIVE: We sought to compare hair DIF in patients with pemphigus vulgaris in clinical remission with conventional DIF for the assessment of immunologic remission. METHODS: A total of 55 patients with pemphigus vulgaris fulfilling the following inclusion criteria were enrolled: absence of any lesion and daily prednisolone dosage equal or less than 10 mg without adjuvant drug in the preceding 6 months. Biopsy specimen and plucked hair were processed for DIF. Intercellular deposition of IgG and/or C3 was considered positive. RESULTS: Conventional DIF and hair DIF were positive in 28 (50.9%) and 36 (65.5%) patients, respectively. Hair DIF had a sensitivity of 0.79 (95% confidence interval [CI] 0.59-0.92), a specificity of 0.48 (95% CI 0.29-0.68), a positive predictive value of 0.61 (95% CI 0.44-0.77), and a negative predictive value of 0.68 (95% CI 0.43-0.87). LIMITATIONS: Small sample size is the main limitation of this study. CONCLUSIONS: The sensitivity of hair DIF was not high enough to allow us to suggest it as a substitute for conventional DIF. On the other hand, one cannot disregard positive cases of hair DIF in the setting of negative biopsy DIF. As hair plucking is less invasive than biopsy, the following approach could be suggested: hair DIF may be repeated in patients in clinical remission until negative; then conventional DIF should be performed, too. The physician can decide to stop treatment only when DIF assays on both substrates are negative.

Autoimmune Bullous Disease Quality of Life (ABQoL) questionnaire: Validation of the translated Persian version in pemphigus vulgaris.

BACKGROUND: Autoimmune bullous diseases are a group of rare, chronic, blistering diseases, with pemphigus vulgaris (PV) being the most common type in Iran. Skin and mucosal involvement and therapy may have a dismal impact on the quality of life of affected patients. OBJECTIVE: We aimed to assess the validity and reliability of the Farsi (Persian) version of the Autoimmune Bullous Quality of Life (ABQoL) index in Iranian patients with PV. METHODS: Forward and backward translation procedures were used to prepare the Persian version of the ABQoL questionnaire. A total of 180 patients were asked to complete the questionnaires, and 24 cases repeated the test after 2 weeks. For validity and reliability, an exploratory factor analysis was performed along with a parallel analysis to determine the number of factors. The multi-trait, multi-method matrix assessed convergent and discriminant validity. To evaluate internal reliability and reliability over time, Cronbach's alpha and intraclass correlation coefficients were reported. RESULTS: Two factors explained a total variance of 54.9% in the data. The first and second factors comprised questions 1-3, 5, 7, 9, and 12-17 (symptoms-social) and questions 4, 6, 8, 10, and 11 (mucosal-psychological), respectively. The internal reliability of the Persian version of the ABQoL index was also acceptable, with a Cronbach's alpha of .855 for total items, .918 for the first factor, and .6 for the second factor. Our results suggest an acceptable convergent and discriminant validity of the Persian version of the ABQoL questionnaire. CONCLUSION: The Persian version of ABQoL index is a valid and reliable tool to monitor quality of life in patients with PV. Further studies are needed to assess the sensitivity of this instrument to changes in different disease activity and correlation with more general tools for the measurement of quality of life.

Family impact of pemphigus disease in an Iranian population using the Family Dermatology Life Quality Index.

BACKGROUND: Pemphigus vulgaris (PV) is a rare but seriously disabling disorder of the skin and mucous membranes that can gravely impact the quality of life (QoL) of patients. OBJECTIVE: The aim of the present study was to identify how family members of patients with PV are affected by the disease. METHODS: A total of 118 patients with confirmed PV and one of their family members (caregivers) were enrolled in the study. To calculate disease severity, the Autoimmune Bullous Skin Disorder Intensity Score was used. The Persian version of the Dermatology Life Quality Index (DLQI) questionnaire was used to evaluate the QoL of patients and the Family Dermatology Life Quality Index (FDLQI) to evaluate the QoL of caregivers. RESULTS: The mean age of patients was 43.14 ±â€¯12.5 years. Ninety patients (76.3%) were female. Eighty-one patients (68.6%) had the mucocutaneous phenotype and 37 cases (31.4%) the mucosal phenotype. The DLQI score was 10.1 ±â€¯7.1 for patients. The DLQI score was higher for patients with the mucocutaneous phenotype (11.8 ±â€¯7.5) than those with the mucosal phenotype (6.4 ±â€¯4.9; p < .001). QoL was significantly affected by disease severity. FDLQI score was 13 ±â€¯7 for caregivers, and was significantly higher in older caregivers and married ones. There was a positive correlation between patients' admission frequencies and FDLQI score. FDLQI score was also significantly affected by the Autoimmune Bullous Skin Disorder Intensity Score of patients' disease severity. The QoL of patients and their caregivers showed a significant positive correlation. CONCLUSION: The QoL of patients and their families are impaired significantly, and is considerably prominent in the mucocutaneous phenotype of PV and more severe forms. LIMITATION: Pemphigus Disease Area Index (PDAI) and Autoimmune Bullous Disease QoL (ABQoL) were not used in this study.

Erosions on a prolapsed uterine in an old woman: an unusual manifestation of pemphigus vulgaris.

Vaginal involvement in pemphigus vulgaris has previously been described. In all those cases a pelvic examination was needed to explore the lesions. We describe a patient with pemphigus vulgaris who had pemphigus erosions on a prolapsed uterus (i.e., on the everted surface of vagina). The patient had widespread lesions of pemphigus in other mucosal and cutaneous sites. Biopsy, antibodies against desmoglein 1 and 3, and direct and indirect immunofluorescence were confirming. The erosions on the prolapsed uterus were resistant to treatment; other mucosal and cutaneous lesions responded rapidly to prednisolone and azathioprine. After lowering the dose of prednisolone the patient was referred to a gynecologist for a vaginal hysterectomy. This case was unique because her vaginal lesions could be easily examined and followed.

Colchicine versus placebo in Behçet's disease: randomized, double-blind, controlled crossover trial.

The effect of colchicine was evaluated in a large cohort of Behçet's disease (BD) patients and compared to placebo. In a randomized, double-blind, controlled crossover trial, 169 patients without major organ involvement were selected consecutively. They fulfilled the International Criteria for Behçet's Disease. Patients were randomly assigned to colchicine or placebo. At 4 months, they were swapped over (colchicine to placebo, placebo to colchicine) for another 4 months. The primary outcome was the overall disease activity index, the IBDDAM. The secondary outcome was the responses of the individual symptoms. A Student's paired t test was used to evaluate results within each group, and an ANOVA to check for differences between colchicine and placebo. Analysis was performed using the "intention to treat" method. For placebo, IBDDAM worsened from 3.17 to 3.63 (t = 1.750, P = 0.08). For colchicine, IBDDAM improved from 3.35 to 2.75 (t = 4.143, P < 0.0001). Oral aphthosis, genital aphthosis, pseudofolliculitis, and erythema nodosum improved significantly with colchicine but not with placebo. According to the ANOVA, the difference in IBDDAM between colchicine and placebo was highly significant (F = 14.674, P = 0.00016). The difference between the results for males and females was not significant (F = 0.181, P = 0.67). In conclusion, colchicine, but not placebo, significantly improved the overall disease activity index. The difference between the results for colchicine and placebo was also statistically significant.