Clinical correlations of striatal hand deformities in Parkinson's disease.

BACKGROUND: Hand deformities have been recognized since the 19th century as part of the postural abnormalities observed in Parkinson's disease (PD). However, their pathogenesis and clinical correlations are poorly understood. METHODS: We evaluated 104 hands of 52 consecutive patients with PD by high-resolution photographs taken from the radial aspect of each hand; the degree of flexion of the 2nd metacarpophalangeal (MCP) joint was measured by software. The presence of classical striatal hand deformity (CSHD) was also evaluated, defined as MCP flexion, proximal interphalangeal joint extension, and distal interphalangeal joint flexion. RESULTS: Patients with PD had a mean age of 63.3 ± 12.7 years, and 29 (56%) were male. The degree of MCP joint flexion in both hands showed moderate correlation with the MDS-UPDRS-III motor score (r = 0.518, P < 0.001), mainly related to ipsilateral rigidity and ipsilateral bradykinesia scores, and fair correlation with the Hoehn-Yahr stage. A CSHD only correlated with a younger age at onset of PD (P = 0.049). These hand deformities were not markers of dyskinesia, levodopa equivalent dose, or cognitive dysfunction. CONCLUSIONS: Metacarpophalangeal joint flexion is the most common hand deformity in PD and correlates with rigidity and bradykinesia. A CSHD was only related to a younger age at onset.

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.

From art to science: the functional damage due to thumb osteoarthritis finely described by Velazquez 300 years before its clinical description.

Velazquez showed to know the entity of thumb osteoarthritis by finely describing it in one of his paintings. The concepts of anatomical damage, loss of strenght, and functional impairment are transmitted to the observer.

[Porphyria cutanea tarda as extrahepatic manifestation of chronic hepatitis C: a case report]. / Porfiria cutánea tarda como manifestación extrahepática de hepatitis Ccrónica: reporte de un caso.

The present case is a 56 year old male who present hyperpigmented and hypopigmented scars in both hands, associated with the presence of milia cysts. It was studied the metabolism of porphyrins and skin biopsy of the lesions which were compatible with porphyria cutanea tarda. In the initial laboratory, elevated transaminases values were found and subsequently identified chronic infection of hepatitis C virus. In order to treat viral infection and resolve the dermal commitment; considered extrahepatic manifestation of hepatitis C virus, treatment was started with pegylated interferon and ribavirin, with favorably development and rapid viral response, with undetectable viral load until now (24 weeks of treatment), decreased level of serum transaminases and improvement of skin lesions.

Advantages of a combined rheumatoid arthritis magnetic resonance imaging score (RAMRIS) for hand and feet: does the RAMRIS of the hand alone underestimate disease activity and progression?

BACKGROUND: To evaluate a combined rheumatoid arthritis magnetic resonance imaging score (RAMRIS) for hand and foot (HaF-score) in rheumatoid arthritis (RA). METHODS: Magnetic resonance imaging (MRI, 0.2 Tesla) of the dominant hand and foot of 26 ACPA positive RA patients before and 6 months after initiation of methotrexate was obtained. RAMRIS of the hand was complemented by corresponding scoring of the foot (MTP I-V; HaF-score). Disease Activity Score 28 (DAS28) and a tender and swollen joint count (JC) of the joints scored in MRI were recorded. Changes in these scores (Δ) were assessed. RESULTS: ΔHaF-score correlated significantly with ΔDAS28 (r = 0.820, 95%-CI 0.633-0.916). Correlations to ΔDAS28 were best for changes in the synovitis subscore (0.648) and bone marrow edema (0.703). Correlations to ΔDAS28 were significantly better for of the ΔHaF-score than ΔRAMRIS (0.499, 0.139-0.743, p = 0.0368).All patients with at least moderate response (EULAR criteria, n = 11) had continuing disease activity on MRI, including five cases with new erosions, three of them at the feet. Improvements of the hand JC or foot JC were seen in 16 and 15 cases, respectively. However, MRI of the hand or feet improved in only 10 and 9 cases, respectively. No patient fulfilled SDAI remission criteria. CONCLUSIONS: The HaF-score identifies patients with continuing disease activity despite clinical response that would have been missed by consideration of the traditional RAMRIS or the DAS28 alone. Response as opposed to remission may be an insufficient goal in RA as all patients showed continuing disease activity, especially at the feet.

Report of a family with idiopathic knuckle pads and review of idiopathic and disease-associated knuckle pads.

