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JAMA Dermatology Mar 2020Necrobiotic xanthogranuloma (NXG) is a non-Langerhans cell histiocytosis classically associated with paraproteinemia attributable to plasma-cell dyscrasias or...
IMPORTANCE
Necrobiotic xanthogranuloma (NXG) is a non-Langerhans cell histiocytosis classically associated with paraproteinemia attributable to plasma-cell dyscrasias or lymphoproliferative disorders. Despite the morbidity of NXG, the literature is limited to case reports and small studies, and diagnostic criteria are lacking.
OBJECTIVE
To evaluate the characteristics of NXG and propose diagnostic criteria.
DESIGN, SETTING, AND PARTICIPANTS
This multicenter cross-sectional study was conducted at tertiary academic referral centers and followed by a systematic review and a consensus exercise. The multicenter cohort included patients with NXG diagnosed at the Brigham and Women's and Massachusetts General Hospitals (2000-2018), the University of Iowa Hospitals and Clinics (2000-2018), and the University of Pennsylvania Health System (2008-2018). The systematic review was conducted in 2018 and included patients with NXG identified in the Cochrane, Ovid EMBASE, PubMed, and Web of Science databases. The consensus exercise was conducted by 8 board-certified dermatologists to identify diagnostic criteria.
MAIN OUTCOMES AND MEASURES
Demographic factors, comorbidities, clinical features, and treatment response.
RESULTS
Of 235 included patients with NXG (34 from the multicenter cohort and 201 from the systematic review results), the mean (SD) age at presentation was 61.6 (14.2) years; 147 (62.6%) were female. Paraproteinemia was detected in 193 patients (82.1%), most often IgG-κ (117 patients [50.0%]). A malignant condition was detected in 59 patients (25.1%), most often multiple myeloma (33 patients [14.0%]). The overall rate of paraproteinemia and/or a malignant condition was 83.8% (197 patients). In the multicenter cohort, evolution of paraproteinemia into multiple myeloma was observed up to 5.7 years (median [range], 2.4 [0.1-5.7] years) after NXG presentation. Cutaneous lesions consisted of papules, plaques, and/or nodules, typically yellow or orange in color (113 of 187 [60.4%]) with a periorbital distribution (130 of 219 [59.3%]). The eye was the leading site of extracutaneous involvement (34 of 235 [14.5%]). In the multicenter cohort, intravenous immunoglobulin had the best treatment response rate (9 of 9 patients [100%]), followed by antimalarial drugs (4 of 5 patients [80%]), intralesional triamcinolone (6 of 8 patients [75%]), surgery (3 of 4 patients [75%]), chemotherapy (8 of 12 patients [67%]), and lenalidomide or thalidomide (5 of 8 patients [63%]). The consensus exercise yielded 2 major criteria, which were (1) clinical and (2) histopathological features consistent with NXG, and 2 minor criteria, consisting of (1) paraproteinemia, plasma-cell dyscrasia, and/or other associated lymphoproliferative disorder and (2) periorbital distribution of cutaneous lesions. In the absence of foreign body, infection, or another identifiable cause, fulfillment of both major and at least 1 minor criterion were proposed to establish the diagnosis of NXG.
CONCLUSIONS AND RELEVANCE
Necrobiotic xanthogranuloma is a multisystem disorder associated with paraproteinemia and malignant conditions. The proposed diagnostic criteria may advance clinical research and should be validated.
Topics: Aged; Cohort Studies; Cross-Sectional Studies; Female; Humans; Male; Middle Aged; Multiple Myeloma; Necrobiotic Xanthogranuloma; Paraproteinemias; Retrospective Studies
PubMed: 31940000
DOI: 10.1001/jamadermatol.2019.4221 -
The Netherlands Journal of Medicine Sep 2009
Topics: Adolescent; Diagnosis, Differential; Edema; Female; Herpesvirus 4, Human; Humans; Infectious Mononucleosis; Orbital Diseases
PubMed: 19767662
DOI: No ID Found -
International Journal of... Jan 2015To review radiographic studies of pediatric patients presenting with periorbital infections and to evaluate sinonasal anatomical factors and clinical course related to...
