Comprehensive assessment of alterations in hand deformities over 11 years in patients with rheumatoid arthritis using cluster analysis and analysis of covariance.

BACKGROUND: Although drug therapy for rheumatoid arthritis (RA) has recently improved, treating patients with established disease, whose hands have three major deformities (thumb deformity, finger deformities, and ulnar drift), remains a challenge. The underlying complex pathophysiology makes understanding these deformities difficult, and comprehensive assessment methods require accumulated skill with long learning curves. We aimed to establish a simpler composite method to understand the pathophysiology of and alterations in the hand deformities of patients with RA. METHODS: We established a rheumatoid hand cohort in 2004 and clinically evaluated 134 hands (67 patients). We repeated the evaluations in 2009 (100 hands of 52 patients) and 2015 (63 hands of 37 patients) after case exclusion. Thumb deformities, finger deformities (swan-neck and boutonnière deformity), and ulnar drift were semi-quantitated and entered as parameters into a two-step cross-sectional cluster analysis for the data in 2004. The parameters in each cluster were plotted at each evaluation point. Two-way analysis of covariance was used to examine whether differences existed between evaluation points and clusters of deformity parameters. RESULTS: Five clusters most appropriately described hand deformity: (i) cluster 1, minimal deformity; (ii) cluster 2, type 1 thumb deformity; (iii) cluster 3, thumb deformity and severe boutonnière deformity; (iv) cluster 4, type 2 or 3 thumb deformity and severe ulnar drift; and (v) cluster 5, thumb deformity and severe swan-neck deformity. Clusters 1 and 2 had higher function than cluster 5, and cluster 3 had moderate function. Clusters 1-4 had similar disease duration but showed different paths of deformity progression from disease onset. Clusters 1 and 2 represented conservative deformity parameters and clusters 3, 4, and 5 represented progressive deformity parameters. Over time, thumb deformity evolved into other types of deformities and swan-neck deformity worsened significantly. CONCLUSIONS: Our comprehensive analysis identified five deformity patterns and a progressive course in the rheumatoid hand. Knowledge of the characteristics of progressive deformity parameters may allow rheumatologists to more easily implement practical interventions and determine functional prognosis.

Atypical Chronic Inflammatory ANCA-Positive Deforming Arthritis After Cocaine-Levamisole Exposure.

BACKGROUND/OBJECTIVE: Immunostimulatory drugs including immune checkpoint inhibitors and levamisole can induce inflammatory disease including vasculitis, rashes, tissue necrosis, and arthritis. METHODS: This prospective cohort study determined the 5-year outcomes of cocaine-levamisole-induced inflammatory disease as to outcomes and survival. Thirty-one consecutive cocaine-levamisole autoimmune patients and 45 primary vasculitis patients were characterized as to clinical differentiating features, antineutrophil cytoplasmic antibody (ANCA) status, treatment, the presence of acute and chronic arthritis, and 5-year outcome. RESULTS: Seventy-one percent (22/31) of cocaine-levamisole vasculopathy cases were ANCA positive (86% p-ANCA and 14% c-ANCA), whereas 53% (23/45) of the primary vasculitis were ANCA positive (p = 0.04). The ANCA-positive cocaine-levamisole cohort at onset were characterized by younger age (45 ± 12 vs 53 ± 14 years, p = 0.04), superficial skin necrosis (82% vs 54%, p = 0.036), depressed complement C3 (27% vs 4%, p = 0.33), antiphospholipid antibodies (50% vs 4%, p < 0.001), neutropenia (18% vs 0%, p = 0.044), and elevated antimyeloperoxidase (MPO) antibody levels (100% vs 67%, p < 0.001). Chronic cocaine-levamisole disease was characterized by severe cicatrical deformities of the face and extremities (45.5% vs 8.3%, p = 0.005). Arthralgias (71% vs 82%, p = 0.19) and acute arthritis (33% vs 32%, p = 0.25) were similar between the 2 groups. However, a substantial proportion cocaine-levamisole-induced autoimmune patients (18% vs 0%, p = 0.045) developed a chronic deforming inflammatory arthritis that was rheumatoid factor, anti-cyclic-citrillinated antibody antibody, and HLA-B27 negative, but p-ANCA-and MPO antibody positive. CONCLUSIONS: Patients exposed to cocaine-levamisole may develop serious chronic sequelae including cicatrical cutaneous and facial deformities and an atypical seronegative, p-ANCA and MPO antibody-positive, HLA-B27-negative chronic deforming inflammatory arthritis.

