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Annular pancreas, the second most common anomaly of the pancreas, is the development of a ring of pancreatic tissue that surrounds and often embraces the duodenum. It was first described by Tiedelmann in 1818 and named “annular pancreas” by Ecker in 1862 [1]. It is usually present in childhood, with symptoms due to duodenal obstruction. In 50% of cases, clinical manifestations become visible after childhood, with abdominal pain, chronic duodenal obstruction, peptic ulceration, obstructive jaundice, pancreatitis, and pancreatic head mass [2, 3]. Therefore, duodeno-jejunostomy is usually applied to relieve strictures caused by such an annulus. Bypass surgery is the treatment of choice [4]. Primary duodenal carcinoma is rare and represents 0.3% of all gastrointestinal malignancies and 25–35% of malignant tumours of the small intestine [5, 6]. Resectability and the presence of distant metastatic disease are the strongest determinants of outcome for patients with duodenal adenocarcinoma. In cases of cancers of the first and second part of the duodenum, the most common procedure is pancreaticoduodenectomy. Despite advancements in techniques of diagnosis and resection and decreased perioperative mortality and morbidity, 5-year survival varies from 37% [7] to 57% [8]. Malignancy in the setting of annular pancreas is an unusual event, and hence only a few cases associated with pancreatic carcinoma, ampullary carcinoma, and cholangiocarcinoma have been reported [9–13]. Only 1 case of annular pancreas associated with duodenal carcinoma has been reported, but without description of the follow-up [14]. We report a case of duodenal carcinoma in a patient with an annular pancreas treated with radical surgery with complete follow-up data. A 53-year-old lady suffered from nausea, post-prandial fullness, and vomiting, and weight loss. She was diagnosed (endoscopy Figure 1, CT scan of the abdomen Figure 2) to have duodenal obstruction with gastric bezoar. The upper intestinal contrast study findings of annular filling defect in the duodenum, prestenotic dilatation, and reverse peristalsis in the proximal segment were suggestive of annular pancreas. A pathology report of the endoscopic biopsy material taken from the stenotic part of the duodenum revealed suspicion of malignancy, i.e. atypic cells. The patient was scheduled for a laparotomy with intraoperative pathological investigation of the suspected lesion, and if malignancy is proven a pancreaticoduodenectomy is anticipated. During the operation a band of fibrous (probably pancreatic) tissue of hard texture encircling the second part of the duodenum was found (Figure 3). Division of the pancreatic ring was done and the abnormal tissue sent for immediate pathology. Once malignant diagnosis was confirmed, and classical Kausch-Whiple pancreaticoduodenectomy with standard lymphadenectomy was done without perioperative complications. The resection specimen had been routinely fixed in buffered 10% formaldehyde and embedded in paraffin wax (Figure 4). Operative time was 300 min, and blood loss was 420 ml. The postoperative course was uneventful; therefore, the patient was discharged from the hospital on the sixteenth postoperative day. A definitive pathological report confirmed tubular carcinoma (G2) of the duodenum associated with the annular pancreas with no lymph node involvement (pT2N0M0) (Figures 5–8). No adjuvant chemotherapy was administered. The patient was followed-up every 3 months thereafter for the first 3 years. In the first (2008) and second year (2009) of the follow-up, abdominal CT-scan was done without signs of recurrence. After 54 months the patient became heavily jaundiced and cachectic, and was therefore hospitalised. Advanced loco-regional recurrence was found on MR cholangiogram, with no liver metastases. The patient died, and no autopsy was performed. Figure 1 Endoscopic view of the duodenum showing ulceration with duodenal stenosis at the second portion Figure 2 Computed tomography scan image revealing a significant dilatation of the stomach with bezoar and the proximal duodenum Figure 3 Intraoperative view of the annular pancreas encircling the second part of the duodenum Figure 4 Cross-sectional view showing the duodenum surrounded by the pancreas tissue (annular pancreas) Figure 5 Tubular adenocarcinoma of the duodenum. Neoplastic tubules border on normal duodenal glands (four-micron sections of representative blocs of the lesion were stained with haematoxylin and eosin (H + E); 100×) Figure 