Botulinum Toxin Injection for Lower Face and Oral Cavity Raynaud Phenomenon After Mandibulectomy, Free Fibula Reconstruction, and Radiation Therapy.

Isolated lingual and lower face Raynaud phenomenon without primary Raynaud of the digits is a very rare condition associated with chemoradiation therapy (RT) in previous reports. The condition, which more commonly presents in patients with a history of Raynaud disease, is often self-limiting, but vasodilating agents and steroids have been suggested as possible treatment options. Spasmodic torticollis is a different, more common entity, also associated with history of RT or previous head and neck surgery. We present a rare case of a patient who developed Raynaud phenomenon of the lower face and tongue in the presence of spasmodic torticollis after mandibulectomy and free fibula reconstruction followed by RT to the oral cavity and neck. Possible causes, pathophysiologic mechanisms and treatment options are discussed. This is the first report of botulinum toxin treatment of isolated secondary Raynaud phenomenon of the lower face and tongue.

Evidence for the Use of Epoprostenol to Treat Raynaud's Phenomenon With or Without Digital Ulcers.

OBJECTIVE: To review the evidence for using intravenous (IV) epoprostenol to treat Raynaud's phenomenon (RP). DATA SOURCES: The databases MEDLINE (1946 to March 2016), PubMed, and International Pharmaceutical Abstracts were searched using the terms epoprostenol, Flolan, Raynaud's disease, and CREST syndrome. Further literature sources were identified by reviewing article citations. STUDY SELECTION AND DATA EXTRACTION: All English-language, clinical trials and case series evaluating IV epoprostenol for the management or treatment of RP were included. Lower-quality evidence were incorporated due to limited information. DATA SYNTHESIS: Seven small uncontrolled studies/case series, 1 small placebo controlled study, and 1 larger randomized trial were identified and included. There was no consistent measurement of efficacy utilized, but improvements in hand temperature, RP attack duration and frequency were commonly associated with IV epoprostenol treatment (5 trials). There were conflicting data regarding effect sustainability, with 5 trials showing long-term effects and 3 showing immediate effects. Fewer ischemic ulcers developed during treatment with IV epoprostenol in 1 trial compared to conventional treatment. Ulcer healing ocurred in 2 trials. Common adverse effects included hypotension, headache, flushing, gastrointestinal symptoms, and jaw pain. CONCLUSIONS: Available evidence supports the use of IV epoprostenol for treatment of severe RP in patients refractory or intolerant to standard therapies. The dose, titration schedule, and duration of IV epoprostenol utilized in studies varied, but a conservative approach to initiation should be considered. Patients who do not respond to intermittent infusions and have severe digital ischemia may require more aggressive regimens.

Case Report: Systemic Sclerosis After Covid-19 Infection.

The coronavirus disease (COVID-19) is a respiratory tract infection caused by the new virus SARS-CoV-2. The acute phase of the infection may in certain individuals be followed by another longer phase of disease (long COVID) of unknown etiology probably associated in certain cases with autoimmune activation. It has been shown that COVID-19 can trigger autoantibody production and in genetically predisposed patients may cause the onset or exacerbation of autoimmune diseases. We are reporting a case of mild COVID-19 infection complicated by autoantibody production and cutaneous and gastrointestinal symptoms and subsequently diagnosed with systemic sclerosis (SSc). A 47-year-old man with no history of any autoimmune diseases and in good health became sick together with his family on the 12th of November with mild symptoms: tiredness, fever, cough, and sore throat. Oropharyngeal swab for SARS-CoV-2 tested positive. He was isolated at home and did not require hospitalization. Three weeks later he presented with clinical manifestation compatible with suspicion of SSc. He briefly presented with skin rush, periorbital edema and conjunctivitis, vomiting, dysphagia, burning sensation in the skin, above all in the fingertips and around the mouth, puffy fingers, Raynaud's phenomenon, pain at the fingertip of the middle finger where a depressed area was noticed without a clear ulceration. ANA showed a strongly positive nucleolar pattern. Anti-PM/Scl 75 and PM/Scl 100 resulted positive. High-resolution computed tomography (HCRT) showed early stage of interstitial lung disease (ILD). The patient was diagnosed with SSc based on the persistence of autoantibodies and the clinical and radiological pictures according to the ACR/EULAR classification (scores: puffy finger, 2; ILD, 2; Raynaud's phenomenon, 3; SSc related antibodies, 3; total 10). There are several cases described in the medical literature of possible new onset of SLE after COVID-19 infection. This is the first case that describes a possible new onset of SSc. Conclusion: SARS-CoV-2 may trigger systemic sclerosis.

