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La atrofodermia idiopática de Pasini y Pierini es una entidad poco frecuente y de etiología aún no esclarecida, se presenta con una frecuencia hasta seis veces mayor en mujeres que en hombres y una posible asociación con la esclerodermia localizada (morfea). Paciente femenina de 30 años, quien consultó por una lesión asintomática de dos años de evolución en el glúteo izquierdo. En el examen físico se evidenció una placa ovalada, deprimida y acrómica en su centro, que mide cinco por diez centímetros. La paciente había sido tratada previamente con múltiples terapias tópicas sin obtener mejoría clínica. Se realizó la biopsia de piel que demostraba cambios mínimos en epidermis, homogenización y adelgazamiento de colágeno sin afección de anexos. Se hizo correlación con los hallazgos clínicos y se decidió iniciar tratamiento con esteroides intralesionales de alta potencia (acetónido de triamcinolona). Posterior a la administración de dos aplicaciones del medicamento, con cuatro semanas de diferencia entre ellas, se evidenció la resolución completa de la dermatosis. Un mes después de la última dosis la paciente no mostró recidivas
diopathic atrophoderma of Pasini and Pierini is a rare entity of unclear etiology, occurring as much as six times more frequently in women than in men, with a possible association with localized scleroderma (morphea). It is about a 30 years old woman who consulted with an asymptomatic lesion of two years of evolution on the left gluteal region. Physical examination revealed an oval plaque, depressed and acromic in its center, measuring five by ten centimeters. A 30 years old female patient who consulted about an asymptomatic lesion of two years of evolution on the left gluteal region. Physical examination revealed an oval plaque, depressed and acromic in its center, measuring five by ten centimeters. The patient was previously treated with multiple topical therapies without clinical improvement.Skin biopsy showed minimal changes in the epidermis, homogenization, and thinning of the collagen without adnexal involvement. After a correlation was made with the clinical findings, starting treatment with high-potency intralesional steroids (triamcinolone acetonide) was recommended. After administering two applications of the drug, four weeks apart, the complete resolution of the dermatosis was evidenced. One month after the last dose, the patient showed no recurrence
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Humanos , Esclerodermia Localizada , Dermatopatias , El SalvadorRESUMO
Resumen La esclerodermia pertenece a un grupo de enfermedades autoinmunes del tejido conectivo que produce inflamación, disfunción vascular y fibrosis excesiva del tejido de soporte de la piel y los órganos viscerales. A nivel bucal se presentan afecciones como la xerostomía y la microstomía, así como caries y enfermedad periodontal por dificultades en el control de la placa bacteriana. Su tratamiento odontológico requiere un manejo integral, que comprenda las patologías bucales presentes y los síntomas permanentes característicos de la enfermedad. Si bien es una patología muy infrecuente en la práctica general, es necesario enfatizar la importancia de que el odontólogo cumpla un rol multidisciplinario en el tratamiento de estos pacientes. Esta revisión narrativa tiene por objetivo describir las manifestaciones bucomaxilofaciales y el manejo odontológico del paciente con esclerodermia.
Abstract Scleroderma belongs to a group of autoimmune connective tissue diseases that causes inflammation, vascular dysfunction and excessive fibrosis of the supporting skin tissue and visceral organs. In the mouth, conditions such as xerostomia and microstomy are evident, as well as caries and periodontal disease due to difficulty in bacterial plaque control. Its dental treatment requires an integral management, which includes the oral pathologies present and the characteristic permanent symptoms of the disease. Although this pathology is not very common in the general practice, it is necessary to emphasize the importance of the dentist playing a multidisciplinary role in the treatment of these patients. This narrative review aims to describe the oral and maxillofacial manifestations and the dental management of patients with scleroderma.
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La morfea es una rara enfermedad fibrosante de piel y tejidos subyacentes que aparece a cualquier edad, con más frecuencia en mujeres. Es de etiología autoinmune, benigna, con remisión espontánea o recidivante crónica; y se caracteriza por la aparición de áreas de piel esclerosadas, únicas o múltiples, redondeadas o lineales, asintomáticas, de evolución crónica y sin afección sistémica. Se presentó un paciente en edad pediátrica con esta enfermedad, cuya clínica, examen diagnóstico e histología fueron compatibles con la enfermedad de morfea lineal que, a pesar del retraso en su diagnóstico una vez iniciado el tratamiento inmunosupresor presentó una evolución favorable, simultáneamente se le realizó fisioterapia.
