Primary Sjogren's syndrome presenting as an isolated severe autonomic dysfunction treated with steroids.

Primary Sjogren's syndrome (pSS) is an autoimmune connective tissue disorder with multisystem manifestations. We here report a previously healthy woman who presented with autonomic dysfunction in the form of severe dizziness without any apparent sensory neuropathy. Detailed history and examination revealed the signs and symptoms of Sjogren's syndrome such as constipation and dry eyes and mouth, following which anti-SSA and SSB antibodies were found to be positive. Finally, a diagnosis of pSS was established after ruling out all the other causes of autonomic dysfunction in addition to the clinical and laboratory evidence. The patient was treated with the maximum doses of midodrine and fludrocortisone, yet no progress was noticed. Hence, a trial of steroids was started and she showed a significant clinical improvement. Our patient presented with pure autonomic failure associated with Sjogren's syndrome, making it an extremely rare entity.

Unusual sites of Rosai-Dorfman disease in children: 5 years experience from tertiary care paediatric haemato-oncology centre.

Rosai-Dorfman disease (RDD) is a rare histiocytic disorder, usually presenting with massive lymphadenopathy. The involvement of extra nodal sites, bone and nodal sites like mediastinum are rare. Clinical data of three cases of extra nodal RDD who were admitted in our paediatric haemato-oncology unit were analysed in the last 5 years. The extra nodal RDD (maxillary sinus, nasal pharyngeal focus, and external auditory meatus) were diagnosed based on histopathology and positron emission tomography scan. They were treated with steroids but were refractory hence requiring salvage chemotherapy. Currently all three of them are in complete remission. Extra nodal RDD is difficult to diagnose, histopathology and radiology play an important role. Here, we present three cases, of which two were steroid-refractory which were treated with salvage chemotherapy and are in complete remission.

An Unusual Presentation of Herpes Esophagitis in an Immunocompromised Individual.

Herpes simplex infection remains the third most common cause of esophagitis following gastric reflux disease and candida infection. This disease usually occurs in immunocompromised individuals; however, it has been frequently reported in healthy individuals. We present a case of a 39-year-old man who presented to the ER with symptoms unusual of herpes esophagitis. He was presumed to be immunocompromised due to uncontrolled diabetes mellitus and chronic alcohol use. Endoscopy revealed features in favor of candidiasis; however, histopathology displayed characteristic features of herpes infection. Herpes esophagitis should thus be suspected in immunocompromised patients with an independent underlying pathology and treated early with antiviral agents like acyclovir to prevent impending complications.

Solitary cysticercosis of the sternocleidomastoid muscle.

Cysticercosis is a tropical infection caused by the larval form of Taenia solium and is usually known to affect the central nervous system. We report a young man who presented with a swelling in the neck and was treated with surgical excision and antihelminthics.

Laparoscopic management of gastric volvulus, diaphragmatic eventration and wandering spleen in a child.

Gastric volvulus (GV) and wandering spleen (WS) associated with eventration of diaphragm share a common pathological cause of absence or laxity of intraperitoneal ligaments. We herein report a rare case of a 13-year-old child presenting with an acute GV, WS, diaphragmatic eventration and an ectopic ascended kidney managed with a laparoscopic approach.