Mallet Finger.

This article provides an overview of issues associated with traumatic injury to the distal finger that results in extensor tendon disruption or bony avulsion at the base of the distal phalanx. Commonly referred to as mallet finger, drop finger, or baseball finger, terminal extensor tendon injuries are a common presentation to the emergency department. Providers need to be advised of evidence-based management of these extensor tendon injuries in order to prevent decreased function and permanent deformity. Current evidence supports nonoperative interventions, but injuries need to be addressed in a timely manner in order to avoid poor outcomes.

A high incidence of extensor pollicis brevis insertion into the distal phalanx in rheumatoid arthritis patients who required the surgical reconstruction for thumb boutonnière deformity.

Objectives: The aim of the current study was to investigate the pattern of extensor pollicis brevis (EPB) insertion macroscopically and histologically using cadaveric thumbs, and to compare the incidence of different insertions with that of thumb boutonnière deformity in rheumatoid arthritis (RA) patients who required surgical reconstruction.Methods: We examined 103 thumbs of 58 adult cadavers with no evidence of RA, and reviewed the surgical records of 28 thumbs of 23 RA patients who underwent surgical reconstruction for thumb boutonnière deformity. The incidence of different insertion patterns of the cadaveric thumbs and the RA thumbs were compared using the Fisher's exact test.Results: Macroscopic and histologic examination revealed that the insertion patterns of EPB could be divided into three groups: insertion into the base of the proximal phalanx (Type P1), integration of EPB into the dorsal fibrocartilage of the MCP joint (Type P2), and insertion into the distal phalanx (Type D). The incidence of Type D was significantly higher in RA patients with thumb boutonnière deformity (64%) than that in the non-RA cadavers (29%; P < .05).Conclusion: EPB is inserted into the distal phalanx more frequently in RA patients who require surgery for thumb boutonnière deformity than non-RA cadavers, suggesting an additional possible mechanism of this deformity.

Prevalence and characteristics associated with dactylitis in patients with early spondyloarthritis: results from the ESPeranza cohort.

OBJECTIVES: Dactylitis is a typical feature of psoriatic arthritis. However, dactylitis was included as a spondyloarthritis (SpA) feature for both (axial and peripheral) of the ASAS classification criteria, but data about its prevalence are scarce, especially in patients with a recent onset of the disease. Our objective was to determine the prevalence and characteristics associated with dactylitis in patients with early SpA. METHODS: A baseline dataset from the ESPeranza cohort was used. This programme included patients who were suspected of having SpA (age <45 years, symptoms duration of 3-24 months and with inflammatory back pain, or asymmetrical arthritis, or spinal/joint pain plus ≥1 of the SpA features). For this study, 609 patients who were diagnosed with SpA by their physician were included. Descriptive, univariable and multivariable logistic regression analyses were employed to investigate the association between the presence of dactylitis and the characteristics associated with SpA. RESULTS: Fifty-eight (9.5%) patients currently or previously had dactylitis. In the multivariable analysis, dactylitis was independently associated with peripheral arthritis (OR= 4.83; p<0.001), enthesitis (OR= 2.49; p=0.01), psoriasis (OR= 3.62; p<0.01) and the physician's visual analogue scale (OR= 0.82; p=0.01). However, 67% of the patients who had dactylitis did not have peripheral arthritis or psoriasis and 15% had predominantly axial disease. CONCLUSIONS: Dactylitis is a frequent manifestation in patients with SpA, even during the early stages of the disease. Its presence is mainly associated with peripheral manifestations and psoriasis. Nevertheless, dactylitis is not exclusive of patients with PsA or peripheral manifestations.

Thumb boutonnière deformity without rheumatoid arthritis or trauma.

