Cutaneous metaplastic synovial cyst

Metaplastic synovial cyst, a recently described entity, is histologically characterized by a cystic cavity lined by metaplastic synovial tissue that is often hyperplastic and protrudes into its lumen as villous structures. Clinically, these lesions resemble suture granuloma and develop at the site of previous surgical trauma. We have studied 4 cases of this unusual entity and have investigated the immunohistochemical profile in an effort to determine the nature and derivation of their lining tissue. One of our cases is unique since the lesion was associated with a basal cell carcinoma. A history of previous surgery was noted in one, and history of trauma in 2 of the 4 cases. Our immunohistochemical observations confirmed a mesenchymal derivation for the "synovial like villous structures" but non-availability of a specific "synovial" marker does not permit us to conclude with certainty the synovial nature of the metaplastic tissue. Cutaneous metaplastic synovial cysts are unique lesions which deserve attention and should be included in the differential diagnosis of cutaneous cysts.     

Histological Esophagitis (Clinical and Histological Response to Omeprazole in Children)

Many children with esophagitis demonstratehistological changes without gross evidence ofesophagitis by esophagoscopy. The effect of omeprazoleon the histological healing of esophagitis in childrenis unknown. Therefore, the aim of this study wasto determine the effect of omeprazole on refractoryhistological esophagitis in pediatric patients. Eighteenpatients with histological evidence of esophagitis and recurrent symptoms despite therapy withH₂-receptor antagonists and prokinetic agentswere prospectively treated with omeprazole. Dosing wasadjusted by monitoring intragastric pH, andesophagoscopy was repeated after 8-12 weeks of omeprazoletreatment. Two patients did not complete the study dueto either worsening symptoms or hypergastrinemia. Of theremaining patients, 76% were asymptomatic with omeprazole treatment and 24% reportedimprovement in their symptoms. Approximately 40%demonstrated complete histological healing of theiresophagitis. Three patients (17%) had persistentelevations in serum gastrin levels while on omeprazoletreatment, which was associated with both youngerpatient age and higher omeprazole dosing; however, allelevated gastrin levels returned to normal afterdiscontinuation of the medication. All patients had recurrenceof their symptoms after completing a course ofomeprazole, even patients with complete histologicalhealing. Omeprazole is efficacious in treating children with esophagitis refractory toH₂-receptor antagonist and prokinetic agents.However, none of the patients were able to discontinueacid suppressive therapy even after documented healingof their esophagitis.     

Cocaine‐associated atypical haemolytic uraemic syndrome in a genetically susceptible individual

Atypical haemolytic uraemic syndrome (aHUS) is a severe, life‐threatening condition that requires early recognition and urgent treatment. In aHUS rare genetic variants in CFH, CFI, CD46, C3 and CFB predispose to complement over activation. This case describes a case of aHUS in which there was a strong temporal association between disease onset and the use of smoked cocaine. The patient was found to have a rare genetic variant in the CFI gene which may have been unmasked by first‐time exposure to cocaine. The patient stabilized and improved with early administration of eculizumab, supporting the notion of an underlying immunological pathogenesis and the importance of early intervention.