8 Fragment of the pancreas (annular pancreas), which is not involved in cancerous infiltrate (H + E; 100×) Figure 6 Tubular adenocarcinoma of the duodenum. Neoplastic tubules border on normal duodenal glands (four-micron sections of representative blocs of the lesion were stained with haematoxylin and eosin (H + E); 200×) Figure 7 Neoplastic infiltrate of the muscular coat of the duodenum encompasses both an inner circular layer and an outer longitudinal layer (H + E; 100×) Annular pancreas is a rare congenital abnormality, and in adult patients it presents with clinical features that differ from those seen in newborns [15]. Duodenal obstruction is a rare symptom of annular pancreas in adults. It is necessary to remember about the coexistence of the annular pancreas when patients with pancreatic or periampullary malignancies are complicated with unexpected obstruction of the second portion of the duodenum in proportion to the size [9]. Features in the adult patient include peptic ulceration, duodenal obstruction, acute pancreatitis, and obstructive jaundice. Treatment strategies for annular pancreas with obstructive jaundice remain controversial, but usually pancreaticoduodenectomy is necessary [2]. In adult patients pancreatic head malignancy usually produces jaundice, and more rarely duodenal obstruction. Nearly always the main cause of this clinical picture is pancreatic head carcinoma, but pancreas annulare should be taken into consideration. The duodenal ring should always be verified on pathological examination since it may harbour this rare type of adenocarcinoma. In adult patients with duodenal obstruction due to annular pancreas, consideration of associated or coexisting duodenal malignancy is mandatory. Only radical surgery (pancreaticoduodenectomy) may provide long-term survival in such cases.

Mucous membrane pemphigoid (MMP, formerly cicatricial pemphigoid) is a chronic autoimmune, subepithelial blistering disease usually of middle aged or elderly patients, more common in women. The prevalence is uncertain and the aetiology is still unknown. Target antigens are epithelial basement membrane proteins BPAG-1, BPAG-2, laminin-331, and laminin-332 [1]. Oral and conjunctival membranes are most frequently affected. Oesophageal involvement is rare but may be complicated with persistent dysphagia [2]. Herein, we present a patient with MMP with acute dysphagia evaluated by upper gastrointestinal (GIT) endoscopy. This study is based on a case report of a 75-year-old patient with MMP. Clinical and endoscopic examinations as well as direct immunofluorescence (DIF) of conjunctival biopsy were used as methods in this study. A 75-year-old woman was referred to the Dermatology Clinic in November 2011 due to painful erosions in the oral cavity and dysphagia. Her past medical history was significant for dry-eye sensations with visual disturbances diagnosed as ocular MMP in 2007 confirmed by DIF (IgA+ deposits) and treated with dapsone. On physical examination there were erosions and haemorrhagic blisters on the hard palate (Figure 1 A). Inspection of the eyes revealed bilateral conjunctival hyperaemia and fibrous adhesions between bulbar and palpebral conjunctiva (symblepharon) (Figure 1 B). No laboratory changes were found, and autoantibodies in the blood (indirect immunofluorescence) were negative. An endoscopic examination of the upper GIT was performed and revealed: 1) white fibrin with or without flat erosions on the mucosa of cricopharynx, along the oesophagus, and on the lining of the stomach body and prepyloric region; 2) two flat, linear erosions, about 10 mm long on the lower third of the oesophagus; 3) hiatal hernia with line “Z” 4 cm above the diaphragm; and 4) spastic but passable pylorus and erythematous mucosa of duodenal bulb (Figure 1 C). An endoscopic biopsy from the oesophagus showed separation between epithelium and mucosa (Figure 1 D). Given the final diagnosis of MMP with prominent conjunctival and oro-oesophageal involvement, the patient was started on methylprednisolone 16/8 mg per day, which resulted in resolution of oral lesions and dysphagia. She remains stable on maintenance therapy with low-dose methylprednisolone. At 18-months’ follow-up the patient remains under control. Figure 1 A – Haemorrhagic blister and erosion localised on hard palate. B – Eye involvement – symblepharon (arrow). C – Endoscopic features of oesophageal involvement in a 75-year-old patient (see text). D – An endoscopic ... Most patients (89–100%) manifest with oral involvement [1]. The conjunctival