Fibroma of tendon sheath: a clinicopathological and genetic analysis of 134 cases / 中华病理学杂志

Objective: To investigate the clinicopathological features, immunophenotypes and molecular genetics of fibroma of tendon sheath (FTS). Methods: One hundred and thirty-four cases of FTS or tenosynovial fibroma diagnosed in the Department of Pathology, West China Hospital, Sichuan University, Chengdu, China from January 2008 to April 2019 were selected. The clinical and histologic features of these cases were retrospectively reviewed. Immunohistochemistry, fluorescence in situ hybridization (FISH) and reverse transcription-polymerase chain reaction (RT-PCR) were performed on the above cases. Results: There were a total of 134 cases of FTS, including 67 males and 67 females. The patients' median age was 38 years (ranged from 2 to 85 years). The median tumor size was 1.8 cm (ranged from 0.1 to 6.8 cm). The most common site was the upper extremity (76/134, 57%). Follow-up data was available in 28 cases and there was no detectable recurrence. Classic FTS (114 cases) were well-defined and hypocellular. A few spindle-shaped fibroblasts were scattered in the dense collagenous sclerotic stroma. Characteristically elongated slit-like spaces or thin-walled vessels were observed. Most of cellular FTSs (20 cases) were well-defined and the area with increased cellularity of the spindle cells coexisted with classic FTS. There were occasional mitotic figures, but no atypical mitotic figures. Immunohistochemistry was performed in 8 cases of classic FTS and most cases were positive for SMA (5/8). Immunohistochemistry was also performed in 13 cases of cellular FTS and showed 100% positive rate for SMA. FISH was conducted on 20 cases of cellular FTS and 32 cases of classical FTS. USP6 gene rearrangement was found in 11/20 of cellular FTS. Among 12 cases of CFTS with nodular fasciitis (NF)-like morphological feature, 7 cases showed USP6 gene rearrangement. The rearrangement proportion of USP6 gene in cellular FTS without NF-like morphological features was 4/8. By contrast, 3% (1/32) of the classic FTS showed USP6 gene rearrangement. RT-PCR was performed in those cases with detected USP6 gene rearrangement and sufficient tissue samples for RT-PCR. The MYH9-USP6 fusion gene was detected in 1 case (1/8) of the cellular FTSs, while no target fusion partner was detected in the classic FTS. Conclusions: FTS is a relatively rare benign fibroblastic or myofibroblastic tumor. Our study and recent literature find that some of the classic FTS also show USP6 gene rearrangements, suggesting that classical FTS and cellular FTS are likely to be at different stages of the same disease (spectrum). FISH for USP6 gene rearrangement may be used as an important auxiliary diagnostic tool in distinguishing FTS from other tumors.

Papillary thyroid carcinoma with nodular fasciitis-like stroma: a rare variant

Papillary thyroid carcinoma with desmoid-type fibromatosis (PTC-DTF) or nodular fasciitis-like stroma (PTC-NFS) is a rare morphological variant of PTC with a favorable prognosis. There is a paucity of molecular data regarding this entity. We present the case of a 20-year-old female who presented with a palpable mass over the anterior aspect of the neck for the past 3-4 months, which was diagnosed as PTC-NFS. Ultrasonogram of the neck revealed a bulky left lobe of thyroid that contained a well-defined heterogenous lesion measuring around 24 × 26 × 36 mm with involvement of the adjacent isthmus. She underwent a total thyroidectomy with central compartment lymph node dissection. Histological examination revealed a biphasic tumor with epithelial and stromal components resembling nodular fasciitis. Two dissected lymph nodes showed metastasis of the epithelial component only. On immunohistochemistry, BRAF mutant protein expression was evident in the epithelial component only, while β-catenin was negative in both the components. The histopathological diagnosis of papillary thyroid carcinoma with nodular fasciitis-like stroma was offered. Sanger sequencing revealed a BRAFV600E (c.1799T>A, Val600Glu) mutation. Post-operatively, no residual tumor was detected on ultrasound and radioiodine scans. The patient was doing well at follow-up of 9 months. PTC-NFS/DTF is a histological variant of PTC with a favorable prognosis. Our index case was associated with the BRAF mutation, which was restricted to the epithelial component. Thorough sampling of the excised specimen is essential in order not to miss the epithelial component, which, in most reported cases (including ours) appears to be small.