Knuckle pads are a rare, frequently overlooked, thickening of the skin usually overlying the extensor surface of the proximal interphalangeal joints. They are well- circumscribed, benign lesions that generally do not require treatment. Idiopathic knuckle pads must be differentiated from similar appearing lesions or trauma-induced pseudo-knuckle pads. Knuckle pads have been observed in association with autosomal dominant conditions such as Bart-Pumphrey syndrome, Dupuytren's contracture, Ledderhose disease, and Peyronie's disease. To the best of our knowledge, idiopathic familial knuckle pads have not previously been described in the English language literature. We describe a sister and brother with familial idiopathic knuckle pads with no associated conditions.

Unusual unilateral presentation of pachydermodactyly: a case report.

Pachydermodactyly is a rare digital fibromatosis characterized by asymptomatic fusiform soft-tissue swellings of the proximal interphalangeal joints of the hands. It usually affects healthy adolescent males with a negative family history. As a rule, clinical presentation of the disease is bilateral and symmetrical enlargement of the joints. So it can be misdiagnosed with inflammatory rheumatic diseases, especially with juvenile chronic arthritis. A prompt clinical diagnosis of the disease would prevent inappropriate treatment with immunosuppressive agents or steroids and unnecessary expensive diagnostic procedures such as biopsy or magnetic resonance imaging. Once diagnosed, patients should be advised in order to avoid repetitive traumas of the hands, rubbing and cracking of the fingers, obsessive-compulsive use of computer and video games. The joint outcome is always benign. Here, we report a case of pachydermodactyly differs from the typical clinical picture of pachydermodactyly in the unilateral distribution of the lesions.

Anti-cyclic citrullinated peptide antibodies in systemic lupus erythematosus patients with articular involvement: a predictive marker for erosive disease?

A small number of systemic lupus erythematosus (SLE) patients develop an erosive disease. Some studies have suggested an association between anti-cyclic citrullinated (anti-CCP) antibodies and this pattern of arthritis, but their exact significance in SLE patients remains unclear. The aim of this study was to evaluate the prevalence of anti-CCP antibodies in SLE patients with different subsets of articular disease. Among 521 SLE patients followed in this center from 1976 to 2011, those with articular involvement (n=298) were selected to take part in the study. We searched for anti-CCP2 IgG antibodies in 198 patients using a commercial enzyme linked immunosorbent assay (Immunoscan RA, Eurodiagnostica). In 174 patients the results for rheumatoid factor (RF) by nephelometry were retrospectively collected. C reactive protein (CRP) was obtained from clinical records. Patients were classified into 3 groups: erosive, non-erosive deforming, non-erosive non-deforming arthritis. Results of the different tests were compared among the groups. P<0.05 was considered statistically significant. Anti-CCP antibodies were significantly associated with erosive disease. We also found that RF positivity and increased CRP were more frequent in erosive arthritis and erosive or non-deforming arthritis, respectively, than in non-erosive non-deforming arthritis. This study supports the evidence that anti-CCP antibodies could be a useful marker of erosive disease in SLE patients. Increase in RF and CRP could be an additional means of identifying lupus patients with arthritis at risk of a worse prognosis.

[Sequelae of hand burns]. / Séquelles de brûlures des mains.

INTRODUCTION: Hand burns are common. Aesthetic and functional consequences are mixed together. ANATOMY: Palmar and dorsal skin are very different. Palmar skin is thick and strong, dedicated to the prehension. Dorsal skin is very thin, creating a mobility plan dedicated to the flexion of the fingers. AESTHETIC SEQUELAE: They are rarely isolated. Proximal nail retractions can occur after nail burns, with matricial exposition. We remove the scar and a full skin graft is done in order to give a normal proximal nail skin shape. FUNCTIONAL SEQUELAE: They are fingers and wrist retractions. We often use collagen substitute to cover soft tissue defect after scar excision. Commissure retractions are treated by trident plasty. PARTICULAR CASES: Electrical burns: we expose the case of a thumb amputation after necrosis by electrical burn, secondary treated by index policization. Children burns: the bridles appear during the growth, so we must follow this patient for a long time. CONCLUSION: Hand burns are frequent. Functional sequelae are often important: retractions, amputations. The orthopaedic treatment of these retractions is unspecific. We often use collagen substitute in our unit. The patient must be involved in his treatment, because it is very long and difficult.

Phalangeal Intra-Articular Osteochondroma Caused a Rare Clinodactyly Deformity in Children: Case Series and Literature Review.

