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The Journal of the Association of... Nov 2022Systemic sclerosis (SSc) is an autoimmune chronic multisystem disorder with a plethora of cutaneous manifestations. These manifestations often may be the only presenting...
BACKGROUND
Systemic sclerosis (SSc) is an autoimmune chronic multisystem disorder with a plethora of cutaneous manifestations. These manifestations often may be the only presenting complaint. Early identification of these help in diagnosing grievous systemic manifestations and their prompt and appropriate treatment.
AIMS
To study the clinical profile of SSc, modified Rodnan's skin scoring (mRSS), nailfold capillaroscopy (NFC) patterns, antibody profile in the western India population, and their association with cutaneous manifestations.
METHODS
Patients of SSc fulfilling the European League Against Rheumatism (EULAR) 2013 classification of SSc criteria, who attended dermatology outpatient department (OPD) between January 2017 and September 2018 were included in the study. The demographic data, cutaneous features, autoantibody profile, mRSS, and NFC pattern were noted Results: A total of 60 patients (57 females and 3 males; mean age years) of SSc were evaluated. Clinical subtypes were 40 diffuse cutaneous SSc and 20 limited cutaneous SSc. The most common presenting symptoms were Raynaud's phenomenon (RP) (95%) and skin tightening (90%). The common cutaneous findings were sclerodactyly (86.7%), stellate scars (78.3%), parrot-beaked nose (76.7%), mask-like facies (75%), microstomia (56.7%), salt and pepper pigmentation (55%), puffy finger (46.7%), telangiectasia (46.7%), digital ulcer (38.3%), fixed flexion deformity (33.3%), and calcinosis cutis (8.33%). Limited cutaneous systemic sclerosis (lcSSc) had mRSS score of 8.3 ± 4.1 and diffuse cutaneous systemic sclerosis (dcSSc) subset had a score of 28 ± 10.4. Antinuclear antibody (ANA), Anti-topoisomerase antibody (ATA), and anti-centromere antibody (ACA) were positive in 59, 49, and 7 patients, respectively. The NFC patterns were early (23.3%), active (45%), and late (18.3%).
LIMITATION
The sample size of the study was small. We were not able to determine the significance of other less common autoantibodies with scleroderma.
CONCLUSION
The study highlights the importance of identifying early cutaneous findings and the role of a useful diagnostic and prognostic reproducible scoring system (mRSS) and NFC.
Topics: Male; Female; Humans; Autoantibodies; Microscopic Angioscopy; Scleroderma, Systemic; Scleroderma, Diffuse; Antibodies, Antinuclear; Scleroderma, Localized
PubMed: 37355940
DOI: 10.5005/japi-11001-0136 -
Journal of the American Academy of... Nov 2023Reduced oral aperture (ROA), resulting from systemic sclerosis (SSc), is a debilitating condition with limited treatment options. Improvement in oral function has been...
BACKGROUND
Reduced oral aperture (ROA), resulting from systemic sclerosis (SSc), is a debilitating condition with limited treatment options. Improvement in oral function has been reported with perioral administration of botulinum toxin type A.
OBJECTIVE
To prospectively evaluate the efficacy of onabotulinumtoxinA (onabotA) injection in improving oral opening and quality of life in SSc patients with ROA.
METHODS
Seventeen women with SSc and ROA were treated with 16 units of onabotA in 8 different sites around the cutaneous lips. Measurements of maximum mouth opening were taken before treatment, at 2 weeks posttreatment, and at 3 months posttreatment. Function and quality of life were also assessed via surveys.
RESULTS
Interincisor and interlabial distances were significantly increased 2 weeks after treatment with onabotA (P < .001) but not 3 months after. Subjective improvement in quality of life was noted.
LIMITATIONS
This single-institution study enrolled 17 patients and did not have a placebo control group.
CONCLUSION
OnabotA appears to have a strong short-term symptomatic benefit in patients with ROA due to SSc, with possible benefit to quality of life.