OBJECTIVE
To review radiographic studies of pediatric patients presenting with periorbital infections and to evaluate sinonasal anatomical factors and clinical course related to this disease process.
METHODS
Retrospective study review of computed tomography (CT) scans in 100 patients less than 18 years old, admitted to a tertiary children's hospital with the diagnosis of an orbital infection. CT scans were reviewed for anatomic variants and Lund-Mackay scores were calculated. An independent chart reviews of the treatment course and need for surgical intervention was performed.
RESULTS
Of 100 patients, 67% were male, 60% had left-sided infections, and 30% of patients were treated with surgical drainage. Adenoid hypertrophy (61%), inferior turbinate hypertrophy (80%) and septal deviation (47%) were common, but did not show statistical correlation with the need for surgical intervention. Dehiscence of the lamina papyracea was identified in 21% of patients treated without surgery and in 76% of those requiring surgery (P 0.0048). The average overall Lund-Mackay score was 11.8 and did not correlate with the need for surgical intervention.
CONCLUSIONS
To our knowledge, this is the first study to evaluate the incidence of sinonasal anatomic abnormalities in children presenting with periorbital infections. This study also demonstrated that lamina papyracea dehiscence is a common finding and is associated with higher rates of surgical intervention. Such findings may have an important role in the diagnosis, surveillance and management of sinus disease in the pediatric population.
PubMed: 26366442
DOI: 10.13188/2380-0569.1000004 -
Herpes simplex, herpes zoster and periorbital erythema flares after SARS-CoV-2 vaccination: 4 cases.Annales de Dermatologie Et de... Mar 2022
Topics: COVID-19; COVID-19 Vaccines; Chickenpox; Erythema; Herpes Simplex; Herpes Zoster; Humans; SARS-CoV-2; Vaccination
PubMed: 34711399
DOI: 10.1016/j.annder.2021.07.009 -
JAMA Dermatology Feb 2021To date, no study has characterized the mucocutaneous features seen in hospitalized children with multisystem inflammatory syndrome in children (MIS-C) or the temporal...
IMPORTANCE
To date, no study has characterized the mucocutaneous features seen in hospitalized children with multisystem inflammatory syndrome in children (MIS-C) or the temporal association of these findings with the onset of systemic symptoms.
OBJECTIVE
To describe the mucocutaneous findings seen in children with MIS-C during the height of the coronavirus disease 2019 (COVID-19) pandemic in New York City in 2020.
DESIGN, SETTING, AND PARTICIPANTS
A retrospective case series was conducted of 35 children admitted to 2 hospitals in New York City between April 1 and July 14, 2020, who met Centers for Disease Control and Prevention and/or epidemiologic criteria for MIS-C.
MAIN OUTCOMES AND MEASURES
Laboratory and clinical characteristics, with emphasis on mucocutaneous findings, of children who met criteria for MIS-C. The characterization of mucocutaneous features was verified by 2 board-certified pediatric dermatologists.
RESULTS
Twenty-five children (11 girls [44%]; median age, 3 years [range, 0.7-17 years]) were identified who met definitional criteria for MIS-C; an additional 10 children (5 girls [50%]; median age, 1.7 years [range, 0.2-15 years]) were included as probable MIS-C cases (patients met all criteria with the exception of laboratory test evidence of severe acute respiratory syndrome coronavirus 2 [SARS-CoV-2] infection or known exposure). The results of polymerase chain reaction tests for SARS-CoV-2 were positive for 10 patients (29%), and the results of SARS-CoV-2 immunoglobulin G tests were positive for 19 patients (54%). Of the 35 patients, 29 (83%) exhibited mucocutaneous changes, with conjunctival injection (n = 21), palmoplantar erythema (n = 18), lip hyperemia (n = 17), periorbital erythema and edema (n = 7), strawberry tongue (n = 8), and malar erythema (n = 6) being the most common findings. Recognition of mucocutaneous findings occurred a mean of 2.7 days (range, 1-7 days) after the onset of fever. The duration of mucocutaneous findings varied from hours to days (median duration, 5 days [range, 0-11 days]). Neither the presence nor absence of mucocutaneous findings was significantly associated with overall disease severity.