Background: Various factors are discovered in the development of clinodactyly. The purpose of this retrospective study was to present a group of children with a rare clinodactyly deformity caused by phalangeal intra-articular osteochondroma and evaluate the efficacy of various treatment methods. Methods: All child patients that were treated for finger problems in our center between Jan 2017 and Dec 2020 were reviewed. A detailed analysis was made of the diagnosis and treatment methods in eight rare cases. X-rays and histopathology were applied. Results: A preliminary analysis of 405 patients in total was performed, and we included eight cases in our final analysis. This cohort consisted of 2 girls and 6 boys, with a mean age of 5.74 ± 3.22 years (range: 2y5m to 11y). Overall, four patients had their right hand affected and four patients had their left hand affected. One patient was diagnosed as having hereditary multiple osteochondroma (HMO) while the other seven patients were all grouped into solitary osteochondroma. Osteochondroma was proven in all of them by histopathology examination. Preoperative X-rays were used to allow identification and surgery planning in all cases. All osteochondromas were intra-articular and in the distal end of the phalanges, which is located opposite the epiphyseal growth area. All of the osteochondromas developed in half side of the phalanges. The angulation in the finger long axis was measured, and resulted in a mean angulation of 34.63 ± 24.93 degree (range: 10.16-88.91 degree). All of them received surgery, resulting in good appearance and fingers straightening. No recurrence was recorded. Conclusions: This retrospective analysis indicates that 10 degrees can be selected as the angulation level for diagnosis of clinodactyly deformities. What's more important, the abnormal mass proven by X-rays should be included as the classical direct sign for diagnosis. The first choice of treatment is surgery in symptomatic osteochondromas.

Multicentric reticulohistiocytosis: A case report and review of the literature.

We report a case of multicentric reticulohistiocytosis (MRH) demonstrating classic clinical and histologic findings. This rare idiopathic disease is manifested by severe destructive polyarthritis and papulonodular mucocutaneous lesions. The characteristic clinical and histologic findings are discussed as well as the workup and treatment of the disease.

Disabilities in leprosy: an open, retrospective analyses of institutional records.

BACKGROUND AND OBJECTIVES: Leprosy remains a leading cause of peripheral neuropathy and disability in the world. Primary objective of the study was to determine the incidence of deformities present at a time of diagnosis and new deformities that patients develop over follow up period. MATERIAL AND METHODS: An open, retrospective cohort study was performed at a tertiary medical center in western India. Recruitment phase of the study was of 2 years (2009-2010) followed by observation/follow up phase of 7 years till 31st December 2017. New patients with leprosy and released from treatment cases who presented with deformity as defined by WHO disability grade (1998) and subsequently developing new deformities during the follow up period of up to 7 years were included in the study. RESULTS: The study included 200 leprosy patients. Of the total 254 deformities, 168 (66.14%) deformities were noticed at the moment of diagnosis, 20 (7.87%) deformities occurred during the follow up phase. Of all patients, 21.25% had Grade 1 deformity and 6.31% had Grade 2 or more severe deformity. Deformities of hand were most common in 44.48%, followed by feet 39.76%, and face 15.74% respectively. LIMITATION OF STUDY: Mode of inclusion of patient was self-reporting during follow up phase so there is possible under reporting of the disabilities. CONCLUSION: New deformities continue to develop in certain forms of leprosy even after release from treatment. Long-term & regular follow up of patients who have been released from treatment is required.

Wrist deformity, bother and function following wrist fracture in the elderly.

OBJECTIVE: Wrist deformity in older people is common following treatment for a wrist fracture, particularly after non-surgical treatment. A cohort of older wrist fracture patients were surveyed by telephone regarding perceived deformity, bother with deformity and patient-reported wrist function. The objectives were to: (1) determine whether older patients with wrist fractures perceived a deformity of their wrist and if they were bothered by it; (2) test if there were associations between deformity and treatment-type and between deformity and function; (3) test for associations between bother and treatment-type and between bother and function; (4) measure the test-retest reliability of the 'bother' question. RESULTS: Of 98 eligible patients who were invited to participate, 41 responded. Out of 41, 14 (34%) believed they had a deformity and 4 (10%) reported that they were bothered by the appearance of their wrist. Deformity was associated with non-surgical treatment (RR = 3.85, p = 0.006) but was not significantly associated with functional outcomes (p = 0.15). All those who were bothered belonged to the non-surgical treatment group. Bother was significantly associated with poorer functional outcomes (p = 0.006) and this association was clinically significant (MD = 35 points). The deformity and bother questions were found to have excellent test-retest reliability; κ = 1.00 and κ = 0.92, respectively.

From ankylosis to pencil-in-cup deformity in psoriatic arthritis: a case report.

A 56-year-old woman with psoriatic arthritis is presented whose initially ankylosed digit was later found to develop pencil-in-cup change. The patient was treated over that period with etanercept and had no signs of active arthritis. The possible mechanisms for these changes are discussed.