Morphea is a rare fibrosing disease of the skin and underlying tissues which is more common in women and can appear at any age. It is an autoimmune and benign disease with spontaneous remission or chronic relapsing course; it is characterized by the appearance of single or multiple, rounded or linear, asymptomatic, chronically evolving sclerosed skin areas without systemic involvement. We present a female pediatric patient with this disease, whose symptoms, diagnostic examination and histology were compatible with linear morphea that, despite the delay in its diagnosis, she had a favorable evolution once immunosuppressive treatment was started, and physiotherapy was simultaneously performed.
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Esclerodermia Localizada , Saúde da CriançaRESUMO
El síndrome SAPHO es una enfermedad autoinflamatoria crónica que engloba una serie de manifestaciones cutáneas y osteoarticulares que incluyen sinovitis, acné, pustulosis, hiperostosis y osteítis. Se presenta el caso de un paciente masculino de 18 años de edad, con antecedente de esclerodermia localizada diagnosticada por biopsia hace 3 años, quien debutó con lesiones cutáneas y osteoarticulares 6 meses después de suspender tratamiento con metotrexate y colchicina, para dicho antecedente. Se reportó una resonancia magnética sugestiva de una sacroileítis con presencia de edema óseo; y la gammagrafía ósea reveló zonas de captación con imagen en cabeza de toro en articulación esternoclavicular, sugiriendo síndrome de SAPHO. Se decidió reincorporar el metotrexate y colchicina, evolucionando satisfactoriamente. No se encontraron reportes similares concomitando estas dos entidades que tienen como punto en común el tratamiento con los mismos fármacos.
SAPHO syndrome is a chronic autoinflammatory disease that encompasses a series of cutaneous and osteoarticular manifestations that include synovitis, acne, pustulosis, hyperostosis, and osteitis. We present the case of an 18-year-old male patient, with a history of localized scleroderma diagnosed by biopsy 3 years ago, who debuted with skin and osteoarticular lesions 6 months after discontinuing treatment with methotrexate and colchicine, for said history. An MRI suggestive of sacroiliitis with the presence of bone edema was reported, and bone scintigraphy revealed uptake areas with a bull's head image in the sternoclavicular joint, suggesting SAPHO syndrome. It was decided to reincorporate methotrexate and colchicine, evolving satisfactorily. No similar reports were found coexisting with these two entities that have treatment with the same drugs as a point in common.
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A esclerodermia localizada (morfeia linear) é uma doença rara que causa distrofia e assimetria faciais em mulheres jovens. É relatado o caso de uma paciente de 20 anos que foi tratada com enxertos compostos de gordura. Após a coleta do lipoaspirado de gordura subcutânea profunda, a microgordura e a nanogordura não filtrada foram processadas separadamente para formar enxertos compostos de gordura. Estes foram meticulosamente transplantados em camadas, para reconstruir o subcutaneo da face da paciente. Após um ano, observou-se boa pega de gordura com correção bem-sucedida da assimetria e restabelecimento do equilíbrio facial.
Localized scleroderma (linear morphea) is a rare disease that causes facial dystrophy and asymmetry in young women. We report the case of a 20-year-old female patient who was treated with novel composite fat grafts. After extracting lipoaspirate from deep subcutaneous fat, the microfat and the unfiltered nanofat were processed separately, and amalgamated, obtaining composite fat grafts. These were meticulously transplanted layer by layer to rebuild the subcutaneous tissue of the patient's face. After one year, a good volume of fat retention was observed with successful correction of asymmetry and restoration of facial balance.
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The positive role of hospice social work in responding to the needs of terminal cancer patients and their families has been highlighted, and has also triggered discussions on localized ethical issues. Taking the casework of terminal cancer patients as an example, this paper analyzed the specific manifestations and causes of ethical dilemmas in the practice of hospice social work, and then provided ideas for coping with them. It was found that hospice care casework workers face four ethical dilemmas, including quality of life dilemmas, emotional boundaries dilemmas, power relationship dilemmas, and cultural respect dilemmas. Service managers can enhance the ethical issue response ability of front-line workers by improving the collaborative service mechanism between medical and social institutions, constructing a whole-process professional supervision system, conducting skills training for caring social workers, and expanding patient and family life education services. The ethical dilemmas in hospice social work service are localized, and will affect the service effect. The response to these issues requires the joint participation of the government, society, universities, hospitals, and front-line social workers.