CONCLUSIONS AND RELEVANCE
In this case series of hospitalized children with suspected MIS-C during the COVID-19 pandemic, a wide spectrum of mucocutaneous findings was identified. Despite their protean and transient nature, these mucocutaneous features serve as important clues in the recognition of MIS-C.
Topics: Adolescent; COVID-19; Child; Child, Preschool; Female; Humans; Infant; Male; Mucous Membrane; New York City; Retrospective Studies; Skin Diseases; Systemic Inflammatory Response Syndrome
PubMed: 33295957
DOI: 10.1001/jamadermatol.2020.4779 -
Clinical Microbiology and Infection :... Feb 2011Skull-base osteomyelitis (SBO) occurs secondary to invasive bacterial and fungal infection. Distinguishing between fungal and bacterial aetiologies of SBO has...
Skull-base osteomyelitis (SBO) occurs secondary to invasive bacterial and fungal infection. Distinguishing between fungal and bacterial aetiologies of SBO has significant therapeutic implications. An 18-year (1990-2007) retrospective review of patients with SBO presenting to Westmead Hospital was performed. Epidemiological, clinical, laboratory and radiology data were collated. Twenty-one patients (median age 58 years) with SBO were identified: ten (48%) had bacterial and 11 (52%) had fungal SBO. Diabetes mellitus (57%) and chronic otitis externa (33%) were the most frequent co-morbidities; immunosuppression was present in five cases (24%). Cranial nerve deficits occurred in ten (48%) patients. The commonest pathogens were Pseudomonas aeruginosa (50% bacterial SBO) and a zygomycete (55% fungal SBO). Compared to bacterial SBO, fungal SBO was more frequently associated with underlying chronic sinusitis, sinonasal pain, facial/periorbital swelling and nasal stuffiness or discharge and the absence of purulent ear discharge (all p <0.05). Bacterial SBO was more frequently associated with deafness, ear pain or ear discharge (all p <0.05). Median time to presentation was longer in patients with bacterial SBO (26.3 weeks vs. 8.1 weeks, p 0.08). Overall 6-month survival was 88% (14/18 patients). All four deaths occurred in patients with fungal SBO. Immunosuppression was a risk factor for death (p <0.05). Early diagnostic sampling is recommended in patients at increased risk of fungal SBO to enable optimal antimicrobial and surgical management.
Topics: Adult; Aged; Aged, 80 and over; Bacteria; Bacterial Infections; Comorbidity; Female; Fungi; Hospitals; Humans; Male; Middle Aged; Mycoses; Osteomyelitis; Retrospective Studies; Risk Factors; Skull Base; Survival Analysis
PubMed: 20384699
DOI: 10.1111/j.1469-0691.2010.03231.x -
BMJ Case Reports Feb 2013Mucormycosis is a rare fungal infection that affects immunocompromised patients, and the rhinoorbitocerebral presentation is the most common clinical form of the... (Review)
Review
Mucormycosis is a rare fungal infection that affects immunocompromised patients, and the rhinoorbitocerebral presentation is the most common clinical form of the disease, often associated with diabetes mellitusThe treatment is complex and involves amphotericin B and surgery. Studies show increasing success without or with minimal surgeries. The authors present the case of a diabetic woman with a 1-month history of intranasal and right periorbital pain associated with progressive deficit of various cranial nerves, sudden amaurosis and homolateral ptosis. Rhizopus oryzae species was identified in pus in the nasal mucosa. She was treated with antifungal therapy and minimal surgical debridement with success. The authors decided on publication because of the rarity of this entity, alerting for the need of a high suspicion index for the diagnosis, which should be made as early as possible due to the high mortality rate, as well as presenting data about the increasing discussion of therapeutic strategies, with some new approaches that prioritise minimal surgeries.
Topics: Amphotericin B; Central Nervous System Fungal Infections; Debridement; Diabetes Mellitus, Type 2; Female; Humans; Immunocompromised Host; Middle Aged; Mucormycosis; Nose Diseases; Orbital Diseases; Rhizopus; Treatment Outcome; Turbinates
PubMed: 23389725
DOI: 10.1136/bcr-2013-008552 -
BMC Ophthalmology Mar 2022We report a rare case of orbital subperiosteal hematoma associated with frontal and ethmoidal sinusitis. Common concerns involving the orbital subperiosteal space...
