Isolated splenic sarcoidosis: a rare cause of hypercalcaemia in a patient with type 1 diabetes

A 30-year-old man was admitted to Intensive Therapy Unit (ITU) with status epilepticus secondary to severe hypoglycaemia on a background of type 1 diabetes. CT of the brain showed generalised cerebral oedema. He was successfully stepped down to a medical ward after 2 weeks. He was noted to have persistently high calcium, which required multiple doses of pamidronate. Parathyroid hormone level was appropriately low. CT of the thorax, abdomen and pelvis showed mild hepatosplenomegaly with small pathological lymph nodes throughout the abdomen. Bone marrow biopsy was unremarkable. Lymph node biopsy was difficult to achieve, and therefore a positron emission tomography scan was arranged. This showed an enlarged and hypermetabolic spleen. Differential diagnoses at this point included lymphoma and sarcoidosis. He underwent diagnostic splenectomy, and the diagnosis of sarcoidosis was confirmed histologically. Calcium level remained normal 17 months after splenectomy.

Symptomatic isolated axillary lymph node sarcoidosis: an unusual presentation

An 82-year-old woman admitted following a 4-week history of feeling unwell, abdominal pain and constipation. Initial investigations revealed severe hypercalcaemia with suppressed parathyroid hormone and elevated 1,25-dihydroxycholecalciferol. ACE was also raised. CT scans of the head, chest, abdomen and pelvis were normal. Fluorodeoxyglucose-positron emission tomography scan showed metabolically active right axillary lymphadenopathy which when biopsied under ultrasound guidance confirmed sarcoidosis. The patient was started on high-dose prednisolone with resolution of symptoms within 2 weeks. Isolated lymph node sarcoidosis is uncommon, and the reported usual sites are lymph nodes in the head and neck. Rarely has it been reported in the axillary lymph nodes.

Adult-onset perianal Langerhans cell histiocytosis presenting as pruritus ani: a case report and review of the literature

Background Langerhans cells belong to the histiocytic system and give rise to two tumors: Langerhans cell histiocytosis and Langerhans cell sarcoma. Clinical aggressiveness and degree of atypia distinguish the two neoplasms. Langerhans cell histiocytosis can infiltrate a single or multiple organ systems and particularly affects bone, skin, and lymph nodes. Perianal cutaneous Langerhans cell histiocytosis is a rare condition in adults, with 15 cases reported in the literature. Case We present the case of a 50-year-old hispanic man who presented with a 9-month history of pruritus ani and a personal history of diabetes insipidus. Punch biopsy confirmed a lesion of Langerhans cells origin but could not exclude Langerhans cell sarcoma because of limited sample size. An additional biopsy was planned as well as a positron emission tomography scan to determine the extent of disease spread. While the patient failed to follow up for repeat biopsy, the positron emission tomography scan was performed and was negative for metastatic disease. A stable perianal lesion of Langerhans cell histiocytosis with benign clinical features in a 50-year-old male despite lack of treatment is extremely rare and has not been described in the literature so far. Here, we review the presentation and workup of patients with Langerhans cell histiocytosis, review the relevant literature, and discuss treatment planning. Conclusion Perianal Langerhans cell histiocytosis is rare, and there should be a high index of suspicion with chronic or new perianal lesions, especially in a patient with a history of diabetes insipidus. It is also important to consider the patient’s full clinical course when it is not possible to reach a definitive pathological diagnosis before management.

Sarcoidosis with Rare Extrapulmonary Involvement

Sarcoidosis is a systemic granulomatous disease in which medullary involvement is a rare extrapulmonary manifestation. We present the case of a 37-year-old man with right abdominal and dorso-lumbar pain lasting for months. Computerized tomography showed renal microlithiasis and retroperitoneal, hilar and mediastinal adenopathies. Laboratory results showed an elevated erythrocyte sedimentation rate, IgG, ?2-microgobulin and angiotensin-conversion enzyme, serum calcium in the upper limit and hypercalciuria. There was a slight elevation of the CD4/CD8 ratio in bronchoalveolar lavage, without lymphocytic alveolitis. An endobronchial ganglion biopsy was inconclusive. A positron emission tomography scan demonstrated supra and infra-diaphragmatic, splenic and medullary involvement, suggesting lymphoproliferative disease (LPD). A bone marrow biopsy (BMB) revealed sarcoid-like epithelioid cell granulomas, excluding LPD. Sarcoidosis was assumed and corticosteroids were started. Although cytopenias were not present, the extensive ganglion, splenic and medullary involvement made LPD exclusion imperative, while BMB allowed for a definitive diagnosis.

The diagnostic challenge of acute Q fever endocarditis

A 79-year-old man was admitted to our ward with symptomatic heart failure 2 months after aortic valve replacement due to severe aortic stenosis. On the third day following admission, he became febrile (>38°C) while manifesting an increase in inflammatory markers. Endocarditis was suspected despite negative blood cultures. Echocardiogram (transthoracic and transesophageal) did not describe any vegetations. No hypermetabolic lesions were described on the Positron emission tomography scan. Empirical antibiotics were started but the fever persisted. Serologies revealed a Coxiella burnetii IgG phase II titre of 1:800, high erythrocyte sedimentation rate. Positive antinuclear antibodies, antibeta2 IgM and anticardiolipin supported a diagnosis of acute Q fever endocarditis. Doxycycline and hydroxychloroquine were started with total resolution of symptoms. This case illustrates the difficulty of diagnosing Q fever endocarditis during its acute phase, not only because vegetations can be minimal or absent, a challenge that is further compounded by a complex presentation of immunological markers.