Boutonnière deformity of the thumb without rheumatoid arthritis or trauma is not widely recognised. This study aimed to investigate its prevalence, relation to sex and age, and identifying factors associated with the extensor mechanism using ultrasonography. We examined 248 thumbs from 127 participants who were asymptomatic for hand disorders and had no history of hand injury. Boutonnière deformity was identified in 20 thumbs of 17 participants and was significantly more prevalent among elderly participants than among young participants. No significant differences in sex or hand dominance were noted. The deformity had a significant effect on range of motion and grip and pinch strengths. The extensor pollicis brevis (EPB) tendon was significantly narrower in affected thumbs than in non-affected thumbs. The prevalence of boutonnière deformity without rheumatoid arthritis or trauma was 13%, and the deformity was associated with advanced age and a narrow EPB tendon. LEVEL OF EVIDENCE: III.

History, Prevalence and Assessment of Limited Joint Mobility, from Stiff Hand Syndrome to Diabetic Foot Ulcer Prevention: A Narrative Review of the Literature.

BACKGROUND: Limited Joint Mobility (LJM) is a dreaded complication of Diabetes Mellitus (DM). During the last half century, LJM has been studied in patients of different age because it has been considered useful for the monitoring of a patient's condition and for the prevention of vascular disease and diabetic foot. OBJECTIVES: The main aims of this review are to describe the relationship between DM and joint mobility as well as its prevalence and assessment. We have also investigated the role of LJM in the development of diabetic foot ulcers. METHODOLOGY: An in-depth literature search was conducted to identify studies that examined the prevalence and characteristics of LJM in patients with DM of different types, age, durations and chronic complications. RESULTS: Many factors (therapy improvements, population characteristics and different evaluation methods) concur to hinder an exact assessment of the prevalence of LJM. However, it has been confirmed that LJM is widespread among patients with DM and may affect more than two-thirds of them in addition to being a major risk factor for foot ulcer. Its role in the monitoring of a patient's condition is also important for the definition of risk thresholds such as in patients with diabetic foot. The efficacy of exercise therapy for the treatment of LJM, also in patients at risk of foot ulcer, has not been discussed. CONCLUSION: Difficulties encountered in the definition of the prevalence of LJM may hinder its study and the establishment of preventive interventions. However, LJM plays a key role in the monitoring of patients, especially those at risk for ulcer.

Deforming Arthropathy in Thai Patients With Systemic Lupus Erythematosus.

OBJECTIVE: The aim of this study was to determine the prevalence, spectrum, and clinical, radiological, and serologic findings as well as hand functions among Thai systemic lupus erythematosus (SLE) patients with deforming arthropathy. METHODS: All SLE patients attending the rheumatology clinic between January and December 2012 were interviewed, with their complete history and a physical examination being taken. Those with hand deformities were invited to join the study. RESULTS: Forty (8.7%) of 458 SLE patients had deforming arthropathy, with 13 (2.8%) of them having erosive arthritis (EA group) and 27 nonerosive arthropathy (NEA group) (8 [1.8%] with Jaccoud arthropathy [JA subgroup] and 19 [4.1%] with mild deforming arthropathy [MDA subgroup]). Three of 13 EA patients (0.7% of all SLE patients) had high titer of rheumatoid factor (RF) and anti-cyclic citrullinated peptide (CCP) antibodies that might represent true overlapping between rheumatoid arthritis and SLE. There were no statistically significant differences in autoantibodies, RF, or anti-CCP between the EA and NEA groups or the JA and MDA subgroups, except for discoid rash that was seen more commonly in the MDA subgroup. Rheumatoid factor and anti-CCP were not present in the JA subgroup. Hand joint destruction and deformities were seen more commonly in the EA group and JA subgroup. The hand grip and palmar pinch strength decreased moderately, with hand functions quite well preserved in all groups. CONCLUSIONS: Deforming arthropathy was not uncommon in Thai SLE patients, but true overlapping between rheumatoid arthritis and SLE was rare. Despite significant hand joint deformities and moderately decreased hand grip and palmar pinch strength, preservation of hand functions was generally apparent.

Clinical differences between Thai systemic sclerosis patients with positive versus negative anti-topoisomerase I.

