Solving a diagnostic dilemma in a patient with periodic fever-When the pieces of the puzzle finally fit.

The lack of pediatric subspecialists locally prior to 5 years ago, meant that some of our patients with rare, relapsing conditions were left behind. Familial Mediterranean fever can be diagnosed clinically and supported via genetic panel studies. Although neurological symptoms can be non-specific, this system symptomatology may lead patients and carers to seek medical attention. When neurological symptoms progress, seemingly refractory to first-line treatment, or suggestive of colchicine resistance, CNS demyelination should be considered by the neurologist. Abstract: Familial Mediterranean fever (FMF) is an inherited disorder with episodic fevers accompanied by pain in the abdomen, joints, or chest. It is a clinical entity that can be confirmed with a specific genetic mutation. Neurological symptoms have not been a focal point in clinical case descriptions. We aim to present the long road to diagnosing our patient, where the diagnostic clues centered around her neurological symptoms.

Shining Light on the Unusual: A Case Report of a Rare Presentation of Abdominal Wall Erysipelas.

We present a clinical case detailing the presentation of erysipelas in a 52-year-old immunocompetent female, wherein the infection displayed an unusual localization encompassing the skin of the anterior abdominal area and breast. The patient exhibited a favorable response to medical treatment. It is paramount to underscore the significance of recognizing such cases, which demand a heightened level of clinical suspicion to facilitate swift diagnosis and effective management strategies.

Demonstrating the benefit of a cellulitis-specific patient reported outcome measure (CELLUPROM©) as part of the National Cellulitis Improvement Programme in Wales.

PURPOSE: Despite a known risk of cellulitis recurrence, the management of the wider impact and risk factors has been neglected. The innovative National Cellulitis Improvement Programme (NCIP) addresses this by providing evidence-based and individualised care to improve patient reported outcomes and reduce the risk of recurrence. The aim of this paper is to examine the longer-term impact of cellulitis and to identify a suitable and clinically relevant Patient Reported Outcome Measure (PROM). METHODS: A review of existing cellulitis-specific PROMs was undertaken, alongside literature detailing the patient-focused impact of cellulitis, to identify a suitable PROM for clinical use. A group of expert therapists and patient representatives (n = 14) shared their individual and collective experiences over a series of events to discuss and debate the impact of cellulitis and review available PROMs. CELLUPROM© is introduced with anonymised PROM data and case study information reported to establish the impact of CELLUPROM© within usual NCIP care. RESULTS: No cellulitis-specific PROMs were identified. Literature focused on the signs and symptoms of an acute episode of cellulitis, with outcome measures primarily used to evidence the impact of an intervention. An enduring physical, social and emotional impact of cellulitis was identified in this study, providing the basis for the new cellulitis-specific PROM (CELLUPROM©), which has been implemented with good effect in clinical care. CONCLUSION: This study has highlighted the lasting impact of cellulitis. Using CELLUPROM© within the risk-reduction NCIP has helped develop Value-Based Healthcare and support programme evaluation.

Current challenges in diagnosing and treating infectious skin diseases - a case series. / Choroby zakazne skóry jako wciaz aktualny problem diagnostyczny i terapeutyczny ­ przeglad nietypowych przypadków klinicznych.

Infectious skin diseases constitute a significant public health problem. Despite the systematic development of many modern diagnostic and therapeutic tools, they still pose a serious challenge for clinicians. Due to their prevalence and mild course in most cases, they are often marginalized, which can delay their diagnosis and treatment initiation. Such an approach in more clinically advanced cases can have serious consequences, sometimes leading to tragic outcomes. This work presents a series of four cases of common infectious skin diseases with an unusually atypical clinical picture: the history of a 49-year-old female patient with recurrent erysipelas of the right lower leg co-occurring with a SARS-CoV-2 infection, a 75-year-old male patient with a generalized form of herpes zoster, a 38-year-old female patient with a complicated severe course of head lice, and a 34-year-old male patient with a severe form of post-steroid mycosis. In each of these cases, difficulties in making the correct diagnosis were highlighted, even though they represent some of the most common bacterial, viral, parasitic, and fungal dermatoses. The paper discusses the risk factors for these diseases, the pathophysiology of their atypical course, the effects and challenges in the therapeutic approach conducted. Infectious skin dermatoses require aggressive treatment and should never be underestimated.

