scholarly journals Lupus pernio (Besnier-Tenneson syndrome): A rare form of sarcoidosis

2018 ◽  
Vol 10 (2) ◽  
Author(s):  
Amal Hubail ◽  
Roza Belkharoeva ◽  
Natalya Tepluk ◽  
Tatyana Belerosova
Keyword(s):  
Psychological Effects ◽  
Management Approach ◽  
Cutaneous Sarcoidosis ◽  
Rare Form ◽  
Rare Presentation ◽  
Lupus Pernio ◽  
Rare Manifestation ◽  
Systemic Sarcoidosis ◽  
Delay In Treatment ◽  
Demographic Features

Lupus pernio (LP) is a chronic non-life threatining type of cutaneous sarcoidosis that can be related to chronic fibrotic sarcoidosis, hyperglobulinemia and hypercalcemia. The aim of this case report is to evaluate the clinical and demographic features of cutaneous sarcoidosis mainly presenting with a rare manifestation of LP. In this paper we report a case of systemic sarcoidosis presenting with LP and a review of the available literature. LP is a rare presentation with infiltrated erythematoviolaceous plaques affecting the nose. We address the main management approach, and possible association with an underlying systemic sarcoidosis. LP is a rare but chronic manifestation of systemic sarcoidosis that needs to be treated in order to prevent cosmetic defects and psychological effects. It is important to recognize such a condition early in order to avoid a delay in treatment and worsening of the condition, both physically and psychologically. Further research regarding the diagnostic approach and management is required to understand this condition thoroughly.

scholarly journals Manifestations of Besnier-Boeck-Schaumann sarcoidosis on skin

2020 ◽  
pp. 83-86
Author(s):  
V. G. Akimov
Keyword(s):  
Differential Diagnosis ◽  
Erythema Nodosum ◽  
Diagnosis And Treatment ◽  
Unknown Etiology ◽  
Cutaneous Sarcoidosis ◽  
Clinical Forms ◽  
Lupus Pernio ◽  
Multisystemic Disease ◽  
Nodular Sarcoidosis ◽  
Systemic Sarcoidosis

Besnier-Boeck-Schaumann sarcoidosis is a multisystemic disease of unknown etiology. Only about 25 % of patients have cutaneous involvement. Every patient with cutaneous sarcoidosis should be evaluated for possible systemic sarcoidosis. This article contains descriptions and original images of clinical forms of the disease: miliary sarcoidosis, circinate sarcoidosis, nodular sarcoidosis, angiolupoid (Brocq-Potrier), lupus pernio (Besnier-Tennesson), erythema nodosum, subcutaneous sarcoidosis (Darier-Roussy). Differential diagnosis and treatment of patients are discussed.

scholarly journals When systemic lupus erythematosus affects vision: a rare presentation of this condition

2020 ◽  
Vol 13 (1) ◽  
pp. e229382
Author(s):  
Tiago Gama Ramires ◽  
Luísa Vieira ◽  
Nuno Riso ◽  
Maria Francisca Moraes-Fontes
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Blurred Vision ◽  
Rare Presentation ◽  
Complement Components ◽  
Oral Ulcers ◽  
Dsdna Antibody ◽  
Rare Manifestation ◽  
Retinal Vascular Tortuosity

A 23-year-old woman with fever, oral ulcers, arthalgias and weight loss of 2-week duration suddenly developed blurred vision, with reduced visual acuity, cotton wool exudates and retinal vascular tortuosity. Laboratory testing revealed anaemia, lymphopaenia, positive antinuclear antibody and high anti-dsDNA antibody titre with low complement components. There was no evidence of infection, clinching the diagnosis of lupus retinopathy. Steroid therapy alone was highly effective and was also accompanied by a normalisation of haemoglobin and lymphocyte counts, after which azathioprine was added. Hydroxychloroquine was introduced after resolution of retinal changes. Immunosuppressive therapy was progressively tapered over the course of 12 months and then discontinued, and the patient remains in remission 48 months after the initial presentation. Our patient exemplifies a very rare manifestation of systemic lupus erythematosus. We emphasise the importance of its early detection and complexity of treatment in order to reduce visual morbidity.

