Sarcoidosis-Associated Sensory Ganglionopathy and Harlequin Syndrome: A Case Report.

Background and Objectives: Sensory ganglionopathy is a rare neurological disorder caused by degeneration of the neurons composing the dorsal root ganglia. It manifests as various sensory disturbances in the trunk, proximal limbs, face, or mouth in a patchy and asymmetrical pattern. Harlequin syndrome is characterized by unilateral flushing and sweating of the face, neck, and upper chest, concurrent with contralateral anhidrosis. Here, we present and discuss a clinical case of sarcoidosis-associated ganglionopathy and Harlequin syndrome. Case presentation: A 31-year-old woman complained of burning pain in the right side of the upper chest and the feet. She also experienced episodes of intense flushing and sweating on the right side of her face, neck, and upper chest. Three years before these symptoms began, the patient was diagnosed with pulmonary sarcoidosis. On neurological examination, sensory disturbances were present. In the trunk, the patient reported pronounced hyperalgesia and allodynia in the upper part of the right chest and some patches on the right side of the upper back. In the extremities, hypoalgesia in the tips of the fingers and hyperalgesia in the feet were noted. An extensive diagnostic workup was performed to eliminate other possible causes of these disorders. A broad range of possible metabolic, immunological, and structural causes were ruled out. Thus, the final clinical diagnosis of sarcoidosis-induced sensory ganglionopathy, small-fiber neuropathy, and Harlequin syndrome was made. Initially, the patient was treated with pregabalin and amitriptyline, but the effect was inadequate for the ganglionopathy-induced pain. Therefore, therapeutic plasma exchange as an immune-modulating treatment was selected, leading to partial pain relief. Conclusions: This case report demonstrates the possible autoimmune origin of both sensory ganglionopathy and Harlequin syndrome. It suggests that an autoimmune etiology for these disorders should be considered and the diagnostic workup should include screening for the most common autoimmune conditions.

Primary Oral Presentation of Sarcoidosis in a Pediatric Patient.

Sarcoidosis is a multisystem granulomatous disease of unknown etiology that commonly affects the lungs, lymph nodes, and skin. The disease often presents in patients between the third and sixth decade and its pathology is defined by the presence of noncaseating granulomas within organs throughout the body. Oral and neurologic involvement of sarcoid is extremely rare and occurs in approximately 1% and 5% of patients with the disease, respectively. A case of sarcoidosis involving the gingiva and submandibular lymph nodes is described in a 14-year-old girl. Further neural involvement of the disease was recognized after initial biopsy examinations and systemic evaluation. This presentation is especially rare given the patient's lack of symptoms, age at diagnosis, and initial oral manifestations.

Foreign-Body Granulomata Caused by Injected Permanent Filler Masquerading as Cutaneous Sarcoidosis.

Foreign-body granuloma formation following filler injections is most commonly seen with permanent fillers; these reactions can occur years following the injections and often require either an intralesional steroid injection or surgical excision. The authors present a case of a 75-year-old woman with a history of systemic sarcoidosis previously treated with numerous immunosuppressive medications who was examined for bilateral infraorbital nodules and swelling that were unresponsive to treatment. She underwent a bilateral anterior orbitotomy through a transconjunctival approach with mass excision. The histologic analysis was consistent with foreign-body granulomata juxtaposed to implantable material, specifically ArteFill, which was injected many years prior. There were no separate noncaseating granulomas to suggest sarcoidosis as the underlying etiology. It is important to consider prior filler injections in patients with sarcoidosis who present with subcutaneous nodules as this changes management and may prevent the need for more aggressive immunosuppressive treatment.

Sarcoidosis following successful treatment of pemphigus vulgaris with rituximab: a rituximab-induced reaction further supporting B-cell contribution to sarcoidosis pathogenesis?

The anti-CD20 peripheral B-cell depleting monoclonal antibody, rituximab, has been shown to be a safe and effective treatment for refractory pemphigus vulgaris (PV), a potentially fatal autoimmune blistering disease. We report a patient who developed skin nodules and arthralgias following successful treatment of refractory PV with rituximab. Clinical, serological and histological findings were consistent with a diagnosis of sarcoidosis. The nodules promptly responded to treatment with corticosteroids, and resolved without recurrence when the medication was tapered several months later. The temporal onset of sarcoidosis following treatment with rituximab and the eventual resolution, coupled with the remarkable similarities between the B-cell immunological environment expected in our patient during the post-rituximab period and the immunological environment described in patients with idiopathic sarcoidosis, strongly implicates exposure to rituximab as the trigger for sarcoidosis development in our patient. We propose that rituximab-induced sarcoidal granulomas may be a rare adverse effect of treatment with this medication, providing further support for an important role of B cells in the pathogenesis of sarcoidosis. With better understanding of the circumstances surrounding sarcoidosis development following rituximab administration, this medication could potentially be used to induce sarcoidosis in animal research models to study the immunopathogenesis of this disease.