BACKGROUND
We report a rare case of orbital subperiosteal hematoma associated with frontal and ethmoidal sinusitis. Common concerns involving the orbital subperiosteal space include abscess, hematoma and tumor.
CASE PRESENTATION
A patient presented to our clinic with periorbital swelling and limited extraocular muscle movement in her left eye. Computed tomography revealed a superior subperiosteal mass with frontal and ethmoidal sinusitis. We diagnosed the patient with subperiosteal hematoma and surgical evacuation was performed via superior orbitotomy. Brown serous discharge was drained and biopsy demonstrated fibrin clots. The final diagnosis was orbital subperiosteal hematoma and the patient was discharged with symptoms resolved.
CONCLUSION
Orbital subperiosteal hematoma is difficult to distinguish from abscess owing to its rarity and similar presentation. Computed tomography is helpful in diagnosis, and surgical evacuation during the early stages is essential to achieving a good outcome.
Topics: Abscess; Ethmoid Sinusitis; Female; Hematoma; Humans; Orbit; Orbital Diseases; Sinusitis
PubMed: 35241018
DOI: 10.1186/s12886-022-02324-y -
International Medical Case Reports... 2022There has been a rise in secondary invasive fungal infections reported in COVID-19 patients globally. We report the first published case of COVID-19 associated...
BACKGROUND
There has been a rise in secondary invasive fungal infections reported in COVID-19 patients globally. We report the first published case of COVID-19 associated rhino-orbital-cerebral mucormycosis in Africa in a newly diagnosed diabetic female who presented with diabetic ketoacidosis (DKA) and discuss the prevalence and risk factors of fungal co-infection with the clinical presentation, diagnosis, and management of mucormycosis in COVID-19.
CASE PRESENTATION
A 39 years old female patient was admitted to ICU with a diagnosis of severe COVID-19 and newly diagnosed diabetes mellitus (DM) with DKA based on HgbA1c of 13.8% and positive RT-PCR. The patient was treated with dexamethasone in line with evidence in the RECOVERY trial and developed right facial and orbital swelling on her second hospital day. Brain MRI showed characteristic peri-sinonasal invasion with central nervous system (CNS) involvement, features suggestive of invasive fungal infection. Despite all medical and surgical treatments including liposomal amphotericin B and debridement, the patient died within 7 days of symptom onset.
CONCLUSION
Clinicians should be aware of the potential for Rhino-Orbital-Cerebral Mucormycosis (ROCM) as a complication of COVID-19, especially in steroid taking diabetics who develop periorbital swelling and sinusitis. Timely diagnosis and multidisciplinary treatment are very critical.
PubMed: 35615254
DOI: 10.2147/IMCRJ.S364591 -
Open Forum Infectious Diseases May 2022Lemierre's syndrome (LS) is a rare and potentially fatal condition that predominantly affects young adults with oropharyngeal infection. is the usual etiology and...
Lemierre's syndrome (LS) is a rare and potentially fatal condition that predominantly affects young adults with oropharyngeal infection. is the usual etiology and classically causes internal jugular vein septic thrombophlebitis, frequently complicated by septic emboli to several organs (most classically to the lungs). Lemierre-like syndrome (LLS) describes the same constellation of symptoms and pathophysiology as Lemierre's syndrome; however, spp. are not the cause, and the source of infection may be nonoropharyngeal. We present a case with an unusual etiology of LLS: a patient with untreated preseptal cellulitis and associated methicillin-resistant (MRSA) bacteremia in the setting of injection drug use. Physical exam revealed tachypnea and rhonchi with severe periorbital and bilateral eyelid edema. Imaging demonstrated bilateral preseptal and orbital cellulitis with thrombosis of both internal jugular veins and bilateral pulmonary cavitary lesions consistent with septic pulmonary emboli. She was managed with anticoagulation and parenteral antibiotics. To our knowledge, this is the first case of LLS originating from preseptal cellulitis without evidence of preceding pharyngitis. While facial and orbital infections are rare etiologies of LLS, the potentially devastating sequelae of LLS warrant its inclusion in differential diagnoses.
PubMed: 35531375
DOI: 10.1093/ofid/ofac143