Bilateral Retinal Vasculitis in Association with Systemic Sclerosis sine Scleroderma. A case report and brief review of the literature.

Abstract Background: Systemic sclerosis sine scleroderma is a very rare subset characterized by the total or partial absence of cutaneous manifestations of systemic sclerosis with the occurrence of internal organ involvement and serologic abnormalities. The purpose of this article is to report a case showing a very rare association of bilateral retinal vasculitis (RV) and systemic sclerosis sine scleroderma (ssSSc). We show the description of a case report and revision of other medical histories, prospective and retrospective studies. Findings: A 40-year-old woman presented at our Hospital for blurry vision with her left eye (OS). Her best-corrected visual acuity was 20/20 in both eyes. Funduscopy examination showed bilateral occlusive vasculitis with proliferative retinopathy and vitreous hemorrhages. Retinography showed temporary sheathed and some occluded veins, surrounded by large number of retinal hemorrhages in both eyes. In fluorescein angiography were found areas of not perfused retina, also with telangiectasia and staining of retinal vessel’s walls. We found isolated microaneurysms in these ischemic areas. The ophthalmological treatment was confluent photocoagulation of the ischemic retina of both eyes, and vitrectomy of the right eye to treat his vitreous hemorrhage. The systemic treatment was corticosteroids and Adalimumab during two years; afterwards the stability was achieved. There was a lack of response to cyclosporine and azathioprine. The diagnosis comes years later after presenting: anticentromere antibodies, scleroderma pattern on capillaroscopy and esophageal manometry showing esophageal dysmotility with absent contractility.Conclusion: We report a rare association of RV and sclerosis sine scleroderma. RV can precede the development of ssSSc by several years.

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Acquired retinoschisis and vitreous hemorrhage as unusual findings in choroideremia: Case report

European Journal of Ophthalmology ◽

10.1177/1120672120946576 ◽

2020 ◽

pp. 112067212094657

Author(s):

Dario Pasquale Mucciolo ◽

Vittoria Murro ◽

Dario Giorgio ◽

Andrea Sodi ◽

Ilaria Passerini ◽

...

Keyword(s):

Case Report ◽

Retinal Pigment Epithelium ◽

Pigment Epithelium ◽

Retinal Pigment ◽

Vitreous Hemorrhage ◽

Advanced Stage ◽

Vascular Abnormalities ◽

Diagnostic Difficulties ◽

The Right

Purpose: To report a case of choroideremia characterized by peripheral retinoschisis with vascular abnormalities and vitreous hemorrhage. Observations: A 58-year-old man affected by advanced-stage choroideremia was diagnosed with peripheral retinoschisis in both eyes. Vitreous hemorrhage was present in the right eye with a peculiar clot-like lesion at the periphery. At the 1-year follow-up, the vitreous hemorrhage had reabsorbed and the vascular clot-like lesion in the periphery had almost completely disappeared. Conclusion and importance: We have reported fundoscopic and OCT features of peripheral-acquired retinoschisis with vascular abnormalities in a patient with choroideremia. OCT examination is extremely useful in clinical evaluation of the peripheral retinal alterations in these cases, where the absence of the retinal pigment epithelium and the choriocapillaris pose many diagnostic difficulties.

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PSORIATIC ARTHRITIS AND SYSTEMIC SCLEROSIS, A RARE ASSOCIATION: A CASE REPORT

10.47660/cbr.2020.17371 ◽

2021 ◽

Author(s):

Gabriela Almeida Barbosa ◽

Thyago Talles de Almeida Santana ◽

Maria Roberta Melo Pereira Soares ◽

Alessandra de Sousa Braz ◽

Eutília Andrade Medeiros Freire ◽

...

Keyword(s):

Case Report ◽

Systemic Sclerosis ◽

Psoriatic Arthritis ◽

Rare Association

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SYSTEMIC SCLEROSIS AND PULMONARY SARCOIDOSIS, A RARE ASSOCIATION: A CASE REPORT

10.47660/cbr.2021.2189 ◽

2021 ◽

Author(s):

Valéria Bezerra da Silva ◽

Tâmara Melo Santos ◽

Gabriela Almeida Barbosa ◽

Robson Antônio Gonçalves ◽

Maria Roberta Mele Pereira Soares ◽

...