Risk factors and interventions: Fournier gangrene in a tertiary hospital: A 10-year experience

@#<p style="text-align: justify;"><strong>Objective.</strong> The study aimed to describe the patient demographic characteristics, clinical factors, surgical interventions, and quality of care parameters in non-survivors and survivors of Fournier's gangrene (FG).</p><p style="text-align: justify;"><strong>Methods.</strong> A total of 131 cases of Fournier's gangrene (FG) were included in a retrospective chart review in the Philippine General Hospital over 10 years using the Department of Surgery research database. We collected data for various direct and derived variables from the identified population. The primary outcome was mortality rate, while other factors studied were genital and colorectal manipulation, bowel diversion, laboratory parameters (white blood cell count, creatinine, hemoglobin).</p><p style="text-align: justify;"><strong>Results.</strong> The mortality rate was 15%. Diabetes mellitus was common comorbidity among patients with Fournier's disease. The following were statistically more common in the non-survivor group: female sex, concomitant bowel diversion surgery. Admission data in the non-survivor group showed a lower serum hemoglobin, a higher serum creatinine, and an increased percentage of patients with an abnormal white blood cell count; these did not statistically differ between cohorts, however. The median time to first antibiotic infusion was six hours. The median time to surgery was 13 hours.</p><p style="text-align: justify;"><strong>Conclusions.</strong> Among patients with Fournier's gangrene, the proportion of women and those undergoing bowel diversion was higher in those who did not survive. The time of infusion of antibiotics or time to surgery did not differ significantly between survivors and non-survivors.</p>

Nodular fasciitis: a benign mimicker of soft tissue malignancies / Fascitis nodular: un imitador benigno de las neoplasias malignas de tejidos blandos

La fascitis nodular es una masa subcutánea, benigna, autolimitada, que puede simular un sarcoma de tejidos blandos en su presentación clínica e histopatológica. Debido a que la mayoría de las descripciones de esta condición provienen de pacientes caucásicos, es necesario enfatizar su existencia en poblaciones poco representadas en la literatura. Se presenta el caso de un paciente mexicano, masculino, de 49 años, quien fue inicialmente mal diagnosticado y tratado como quiste epidérmico. Tras la recurrencia de la lesión, se realizó una adecuada técnica de biopsia y estudios de imagen, confirmando el diagnóstico de fascitis nodular. El presente reporte resalta la importancia de abordar con cautela las masas subcutáneas que puedan simular tanto tumores benignos como malignos para mejorar el pronóstico de los pacientes.

Nodular fasciitis is a benign, self-limited, subcutaneous tumor that may resemble a soft tissue sarcoma both clinically and histopathologically. Since most descriptions derive from Caucasian patients, further awareness is necessary in underrepresented populations. Here, we present a case of nodular fasciitis in a 49-year-old Mexican male who had been misdiagnosed and mistreated as an epidermal cyst. After recurrence, an adequate biopsy and imaging studies confirmed the diagnosis of nodular fasciitis. This case illustrates the importance of properly studying subcutaneous masses that can mimic a wide array of benign and malignant tumors to improve overall patient prognosis.

Fasceíte necrotizante cervical: relato de caso / Cervical necrotizing fasciitis: case report

Introdução: A Fasceíte Necrotizante Cervical é uma infecção rara, geralmente com evolução rápida e progressiva da fáscia superficial e do tecido adiposo subcutâneo. Pode se desenvolver a partir de uma infecção odontogênica que se dissemina para os planos faciais e profundos. O presente trabalho tem por objetivo relatar um caso clínico de uma paciente do gênero feminino, 49 anos de idade, atendida na Emergência do Hospital Getúlio Vargas-PE, com quadro clínico sugestivo de Fasceíte Necrotizante Cervical de origem Odontogênica. Relato de caso: O tratamento proposto foi antibioticoterapia parenteral de amplo espectro, desbridamento cirúrgico radical com instalação de dreno de penrose transfixante submandibular direito, múltiplas exodontias para remoção do foco primário da infecção, além de curativos orientado pela equipe de infectologia. Considerações finais: Excepcionalmente, as Fasceítes Necrotizantes Cervicais devem ter diagnóstico e manejo imediato, devido aos altos índices de destruição, rápida progressão e mortalidade. Nos casos de Fasceíte Necrotizante de origem odontogênica, a antibioticoterapia, remoção do(s) focos(s) de infecção, desbridamento cirúrgico e monitoramento intensivo constituem a terapêutica adequada... (AU)