Scleroderma: considerations for dental hygienists.

Scleroderma, the general name of a group of progressive diseases affecting the connective tissues is the most deadly of the varying connective tissue disorders. Characterized by abnormal thickening of the skin, this collagen-vascular disease is associated with immune dysfunction. Hallmark signs of scleroderma include fibrosis, vascular instability and initial inflammation resulting from excessive collagen deposition. Oral facial involvement is considerable, necessitating adaptations in patient oral self-care and influencing oral hygiene. Appropriate dental hygiene management of patients with this autoimmune disorder requires an understanding of clinical characteristics, the recognition of oral facial involvement, treatment considerations and pharmacological interventions. With this information, dental hygienists will be better prepared to provide compassionate, safe and effective dental hygiene management and care to patients with scleroderma.

Case of primary Sjogren's syndrome preceded by dystonia.

There are only six cases in literature that describe development of dystonia with Sjogren's syndrome (SS). We describe a case of a 43-year-old woman who presented with symptoms including movement disorder, sensory neurogenic bladder, sensory loss and neuropathic pain, migraine like headaches, musculoskeletal pain, Raynaud's phenomenon and dysautonomia. Symptoms started in 2000, with weakness that progressed to dystonia in 2003. Diagnostic work-up was inconclusive with negative inflammatory serologies, cerebrospinal fluid and MRI for many years. After patient developed sicca syndrome with dry eyes and mouth in 2009, her rheumatoid factor titre was elevated (550 IU/mL), erythrocyte sedimentation rate, anti-Sjogrens syndrome-related antigen A (anti-Ro/SSA) and anti-SSB/La: anti-Sjogrens syndrome-related antigen B (anti-La/SSB) became positive. Lip biopsy confirmed diagnosis of SS. She was diagnosed with primary SS with neurological involvement. Her symptoms responded well to intravenous methylprednisolone. Symptoms stabilised with trials of immune-suppressive therapy. This is a case that demonstrates the delay of diagnosing SS with preceding unique neurological association.

Chronic arthropathy occurring after augmentation mammaplasty.

Augmentation mammaplasty has been associated with a broad spectrum of connective-tissue disease, systemic illness and autoimmune phenomena. The three cases reported herein suggest a possible relationship between silicone gel implants for augmentation mammaplasty (with capsular contractures as complicating feature) and the development of chronic arthropathy.

Silicone mammary implants and connective tissue disease.

The American Food and Drug Administration recently restricted the use of silicone gel-filled mammary implants for breast augmentations and reconstructions because a number of case reports had suggested that there was an association between silicone and connective tissue disease. We have found 36 such case reports published since 1982. Systemic sclerosis is the most common reported diagnosis (n = 15). Implants were removed from 15 patients, in nine of whom symptoms improved. The mechanism behind the postulated relationship is obscure, and most authors focused on an auto-immune-like response to silicone, which acts directly as a hapten or as an adjuvant. We found no conclusive evidence to implicate silicone mammary implants in connective tissue diseases. We do, however, recommend removal of implants from patients with severe connective tissue diseases. Patients with symptoms or active connective tissue disease should not be considered for silicone implants.

[Occupational Raynaud's phenomenon in a dentist: a case report]. / Fenomeno di Raynaud occupazionale in dentista: presentazione di un caso.

After more than 39 years of practice, a dentist showed Raynaud's phenomenon of the hand exposed to vibrations generated by dental instruments. Other possible occupational, pharmacological and systemic disease factors that could cause Raynaud's phenomenon were excluded. Since it has been reported in the literature that dentists and dental technicians could exceed the permissible limits for vibrations, an occupational etiology of this case is suggested on the basis of logical reasoning.

New concepts and technologies in reconstructive hand surgery.

Complex traumatic injuries and degenerative conditions of the hand continue to lead to significant impairment and disability. From technical innovations to regenerative concepts, this article presents the latest advances in the dynamic field of hand surgery in which worldwide efforts are made around the globe to repair, regenerate, or restore each composite tissue forming the hand. The systematic method by which finger replantation is performed, from bony fixation to skin closure, provides a platform for discussion of the newest innovations available to reconstructive hand surgeons.

[Analysis of subjective symptoms of upper extremities in dental technicians].