BACKGROUND: Anti-topoisomerase I antibody (ATA) carries an increased risk of systemic sclerosis (SSc) internal organ involvement. There have been no published comparisons of the clinical characteristics of patients positive and negative for ATA in Thailand, where the positive rate for ATA is higher than among Caucasians. OBJECTIVE: To define the clinical differences between SSc, positive versus negative, for ATA. METHODS: A retrospective cohort study was performed among SSc patients over 18 at Srinagarind Hospital, Khon Kaen University, Thailand, during January 2006-December 2013. SSc-overlap syndrome was excluded. RESULTS: Two hundred and ninety-four SSc patients were included (female : male 2.5 : 1). The majority (68.6%) were the diffuse cutaneous SSc subset (dcSSc). ATA was positive in 252 patients (85.7%), among whom 71.7% had dcSSc and 28.2% limited cutaneous SSc (lcSSc). Using a multivariate analysis, hand deformity had a significantly positive association with ATA (odds ratio [OR] 7.01; 95% CI 1.02-48.69), whereas being anti-centromere (ACA) positive had a negative association (OR 0.17; 95% CI 0.03-0.92). After doing a subgroup analysis of the SSc subset, the median duration of disease at time of pulmonary fibrosis detection among ATA positive dcSSc was significantly shorter than the ATA negative group (1.05 vs. 6.77 years, P = 0.01). Raynaud's phenomenon (RP) at onset was significantly more frequent in lcSSc sufferers who were ATA negative than those who were ATA positive (90.5% vs. 56.9%, P = 0.005). CONCLUSIONS: A high prevalence of ATA positivity was found among Thai SSc patients and this was associated with a high frequency of hand deformity, ACA negativity, a short duration of pulmonary fibrosis in dcSSc and a lower frequency of RP in lcSSc.

Ulnar polydactyly - an analysis of appearance and postoperative outcome.

BACKGROUND/PURPOSE: Ulnar polydactylies present with a wide variation in the size and appearance of the affected finger, ranging from small cutaneous appendages to fully formed additional fingers. Since ulnar polydactyly is rarely associated with functional disabilities, psychological and cosmetic reasons are the main rationale for surgical treatment. The aim of this study was to present the appearance, treatment regimen, and outcome of a series of children treated with ulnar polydactylies. METHODS: Data of all pediatric patients treated between 2000 and 2010 were included. For assessing long-term outcome, patients were contacted via mail and asked to complete a questionnaire concerning location and appearance of the scar as well as functional and cosmetic satisfaction (VAS 0-100). RESULTS: In that period, 32 patients (20 male, 12 female) with 53 ulnar polydactylies were treated. In 20 patients the polydactyly occurred bilaterally (63%), in 10 patients on the left side (31%), and 2 patients presented with right-sided ulnar polydactyly (6%). The vast majority of ulnar polydactylies were floating cutaneous appendages. The mean age of the patients at operation was 8.6 months (range 0 to 10 years). 20 patients (63%) responded to the postal questionnaire. Follow-up time was 4.9 years (range 2.1 to 10 years). The majority of patients reported a flat scar (n=18, 90%). Mean overall satisfaction with the postoperative result using a VAS 0-100 score was 89. CONCLUSION: We were able to report a good postoperative outcome of a series of patients suffering from ulnar polydactylies.

Current concepts in the evaluation and treatment of mallet finger injury.

The mallet finger is a frequently encountered fingertip injury that leads to extensor lag of the distal phalanx. Classification systems stratify these injuries as ranging from soft-tissue disruption of the extensor mechanism alone to those that have articular involvement and volar subluxation. The management of mallet finger injuries varies based on injury pattern and surgeon preference. These treatment options include splinting regimens, closed reduction and percutaneous pinning, and open reduction and internal fixation. Although the final goal of treatment is to establish a congruent joint, the efficacy of each treatment modality has been shown to vary.

[Erosive hand osteoarthritis]. / Arthrose digitale érosive.