Gut microbiota's influence on erysipelas: evidence from a two-sample Mendelian randomization analysis.

Background: Previous studies have suggested a link between gut microbiota and skin diseases, including erysipelas, an inflammatory skin condition. Despite this, the precise nature of the relationship between erysipelas and gut microbiota remains unclear and subject to debate. Methods: We conducted a Mendelian Randomization (MR) analysis using publicly available summary data from genome-wide association studies (GWAS) to explore the potential causal relationship between gut microbiota and erysipelas. Instrumental variables (IVs) were identified using a comprehensive set of screening methods. We then performed MR analyses primarily using the Inverse Variance Weighted (IVW) method, complemented by alternative approaches such as MR Egger, weighted median, simple mode, and weighted mode. A series of sensitivity analyses, including Cochran's Q test, MR-Egger intercept test, Mendelian Randomization Pleiotropy RESidual Sum and Outlier (MR-PRESSO) test, and a leave-one-out test, were executed to ensure the robustness and validity of our findings. Results: We identified potential associations between erysipelas and various gut microbiota, including Alcaligenaceae (OR 1.23; 95% CI 1.06-1.43; p=0.006), Rikenellaceae (OR 0.77; 95% CI 0.67-0.90; p=0.001), and others. Notably, associations with Actinomyces, Lachnospiraceae NC2004 group, Ruminiclostridium 9, Ruminococcaceae UCG014, Odoribacter, and Actinobacteria were also observed. Sensitivity analyses confirmed the robustness of these associations. Conclusion: Our MR analysis suggests both potentially beneficial and harmful causal relationships between various gut microbiota and the incidence of erysipelas. This study provides new theoretical and empirical insights into the pathogenesis of erysipelas and underscores the potential for innovative preventive and therapeutic approaches.

Significantly reduced duration of antibiotic prescription for erysipelas subsequent to the 2019 French guidelines on skin and soft tissue infection: A before-after study.

BACKGROUND: New skin and soft tissue infections (SSTI) guidelines were published in 2019 in France, changing the recommended duration for antibiotic treatment. The objective of the present study was to assess the impact of the publication of the 2019 French guidelines on SSTIs on the duration of antibiotic prescription for erysipelas. METHODS: In a before-after study (a year before and a year after April 1st, 2019), we included all adult patients diagnosed with erysipelas in Reims University Hospital medical wards and the emergency department. We retrospectively retrieved antibiotic prescription duration in the patients' medical files. RESULTS: Among 50 patients in the "before" and 39 in the "after" group, the mean duration of antibiotic prescription was significantly shorter in the "after" group (9.4 ± 2.8 vs. 12.4 ± 3.8 days, p = 0.0001). CONCLUSIONS: A 25% decrease in the duration of antibiotic prescription for erysipelas was observed following the implementation of these guidelines, providing useful information for an antibiotic stewardship policy.

Erysipelas with preferential brain and skin involvement in a Mediterranean bottlenose dolphin Tursiops truncatus.

Infections by Erysipelothrix rhusiopathiae occur in domestic animals and cause the disease known as 'erysipelas'. The ubiquity of Erysipelothrix spp. makes infection possible in a wide range of vertebrates and invertebrates. Cetaceans are highly susceptible to erysipelas, especially those under human care. The number of cases documented in wild cetaceans is low, the pathogenesis is incompletely understood, and the full spectrum of lesions is not well defined. The possible serotypes and species of the genus that can cause disease are unknown. In October 2022, a common bottlenose dolphin Tursiops truncatus stranded in Vilassar de Mar (Catalonia) showing skin lesions consistent with 'diamond skin disease', a characteristic lesion of erysipelas shared by swine and cetaceans. Necropsy was performed following standardized procedures, and multiple samples were taken for histopathology and bacteriology. Erysipelothrix sp. grew in pure culture in many tissue samples. Genetic characterization by multi-locus sequence analysis identified the species as E. rhusiopathiae. Histologically, the main lesions were an intense suppurative vasculitis of leptomeningeal arteries and veins with abundant intramural Gram-positive bacilli and meningeal hemorrhages. Meningeal lesions were considered the cause of death. The affected skin showed moderate suppurative dermatitis. Herein we document a case of erysipelas in a Mediterranean common bottlenose dolphin with unusual lesions in the leptomeningeal vessels and marked skin tropism. To our knowledge, this is the first case of severe brain involvement in erysipelas in a cetacean. We also provide a review of available cases in wild cetaceans, to highlight the characteristics of the disease and improve future diagnosis.