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Objective:To analyze the biochemical failure rate and its predictive factors after radical prostatectomy (RP) in patients with high-risk localized prostate cancer.Methods:The data of 166 patients with high-risk localized prostate cancer who underwent RP surgery in Peking university cancer hospital from January 2015 to November 2021 were retrospectively reviewed. The average age was 65.4±6.2 years old, and the average body mass index (BMI) was 24.86±3.23 kg/m 2. The median prostate-specific antigen (PSA) was 19.84 (10.98, 44.47) ng/ml, PSA density was 0.68 (0.34, 1.32)ng/ml 2, and prostate volume was 31.20 (25.58, 40.23) ml. Biopsy pathology Gleason score according to the International society of Urological Pathology(ISUP) grade group: 18 cases of group 1, 33 cases of group 2, 30 cases of group 3, 51 cases of group 4, and 33 cases of group 5, 1 case was unknown. The percentage of puncture positive needles was (55.4±25.7)%, and the largest linear length of positive lesions was 80.0% (60.0%, 90.0%). Preoperative clinical stage : 14 cases in ≤T 2b stage, 117 cases in T 2c stage, 13 cases in T 3a stage and 22 cases in ≥T 3b stage; 157 cases in N 0 stage, 9 cases in N 1 stage. One hundred and three patients (62.0%) were assessed by traditional imaging and 63(38.0%) were assessed by PSMA PET-CT. The patients underwent laparoscopic radical prostatectomy. 64 patients (38.6%) received neoadjuvant therapy, including 37 received neoadjuvant therapy for 1-3 months, 23 for 4-6 months and 4 for over 6 months. The postoperative pathological characteristics, treatment and prognosis of the patients were analyzed. The primary endpoint was biochemical failure, including biochemical persistence(BCP, defined as PSA≥0.1ng/ml at 4-6 weeks after operation, and confirmed by re-examination at least 1 week interval) and biochemical recurrence(BCR, PSA falling below 0.1ng/ml after operation and then rising ≥0.2 ng/ml without adjuvant therapy or after the end of adjuvant treatment). Results:Compared with preoperative clinicopathological characteristics, 48(28.9%) cases had postoperative pathological ISUP upgrade, 98 (59.0%)cases had T stage upgrade, and 13 (7.8%) cases had N stage upgrade. The rate of positive margins was 53%, and apex margin was the most common positive site (65.9%). The postoperative PSA in 114 patients (68.7%) decreased to less than 0.1ng/ml, of which 74 patients didn't receive the therapy and 40 patients received adjuvant therapy. 52 patients (31.3%) had postoperative PSA more than 0.1ng/ml and among them, 51 cases received salvage treatment. 5 patients (3.0%) underwent PSA progression during adjuvant or salvage endocrine therapy and were considered to have castration resistance. After a median follow-up time of 25.5 (12.0, 40.0) months, 78 patients (48.4%, 78/161) experienced biochemical failure, including 49 BCP and 29 BCR, the median time of biochemical failure was 30.0 (95% CI 14.5-45.5) months. Adjuvant therapy could reduce the rate of BCR (31.1% and 15.8%, P=0.08). Baseline PSA, PSA density, proportion of pathological ISUP ≥4, proportion of pathological T stage ≥T 3a, adjuvant therapy, and positive surgical margins were significantly associated with biochemical failure ( P=0.034, 0.002, 0.004, 0.025, <0.001and 0.047). Multivariate Cox regression analysis showed that adjuvant therapy ( P<0.001, OR=0.12), PSA density ( P=0.03, OR=1.19) and positive surgical margins ( P=0.034, OR=1.80) were independent factors for biochemical failure. Conclusions:Patients with high-risk localized prostate cancer have a high rate of biochemical failure after RP and need to receive RP-based multimodal therapy. Adjuvant therapy, PSA density and positive surgical margins are independent factors associated with postoperative biochemical failure.