Erosive hand osteoarthritis is a form of hand osteoarthritis. Phase of the normal process or separate entity? This remains debated. It is defined by radiographic erosions with a central subchondral bone collapse. It is more frequent than initially described as recently evidenced by ultrasonographic and MRI images which have shown better sensitivity to detect erosions and osteophytes than conventional radiographs. Synovitis, detectable by Doppler ultrasonography, is a common feature. The classical clinical picture is made of inflammatory flares with severe pain, joint swelling and important functional impairment. It leads to severe joint destruction, with major deformations and sometimes ankylosis with mobility restriction. It carries high consequences in terms of aesthetic discomfort, functional impairment and impact on quality of life, justifying the earliest diagnosis as possible and a tight management using, among many other options, local and general anti-inflammatory therapeutics.

Prevalence and clinical characteristics of dactylitis in spondylarthritis: a descriptive analysis of 275 patients.

OBJECTIVES: Dactylitis is a common but little studied feature of spondylarthritis (SpA). Our objective was to assess the prevalence of dactylitis among a cohort of patients with spondylarthritis in a tertiary care centre and to describe the clinical characteristics of dactylitis. METHODS: This was a prospective single centre observational study carried out in 2010. The patients included had been diagnosed as having definite SpA based on Amo's criteria. Each patient was interviewed by a physician. The data collected included prevalence of dactylitis and its clinical characteristics, effectiveness of the different treatments, and association with severe manifestations of SpA, and analysed by descriptive analysis. RESULTS: 275 consecutive SpA patients were assessed: mean age 43.2±13.5 years, mean disease duration 14.0±11.8 years, 169 (61.4%) were men. In all, 59 patients (21.5%) suffered from SpA-associated dactylitis. The localisation of dactylitis was toes in 46 patients (78.0%) and/or fingers in 25 patients (42.4%). The most frequent localisations were the second toe and the second finger. Dactylitis was the first symptom of SpA in 14 patients (5.1%), and 28.8% (n=17) of dactylitis appeared within the first 5 years of disease. Dactylitis was present in 35.1% (n=13) of patients with undifferenciated SpA and in 30.6% (n=15) of patients with psoriatic arthritis. It was significantly associated with history of peripheral arthritis or heel pain. In our population, there was no correlation between dactylitis and HLA B27 status or sex and it was not a marker of severity of disease. CONCLUSIONS: Dactylitis is a frequent manifestation in SpA (21.5%) particularly in peripheral disease and it may be the first manifestation of the disease with localisation being more frequent in the toes.

Camptodactyly: a 10-year series.

Camptodactyly is a non-traumatic, painless, non-neurogenic flexion deformity at the proximal interphalangeal joint of the little finger, which may occur in isolation or in various developmental dysmorphology syndromes. In a ten-year survey of almost 10,000 consecutive neurology outpatient referrals, using a passive case finding strategy, camptodactyly was observed with a frequency of 0.43%. All were cases of isolated camptodactyly, and all but one were asymptomatic. Camptodactyly was more often bilateral, often asymmetric, than unilateral. A family history was common, sometimes with intrafamilial heterogeneity (symmetry, degree of angulation). The pattern of inheritance was not certain, but the predominance of female cases (both directly observed and reported in families) and a paucity of father-to-daughter cases suggested the possibility of either sex-linked dominant transmission or mitochondrial DNA point mutation. There was no evidence for aminoaciduria or taurinuria, as previously reported in some cases. Hence, camptodactyly is relatively common as an incidental finding in patients referred to general neurological outpatient clinics, and thus possibly also in the general population. It is possible that camptodactyly is a heterogeneous disorder. Further studies are required to ascertain whether this is the case, and to probe further the inheritance and pathogenesis of the disorder.

Disability profile in leprosy patients' diagnoses in a rural reference leprosy centre in Ethiopia during 1999-2009.

We assessed the epidemiology of disabilities in leprosy cases treated in a rural hospital over a 10-year period. This is a retrospective data collection using leprosy registers and treatment cards in a rural private mission hospital. Over the 10-year period, 210 patients with leprosy were registered for treatment. One hundred and twenty-eight (61.5%) had disabilities (26.0% grade 1 and 35.6% grade 2): 13.5% ocular disabilities, 44.5% disabilities in hands and 44.7% foot impairment. Patients >19 years had more disabilities (66.7% versus 50.7%) (P = 0.03), especially ocular disabilities (16.7% in >20 versus 6.0% in <20 years) (P = 0.03). This study detected a high prevalence of disabilities.