Compression Therapy in Dermatology. / Terapia compresiva en Dermatología.

The benefit of lower limb compression therapy is not limited to chronic venous insufficiency or/and lymphoedema. Thanks to its anti-edema and anti-inflammatory effects, compression therapy is considered a beneficial adjuvant therapy to treat atypical wounds, inflammatory dermatoses, cellulitis, and traumatic wounds in the absence of contraindications. Strict contraindications are limited to severe peripheral arterial disease and decompensated heart failure. The variability of commercially available compression materials and systems, such as short-stretch bandages, multi-component systems, zinc oxide bandages, medical adaptive compression systems, ulcer compression stockings or medical compression stockings, facilitates the adaptation of compression therapy to the individual needs of each patient. Compared to venous leg ulcers, low pressures of 20mmHg are often sufficient to treat dermatological disorders, with higher patient tolerance and compliance.

Factors associated with cellulitis in lymphoedema of the arm - an international cross-sectional study (LIMPRINT).

BACKGROUND: Lymphoedema is a globally neglected health care problem and a common complication following breast cancer treatment. Lymphoedema is a well-known predisposing factor for cellulitis, but few have investigated the risk factors for cellulitis in this patient cohort on an international level. The aim of this study was to identify the frequency of cellulitis in patients with lymphoedema of the arm, including potential risk factors for cellulitis. METHODS: An international, multi-centre, cross-sectional study including patients with clinically assessed arm lymphoedema. The primary outcome was the incidence of cellulitis located to the arm with lymphoedema within the last 12 months, and its potential associated risk factors. The secondary outcome was life-time prevalence of cellulitis. Adults with clinically-assessed arm lymphoedema/chronic oedema (all causes) and able to give informed consent were included. End-of-life-patients or those judged as not in the patient's best interest were excluded. Both univariable and multivariable analysis were performed. RESULTS: A total of 2160 patients were included from Australia, Denmark, France, Ireland, Italy, Japan, Turkey and United Kingdom. Secondary lymphoedema was present in 98% of the patients; 95% of these were judged as related to cancer or its treatment. The lifetime prevalence of cellulitis was 22% and 1-year incidence 11%. Following multivariable analysis, factors associated with recent cellulitis were longer swelling duration and having poorly controlled lymphoedema. Compared to having lymphoedema less than 1 year, the risk increased with duration: 1-2 years (OR 2.15), 2-5 years (OR 2.86), 5-10 years (OR 3.15). Patients with well-controlled lymphoedema had a 46% lower risk of cellulitis (OR 0.54, 95% CI 0.39-0.73, p < 0.001). More advanced stages of lymphoedema were associated with cellulitis even after adjustment for swelling duration and control of swelling by logistic regression (stage II OR 5.44, stage III OR 9.13, p = 0.002), demonstrated in a subgroup analysis. CONCLUSION: Patients with advanced arm lymphoedema are at particular risk of developing cellulitis. Prevention of lymphoedema progression is crucial. The results lend towards a positive effect of having well-treated lymphoedema on the frequency of cellulitis.

Systemic Erysipelas Outbreak among Free-Ranging Bottlenose Dolphins, San Diego, California, USA, 2022.

We diagnosed fatal Erysipelothrix rhusiopathiae sepsis in 3 stranded bottlenose dolphins (Tursiops truncatus) during summer 2022, in San Diego, California, USA. The previously undetected disease in this relatively small, regional population of dolphins most likely indicates an environmental or biological change in the coastal ocean or organisms.

[Practicability of the German guidelines on skin and soft tissue infections]. / Praktikabilität der Leitlinie zu Haut- und Weichgewebeinfektionen.