Introduction: Cervical Necrotizing Fasciitis is a rare infection, usually with rapid and progressive evolution of the superficial fascia and subcutaneous adipose tissue. It can develop from an odontogenic infection that spreads to the facial and deep planes. This study aims to report a clinical case of a 49-year-old female patient, seen at the Emergency Department of Hospital Getúlio Vargas-PE, with a clinical condition suggestive of Cervical Necrotizing Fasciitis of Odontogenic origin. Case report: The proposed treatment was broad-spectrum parenteral antibiotic therapy, radical surgical debridement with the installation of a right submandibular transfixing penrose drain, multiple extractions to remove the primary focus of the infection, in addition to curatives guided by the infectology team. Final considerations: Exceptionally, cervical necrotizing fasciitis must have an immediate diagnosis and management, due to the high rates of destruction, rapid progression and mortality. In cases of Necrotizing Fasciitis of odontogenic origin, antibiotic therapy, removal of the foci (s) of infection, surgical debridement and intensive monitoring constitute the appropriate therapy... (AU)

Eosinophilic fasciitis: an atypical presentation of a rare disease

SUMMARY Eosinophilic fasciitis, or Shulman's disease, is a rare disease of unknown etiology. It is characterized by peripheral eosinophilia, hypergammaglobulinemia, and high erythrocyte sedimentation rate. The diagnosis is confirmed by a deep biopsy of the skin. The first line of treatment is corticotherapy. We present a rare case of eosinophilic fasciitis in a 27-year-old woman with an atypical presentation with symmetrical peripheral edema and a Groove sign. The patient responded well to treatment with corticosteroids at high doses and, in this context, was associated with hydroxychloroquine and azathioprine. After two and a half years, peripheral eosinophilia had increased, and more of her skin had hardened. At that time, the therapy was modified to include corticoids, methotrexate, and penicillamine. It is of great importance to publicize these cases that allow us to gather experience and better treat our patients.

RESUMO A fasciite eosinofílica ou doença de Shulman é uma doença rara de etiologia desconhecida. É caracterizada por eosinofilia periférica, hipergamaglobulinemia e velocidade de sedimentação eritrocitária elevada. O diagnóstico é confirmado por biópsia profunda da pele. O tratamento de primeira linha é a corticoterapia. Apresentamos um caso raro de fasciite eosinofílica numa mulher de 27 anos com uma apresentação atípica com edema periférico simétrico e sinal de Groove. A paciente respondeu bem ao tratamento com corticoides, mas em doses elevadas, e, nesse contexto, associou-se hidroxicloroquina e azatioprina. Ao fim de dois anos e meio verificou-se aumento de eosinofilia e novamente pele mais endurecida. Nessa altura alterou-se a terapêutica para corticoides, metrotexato e penicilamina. É de grande importância a divulgação desses casos que nos permitem reunir experiência e assim melhor tratar os nossos doentes.

Nodular Fasciitis on the Scalp: A Case Report and Literature Review / 대한피부과학회지

No abstract available.

Clinical observation of mild to moderate lumbodorsal fascitis treated with distal acupoints along meridian plus exercising combined with penetration needling on meridians of back / 中国针灸