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Objective:To investigate the safety and efficacy of MR-guided focused ultrasound surgery (MRgFUS) in treating localized prostate cancer.Methods:Six patients with localized prostate cancer who underwent MRgFUS treatment from August 2020 to September 2021 in Beijing Hospital were prospectively enrolled in this study. The patients were all over 18 years old, with an average age of (68±10) years, and had not received any prior treatment for prostate cancer. Pretreatment pelvic MR and CT scans were performed to determine the region of treatment (ROT). Different urinary catheterization measures were given based on the location of the lesion. Under general anesthesia, the lesions were treated by MRgFUS using a specialized rectal ultrasound transducer on the treatment bed. The patients were followed up at 1, 3, and 6 months after treatment and annually thereafter. During follow-up, prostate-specific antigen (PSA) levels, pelvic MR scans, International Prostate Symptom Score (IPSS), International Index of Erectile Function-15 (IIEF-15) scores, and adverse events were assessed.Results:(1) All six patients underwent MRgFUS treatment for six lesions, with an average duration of (126±56) minutes, an average number of (7.3±3.2) focal ultrasound pulses per lesion, and an average non-perfusion volume of (3.8±1.1) cm 3, which covered the entire treatment target area. No treatment-related adverse events were reported. (2) The PSA levels at baseline, 1, 3, 6, and 12 months after treatment were (6.6±0.8), (3.6±1.3), (3.4±3.0), (2.5±1.7), and (2.3±1.8) ng/ml, respectively. PSA levels increased in 2 out of 6 patients during follow-up, and pelvic MR scan revealed recurrent lesions, while PSA levels continued to decrease in the remaining 4 patients, and pelvic MR scan were normal. (3) The IPSS scores at baseline, 1, and 3 months after treatment were 13.0 (4.0, 16.0), 10.0 (4.0, 12.0), and 5.0 (3.0, 6.0) points, respectively. For the three sexually active patients, the IIEF-15 scores at baseline were 40, 51, and 14 points, respectively, and IIEF-15 at 1 month after treatment were 9, 8, and 14 points, respectively, and at 3 months after treatment were 9, 66 and 26 points, respectively. (4) One patient was diagnosed with recurrence 10 months after treatment, and another patient was found to have a new lesion 6 months after treatment. Conclusions:MRgFUS might be a safe, non-invasive, and effective treatment for localized prostate cancer, but regular follow-up is vital for detecting tumor recurrence.
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SPeripheral sympathectomy is a procedure which has shown high rates of decreasing ischemic pain, recover functionality and wound healing, preventing the progression of the disease and further complications. We present a female patient with severe Raynaud´s phenomenon secondary to localized cutaneous systemic sclerosis complicated who presented digital ulcer treated with a sympathectomy of the radial and ulnar artery at the wrist level, undergoing post-operative follow-up.
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Humanos , Feminino , Pessoa de Meia-Idade , Doença de Raynaud/cirurgia , Simpatectomia/métodos , Artéria Ulnar/inervação , Osteomielite , Doença de Raynaud/etiologia , Fluxo Sanguíneo Regional/fisiologia , Esclerodermia Localizada , Escleroderma Sistêmico , Seguimentos , Artéria Radial/inervaçãoRESUMO
La morfea lineal en golpe de sable es una entidad dermatológica caracterizada por la inflamación esclerosante y progresiva del tejido cutáneo en la región frontal y/o en el cuero cabelludo. La cefalea y las crisis convulsivas son dos de los síntomas extracutáneos más frecuentes y están causados por el crecimiento subyacente de la lesión. Es importante un diagnóstico temprano para frenar la progresión e intentar evitar las complicaciones secundarias, principalmente neurológicas. El diagnóstico se basa en el cuadro clínico y el estudio histológico, que permite la confirmación definitiva. El tratamiento de elección es la terapia combinada con corticoides orales y metotrexato. Aun con el tratamiento farmacológico adecuado, esta patología puede presentar un curso recidivante y dejar secuelas a largo plazo. Se presenta el caso de una niña en quien se realizó un diagnóstico rápido de esta enfermedad, a pesar de un cuadro clínico inespecífico. Fue tratada con metotrexato oral con buena respuesta, sin efectos secundarios.