The guidelines on calculated parenteral initial treatment of bacterial infections in adults from 2018 were the first German language S2k guidelines for these infections. This article summarizes the experiences with respect to their practicality in the clinical routine and the resulting supplementations and comments. In view of the many different terms for soft tissue infections, the guidelines had to first establish some definitions and diagnostic criteria. Among others, the guidelines introduced the provisional term limited phlegmons (phlegmons are usually termed cellulitis in Angloamerican literature) for the frequent initially superficial soft tissue infections with Staphylococcus aureus, which do not always extend to the fascia, in order to differentiate them from erysipelas caused by Streptoccocus, which in contrast to phlegmons always respond to penicillin. The general symptoms present in erysipela are a practical differential criterion. Somewhat more complex are the definitions and recommendations for the severe forms of phlegmon, which involve the fascia and are accompanied by necrosis, so that here the practicality of the guidelines needs to prove its worth over time. The guidelines also give recommendations how to proceed in case of alleged or confirmed hypersensitivity to beta-lactam antibiotics. Currently, relevant guidelines recommend, and it is correspondingly here elaborated, that in acute cases a beta-lactam antibiotic with side chains other than those in the suspected drug may present an alternative without prior testing. Therefore, cefazolin, that does not share any side chains with other beta-lactam antibiotics, could be administered under appropriate precautionary measures. The term cellulitis is avoided in the guidelines. Since it is used frequently, and also for non-infectious dermatoses, the various meanings are discussed and distinguished from each other.

Immunogenicity and Protection by DnaK and SpaA Recombinant Proteins Against Erysipelothrix Rhusiopathiae in a Murine Model.

BACKGROUND/AIMS: Swine erysipelas is a disease caused by Erysipelothrix rhusiopathiae, a Gram-positive bacillus, which has great economic importance because it leads to the loss of the swine herd. To control this disease, animals are immunized with a cellular vaccine of killed or attenuated E. rhusiopathiae, but even with herd vaccination, cases of swine erysipelas outbreaks have been reported in the United States, China and Japan, leading to the search for other antigenic components of the bacteria that may promote greater protection against E. rhusiopathiae. The surface protein SpaA from E. rhusiopathiae has been shown to be a candidate to constitute a subunit vaccine, since it has already been reported to induce a host immune response against the bacterium. DnaK, a hsp70 molecular chaperone, also seems to be a good candidate in the composition of a vaccine, as it has been demonstrated to be an antigenic protein of the bacteria. METHODS: This work evaluated the immunogenicity and protection induced by the E. rhusiopathiaee SpaA and DnaK recombinant proteins in a murine model, by intramuscular administration to mice with two doses of 100 µg at 21-day interval between them. The candidate proteins were tested either separately and together, compared with the commercial vaccine and the non-vaccination condition, and mice were challenged with a virulent strain of E. rhusiopathiae. Serum was collected to assess the produced antibodies and peripheral blood cells, whereas spleen and kidney tissues were assayed for E. rhusiopathiae presence by colony counting. RESULTS: A survival curve of the animals was performed, which confirmed the protection induced by the proteins. IgG antibodies increased in the animal serum inoculated with the proteins when compared to the control, and a significant delay in disease symptoms was observed. CONCLUSION: These results suggest that E. rhusiopathiae DnaK and SpaA are immunogenic in mice and interfere with the disease development.

First Serologic Survey of Erysipelothrix rhusiopathiae in Wild Boars Hunted for Private Consumption in Portugal.

Erysipelothrix rhusiopathiae is a relevant zoonotic infectious agent causing swine erysipelas (SE) in wild boar. In Portugal, there is no information on its occurrence. For this reason, this study aims to perform a first serosurvey of SE in hunted wild boars in Portugal. During the 2019/2020 hunting season, 111 sera from hunted wild boar were collected and analysed serologically in the laboratory with a commercial ELISA kit. No animals were eviscerated and examined after the hunt. The hunters took it all for private consumption. The results identified 18 animals that were exposed to SE, corresponding to a seroprevalence of 16.2% (95% CI: 19.9-24.4%). No statistical significance was observed on the effect of gender and age on seropositivity. However, wild boar hunted in Pinhel County, had five times more likely to be seropositivity (p-value < 0.05; OD = 5.4). Apart from its potential debilitating capacity and chronicity in the wild boar population, SE is also a very serious occupational zoonosis. Thus, the result of this first serosurvey in Portugal should raise awareness and alert competent national veterinary authorities and those involved in the hunting sector, especially hunters who directly handle these carcasses. Further studies should be conducted to better understand the role of wild boar as a reservoir and spillover of this disease to other animals and humans.

Erysipeloid Leishmaniasis: A Case Series.