OBJECTIVE@#To compare the therapeutic effect between distal acupoints along meridian plus exercising combined with penetration needling on meridians of back and simple penetration needling on meridians of back for mild to moderate lumbodorsal fascitis.@*METHODS@#A total of 60 patients with mild to moderate lumbodorsal fascitis were randomized into an observation group and a control group, 30 cases in each one. In the control group, penetration needling on meridians of back was applied at acupoints of the Governor vessel (TL) and the first line of bladder meridian, penetration needling was performed from the top down along the governor vessel and the first line of bladder meridian of the lumbar back pain (from one acupoint down to another acupoint), until there was no pain. In the observation group, distal acupoints along meridian plus exercising were adopted on the base of treatment in the control group. The distal acupoints along meridian plus exercising was applied at Cuanzhu (BL 2) for 30 min, at the same time, lumbar back anteflexion, hypsokinesis and turning sides were used in combination for 10 min. And then penetration needling on meridians of back was performed. The treatments were given once a day, 5 consecutive treatments a week, 1 week as a course and 2 courses were required. The visual analogue scale (VAS) score and Oswestry disability index (ODI) before treatment, after treatment and 1month in followup were observed in the two groups, and the clinical effects were compared.@*RESULTS@#Compared before treatment, the VAS score and ODI were reduced after treatment in the two groups (<0.01). The changes of the VAS score and ODI in the observation group were larger than those in the control group (<0.01, <0.05). In follow-up, the VAS score and ODI in the observation group were lower than those in the control group (<0.05). The total effective rate in the observation group was 90.0% (27/30), which was superior to 83.3% (25/30) in the control group (<0.05).@*CONCLUSION@#Distal acupoints along meridian plus exercising combined with penetration needling on meridians of back have a better therapeutic effect than simple penetration needling on meridians of back in the treatment of mild to moderate lumbodorsal fascitis.

Manejo diagnóstico-terapéutico de las lesiones mamarias atípicas / Diagnostic-therapeutic Management of Atypical Breast Lesions

Las lesiones mamarias se dividen histológicamente en dos grandes grupos, malignas y benignas. Las lesiones malignas pueden ser de origen ductal o lobulillar, siendo el carcinoma ductal infiltrante la neoplasia invasiva más frecuente. Las lesiones benignas se clasifican en no proliferativas, proliferativas sin atipias y proliferativas con atipias. Dentro de los dos últimos grupos se encuentran entidades que conllevan un alto riesgo de desarrollar carcinoma de mama, como pueden ser la hiperplasia ductal atípica, la cicatriz radial o la neoplasia lobular. Revisamos en qué consisten dichas entidades y cuáles son sus características principales en imagen, fundamentalmente en mamografía y ecografía. Si tras realizar una biopsia se obtiene uno de esos diagnósticos histológicos, es importante analizar las características imagenológicas y el tipo de procedimiento realizado (número de cilindros obtenidos, calibre de aguja...), para realizar un adecuado manejo posterior. En algunos casos la actitud a seguir será la extirpación quirúrgica completa de la lesión, mientras que en otros se podrá realizar una extirpación percutánea (mediante biopsia con aguja de vacio), o incluso seguimiento estricto por imagen. Mediante diferentes casos mostraremos nuestra experiencia y analizaremos la literatura vigente para recordar esas entidades y llegar a un consenso adecuado sobre el manejo de las mismas.

Breast lesions are divided histologically into two large groups, malignant and benign. Malignant lesions may be of ductal or lobular origin, with infiltrating ductal carcinoma being the most frequent invasive neoplasm. Benign breast lesions are classified as proliferative, proliferative without atypia and proliferative with atypia. Within the last two classifications are entities that carry a high risk of developing breast carcinoma, such as atypical ductal hyperplasia, radial scar or lobular neoplasia. We review what these entities consist of and what are their fundamental characteristics in image, fundamentally in mammography and ultrasound. When we perform one of these histological diagnoses after a biopsy, it is important to analyze the radiological characteristics and the type of procedure performed (number of cylinders, needle gauge ...) to perform an appropriate posterior management. In some cases the attitude to be followed will be the complete surgical removal of the lesion, while in others a percutaneous excision (through vacuum needle biopsy) or even strict image follow-up may be performed. Through different cases we will show our experience and analyze current literature to remember these entities and reach an adequate management consensus.