Our review of the literature showed no report on the subjective symptoms of the upper extremities in dental technicians. This study was therefore undertaken on 164 dental technicians in Gifu Prefecture to investigate the relationship between the subjective symptoms of the hands, arms and shoulders and work of dental technicians. The following results were obtained. 1) The prevalence of Raynaud's phenomenon and numbness of the fingers was 5.5 and 18.5 %, respectively. A significant positive correlation was observed between the prevalence and frequency of numbness and cold sensation in the fingers and the daily usage time (h) of high-speed type machines. In addition, pain in the fingers and around the wrists, and pain and numbness in the arms were significantly related to the daily usage time (h) of low-speed type machines. These results suggest that the usage of high- and low-speed type machines may be a cause of vibration syndrome among dental technicians. 2) A high prevalence of pain in the shoulders (30.8%), neck (30.1%), and back (36.3%) was observed in dental technicians. It is considered that these symptoms are related to the working posture and usage of machines.

Detection of immune complexes in systemic sclerosis and Raynaud's phenomenon.

Phagocytosed immune complexes in polymorphonuclear leukocytes of nineteen patients with Raynaud's phenomenon were detected by scoring the cells for IgG and C3 inclusions. Parallel estimations of circulating immune complexes were made by liquid phase C1q binding and polyethylene glycol (PEG) precipitation methods. The results were compared with the degree of cold stress-induced vasospasm measured by quantified infra-red thermography. Thirteen of nineteen patients with Raynaud's phenomenon compared with only one of seventeen normal controls showed significantly elevated levels of phagocytosed IgG. The correlation of IgG and C3 scores for individual patients was high and the IgG and C3 scores correlated well with the thermographic assessment of the severity of the Raynaud's phenomenon. Two of the patients had elevated levels of circulating immune complexes detectable by raised levels of C1q binding, four patients had raised IgG and three patients raised IgM complexes detectable by PEG precipitation. None of the controls had elevated levels of circulating immune complexes detectable by either method. There was no correlation between the levels of circulating and phagocytosed immune complexes.

Association of mixed connective tissue disease, Sjögren's syndrome and autoimmune hepatitis: report of a case.

A 51-year-old female was admitted to the hospital due to liver dysfunction. She had suffered from arthralgia, Raynaud's phenomenon, dry mouth and dry eyes. Laboratory data showed positive LE cells and anti-RNP. The diagnosis of mixed connective tissue disease (MCTD) was made according to the criteria of the international symposium on MCTD and Antinuclear Antibodies (August 1986, Tokyo). Liver biopsy showed chronic active hepatitis. The results of sialography, lip biopsy and rose-bengal test were compatible with Sjögren's syndrome (SjS). This is the report of a rare case with MCTD associated with SjS and autoimmune hepatitis.

A prospective evaluation of 118 patients with the fibromyalgia syndrome: prevalence of Raynaud's phenomenon, sicca symptoms, ANA, low complement, and Ig deposition at the dermal-epidermal junction.

One hundred eighteen consecutive patients with fibromyalgia were evaluated prospectively. Immunoglobulin deposits at the dermal-epidermal junction were found in only 4 of 36 patients. Twelve patients (14%) had at least one positive antinuclear antibody (ANA) test. Eight patients (7%) had at least one low C3. Thirty-five patients (30%) has a history of Raynaud's phenomenon and 22 (18%) had sicca symptoms, defined as symptomatic dry eyes and mouth and a positive Schirmer test. The 35 patients with Raynaud's phenomenon had a significantly higher prevalence of sicca symptoms, positive ANA or low C3 compared to the 83 patients who did not have Raynaud's phenomenon. Thus, a subset of patients with fibromyalgia may have features suggestive of a systemic connective tissue disorder.

Silicone granuloma in acral skin in a patient with silicone-gel breast implants and systemic sclerosis.

A 68-year-old white woman was referred to us by her rheumatologist for possible participation in a clinical study of photopheresis for scleroderma. In February 1993, she noticed edema of her distal phalanges, Raynaud's phenomenon in both hands, flu-like symptoms, fatigue, intermittent diarrhea, abdominal pain, tearing in both eyes, dyspnea on exertion, dysphagia, and odynophagia. Bilateral silicone-gel breast implants had been placed 12 years before; 2 months before her present evaluation, they were removed and found to be ruptured. Physical examination revealed edema, limited to the fingers and hands bilaterally, and slight induration of the skin on the dorsum of both hands and distal forearms. The remainder of the physical examination was normal. According to our study protocol, a skin biopsy specimen from the dorsum of the right hand was taken, but all other laboratory investigations were refused. Histopathologic examination revealed multiple clear spaces of varying sizes in the dermis and multinucleated macrophages containing small refractile particles, characteristic of silicone granuloma (Figs. 1 and 2); however, the specimen showed no evidence of scleroderma. X-ray energy dispersive analysis by scanning electron-microscopy confirmed the presence of elemental silicon in the small refractile particles. The patient did not receive any treatment after her diagnosis and shortly thereafter, she was lost to follow-up.