Cutaneous leishmaniasis can present in many different clinical forms. Diagnosis of atypical forms is often delayed. It is useful to keep in mind the diagnosis of cutaneous leishmaniasis, a mimicking disease, to reduce unnecessary treatment and patient morbidity. Erysipeloid leishmaniasis should be considered when presented as long-term erysipelas-like lesions that do not respond to antibiotics. We want to present our five patients with erysipeloid leishmaniasis, one of the atypical clinical forms.

Misdiagnosis of Uncomplicated Cellulitis: a Systematic Review and Meta-analysis.

BACKGROUND: Cellulitis is a clinical diagnosis with several mimics and no gold standard diagnostic criteria. Misdiagnosis is common. This review aims to quantify the proportion of cellulitis misdiagnosis in primary or unscheduled care settings based on a second clinical assessment and describe the proportion and types of alternative diagnoses. METHODS: Electronic searches of Medline, Embase and Cochrane library (including CENTRAL) using MeSH and other subject terms identified 887 randomised and non-randomised clinical trials, and cohort studies. Included articles assessed the proportion of cellulitis misdiagnosis in primary or unscheduled care settings through a second clinical assessment up to 14 days post initial diagnosis of uncomplicated cellulitis. Studies on infants and patients with (peri-)orbital, purulent and severe or complex cellulitis were excluded. Screening and data extraction was conducted independently in pairs. Risk of bias was assessed using a modified risk of bias tool from Hoy et al. Meta-analyses were undertaken where ≥ 3 studies reported the same outcome. RESULTS: Nine studies conducted in the USA, UK and Canada, including a total of 1600 participants, were eligible for inclusion. Six studies were conducted in the inpatient setting; three were in outpatient clinics. All nine included studies provided estimates of the proportion cellulitis misdiagnosis, with a range from 19 to 83%. The mean proportion misdiagnosed was 41% (95% CI 28 to 56% for random effects model). Heterogeneity between studies was very high both statistically (I2 96%, p-value for heterogeneity < 0.001) and clinically. Of the misdiagnoses, 54% were attributed to three conditions (stasis dermatitis, eczematous dermatitis and edema/lymphedema). DISCUSSION: The proportion of cellulitis misdiagnosis when reviewed within 14 days was substantial though highly variable, with the majority attributable to three diagnoses. This highlights the need for timely clinical reassessment and system initiatives to improve diagnostic accuracy of cellulitis and its most common mimics. TRIAL REGISTRATION: Open Science Framework ( https://osf.io/9zt72 ).

[Diagnosis of severe skin infections, epidemiology and clinical semiology]. / Diagnostic des infections cutanées graves, épidémiologie et sémiologie clinique.

DIAGNOSIS OF SEVERE SKIN INFECTIONS, EPIDEMIOLOGY AND CLINICAL SEMIOLOGY. Serious skin infections are mainly represented by necrotizing soft-tissue infections (NSTI). They are rare but associated with a high mortality rate and severe long-term sequelae. Despite their relatively low incidence, most physicians may see at least one case of NSTI throughout their career. The main difficulty lies in establishing an early diagnosis with a rapid distinction of necrotizing soft tissue infections from non-necrotizing soft tissue infection. Early diagnosis and surgical management are major prognostic factors.

DIAGNOSTIC DES INFECTIONS CUTANÉES GRAVES, ÉPIDÉMIOLOGIE ET SÉMIOLOGIE CLINIQUE. Les infections cutanées graves sont essentiellement représentées par les infections nécrosantes des tissus mous, ou dermohypodermites bactériennes nécrosantes-fasciites nécrosantes (DHBN-FN). Elles sont rares mais associées à une mortalité élevée et à de lourdes séquelles à long terme. Malgré leur incidence relativement faible, la plupart des médecins peuvent être amenés à voir au moins un cas de DHBN-FN au cours de leur carrière. La principale difficulté réside dans le fait d'établir un diagnostic précoce, avec une distinction rapide du caractère nécrosant de la dermohypodermite bactérienne. La précocité du diagnostic et de la prise en charge chirurgicale sont des facteurs pronostiques majeurs.

[Differential diagnoses severe skin infections]. / Diagnostics différentiels des infections cutanées graves.

DIFFERENTIAL DIAGNOSES SEVERE SKIN INFECTIONS. The diagnosis of necrotizing soft tissue infection is a difficult clinical diagnosis, confirmed by surgical exploration and requiring urgent surgical treatment. The main differential diagnoses are non-necrotizing soft tissue infection, pyoderma gangrenosum, acute leg ischaemia, compartment syndrome and diabetic foot infection. It is important to know how to recognise these differential diagnoses because the management sometimes differs radically with, in the case of pyoderma gangrenosum, a risk of aggravation in the event of surgery.