Keyword(s):

Case Report ◽

Systemic Sclerosis ◽

Pulmonary Sarcoidosis ◽

Rare Association

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Basal Cell Carcinoma Involving a Ventriculoperitoneal Shunt: Case Report and Literature Review

International Surgery ◽

10.9738/intsurg-d-14-00271.1 ◽

2017 ◽

Vol 102 (1-2) ◽

pp. 77-80

Author(s):

David S. Sparks ◽

Brandon M. Adams ◽

Michael Wagels

Keyword(s):

Case Report ◽

Basal Cell Carcinoma ◽

Cell Carcinoma ◽

Surgical Management ◽

Basal Cell ◽

Subcutaneous Tissue ◽

Rare Association ◽

Synthetic Materials ◽

Pathologic Assessment ◽

The Right

Ventriculoperitoneal shunts (VPSs) are commonly used for the management of raised intraventricular pressure, especially in the context of hydrocephalus. Malignant invasion involving a VPS is an exceedingly rare association, only reported three times in the literature to date. We present the involvement of a VPS with a recurrent basal cell carcinoma (BCC), which has not been described previously. It was theorized that previously breached tissue planes associated with the VPS tract might facilitate local spread of the BCC. As such, this case represented an exceedingly rare association that has important conceptual implications for definitive surgical management. A 51-year-old paraplegic man with spina bifida and hydrocephalus, who had been immunosuppressed for 7 years following a renal transplantation, presented to our clinic with a recurrent BCC involving an inactive VPS in the right neck. Surgical management involved an excision of the scar with a peripheral margin of 1 cm, along with removal of the involved shunt tract and intraoperative pathologic assessment. Definitive histopathology revealed a focus of infiltrating sclerosing BCC involving the subcutaneous tissue and abutting the VPS. As the scope for synthetic materials in surgery continues to expand, so does the multitude of interesting complications that appear to arise from interactions between host tissue and foreign bodies. This case report describes a fascinating relationship between a recurrent BCC and a VPS, providing a guide for future management to ensure adequate surgical clearance. The case would be of interest to head/neck surgeons, neurosurgeons, and plastic surgeons.

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Síndrome de superposição e associação de paniculite e amiloidose – Relato de Caso - Overlap syndrome and association of panniculitis and amyloidosis - case report

Revista Educação em Saúde ◽

10.29237/2358-9868.2017v5i1.p78-80 ◽

2017 ◽

Vol 5 (1) ◽

pp. 78

Author(s):

Tatiana De Souza Pina Lobo ◽

Luciana Rodrigues de Alencar ◽

Thiago De Carvalho Barros ◽

Adriana Guimarães de Farias ◽

Nilzio Antônio da Silva ◽

...

Keyword(s):

Rheumatoid Arthritis ◽

Case Report ◽

Systemic Sclerosis ◽

Skin Biopsy ◽

Clinical Case ◽

Overlap Syndrome ◽

Antiinflammatory Drugs ◽

Amyloid Deposits ◽

Artrite Reumatoide ◽

The Right

ResumoObjetivo: Descrever um caso clínico de uma mulher de 46 anos, com superposição de esclerose sistêmica e artrite reumatoide, que desenvolve paniculite septal e amiloidose. Relato do caso: Paciente do gênero feminino, 46 anos, com diagnóstico de síndrome de superposição (esclerose sistêmica e artrite reumatóide) há oito anos, evolui com dor e aumento de volume das pernas. Aventada a hipótese de paniculite, a paciente foi medicada com anti-inflamatórios, não obtendo melhora. Submetida à biópsia de pele da perna direita, o quadro histopatológico foi compatível com amiloidose e discreta paniculite septal. Considerações finais: A presença de paniculite, de difícil resposta ao tratamento antiinflamatório, deve suscitar a pesquisa de doenças infiltrativas, tais como a amiloidose. A presença de depósito amiloide na derme implica na investigação de doença sistêmica.Palavras-chave:Esclerose sistêmica. Artrite reumatoide. Paniculite. Amiloidose.AbstractObjective: To describe a clinical case of a 46-year-old woman, with overlap of systemic sclerosis and rheumatoid arthritis who develop panniculitis septal and amyloidosis. Case report: A 46-year-old female patient with a diagnosis of overlap syndrome (systemic sclerosis and rheumatoid arthritis) eight years ago evolves with pain and enlargement of the legs . After the hypothesis of panniculitis, the patient was medicated with antiinflammatory drugs and did not obtain improvement. Subjected to skin biopsy of the right leg, histopathological findings were compatible with amyloidosis and discrete panniculitis septal. Final considerations: The presence of panniculitis, which is difficult to respond to antiinflammatory treatment, should lead to the investigation of infiltrative diseases, such as amyloidosis. The presence of amyloid deposits in the dermis implies the investigation of systemic disease.Keyword:Systemic sclerosis. Rheumatoid arthritis. Panniculitis. Amyloidosis.