Late deformities following the transfer of the flexor carpi ulnaris to the extensor carpi radialis brevis in children with cerebral palsy.

PURPOSE: The transfer of flexor carpi ulnaris (FCU) to extensor carpi radialis brevis (ECRB) (the Green transfer) in children with cerebral palsy (CP) is a surgical option frequently used to address the typical wrist flexion deformity that is often present in these patients. We hypothesize that late deformities may occur when these transfers are performed in patients before skeletal maturity. The purpose of this investigation was to determine the frequency of these deformities and the factors that influence their development. METHODS: We performed 41 FCU to ECRB tendon transfers in children with CP at our institution between 1987 and 2005 and retrospectively reviewed them. A total of 24 patients with 25 transfers had a minimum 2-year follow-up and were included in the study population. We identified patients who developed a late deformity after tendon transfer. We analyzed medical records of these patients to identify factors associated with the development of a deformity. RESULTS: Of the 25 transfers, 12 developed a late deformity between 10 and 105 months postoperatively. The deformities that developed were extension deformities (8), supination deformities (one), and recurrent flexion deformities (3). Of the 12 patients with deformity, 9 required revision surgeries. Of these 12 patients, 9 who were less than 13 years of age at the time of transfer developed a late deformity, compared with 3 who were older than 13 years of age. CONCLUSIONS: The FCU to ECRB tendon transfer remains a viable option to address the wrist flexion deformity seen in patients with CP. Care should be taken when performing this tendon transfer in patients less than 13 years of age because they may develop a postoperative deformity, commonly an extension deformity. We believe that these deformities develop when the patient enters a growth spurt and the transferred muscle-tendon unit does not lengthen at the same rate as the involved upper extremity. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

A longitudinal follow-up of hand involvement and activities of daily living in early systemic sclerosis.

OBJECTIVE: To investigate the development of hand involvement and activities of daily life (ADL) in early systemic sclerosis (SSc), and to examine the usefulness of the Hand Mobility in Scleroderma (HAMIS) test in a longitudinal study. METHODS: Forty-three patients with disease duration no longer than 3 years and at least 4 years of follow-up formed the study group. Based on skin involvement, 31 patients were classified as limited cutaneous SSc (lcSSc) and 12 as diffuse cutaneous SSc (dcSSc). The patients were assessed regarding hand function [mobility (HAMIS), grip force (Grippit), and self-assessed hand function visual analogue scale (VAS)], vascular involvement [Raynaud's phenomenon (RP)], skin involvement [modified Rodnan skin score (mRss)], and ADL capacity [the scleroderma Functional Score (FS)]. RESULTS: Hand mobility and ADL capacity were in general good and did not change significantly. However, at the individual level 72% of the patients showed a change in HAMIS score. Grip force and perceived hand function were moderately impaired at baseline and during the follow-up. Skin involvement in the hand/arm improved significantly (p<0.001). During the observation period, the hand/arm mRss and HAMIS score changed in parallel (r(s) = 0.58, p<0.001). ADL capacity correlated significantly with grip force, self-assessed hand function, and RP at baseline, and also with HAMIS at follow-up. CONCLUSIONS: In this study hand mobility and ADL capacity were maintained during the first years of SSc. HAMIS was found to be a feasible test for longitudinal assessment of hand mobility in SSc.

[Conjugal leprosy]. / Lepra conyugal.