PubMed: 37301288
DOI: 10.1016/j.jaad.2023.04.069 -
The American Journal of Case Reports May 2023BACKGROUND Agnathia-otocephaly complex (AOC) is a rare congenital malformation due to a first-branch arch disorder and has been considered lethal. However, milder...
BACKGROUND Agnathia-otocephaly complex (AOC) is a rare congenital malformation due to a first-branch arch disorder and has been considered lethal. However, milder variants of the isolated type of AOC have been reported as nonlethal. The ex-utero intrapartum treatment (EXIT) procedure is basically indicated for a fetus with a high risk of airway obstruction immediately after birth; it is not indicated for all AOC cases but is chosen to treat cases until the airway can be evaluated to achieve a better prognosis. CASE REPORT A 37-year-old woman was referred with reported fetal facial deformity and polyhydramnios at 27 weeks of gestation. Our fetal ultrasound scans showed agnathia, microstomia, and synotia, but not holoprosencephaly. Isolated AOC was diagnosed prenatally. Magnetic resonance imaging and microbubble tests revealed delayed fetal lung maturation, although it was not completely unmatured. With patient agreement, an emergency cesarean section with EXIT was performed because of clinical chorioamnionitis at 35 weeks of gestation. Tracheostomy was attempted for 16 min during EXIT and was completed 4 min after delivery. Despite this, the neonate died 12 h after delivery from severe respiratory failure and a tension pneumothorax caused by a hypoplastic lung. CONCLUSIONS There is controversy surrounding the non-lethality of all isolated AOC cases and the non-contraindication of EXIT procedures. Our case was estimated as the milder variant, and the EXIT procedure was indicated; however, the neonate died of the hypoplastic lung. The evaluation methods of lung maturation are inconsistent, and the indication of the invasive EXIT procedure must be carefully considered.
Topics: Infant, Newborn; Humans; Pregnancy; Female; Adult; Cesarean Section; Craniofacial Abnormalities; Airway Obstruction; Ultrasonography, Prenatal
PubMed: 37165610
DOI: 10.12659/AJCR.939016 -
Cureus Mar 2023A dimensionally accurate impression is one of the primary determinants for the precise fabrication of complete denture prostheses in microstomia patients. This can be...
A dimensionally accurate impression is one of the primary determinants for the precise fabrication of complete denture prostheses in microstomia patients. This can be achieved with the help of sectional trays. This technical report describes the fabrication of a sectional impression tray with a reusable sectional handle using magnets. The handles can be sterilized and reused, thus saving clinical time for future use. The proposed method provides ease of reassembling and disassembling, easy placement, and guided orientation of the two tray segments.
PubMed: 37056534
DOI: 10.7759/cureus.36038 -
Cureus Mar 2023Commissuroplasty is a procedure that is performed to correct deformities at the corner of the mouth or oral commissure. Herein, we report a case of postoperative...
Commissuroplasty is a procedure that is performed to correct deformities at the corner of the mouth or oral commissure. Herein, we report a case of postoperative microstomia treated with commissuroplasty using split dry lips. In a surgical procedure, the dry lip was divided into orbicularis oris muscle cutaneous flaps and transpositioned into the cleft formed. The deformation of the corners of the mouth improved, and mouth opening improved enough to wear dentures. We believe that this method enables commissuroplasty that combines aesthetics with function.
PubMed: 37051003
DOI: 10.7759/cureus.35980 -
Journal of Scleroderma and Related... Feb 2023Decreased maximal mouth opening is a common and disabling manifestation in systemic sclerosis patients. We aimed to study the course of maximal mouth opening,...
OBJECTIVE
Decreased maximal mouth opening is a common and disabling manifestation in systemic sclerosis patients. We aimed to study the course of maximal mouth opening, determinants of smaller maximal mouth opening over time and the burden of smaller maximal mouth opening on mouth handicap.