Fascitis eosinofílica: reporte de un caso / Eosinophilic fasciitis: a case report

RESUMEN La fascitis eosinofílica es una enfermedad rara del tejido conectivo que se caracteriza por induración y engrosamiento progresivo y simétrico de la piel y del tejido celular subcutáneo localizado, principalmente, en las extremidades. Además de las manifestaciones cutáneas hay compromiso articular, muscular y, en casos excepcionales, compromiso sistémico. Su diagnóstico se basa en los hallazgos clínicos, eosinofilia en sangre periférica y la toma de una biopsia profunda de piel, que incluya la fascia donde se evidencia un infiltrado compuesto por linfocitos y eosinófilos. El tratamiento de elección son los esteroides sistémicos acompanados de medicamentos inmunosupresores.

ABSTRACT Eosinophilic fasciitis is a rare connective tissue disease. It is characterised by a progressive and symmetrical induration and thickening of the skin and soft tissues of the limbs. In addition to the skin manifestations, the joints and muscles are also involved, and in rare cases there can be systemic involvement. The diagnosis of EF is based on clinical findings, the presence of peripheral blood eosinophilia, and a full-thickness biopsy that should include the deep fascia in order to show the inflammatory infiltration that is mostly composed of lymphocytes and eosinophils. Systemic corticosteroids remain the treatment of choice and may be combined with an immunosuppressive drug.

Initial Nutritional Status and Clinical Outcomes in Patients With Deep Neck Infection

OBJECTIVES: The current study aims to determine the correlation between nutritional status upon presentation and disease severity, as well as treatment and survival outcomes. METHODS: Patients who were diagnosed with deep neck infection, underwent at least one surgical drainage/debridement, and had more than 1 week of hospitalization at a tertiary medical center from 2007 to 2015 were retrospectively included. Thereafter, initial serum albumin, C-reactive protein (CRP), and body mass index (BMI) were reviewed. RESULTS: A total of 135 patients were included in the final analysis. Accordingly, the proportion of patients with simultaneous mediastinitis (21.0%), necrotizing fasciitis (12.9%), disease extent >1 cervical level (72.6%), mean CRP (22.4 mg/dL), mean length of hospitalization (25.0 days), and mean 1-week follow-up CRP (7.2 mg/dL) was significantly higher in the hypoalbuminemia group (initial serum albumin 1 cervical level (2.12), initial serum CRP over 20 mg/dL (3.79), hospitalization of more than 14 days (4.10), 1-week follow-up CRP over 5 mg/dL (3.78), and increased duration for an over 50% decrease in initial CRP (2.70) (all P < 0.05). Although intravascular albumin replenishment decreased the proportion of patients with hypoalbuminemia after 2 weeks (P < 0.05), it did not significantly predict better treatment outcomes. CONCLUSION: Among the markers reflecting an individual's nutritional state, an initial serum albumin of less than 3.0 g/dL was an independent serologic marker predicting increased disease severity and complications in patients with deep neck infection.

A Case of Acutely Developed Ischemic Fasciitis Caused by Surgical Positioning / 대한피부과학회지

No abstract available.

Proliferative Fasciitis on the Dorsum of the Hand: A Case Report / 대한피부과학회지

No abstract available.

Injerto dermo-epidérmico como tratamiento del siliconoma de extremidad inferior / Dermoepidermal graft as treatment of lower extremity siliconoma

Resumen Introducción A pesar de la evidencia sobre los malos resultados y riesgos del uso de inyecciones subcutáneas de silicona para mejorar el contorno corporal, esta técnica sigue siendo utilizada por personas no calificadas. Caso clínico Paciente de 56 años quien consultó por cuadro de celulitis en pierna izquierda. Se obtuvo el antecedente de intervención con silicona en cara, dorso de las manos, glúteos y piernas el año 2000. El cuadro progresó con múltiples abscesos en la totalidad de la pierna y sepsis. Se realizaron aseos quirúrgicos exponiendo planos fascial y muscular, se utilizó terapia local con sistemas de presión negativa y posterior injerto dermo-epidérmico (IDE). El manejo multidisciplinario se realizó en unidades críticas. Los resultados del manejo fueron satisfactorios, salvando la extremidad con cobertura total, logrando el alta luego de 4 meses. Conclusión A pesar de conocerse los peligros de la inyección de silicona, este sigue siendo un procedimiento frecuente. En nuestra paciente se manifestó como fascitis de la pierna, requiriendo tratamiento quirúrgico agresivo. Conocer esta entidad, su diagnóstico y tratamiento es fundamental para tratar pacientes con complicaciones graves.