OBJECTIVES: Determining the occurrence of leprosy amongst couples and establishing the type of leprosy in index and secondary cases. METHODS: The characteristics of leprosy were studied between 1980 and 2000 in Atlántico Department located on the Colombian Caribbean Coast. Clinical histories of patients registered in the Leprosy Control Programme were reviewed. Clinical exams were performed and personal interviews carried out with couples suffering from leprosy. RESULTS: Twenty-six married couples were found to be suffering from leprosy, representing 5.4% of leprosy cases detected during the study period. They were aged from 20 to 89. 65.4% of the index cases were men and 22 (84.6%) were multibacillary, lepromatous cases; two had indeterminate leprosy and two were suffering from tuberculoid leprosy. Sixty-one percent of the secondary cases were paucibacillary patients, 42% of them being tuberculoid. No paucibacillary cases were associated with multibacillary leprosy in the couples. Only two couples had consanguinity. None of the couples had had leprosy at the time of being married. The time between leprosy being detected in index cases and the disease being detected in secondary patients varied from 5 to 40 years. Disabilities were more common in the feet and to a higher degree in lepromatous index cases who had had the disease for a longer time than the secondary cases because these were more frequently paucibacillary patients who had a disease having shorter evolution. CONCLUSIONS: In spite of ongoing, prolonged contact, conjugal leprosy is not frequent and requires several years to develop in the second person. Lepromatous leprosy in index cases was more frequently associated with leprosy in the couple. When the primary case was paucibacillary, no multibacillary leprosy occurred in the secondary case.

Lepra Conyugal / Conjugal leprosy

OBJETIVOS: Determinar la ocurrencia de lepra entre cónyuges y precisar las características de la enfermedad en el caso índice y en el secundario. MÉTODOS: Estudiamos sus características en una zona de la Costa Caribe colombiana (Departamento del Atlántico), durante 1980-2000, a través de la revisión de las historias clínicas del Programa Control de Lepra, del examen clínico y de entrevistas con los afectados. RESULTADOS: Encontramos 26 parejas con lepra conyugal, que representan el 5,4 % de los casos diagnosticados durante el periodo, con edades entre 20 y 89 años. El 65,4 % de los casos índices fueron hombres y 22 (84,6 %) lepromatosos; dos tenían lepra indeterminada y dos tuberculoide. Los casos secundarios fueron tuberculoides en su mayoría (42,3 %) y, en conjunto, 61 % paucibacilares. Ningún caso índice paucibacilar se asoció con lepra multibacilar en el cónyuge. Sólo dos parejas tenían consanguinidad. Ninguno de los cónyuges llegó enfermo al matrimonio y su convivencia entre la aparición de la enfermedad en el caso índice y el secundario, varió entre 5 y 40 años. La convivencia de los cónyuges varió entre 5 y 44 años. Las discapacidades se presentaron con mayor frecuencia en los pies y fueron más graves en los casos índices lepromatosos, con enfermedad de larga evolución, que en los secundarios, porque éstos fueron paucibacilares con mayor frecuencia y se detectaron cuando su enfermedad tenía menor tiempo de evolución. CONCLUSIONES: La lepra conyugal es poco frecuente, requiere varios años de convivencia para presentarse en el segundo cónyuge y es más común cuando el caso primario es lepromatoso. Cuando un cónyuge es paucibacilar su pareja no desarrollara lepra multibacilar.

Hand involvement in Behçet's disease.

AIMS: To study the clinical hand findings in Behçet's disease (BD) and to observe scintigraphic changes of these areas. METHODS: Fifty-seven randomly selected BD patients and the patients in the control group (N=40) were evaluated by two blind rheumatologists. The hands were examined for the presence of pain, tenderness, swelling, effusion, erythema, warmth, range of motion and limitation of motion, deformities and muscle atrophy. Then scintigraphic examination of the hands was performed. Control hand scintigrams were obtained from 40 age- and sex-matched patients and were examined by the same two observers. RESULTS: Thirty-two of the 57 patients (56.1%) showed Behçet's clinical hand findings. Terminal phalangeal pulp atrophy was observed in 17 (29.81%), rheumatoid-like hand findings were observed in 16 (28.1%), dorsal interosseos atrophy was observed in 12 (20.05%) and erythema over the digits was observed in 12 (20.05%). Twenty-four patients (42.1%) had scintigraphic involvement. The disease duration was observed to be an important factor for hand findings (P=0.040) and scintigraphic involvement (P=0.011). CONCLUSION: High prevalence of hand involvement in BD and its relationship with disease duration is demonstrated. Hand involvement tends to be overlooked and careful examination is required in the evaluation of BD. The scintigraphic involvement detected in hands requires special consideration, too.