METHODS
Consecutive systemic sclerosis patients participating in the prospective Leiden Combined Care in systemic sclerosis cohort were included. Annual clinical assessment included maximal mouth opening measurement and mouth handicap evaluation (Mouth Handicap in Systemic Sclerosis scale). Presence of microstomia (maximal mouth opening < 30 mm) was studied. Maximal mouth opening over time was assessed on group level and for all patients individually. Baseline characteristics were analysed for their association with smaller maximal mouth opening over time (linear mixed-effects models). Furthermore, cross-sectional association between maximal mouth opening with Mouth Handicap in Systemic Sclerosis scale was assessed (linear regression analysis).
RESULTS
A total of 382 systemic sclerosis patients were studied with median follow-up time of 2.0 years (interquartile range = 0.0-3.0). At baseline, mean maximal mouth opening was 42.2 ± 8.0 mm and 7% suffered from microstomia. Annual decrease of > 5.0 mm in maximal mouth opening during follow-up occurred in 63 patients and was accompanied by increase in disease severity. Disease characteristics at baseline independently predictive for smaller maximal mouth opening over time were: more extended skin subtype; peripheral vasculopathy; pulmonary, renal and gastrointestinal involvement. Smaller maximal mouth opening was significantly associated with more reported mouth handicap.
CONCLUSION
The course of maximal mouth opening is stable in a majority of systemic sclerosis patients. Still, maximal mouth opening over time was smaller in patients with more severe organ involvement. Although microstomia was infrequent, a smaller maximal mouth opening was significantly associated with more mouth handicap, indicating the importance to address maximal mouth opening in routine care of systemic sclerosis patients.
PubMed: 36743815
DOI: 10.1177/23971983221138177 -
The EPMA Journal Mar 2023This study assessed sleep quality in patients with burn scars and investigated risk factors of sleep disorders to guide clinical therapy. From the strategy of...
PURPOSE
This study assessed sleep quality in patients with burn scars and investigated risk factors of sleep disorders to guide clinical therapy. From the strategy of predictive, preventive, and personalized medicine (PPPM/3PM), we proposed that risk assessment based on clinical indicators could prompt primary prediction, targeted prevention, and personalized interventions to improve the management of sleep disorders present in patients with burn scars.
METHODS
This retrospective study recruited patients with burn scars and healthy volunteers from the Shanghai Burn Treatment Center between 2017 and 2022. Relevant information and data, including demographic characteristics, scar evaluation, and sleep quality, were obtained through the hospital information system, classical scar scale, and self-report questionnaires. Sleep quality was assessed using the Pittsburgh Sleep Quality Index (PSQI) and monitored using a cardiopulmonary-coupled electrocardiograph. Pain and pruritus were assessed using the visual analog scale (VAS). Scar appearance was assessed using the modified Vancouver scar scale (mVSS).
RESULTS
The sample was comprised of 128 hypertrophic scar (HS) patients, with 61.7% males, a mean age of 41.1 ± 11.6 years, and burn area of 46.2 ± 27.9% total body surface area (TBSA). Patients with PSQI ≥ 7 accounted for 76.6%, and the global PSQI score was 9.4 ± 4.1. Objective sleep data showed that initial enter deep sleep time, light sleep time, awakening time, light sleep efficiency, and sleep apnea index were higher but deep sleep time, sleep efficiency, and deep sleep efficiency were lower in HS patients than that in healthy controls. Preliminary univariate analysis showed that age, hyperplasia time of scar, narrow airway, microstomia, VAS for pain and pruritus, and mVSS total (comprised of pigmentation, vascularity, height and pliability) were associated with the PSQI score ( < 0.1). Multivariable linear regression showed narrow airway, VAS for pain and pruritus, and mVSS specifically height, were the risk factors for PSQI score ( < 0.1).
CONCLUSIONS
This study model identified that narrow airway, pain, pruritus and scar appearance specifically height may provide excellent predictors for sleep disorders in HS patients. Our results provided a basis for the predictive diagnostics, targeted prevention, and individualized therapy of somnipathy predisposition and progression of HS patients in the setting of PPPM/3PM health care system, which contributed to a paradigm shift from reactive cure to advanced therapy.