Introduction Despite the evidence of poor performance and risks of using silicone subcutaneous injections to improve body contour, this technique is still used by untrained people, generating an iatrogenic entity with serious complications. Case report 56 year old female presented left lower extremity cellulitis. A history of previous intervention with silicone injections in face, back of hands, buttocks and legs in year 2000 was obtained. An inflammatory process progressed locally and systemically with multiple abscesses, subcutaneous thickness in the entire leg and sepsis, requiring multiple surgical procedures, reaching fascial and muscle planes exposition of the left leg. The patient underwent local therapy with negative pressure systems and subsequent dermoepidermal graft. Multidisciplinary management was performed on a critical unit. The results were satisfactory, preserving the limb with full coverage. She was discharged after four months of inpatient management. Conclusion Despite knowing the risks of silicone injection (industrial or medical) it remains a common procedure. In our patient she presented as leg fasciitis, requiring aggressive surgical treatment. Knowing this entity, its diagnosis and treatment is essential to treat patients with serious complications.

Pyomyositis and fasciitis by carbapenem resistant microorganism in a patient with severe tophaceus gout / Piomiositis y fascitis asociadas a microorganismo resistente a carbapenémicos, en un paciente con gota tofácea severa

Abstract Pyomyositis, defined as a suppurative muscle infection, is a rare entity that is classified as tropical or non-tropical according to the geographical region. The case of a patient with a history of severe tophaceous gouty arthritis, coming from tropical wet climate in Colombia, who presented with suggestive cellulitis, with a torpid evolution despite standard dual management. An associated abscess was documented, and initially abscessed cellulitis and septic arthritis were suspected, the latter was finally ruled out, once teh muscle origin of the abscess becames evident by means of computed tomography and ultrasonography. Intraoperatively, an abscess with fascia involvement and intramuscular tophaceous disease was observed. Surgical drainage and repeated washes were required. Carbapenem resistant Klebsiella pneumoniae was isolated, requiring last-line Gouty arthritis antibiotic therapy for a prolonged period of time.

Resumen La piomiositis es una infección supurativa del músculo estriado, rara, que se clasifica según la región geográfica de presentación, en tropical y no tropical. Se presenta el caso de un paciente con antecedente de artritis gotosa tofácea severa, proveniente de zona tropical, quien comienza con cuadro sugestivo de celulitis, con evolución tórpida pese a manejo estándar dual; se documenta un absceso de ubicación no clara, sospechando en principio celulitis abscedada y artritis séptica, finalmente descartándose esta última y haciéndose evidente origen muscular del absceso por medio de tomografia computarizada y ultrasonografía. Quirúrgicamente se evidenció absceso con compromiso de fascia, enfermedad tofácea intramuscular; requirió drenaje quirúrgico y lavados, documentándose germen Klebsiella pneumoniae resistente a carbapenémicos, requiriendo antibioticoterapia de última línea por un periodo prolongado.

A Case of Vibrio vulnificus Sepsis: A Potential Use of Extracorporeal Membrane Oxygenation

Necrotizing fasciitis caused by Vibrio vulnificus can rapidly progress to septic shock and death. Hence, early surgical debridement of the involved tissue is vital. However, this can be a challenging task due to the coagulopathy and unstable conditions often associated with these patients. Herein, we present a patient with necrotizing fasciitis caused by V. vulnificus who received extracorporeal membrane oxygenation (ECMO) support for refractory hypotension. After initiating ECMO, his vital signs stabilized, and lactate, C-reactive protein, and procalcitonin levels continued to decrease. He underwent several rounds of surgical debridement and vacuum-assisted drainage on both lower legs. On ECMO day 15, he was successfully weaned off the device and his condition was uneventful for several days. However, on the 24th day of intensive care unit (ICU), he was again placed on ECMO due to clinical deterioration. On ICU day 32, he underwent bilateral below-knee amputations due to delayed wound healing. Unfortunately, he subsequently developed multi-organ failure and died. Nonetheless, this case is instructive regarding the potential use of ECMO. We suggest that ECMO could provide the necessary time for sepsis patients to undergo aggressive medical and surgical interventions.