Osteolysis of unknown origin: a case report.

BACKGROUND: Sarcoidosis is a granulomatous disease that may affect any organ of the body. The most frequent loci of manifestation are the lungs. However, there are individual cases where bones are affected. The literature describes cases in which swelling or fistula were the first findings of a bone lesion. This is the first case reporting an osteolysis in both angles of the mandibles which led to the diagnosis of sarcoidosis with multi-organ involvement. CASE PRESENTATION: The authors present a 74 years old European female patient without previous diagnosis of sarcoidosis who presented with pain in the area of the jaw angles. There were no further clinical symptoms. Bone biopsy following radiological investigation demonstrated non-caseating granulomas consistent with sarcoidosis of the bone. Further evaluation confirmed multi-organ disease with involvement of lungs, intrathoracic lymph nodes, and the central nervous system. CONCLUSION: This case report shows that diagnosis of a severe disease can be missed if systematic clinical signs are not given. Furthermore, an accurate anamnesis and examination is required to receive an early diagnosis which often needs an interdisciplinary approach.

[Neurosarcoidosis--case report]. / Neurosarkoidoza--opis przypadku.

Sarcoidosis is a granulomatous disease of unknown cause. It could affect many organs, including nervous system. A forty two years old female patient with neurosarcoidosis is described. The disease began with the lung and thoracic lymph nods involvement. After mediastinoscopy sarcoidosis was histopathologicaly confirmed. A few months after the diagnosis of neurological symptoms appeared in the form of seizure, the vibration of the eyelids and numbness around the mouth. MRI revealed changes in the brain typical for neurosarcoidosis. After corticosteroids treatment excellent recovery was achieved, confirmed in next MRI.

Sarcoidosis and Sjögren's syndrome: clinical and salivary evaluation.

BACKGROUND: Sarcoidosis and Sjögren's syndrome are two different diseases; however, when affecting the salivary glands, both diseases exhibit similar clinical signs and symptoms, which often complicates the diagnosis. The purpose of this study was to investigate the possibility of using salivary electrophoresis to differentiate between the two diseases. METHODS: Saliva was collected from patients with sarcoidosis and patients with Sjögren's syndrome. Salivary flow rate, total protein, and electrophoretic profiles were examined. RESULTS: Mean salivary flow rate was 0.41 ± 0.07 ml/min/gland vs. 0.43 ± 0.07 ml/min/gland; total salivary protein was 130.0 ± 29.2 mg% vs. 104.0 ± 8.8 mg% for sarcoidosis vs. Sjögren's syndrome, respectively. No differences were observed in salivary flow rate, total salivary protein, or electrophoretic profile between patients with sarcoidosis and patients with Sjögren's syndrome (P = 0.768, 0.718, and 1.000, respectively). CONCLUSIONS: Salivary protein electrophoresis does not appear to be useful to differentiate between sarcoidosis and Sjögren's syndrome.

Miliary sarcoidosis with secondary Sjogren's syndrome.

We report a case of miliary sarcoidosis with secondary Sjogren's in a 45-year-old male who presented with symptoms of sicca syndrome in the form of dryness of eyes and mouth with parotid swelling. Computed tomography thorax showed mediastinal and hilar lymphadenopathy, bilateral miliary opacities in lung parenchyma. Whole body FDG PET/CT showed involvement of both parotids, liver, diffuse uptake in lungs, mediastinal and retroperitoneal lymph nodes. Patient is on treatment with prednisolone and has responded well.

Diagnostic considerations of calcified lymph nodes.

Dentists are responsible for the diagnosis of abnormalities involving both hard and soft tissues in and around the oral cavity. In addition to a clinical examination, the radiographic evaluation will often highlight abnormalities within the radiographic field. A relatively uncommon radiographic abnormality is calcification of lymph nodes, which may be associated with a number of pathologic processes, both local and systemic. We present and discuss lymph node calcifications and the diagnostic considerations associated with them in order to assist practitioners in managing their patients appropriately.