mucosa is the second most frequently involved site (66%) [1]. Mucous membrane pemphigoid typically begins with blisters or erosions on mucous membranes or skin. Symptoms of MPP evolve over several weeks with subsequent exacerbation and remission periods. The primary lesion is a serous or haemorrhagic blister of variable size that ruptures in 24 to 48 h producing erosion or ulceration that heals slowly and usually tends toward scarring [1]. Mucous membrane pemphigoid is characterised by deposits of IgG, IgA, or C3 complement along the basement membrane zone. Antigens of epithelium are regarded as targets for autoantibodies. These autoantigens are basically BPAG-1, BPAg-2, laminin-331, and laminin-332, which belong to epithelial proteins [3]. Oral lesions present as painful erosions on buccal mucosa and the palate or as desquamative gingivitis, which is very typical of MMP [4–6]. When dysphagia is observed, it is usually secondary to the involvement of hypopharynx. Oesophageal affection is present in up to 15% of cases and rarely as first or isolated presentation [4]. Endoscopic features of oesophageal involvement vary at different stages of the disease and include longitudinal folding of the oesophageal mucosa and diffuse nonspecific inflammatory changes (mucosal oedema, areas of inconstant narrowing and spasm, erosions, superficial ulcerations), which heal with scarring, leading to the formation of adhesions, transverse and circumferential webs, and various sizes of strictures. Oesophageal involvement may be completely asymptomatic or complicated with dysphagia and even life-threatening bleeding from upper GIT [7, 8]. It may manifest as various types of lesions including esophagitis dissecans superficialis (EDS), as a most severe but rare form. Typical features of EDS are vertical fissures and sloughing of whitish superficial epithelium, resulting in dysphagia, odynophagia, and heartburn, but haematemesis or vomiting oesophageal casts occurs rarely [7]. Adhesions appear as healing progresses and scar formation develops. Strictures result from recurrent episodes of disease may be single or multiple and of variable length, sometimes requiring endoscopic dilatation or surgery [7]. Bullae are rarely seen because endoscopy is usually performed at a late stage when scarring has already occurred [4]. Upper endoscopy is not a routine method in the investigation of patients with MMP. It should be indicated in patients with odynophagia, epigastric pain, or bleeding of the gastrointestinal tract [8]. The course of MMP is progressive. The disease seldom goes into complete remission spontaneously, and the aim of therapy is to prevent progression. Treatment strategies depend on the extent and severity of the disease [4]. Most patients require systemic therapy, and corticosteroids (prednisone or methylprednisolone) are the drugs of choice. Immunosuppressants and Dapsone have also been used with success [1, 5]. Some studies show promising results with the use of mycophenolate mofetil or etanercept [9, 10]. The presented case shows the importance of endoscopic diagnostics in acute dysphagia, regardless of the underlying disease. It is also worth emphasising that patients with MMP must be followed up because the disease may relapse, often in a different localisation.

Cat scratch disease (CSD) - bartonellosis, is zoonosis caused by the intracellular gram negativebacterium Bartonellahenselae or Bartonellaquintana. The pathogens of this disease enter the human body usually as a consequence of a bite or scratch by young cats which are the natural source of such bacteria. The illness proceeds asymptomatically or with topical symptoms of infection such as a lump, spot or blister. Within 14 days a high fever and topical lymphadenopathy are observed. Lymph nodes are sore and start suppurating. In half of patients, these symptoms may resemble malignancy, and in single cases there are symptoms associated with the musculoskeletal system, such as: osteitis, arthitis and myositis. In paper presented case of 9 year-old girl patients, treated in Oral Surgery Unit due to odema and lymphadenopathy in right submandibular space. Primary surgical treatment of deciduous teeth was conducted without recovery. In few months follow-up, biopsy of lymph node of submandibular group was taken and provisional diagnosis of cat scratch disease was set. Patient was referred to the Infectious Diseases Unit where serological test confirmed cat scratch disease, and pharmacological treatment was conducted with success and recovery of young patient.