DIAGNOSTICS DIFFÉRENTIELS DES INFECTIONS CUTANÉES GRAVES. Le diagnostic des dermohypo dermites bactériennes nécrosantes-fasciites nécrosantes (DHBN-FN) est un diagnostic clinique difficile, confirmé par l'exploration chirurgicale et qui nécessite un traitement chirurgical en urgence. Les principaux diagnostics différentiels sont les dermohypodermites bactériennes non nécrosantes (DHBNN), le pyoderma gangrenosum, l'ischémie aiguë de jambe, le syndrome des loges et l'infection du pied diabétique. Il est important de savoir reconnaître ces diagnostics différentiels car la prise en charge diffère parfois radicalement avec, dans le cas du pyoderma gangrenosum, un risque d'aggravation en cas de chirurgie.

Serotypes, Antibiotic Susceptibility, Genotypic Virulence Profiles and SpaA Variants of Erysipelothrix rhusiopathiae Strains Isolated from Pigs in Poland.

The aim of the study was phenotypic and genotypic characterization of Erysipelothrix rhusiopathiae strains isolated from diseased pigs in Poland and comparison of the SpaA (Surface protective antigen A) sequence of wild-type strains with the sequence of the R32E11 vaccine strain. The antibiotic susceptibility of the isolates was assessed using the broth microdilution method. Resistance genes, virulence genes, and serotype determinants were detected using PCR. The gyrA and spaA amplicons were sequenced to determine nonsynonymous mutations. The E. rhusiopathiae isolates (n = 14) represented serotypes 1b (42.8%), 2 (21.4%), 5 (14.3%), 6 (7.1%), 8 (7.1%), and N (7.1%). All strains were susceptible to ß-lactams, macrolides and florfenicol. One isolate showed resistance to lincosamides and tiamulin, and most strains were resistant to tetracycline and enrofloxacin. High MIC values of gentamicin, kanamycin, neomycin, trimethoprim, trimethoprim/sulfadiazine, and rifampicin were recorded for all isolates. Phenotypic resistance was correlated with the presence of the tetM, int-Tn, lasE, and lnuB genes. Resistance to enrofloxacin was due to a mutation in the gyrA gene. All strains contained the spaA gene and several other genes putatively involved in pathogenesis (nanH.1, nanH.2, intl, sub, hlyA, fbpA, ERH_1356, cpsA, algI, rspA and rspB) Seven variants of the SpaA protein were found in the tested strains, and a relationship between the structure of SpaA and the serotype was noted. E. rhusiopathiae strains occurring in pigs in Poland are diverse in terms of serotype and SpaA variant and differ antigenically from the R32E11 vaccine strain. Beta-lactam antibiotics, macrolides, or phenicols should be the first choice for treatment of swine erysipelas in Poland. However, due to the small number of tested strains, this conclusion should be approached with caution.

PREDICT-crFMF score: A novel model for predicting colchicine resistance in children with familial Mediterranean fever.

OBJECTIVES: To develop a novel scoring system to predict colchicine resistance in Familial Mediterranean fever (FMF) based on the initial features of the patients. METHODS: The medical records of patients were analyzed prior to the initiation of colchicine. After generating a predictive score in the initial cohort, it was applied to an independent cohort for external validation of effectiveness and reliability. RESULTS: Among 1418 patients with FMF, 56 (3.9%) were colchicine resistant (cr) and 1312 (96.1%) were colchicine responsive. Recurrent arthritis (4 points), protracted febrile myalgia (8 points), erysipelas-like erythema (2 points), exertional leg pain (2 points), and carrying M694V homozygous mutation (4 points) were determined as the parameters for predicting cr-FMF in the logistic regression model. The cut-off value of 9 was 87% sensitive and 82% specific to foresee the risk of cr-FMF in the receiver operating characteristic. Validation of the scoring system with an independent group (cr-FMF = 107, colchicine responsive = 1935) revealed that the cut-off value was 82% sensitive and 79% specific to identify the risk of cr-FMF. CONCLUSIONS: By constructing this reliable and predictor tool, we enunciate that predicting cr-FMF at the initiation of the disease and interfering timely before the emergence of complications will be possible.