Breast-implant Related Silicone Lymphadenopathy: Asteroid Bodies do not Always Equal Sarcoidosis!

Silicone breast implants are frequently used for breast augmentation for cosmetic purposes, as well as for breast reconstruction after prophylactic or therapeutic mastectomy. Silicone lymphadenopathy is a well-known complication of silicone breast implants. Silicone droplets are present in the breast tissue through 'silicone bleeding' of the implant or because of implant rupture. These silicone particles can migrate from the breast to the regional lymph nodes. Silicone lymphadenopathy is caused by a substantial foreign body reaction against these silicone particles, and is frequently associated with asteroid body-containing multinucleated giant cells. Similar multinucleated giant cells are often observed in the capsule surrounding the silicone breast implant, and the number of associated asteroid bodies is highly variable. Here, we discuss a series of twelve women with breast implant-related asteroid bodies in their lymph nodes and/or breast tissue. This pictorial essay illustrates that the presence of asteroid bodies in a lymph node does not necessarily suggests a diagnosis of sarcoidosis. Clinical information about the patient having (or having had) silicone breast implants is often lacking. The encounter of asteroid body-containing giant cells in lymph node cytology, biopsies or resections should therefore lead to reflex clinical-pathological correlation, before establishing a final diagnosis.

[Respiratory function impairment during treatment of chronic hepatitis C with peginterferon alfa2 and ribavirin. Two cases presentation]. / Zaburzenia czynnosci ukladu oddechowego podczas leczenia przewleklego zapalenia watroby typu C pegylowanym interferonem i rybawiryna. Prezentacja przypadków.

Pulmonary complications during standard therapy of chronic hepatitis C with pegylated interferon and ribavirin are rare but connected with wide spectrum of diseases from sarcoidosis to interstitial pneumonitis. The clinical course of that complications is often serious. In this paper, presentation of two patients with chronic hepatitis C with different anamnesis was shown. In the first case, without pulmonary burden, during therapy the features of pneumonitis were developed. In the second case, in patient with sarcoidosis, the antiviral therapy was carried out without important pulmonary aggravation. In reference to these observations and based on available literature, the pathogenetic mechanisms underlying pulmonary complications during this kind of therapy were discussed. Additionally, the scheme of pulmonary diagnostic proceeding in patients certified and then treated with peginterferon and ribawirin was proposed.

[Maxillary bone sarcoidosis]. / Sarcoïdose osseuse maxillaire.

INTRODUCTION: Sarcoidosis is an unexplained systemic granulomatosis. Bone localizations of the disease are rare. We report a maxillary localization. CASE: A 35-year-old patient consulted for facial dysmorphia first observed 2 years before. This swelling was associated to nodular panniculitis lesions and xerostomia. The facial asymmetry was due to maxillary gingival and alveolar swelling. It was associated with scarring in the legs and a purple nodular facial lesion. Lip and jaw biopsies revealed epithelioid and giant cell granulomas without caseous necrosis. The panoramic dental X-ray showed diffuse horizontal alveolar ridge lysis and CT scan revealed an osteolytic lesion of the right maxilla associated to a bone-condensing lesion of the left hemi-mandible. DISCUSSION: The diagnosis of sarcoidosis is made in case of epithelioid and giant cell granulomas without caseous necrosis and the ruling out of other possible diagnoses, including tuberculosis. Bone involvement is rare; face and maxillary localization are extremely rare. The recommended treatment is corticosteroids. Facial remodeling surgery is not recommended.

Extensive bony sarcoidosis of the head and neck region: a rare presentation.

We present a rare case of sarcoidosis with extensive bony destruction of the maxillofacial and skull base bones. A 65-year-old woman was referred with an asymptomatic, non-healing dental socket. Examination revealed an oroantral fistula that was biopsied and repaired under general anaesthesia. Investigations included plain and cross-sectional imaging. Serological tests, in particular ACE, were normal. Histology showed benign florid granulomatous inflammation. At 6 months, the patient remained asymptomatic. She was re-referred 3 years later with further bony destruction of her maxilla and mandible. Repeat imaging showed intrathoracic lymphadenopathy and skull base involvement. Repeat biopsy confirmed granulomatous inflammation. Given the pulmonary, histological and radiological findings, a sarcoidosis diagnosis was made. Following multidisciplinary team meetings, the patient was treated with methotrexate and arrangements made for close monitoring. This case highlights the need for a consensus in identifying, treating and developing a follow-up protocol in such patients.