PubMed: 36684850
DOI: 10.1007/s13167-022-00309-x -
International Journal of Surgery Case... Jan 2023The recessive dystrophic epidermolysis bullosa is a severe form of hereditary epidermolysis bullosa characterized by deformities of the skin, blisters and erosions on...
INTRODUCTION AND IMPORTANCE
The recessive dystrophic epidermolysis bullosa is a severe form of hereditary epidermolysis bullosa characterized by deformities of the skin, blisters and erosions on the mucous membranes. Oral manifestations are frequent and extensive vary from small discrete vesicles to large bullae, associated with microstomia, ankyloglossia and a depapilled tongue. The purpose of this case report is to describe oral health status of patient with recessive-dystrophic epidermolysis bullosa, and the measures that dentists should adopt with the purpose of provide an effective dental treatment.
CASE PRESENTATION
We present a clinical case of a patient with recessive-dystrophic epidermolysis bullosa who underwent a bone regularization and whose follow-up was carried out until healing.
CLINICAL DISCUSSION
The management of patients with RDEB in oral surgery requires the adoption of an atraumatic technique and certain precautions to limit the formation of bullae and promote mucosal healing.
CONCLUSION
We can conclude that oral management of patients with recessive dystrophic epidermolysis bullosa presents a challenge for the oral surgeon and the management is generally multidisciplinary and relies essentially on the adoption of a preventive and atraumatic approach.
PubMed: 36580730
DOI: 10.1016/j.ijscr.2022.107848 -
Special Care in Dentistry : Official... 2023Epidermolysis bullosa (EB) is a group of genetic disorders characterized by fragility of the skin and mucosal membranes. Dystrophic EB (DEB) is caused by mutations in...
Impression technique modification and oral contracture release surgery for orthodontic treatment in a patient with severe microstomia due to recessive dystrophic epidermolysis bullosa.
INTRODUCTION
Epidermolysis bullosa (EB) is a group of genetic disorders characterized by fragility of the skin and mucosal membranes. Dystrophic EB (DEB) is caused by mutations in the gene coding for type VII collagen. Among the most frequent oral manifestations in Recessive DEB (RDEB) are oral ulcers and blisters, absence of tongue papillae and palatal rugae, ankyloglossia, oral vestibule obliteration, and microstomia. The following report describes a modified impression technique used in a patient with severe RDEB and severe microstomia to obtain models for orthodontic treatment with aligners.
CASE REPORT
A 25-year-old female patient with severe RDEB was referred for orthodontic treatment. Severe microstomia (8 mm), hindered the use of conventional trays or intraoral scanners to design the aligners. Therefore, a contracture release surgery in combination with a modified impression technique was performed to obtain an optimal impression and subsequent aligners for orthodontic treatment.
DISCUSSION
This case presents an alternative strategy to provide orthodontic treatment with aligners in patients with severe microstomia due to severe RDEB. Reports of orthodontic treatment in people living with EB, especially in RDEB, are still rare, with few publications about fixed braces, early teeth extraction and removable devices, and none using aligners. Most of the impression techniques reported are aimed at oral rehabilitation. The multidisciplinary approach and impression technique reported should broaden the alternatives of orthodontic techniques provided to patients with EB and severe microstomia.
CONCLUSIONS
This article describes an oral contracture release surgery and modified impression technique for obtaining good quality impression for the design of orthodontic aligners in patients with severe microstomia due to severe RDEB.
PubMed: 36504454
DOI: 10.1111/scd.12808 -
Cureus Nov 2022Edentulism is considered a poor health condition and may compromise the quality of life. Prosthodontic replacement of missing teeth results in significant improvement of...
Edentulism is considered a poor health condition and may compromise the quality of life. Prosthodontic replacement of missing teeth results in significant improvement of oral functions. Treating a patient with Down syndrome can pose clinical challenges in handling the emotional aspect as well as in rendering treatment. Careful oral analysis and diagnosis of the existing conditions in such patients will enable and pave the way for clinically acceptable treatment results. This clinical report describes the challenges encountered in the prosthodontic management of an edentulous young patient with Down syndrome.
PubMed: 36483899
DOI: 10.7759/cureus.31148