Clinical Guidelines for the Antibiotic Treatment for Community-Acquired Skin and Soft Tissue Infection

Skin and soft tissue infection (SSTI) is common and important infectious disease. This work represents an update to 2012 Korean guideline for SSTI. The present guideline was developed by the adaptation method. This clinical guideline provides recommendations for the diagnosis and management of SSTI, including impetigo/ecthyma, purulent skin and soft tissue infection, erysipelas and cellulitis, necrotizing fasciitis, pyomyositis, clostridial myonecrosis, and human/animal bite. This guideline targets community-acquired skin and soft tissue infection occurring among adult patients aged 16 years and older. Diabetic foot infection, surgery-related infection, and infections in immunocompromised patients were not included in this guideline.

Eosinophilic fasciitis

Abstract Eosinophilic fasciitis is a rare sclerodermiform syndrome of unknown etiology. It is characterized by the thickening of the muscular fascia and subcutaneous tissue, with a variable infiltration of eosinophils. Peripheral eosinophilia, poly or monoclonal hypergammaglobulinemia and increased erythrocyte sedimentation rate can be seen. Clinical features begin acutely, with local edema and a painful and symmetrical stiffening of the limbs, progressing rapidly to fibrosis, which can limit joint movements. Some cases have a history of strenuous physical exercise or trauma. The diagnosis is confirmed by a deep skin biopsy. Glucocorticoids in high doses is the treatment of choice. We report a typical eosinophilic fasciitis case with peripheral eosinophilia and dramatic response to pulse therapy with methylprednisolone.

Three Cases of Periorbital Nodular Fasciitis

PURPOSE: Herein, we report 3 cases of nodular fasciitis presenting with periorbital mass. CASE SUMMARY: An 18-year-old male presented with a mass in his left lateral temple area 3 months in duration. On physical examination, a subcutaneous mass approximately 20 mm in size was palpable with upper eyelid swelling. Orbital magnetic resonance imaging (MRI) showed T1 isointensity and T2 hyper intensity and a well-circumscribed enhancing lesion. A 36-year-old male presented with a 6-month history of a bump under his right upper eyelid. External examination revealed a 15 mm-sized subcutaneous mass in the right central sub-brow area. Orbital computed tomography showed a homogenous, well-circumscribed mass with moderate enhancement. A 3-year-old boy presented with a mass in his right upper eyelid 4 months in duration. A subcutaneous mass approximately 10 mm in size was palpated at the medial superior orbital rim. Orbital MRI revealed an enhanced mass of irregular shape. Surgical excision was performed for all cases. Histopathological examination showed pathognomonic proliferation of spindle cells and immunohistochemical stains showed the spindle cells were positive for smooth muscle actin, negative for S-100 and negative for CD34, consistent with nodular fasciitis. Recurrence of the tumors after excision in the 3 cases was not observed after 4 months, 3 months and 48 months, respectively. CONCLUSIONS: Nodular fasciitis is rare but can occur at the periorbital region. In particular, nodular fasciitis should be considered as a differential diagnosis of a subcutaneous mass short in duration in children or young adults.

Nodular Fasciitis of External Auditory Canal

Nodular fasciitis is a pseudosarcomatous reactive process composed of fibroblasts and myofibroblasts, and it is most common in the upper extremities. Nodular fasciitis of the external auditory canal is rare. To the best of our knowledge, less than 20 cases have been reported to date. We present a case of nodular fasciitis arising in the cartilaginous part of the external auditory canal. A 19-year-old man complained of an auricular mass with pruritus. Computed tomography showed a 1.7 cm sized soft tissue mass in the right external auditory canal, and total excision was performed. Histologic examination revealed spindle or stellate cells proliferation in a fascicular and storiform pattern. Lymphoid cells and erythrocytes were intermixed with tumor cells. The stroma was myxoid to hyalinized with a few microcysts. The tumor cells were immunoreactive for smooth muscle actin, but not for desmin, caldesmon, CD34, S-100, anaplastic lymphoma kinase, and cytokeratin. The patient has been doing well during the 1 year follow-up period.