Linear morphea in coup de sabre is a dermatological entity characterized by progressive, sclerosing inflammation of the skin tissue in the frontal region and on the scalp. Headache and seizures are two of the most frequent extracutaneous symptoms and they are caused by the growth of the lesion towards underlying structures. An early diagnosis is important to stop cranial progression and try to avoid secondary complications, mainly neurological. The diagnosis is relied on compatible clinical signs and a pathological study that allows a definitive confirmation. The treatment of choice is combination therapy with oral corticosteroids and methotrexate. Despite an adequate pharmacological treatment, this pathology can present a recurrent course and cause long-term sequelae. We present the case of a girl who was diagnosed quickly, despite a not very noticeable symptoms. She has been treated with oral methotrexate with a good response, without side effect
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Humanos , Feminino , Criança , Esclerodermia Localizada/complicações , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/tratamento farmacológico , Metotrexato , Progressão da Doença , CefaleiaRESUMO
Purpose: To describe the role of localized debridement and instillation of amphotericin B for the management of orbital mucormycosis post COVID?19 infection with a view to avoid exenteration. Methods: The records of all patients with orbital mucormycosis post COVID?19 infection in the last 6 months from December 2020 to June 2021 were evaluated, and ten patients were identified who were successfully managed with localized debridement, that is, removing the fungal tissue and necrotic material and amphotericin B gel instillation locally. MRI scan was used to identify the area of fungal infiltration and presence of necrotic material. Early surgery in the form of transconjunctival orbitotomy was performed for disease in the infraorbital fissure area, and superior transcutaneous lid crease approach was employed for disease in the superomedial orbit or medial orbit. Most patients had lid edema, ptosis, and proptosis; this resolved with the medication. Systemic antifungals were given and the follow?up ranged from 1 to 5 months. Results: The ptosis, proptosis, and lid edema subsided in all, except in one patient who had residual ptosis and in one who had residual ophthalmoplegia. Vision deficit did not occur in any patient. All patients were successfully discharged on oral antifungal medication. Conclusion: Localized clearance of the fungal tissue and the necrotic material is a good option to avoid exenteration in cases of orbital mucormycosis, avoiding disfigurement and mental trauma to the patient
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Introdução: a hemiatrofia facial progressiva, também conhecida como síndrome de Parry Romberg, é uma forma rara de esclerodermia, cujo manejo de sequelas é desafiador. Objetivo: diante desse desafio, o objetivo deste estudo foi avaliar pela primeira vez na literatura a eficácia do preenchimento da hidroxiapatita de cálcio (CaHa) na reconstrução da mandíbula de um paciente com síndrome de Parry Romberg. Relato do caso: paciente do sexo feminino, 15 anos, com atrofia progressiva do lado direito da face por síndrome de Parry Romberg. Após o controle da doença, a principal queixa da paciente era assimetria facial, principalmente na região mandibular. Discussão: a CaHa é um preenchedor injetável biocompatível, que é gradualmente reabsorvido e substituído por estroma fibrovascular, formado principalmente por novo colágeno, em um processo que ocorre sem qualquer reação imunológica. Este perfil de segurança torna a CaHa uma boa escolha para a correção de sequelas de esclerodermia estável. Conclusão: este relato permite concluir que a biocompatibilidade do preenchimento de CaHa e a adequação para correção da mandíbula também são aplicáveis em procedimentos reconstrutivos para esclerodermia estável, de forma segura e minimamente invasiva, com ótimos resultados estéticos. O procedimento deve ser programado caso a caso, e um acompanhamento regular também é recomendado.
Introduction: Progressive Facial Hemiatrophy, also known as Parry Romberg Syndrome, is a rare form of linear scleroderma. The management of facial atrophy sequelae is challenging. Objective: This study aims to evaluate for the first time in literature the effectiveness of the calcium hydroxylapatite (CaHa) filler in the jawline reshaping of a patient with Parry Romberg Syndrome. Case report: A 15-year-old woman with progressive atrophy of the right side of the face due to Parry Romberg Syndrome. After disease control, the patient's main complaint was facial asymmetry, mainly in the jawline region. Discussion: CaHa is a biocompatible injectable filler that is gradually resorbed and replaced by fibrovascular stroma, mainly formed for new collagen, in a process that occurs without any immunological reaction. This safety profile makes CaHa a good choice for correcting stable scleroderma defects. Conclusion: This report concludes that CaHa filler biocompatibility and suitability for the jawline correction are also applicable in reconstructive procedures for stable scleroderma, safely and minimally invasively, with optimal aesthetic results. The method must be programmed case-by-case, and a regular follow-up is also recommended.