scholarly journals Extensive erythema elevatum diutinum associated with IgA monoclonal gammopathy: a rare case report

2021 ◽  
Vol 7 (4) ◽  
pp. 593
Author(s):  
Manish Rijhwani ◽  
Divya Yadav ◽  
Manisha Nijhawan ◽  
Arvind Verma
Keyword(s):  
Monoclonal Gammopathy ◽  
Rare Case ◽  
Leukocytoclastic Vasculitis ◽  
Burning Sensation ◽  
Rare Form ◽  
Rare Presentation ◽  
Rare Case Report ◽  
Nodular Lesions ◽  
Erythema Elevatum Diutinum ◽  
Characteristic Features

<p class="abstract">Erythema elevatum diutinum (EED) is a rare form of leukocytoclastic vasculitis with an unclear pathogenesis. Almost 250 cases of EED have been reported in the literature, associated with several diseases and presented with various clinical features. Term 'diutinum' means chronic, describes one of the main characteristic features. The duration is very long, varying between one to more than 39 years. We report a rare presentation of EED in a 58-years-old man who presented with extensive and symmetrical, persistent, erythematous to violaceous   plaques over trunk and limbs and popular-nodular lesions present over pinna and dorsum of hands with burning sensation and itching over few lesions, in association with IgA monoclonal gammopathy.</p>

Systemic Sarcoidosis Revealed by Axillary Electrolysis

2015 ◽  
Vol 19 (4) ◽  
pp. 404-406 ◽  
Author(s):  
Meggie Morand ◽  
Solange Beauregard ◽  
Steve Mathieu
Keyword(s):  
Clinical Manifestations ◽  
Skin Lesions ◽  
Hair Removal ◽  
Granulomatous Disease ◽  
Cutaneous Sarcoidosis ◽  
Systemic Sarcoidosis ◽  
Histopathologic Findings

Background: Sarcoidosis is a noncaseating granulomatous disease that affects a large variety of organs and tissues. Skin sarcoidosis is commonly found in scar and tattooed tissues. Objective: To report this particular case of sarcoidosis following electrolysis hair removal. Method: We report the case of a woman who developed sarcoidosis years after axillary electrolysis hair removal. Results: The diagnosis of cutaneous sarcoidosis was suggested by the clinical manifestations and confirmed by histopathologic findings. Conclusion: Sarcoidosis should be considered during the investigation of skin lesions occurring in scar or traumatized tissue.

scholarly journals Cutaneous Sarcoidosis of the Scrotum: A Rare Manifestation of Systemic Disease

2002 ◽  
Vol 82 (3) ◽  
pp. 216-217 ◽  
Author(s):  
Susan S. McLaughlin ◽  
Allison M. Linquist ◽  
Joseph W. Burnett
Keyword(s):  
Systemic Disease ◽  
Cutaneous Sarcoidosis ◽  
Rare Manifestation

scholarly journals Subcutanaeous emphysema: An extremely rare manifestation of supraglottic squamous cell carcinoma

2021 ◽  
Vol 8 (2) ◽  
pp. 64-67
Author(s):  
Abhishek Subhash Chande ◽  
Shilpa Bawankule ◽  
Pallavi Yelne ◽  
Vinay Verma ◽  
Sujal Patel
Keyword(s):  
Emergency Department ◽  
Squamous Cell Carcinoma ◽  
Cell Carcinoma ◽  
Squamous Cell ◽  
Rare Presentation ◽  
Mass Growth ◽  
Local Extension ◽  
Supraglottic Squamous Cell Carcinoma ◽  
Rare Manifestation ◽  
Common Causes