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Osteopetrosis in a Patient of Systemic Sclerosis Sine Scleroderma: A Rare Association

Indian Journal of Radiology and Imaging ◽

10.1055/s-0041-1740506 ◽

2022 ◽

Author(s):

Saroj K. Pati ◽

Praveen Raja ◽

Ajoy K. Behera ◽

T.G. Ranganath ◽

Narendra K. Bodhey

Keyword(s):

Systemic Sclerosis ◽

Connective Tissue ◽

Male Patient ◽

Connective Tissue Disorder ◽

Unknown Etiology ◽

Cutaneous Manifestations ◽

Rare Association ◽

Serologic Markers ◽

Chief Complaints ◽

Musculoskeletal Systems

AbstractSystemic sclerosis is a connective tissue disorder of unknown etiology. Although it is a multisystemic disorder, skin thickening is considered as a hallmark of the disease. It usually involves the lungs, gastrointestinal, and musculoskeletal systems. However, a rare subset of systemic sclerosis, systemic sclerosis sine scleroderma, is characterized by internal organ involvement and positive serologic markers with the total or partial absence of cutaneous manifestations. We present a rare association of osteopetrosis in a case of systemic sclerosis sine scleroderma, in a 22-year-old male patient, who presented with pulmonary symptoms as his chief complaints, unreported so far in literature.

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Progressive systemic sclerosis with unilateral osteolysis of the mandible: a unique case report and review

Jurnal Radiologi Dentomaksilofasial Indonesia ◽

10.32793/jrdi.v5i3.734 ◽

2021 ◽

Vol 5 (3) ◽

pp. 126

Author(s):

Lusi Epsilawati ◽

Chrisna Ardhya Medika ◽

Eddy Hermanto

Keyword(s):

Case Report ◽

Systemic Sclerosis ◽

Tooth Extraction ◽

Mandibular Condyle ◽

Progressive Systemic Sclerosis ◽

The Body ◽

Entire Body ◽

Posterior Teeth ◽

The Face ◽

The Right

Objectives: This case report is aimed to discuss case findings of Progressive Systemic Sclerosis (PSS), an overview of the characteristics in the form of osteolysis on one side of the mandible, and a theoretical review. Case Report: A 30-year old male patient came to an oral surgeon after tooth extraction. Clinical extraoral examination revealed hyperpigmentation on the right side of the face. A radiological study showed widening periodontal space on posterior teeth, and the angles of the mandibular arch, the jaw branch and the mandibular condyle neck were dissolved in the form of bone resorption. Conclusion: Characteristics of Progressive Systemic Sclerosis (PSS) in radiographs appear in the form of expansion of the periodontal space and osteolysis of the mandibular angle, branch, and even condyle. This disease is caused by an autoimmune disease that affects the entire body, but it can manifest on one side of the body.

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Popliteal artery thrombosis secondary to a tibial osteochondroma

VASA ◽

10.1024/0301-1526/a000101 ◽

2011 ◽

Vol 40 (3) ◽

pp. 251-255 ◽

Cited By ~ 4

Author(s):

Gruber-Szydlo ◽

Poreba ◽

Belowska-Bien ◽

Derkacz ◽

Badowski ◽

...

Keyword(s):

Case Report ◽

Magnetic Resonance Angiography ◽

Popliteal Artery ◽

Doppler Ultrasonography ◽

Histopathological Examination ◽

Previous History ◽

Plain Radiography ◽

Artery Thrombosis ◽

History Of ◽

The Right

Popliteal artery thrombosis may present as a complication of an osteochondroma located in the vicinity of the knee joint. This is a case report of a 26-year-old man with symptoms of the right lower extremity ischaemia without a previous history of vascular disease or trauma. Plain radiography, magnetic resonance angiography and Doppler ultrasonography documented the presence of an osteochondrous structure of the proximal tibial metaphysis, which displaced and compressed the popliteal artery, causing its occlusion due to intraluminal thrombosis..The patient was operated and histopathological examination confirmed the diagnosis of osteochondroma.

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