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Localized peripheral neuropathy amyloidosis is a rare disease that mainly occurred in elder people who present with focal neurological symptoms. AL is the main type of amyloid protein. Biopsy is the golden standard for diagnosis. Mass spectrometry and immunohistochemical analysis help to confirm the type of amyloid protein. This paper retrospectively analyzes the clinical and imaging data, auxiliary examinations, histological, and immunohistochemical markers. The patient, a 34-year-old woman, presented with a right neck mass and weakness of the right arm. Brachial plexus magnetic resonance imaging (MRI) showed a tumor-like lesion in the nerve root at C5 and C6 and in upper trunk. Electrophysiological studies revealed damage in the upper trunk of the brachial plexus. Positive staining with Congo red was found in brachial plexus biopsy. Mass spectrometry showed that the type of amyloid protein was AHL(G-λ). The patient underwent nerve graft for treatment. Meanwhile, literature review revealed that the average onset age of localized spinal nerve amyloidosis was 62.4 years old.The radial nerve was the most susceptible, followed by the lumbosacral plexus. Fifty percent of the type of amyloid protein is AL.Until now, no consolidated treatment is available. Here, we summarize the clinical characteristics of localized peripheral neuropathy amyloidosis in order to raise the awareness of the disease.
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ObjectiveTo observe the clinical effect of Mahuang Xixin Fuzitang combined with acupuncture and moxibustion in the treatment of localized scleroderma. MethodA total of 95 patients with localized scleroderma treated in Wuhan No. 1 Hospital from September 2019 to October 2021 were assigned into a control group （47 patients） and an observation group （48 patients） by random number table method. The control group was treated with Centella triterpenes tablets and heparin sodium cream， and the observation group was additionally treated with Mahuang Xixin Fuzitang combined with acupuncture and moxibustion. Both groups were treated for 8 weeks， and the clinical effect was compared between groups. The traditional Chinese medicine（TCM） syndrome score （local skin sclerosis， loss of skin texture， darkening of skin pigment， scaly dry skin， etc.）， serum levels of soluble interleukin-2 receptor （sIL-2R） and tumor necrosis factor-alpha （TNF-α）， erythrocyte sedimentation rate （ESR）， and eosinophil count （EO） were compared between before and after treatment as well as between groups. Additionally， the adverse reactions were recorded. ResultThe observation group had higher total effective rate than the control group ［95.83% （46/48） vs. 82.98% （39/47）， χ2=4.166 4， P<0.05］. Before treatment， the TCM syndrome score， sIL-2R， TNF-α， ESR， and EO showed no significant differences between the two groups. The 8 weeks of treatment improved the TCM syndrome score， sIL-2R， TNF-α， ESR， and EO. Moreover， the observation group was superior to the control group in these indicators （P<0.05）. During the treatment， the observation group showed 1 case of abnormal liver function and 1 case of nausea and vomiting， and the control group had 1 case of nausea and vomiting， 1 case of abnormal renal function， and 1 case of abnormal liver function. The total adverse reactions of the observation group （4.17%） and the control group （6.38%） had no significant difference （χ2=0.233 9， P=0.062 86）. ConclusionMahuang Xixin Fuzitang combined with acupuncture and moxibustion is safe and effective in the treatment of localized scleroderma.
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ObjectiveTo observe the clinical effect of Mahuang Xixin Fuzitang combined with acupuncture and moxibustion in the treatment of localized scleroderma. MethodA total of 95 patients with localized scleroderma treated in Wuhan No. 1 Hospital from September 2019 to October 2021 were assigned into a control group （47 patients） and an observation group （48 patients） by random number table method. The control group was treated with Centella triterpenes tablets and heparin sodium cream， and the observation group was additionally treated with Mahuang Xixin Fuzitang combined with acupuncture and moxibustion. Both groups were treated for 8 weeks， and the clinical effect was compared between groups. The traditional Chinese medicine（TCM） syndrome score （local skin sclerosis， loss of skin texture， darkening of skin pigment， scaly dry skin， etc.）， serum levels of soluble interleukin-2 receptor （sIL-2R） and tumor necrosis factor-alpha （TNF-α）， erythrocyte sedimentation rate （ESR）， and eosinophil count （EO） were compared between before and after treatment as well as between groups. Additionally， the adverse reactions were recorded. ResultThe observation group had higher total effective rate than the control group ［95.83% （46/48） vs. 82.98% （39/47）， χ2=4.166 4， P<0.05］. Before treatment， the TCM syndrome score， sIL-2R， TNF-α， ESR， and EO showed no significant differences between the two groups. The 8 weeks of treatment improved the TCM syndrome score， sIL-2R， TNF-α， ESR， and EO. Moreover， the observation group was superior to the control group in these indicators （P<0.05）. During the treatment， the observation group showed 1 case of abnormal liver function and 1 case of nausea and vomiting， and the control group had 1 case of nausea and vomiting， 1 case of abnormal renal function， and 1 case of abnormal liver function. The total adverse reactions of the observation group （4.17%） and the control group （6.38%） had no significant difference （χ2=0.233 9， P=0.062 86）. ConclusionMahuang Xixin Fuzitang combined with acupuncture and moxibustion is safe and effective in the treatment of localized scleroderma.