We present the case report of a 60 years old man who presented to the emergency department with complaints of breathlessness for 2 weeks. After all common causes were ruled out, the patient was subjected to CT scan of neck in which he showed laryngeal mass growth which was perforating through the cartilage. There were a few surrounding lymph nodes and after an FNAC, it was found to be squamous cell carcinoma. The patient was referred for chemotherapy and radiotherapy at higher centre. To our knowledge, this is a very rare presentation of local extension of tumour. The patient presenting with carcinoma at a very late stage, succumbed to it 2 months later.

scholarly journals Chronic intestinal pseudo-obstruction due to β2microglobulin-amyloidosis in a patient on high-flux haemodialysis

2020 ◽  
Vol 13 (1) ◽  
pp. e232201
Author(s):  
Pieter De Mulder ◽  
Kristof Cokelaere ◽  
Wim Terryn
Keyword(s):  
Chronic Kidney Disease ◽  
Kidney Disease ◽  
Rare Disease ◽  
Amyloid Fibrils ◽  
High Flux ◽  
Rare Presentation ◽  
Visceral Organs ◽  
Intestinal Pseudo Obstruction ◽  
Delay In Treatment

Dialysis-related amyloidosis (DRA) or β2microglobulin (β2m)-amyloidosis is a disorder caused by the inability to clear a protein called β2m in patients with chronic kidney disease. It results in deposition of β2m as amyloid fibrils, most commonly in bones and joints. Infrequently, visceral organs may be involved. With modern high-flux haemodialysis, DRA has become a rare disease, yet it may occur. We present a case of DRA in an 86-year-old woman. This case is particularly notable for its rare presentation as chronic intestinal pseudo-obstruction. It is of paramount importance to recognise this entity in order to reduce delay in treatment and avoid patients being frustrated not getting a diagnosis.

A rare form of congenital high airway obstruction syndrome and a literature review of ex utero intrapartum treatment

2018 ◽  
Vol 7 (1) ◽  
Author(s):  
Seifeldin Sadek ◽  
Hajoon Chun
Keyword(s):  
Literature Review ◽  
Airway Obstruction ◽  
Mainstem Bronchus ◽  
Viable Option ◽  
Rare Form ◽  
Rare Presentation ◽  
Exit Procedure ◽  
Ex Utero Intrapartum Treatment ◽  
Rare Malformation

Abstract Objective This is a rare presentation of a fetus with a right mainstem bronchus occlusion leading to congenital high airway obstruction syndrome (CHAOS). Methods A review of 15 articles reporting on the use of the ex utero intrapartum treatment (EXIT) procedure. Results A total of 22 reported cases of CHAOS had undergone the EXIT procedure from 1994 to 2016. Seventy-seven percent of fetuses that underwent EXIT were alive at the last point of follow-up, but only 36% were alive without any sequelae. Conclusion CHAOS is a rare malformation, but after the development of EXIT, survival is a viable option. It is associated with other malformations, but its etiology is entirely unknown.

Great auricular nerve schwannoma: a rare presentation and literature review

2021 ◽  
Vol 14 (7) ◽  
pp. e242972
Author(s):  
Soo Oh ◽  
Ahmad Abou-Foul ◽  
Sanjay Patel ◽  
Paul Wilson
Keyword(s):  
Literature Review ◽  
Surgical Management ◽  
Diagnostic Challenge ◽  
The West ◽  
Rare Form ◽  
Rare Presentation ◽  
Great Auricular Nerve ◽  
Nerve Sheath ◽  
Slow Growing ◽  
Cutaneous Innervation

Head and neck schwannomas are a rare form of tumour arising from the nerve sheath. They are often slow growing and asymptomatic, posing a diagnostic challenge for clinicians. The great auricular nerve (GAN) provides cutaneous innervation to the lower pinna, ear lobule and the inferior periauricular area. Hence, surgical management of GAN schwannomas can have sensory and functional dysfunction postoperatively, necessitating good counselling and communication with the patient. We present the first documented case of GAN schwannoma in the West, with literature review and considerations for surgical management.

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