Sarcoidosis with cutaneous granulomatous vasculitis.

Ulceration of non-caseating granulomas is a rare cutaneous presentation of sarcoidosis. Granulomatous vasculitis is classically associated with Wegener's granulomatosis, lymphomatoid granulomatosis or Churg-Strauss syndrome. It is also commonly noted in pulmonary sarcoidosis, but has seldom been reported in cutaneous sarcoidosis, particularly the ulcerative variant. We present a rare case of sarcoidosis with multiple purpuric leg ulcers showing a granulomatous vasculitis histologically.

Gastric sarcoidosis: case report and literature review.

Sarcoidosis involving the gastrointestinal tract is extremely rare. Clinically recognizable gastrointestinal system involvement occurs in 0.1% to 0.9% of patients with sarcoidosis. We encountered a 22-year-old African American female admitted to Johns Hopkins Hospital (Baltimore, Maryland) for a 2-week history of fever, chills, eye pain, and abdominal pain. Her abdominal CT scan showed multiple subcentimeter retroperitoneal lymph nodes. An upper endoscopy was performed and discovered an antral nodule that measured about 7 mm and antral gastritis in which biopsies showed active chronic necrotizing granulomatous gastritis. Biopsies of the antral polyp showed focal intestinal metaplasia and active chronic necrotizing granulomatous pattern. Stains for Helicobacter pylori, acid fast, and fungi were negative. A small-bowel series showed no abnormality. Ophthalmologic evaluation revealed panuveitis with bilateral optic disc edema. The patient was later prescribed 60 mg of prednisone by mouth once a day and subsequently her abdominal pain and fever resolved during follow-up 2 months later. This literature review demonstrates the importance in the diagnosis, pathophysiology, clinical manifestations, types of gastric sarcoidosis, major endoscopic findings, and management of gastric sarcoidosis.

Systemic sarcoidosis revealed by venepunctures: a very rare but rewarding cutaneous manifestation.

A 67-year-old man was referred to our department for the onset of cutaneous lesions following venepunctures. His recent medical history included brief flu-like syndrome, persistent cough, dyspnoea, dry mouth, blurred vision and weight loss. The extensive clinical, biological and radiological check-up showed signs consistent with systemic sarcoidosis: right uveitis, hypercalcemia, renal failure, inflammatory syndrome, elevated levels of ACE, alveolitis with elevated CD4+/CD8+ T cell ratio, hilar and mediastinal lymphadenopathy, bilateral pulmonary infiltrates, mild bronchial obstruction and lowered diffusing capacity of the lungs for carbon monoxide. Multiple biopsy samples (bronchus, accessory salivary glands and one of the skin lesions) eventually confirmed the diagnosis. Corticosteroids resulted in skin lesions resolution in a few days and overall clinical, biological and lung function improvement. The infiltration of scars by granulomatous tissue is well recognised in sarcoidosis but its onset in venepuncture sites is a very rare but easily recognisable condition, which can be helpful for quick diagnosis purpose.

Atteinte osseuse axiale de la sarcoïdose.

INTRODUCTION: Sarcoidosis is a multi-systemic disease characterized by non-caseating granulomas. Bone involvement initially considered as rare and described as a peripheral osteitis of the hands and feet, has recently been reported on the axial skeleton. CASE REPORTS: We report 4 clinical observations of sarcoidosis (3 women, 1 man) with axial bone involvement located to the spine (n = 4), pelvic bone (n = 2), scapular bone (n = 2), sternum (n = 1), mandible (n = 1). Sarcoidosis was already diagnosed in 3 cases. Bone pain was the main symptom, related in 3 cases. Magnetic resonance imaging appeared to be the best imaging test Histological bone analysis revealed typical granulomatous lesions (n = 2). Treatment included corticosteroids (n = 4), hydroxychloroquine (n = 2), and methotrexate (n = 2), with a good efficacy on bone pain in symptomatic patients. CONCLUSION: These 4 cases, as well as recent literature, illustrate bone involvement of sarcoidosis on the axial skeleton. It is symptomatic in around 50% of cases but may be a source of significant disability. Differential diagnosis with neoplasm may require bone histological analysis. This condition appears to be responsive to usual treatments for sarcoidosis.