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Objective:To determine classification and clinical features of morphea.Methods:A retrospective analysis was conducted on epidemiological information about clinical manifestations of and laboratory data from 180 patients with morphea, who visited Zhongshan Hospital, Fudan University from January 2010 to July 2021. Two-independent-sample t test was used to compare the age at onset between genders, and chi-square test to analyze differences in clinical characteristics between different genders and subtypes. Results:Among the 180 patients, 123 were females and 57 were males, with a male-to-female ratio of 1∶2.16. The age at onset of morphea was 28.69 ± 17.97 years for female patients, and 29.90 ± 20.67 years for male patients. Among them, linear morphea was the most common type in this study (68 cases, 37.78%), followed by plaque morphea (63 cases, 35.00%), mixed morphea (28 cases, 15.56%) and deep morphea (21 cases, 11.67%). The disease occurred in all age groups, but the age at onset significantly varied among different clinical subtypes ( F = 5.95, P < 0.001). No significant difference was observed in the age at onset or proportion of clinical subtypes between genders ( F = 0.15, P = 0.696; χ2 =2.88, P = 0.410). Atrophoderma of Pasini and Pierini (APP) was very common (62 cases, 34.44%) in the 180 patients, which mainly manifested as plaques or linear lesions, and 26 out of 45 patients with plaque APP and 11 out of 17 with linear APP were both accompanied by other subtypes of morphea. Among the 75 patients tested for autoantibody profiles, 34 (45.33%) presented with positive results. More diverse types of autoantibodies were found in female patients compared with male patients, and antinuclear antibodies, anti-SSA and anti-SSB antibodies were the most common types. There were various types of comorbidities in female patients, but lichen sclerosus et atrophicus and vitiligo were the most common comorbidities in both genders. Conclusion:High incidence and frequent co-occurrence with other subtypes of APP may be the characteristics of Chinese patients with morphea, and it is recommended to classify morphea into plaque, linear, deep and mixed subtypes.
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We experienced a rare case of a patient who had a bicuspid aortic valve associated with acute aortic dissection limited to the sinus of Valsalva involving the left main coronary artery and acute coronary syndrome. The patient was a 36-year-old male who was identified as having a congenital bicuspid aortic valve. He visited our emergency room with a chief complaint of acute chest/back pain. He was diagnosed with acute coronary syndrome based on ECG findings and underwent an emergency coronary angiography. This test revealed filling defects at the entrance of the left main coronary artery, with aortic dissection limited to the sinus of Valsalva suspected. Emergency chest contrast-enhanced CT (ECG gated) led to his being diagnosed as having an acute aortic dissection limited to the sinus of Valsalva. After an intra-aortic balloon pump was inserted in order to maintain the coronary blood flow by surgery, emergency coronary artery bypass surgery and a modified Bentall procedure (the Carrel patch method) were carried out. His postoperative course was good and he was discharged home on the 19th disease day.
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Juvenile localized scleroderma is a rare childhood disease, the specific pathogenesis of this disease has not been fully clarified.Early recognition and timely diagnosis are crucial, early intervention is associated with good prognosis.But there is a lack of relatively reliable evaluation of disease activity and treatment response.At present, the main diagnostic basis of the disease is clinical manifestations.When there is doubt about the diagnosis, skin or subcutaneous tissue biopsy is required.In addition, high frequency Doppler ultrasound, shear-wave elastography, infrared thermal imaging, MRI, cone beam computed tomography, and dermatology evaluation tools, etc., all can reflect the patient′s condition to a certain extent.However, systematic evaluation should be improved in the process of diagnosis and treatment.In terms of treatment, hormones and methotrexate are still the first-line treatment for the disease, while evidence for second-line therapy is limited.Mycophenolate, hydroxychloroquine, cyclosporine A, tocilizumab and infliximab may play a certain role in the treatment of this disease.
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ABSTRACT A case is presented of an atypical manifestation of localized scleroderma. The patient is a 30-year-old Caucasian women with localized facial scleroderma "coup de sabre", with clinical stability foryears. She was seen in the Emergency Department due to a sudden lack of sensitivity in her left side, and was treated as a stroke. Complementary tests showed intracranial parenchymal images that were finally attributed to the localized scleroderma. After a comprehensive literature review, this presentation was more frequent than expected. This is why neurological symptoms should be carefully evaluated in these patients. This is the first stroke-mimic case reported.
RESUMEN A continuación, presentamos el caso de una paciente con una manifestación atípica para esclerodermia localizada. Mujer de 30 an˜ os con esclerodermia localizada facial en sabré¼, con estabilidad clínica de las lesiones durante an˜ os. En el 2017 acude al servicio de urgencias por un cuadro de alteración sensitiva en hemicuerpo izquierdo que fue tratada como un ictus. En los estudios complementarios se objetivaron lesiones parenquimatosas finalmente atribuibles a la esclerodermia localizada. Tras revisar la literatura, este tipo de lesiones es más frecuente de lo esperado, por lo que parece obligatorio dedicar más atención a los síntomas neurológicos en los pacientes con esclerodermia localizada. Este es el primer caso de stroke-mimic reportado.
Assuntos
Humanos , Feminino , Adulto , Esclerodermia Localizada , Doenças da Pele e do Tecido Conjuntivo , Doenças do Tecido ConjuntivoRESUMO
RESUMEN La esclerodermia cutánea localizada es una enfermedad crónica del tejido conectivo, etiología desconocida, caracterizada por áreas de piel induradas. Existen varias formas. La Morfea es una enfermedad rara con incidencia de 0,3-3 casos por 100.000 habitantes / año. Más común en mujeres, proporción 4:1 mujer/hombre. Mujer, 21 años acude al Servicio de Dermatología del Hospital de Clínicas por cuadro de 3 años de evolución de mancha roja en cadera de lado derecho que luego se vuelve marrón, aparece luego otra lesión similar en muslo y rodilla derechos con misma evolución, sin desencadenante aparente ni síntomas acompañantes. Al examen físico se observa varias placas hipocrómicas algunas con bordes eritematosos, entre 2-3 cm, límites netos y bordes irregulares distribuidos en muslo derecho. Placas hipercrómicas induradas entre 1-6 cm de diámetro, límites netos, bordes regulares en cadera y muslo derechos, y brazo izquierdo. Piel difícil de plegar. Biopsia de piel compatible con Morfea. Recibe tratamiento con hidroxicloroquina, metotrexate, corticoides tópicos, vitamina A, C, E con respuesta y evolución favorable. Esclerodermia localizada aparece en adultos entre 40- 50 años, en comparación con paciente que afectó segunda década de vida. Tronco como localización más frecuente, característica de la paciente, además en miembros superiores e inferiores. Artralgias se presentan en 44% de casos, coincidentes con la paciente. Aumento del factor reumatoideo, eosinofilia, VSG guían hacia diagnóstico de Morfea en fase activa, no apreciables en el caso. Tratamiento con metotrexate como inmunosupresor es la terapéutica con evolución favorable, así como indican estudios, complementados con hidroxicloroquina y vitaminas A y E.
ABSTRACT Localized scleroderma is a chronic connective tissue disease, unknown etiology, characterized by areas of indurated skin. There are several types. It is a rare disease with an incidence of 0.3-3 cases per 100,000 inhabitants / year. More common in Caucasian women, with a 2-4:1 female/male ratio. Woman, 21 years of age goes to the Dermatology Department of the Hospital de Clínicas for the 3-year history of the red woman on the right who later turns brown, a similar lesion appears on the right leg, apparently not trigger or symptoms companions. On physical examination, several hypochromic plaques were observed, some with erythematous borders, between 2-3 cm, net boundaries and irregular borders distributed in the right thigh. Indurated hyperchromic plates between 1 and 6 cm in diameter, net boundaries, regular edges in red and right thighs, and left side. Skin difficult to fold. Skin biopsy compatible with Morphea. Treated with hydroxychloroquine, methotrexate, topical corticosteroids, vitamin A, C, E with response and favorable evolution. Localized scleroderma appears in adults between 40-50 years, in comparison with the patient who affected the second decade of life. The trunk as the most frequent location, found in the patient, also in the upper and lower limbs. Join pains are presented in 44% of cases. Increase of the rheumatoid factor, eosinophilia, VSG guide to the diagnosis of Morphea in active phase, not appreciable in the case. Treatment with methotrexate as an immunosuppressant is the appropriate therapy, as indicated by studies, supplemented with hydroxychloroquine and vitamins A and E.