Clinical Case Summary

We report the case of a 50-year-old woman with a history of arterial hypertension who consulted for progressive dysphagia to solids since seven months, tolerating only liquids in the last week. In addition, retrosternal knotting sensation, regurgitations, weight loss and a painful whitish plaque on the oral mucosa. Hospital admission was decided to complete study and to ensure the nutritional status.
Gastroscopy was performed, observing a punctiform stenosis of the esophageal lumen at 18 cm of the dental arch, which did not allow the passage of the endoscope. We observed erythematous mucosa and fibrinous exudate covering almost all of it (Figure 1), biopsies were taken.
Barium esophagogram showed two strictures, a short one at the proximal esophagus level and a second one of approximately 7 cm in distal third (Figure 2). Biopsies were negative for microbiology, histological study showed Civatte bodies, compatible with erosive lichen planus.
Endoscopic dilatation of stenoses was considered but this entailed a high risk of complications. Finally, it was decided jointly with Dermatology and Systemic Diseases Department to start systemic corticosteroids and oral acitretin. In the following days, patient showed clinical improvement with adecuate tolerance to a liquid-shredded diet. Nowadays, the patient takes methotrexate and oral acitretin, improving findings in gastroscopy and tolerance to diet.
Lichen planus is a common cutaneous-mucosal disease, where symptomatic esophageal involvement is exceptional. The diagnosis is established by histological study, highlighting the degeneration of the keratinocytes of the basal layer of the epithelium "Civatte bodies". Therapeutic options are varied, the first line being the use of systemic corticosteroids, with patients presenting a rapid response but high percentage of relapses. In addition, immunosuppressants can be used, although all of them have low long-term response rate. JAK-kinase inhibitors are being studied.

References

Esophageal stenting for benign and malignant disease: European Society of Gastrointestinal Endoscopy (ESGE) guideline-Uptodate 2021.
Malagelada JR, Bazzoli F, Boeckxstaens G, et al. Directrices globales de la organización mundial de gastroenterología: disfagia: actualización de las pautas globales y cascadas de septiembre de 2014. J Clin Gastroenterol 2015; 49:370.
Le Cleach L, Chosidow O. Práctica clínica. Liquen plano. N Engl J Med 2012; 366:723.

Clinical Case Summary

A 46-year-old woman with a history of using an "Easy forte" supplement. She reported sciatic neuropathy managed with analgesics and anti-neuritics. She reported onset of distension, epigastric pain, and heartburn 2 days prior to admission. After one day, she experienced an increasing, abdominal transfixing pain and inter-scapular region that did not improve with analgesics, with vomiting and oral intolerance. She was evaluated by a gastroenterologist. Physical examination revealed a heart rate 112 bpm, blood pressure 132/74 mm Hg, conscious patient, no pleuropulmonary syndrome, reduced peristalsis in the abdomen, painful superficial and deep palpation in the epigastrium. Laboratory tests showed Hb 11.4, platelets 222,000, urea 23.98, creatinine 1.34 mg/dL, total bilirubin 1.5 mg/dL, lipase 11861 U/L, and LDH 422. Abdominal CT revealed a liver with fatty infiltration, intra and extrahepatic bile ducts, and normal gallbladder. The pancreas was significantly enlarged, with lobulated edges, mesenteric and peri-pancreatic fat striation. Severe acute pancreatitis was diagnosed according to the Atlanta criteria. During the hospitalization, a decrease in Hb levels and indirect hyperbilirubinemia with increased levels of LDH were observed, suggesting hemolytic anemia. Hematology was consulted and peripheral blood smears were performed, revealing schistocytes. Therefore, a PLASMIC score was calculated (6 points), indicating a 72% risk of severe ADAMTS13 deficiency, and treatment for TTP was initiated with methylprednisolone 1 g/3 days and 5 plasma exchanges, leading to improvement. Due to worsening of renal function, 3 hemodialysis sessions were performed, leading to remission. At 96 hours, contrast-enhanced abdominal CT showed no collections or necrosis. Biliary, alcoholic, hypertriglyceridemia, hypercalcemia and drug related-causes were ruled out,. ADAMTS13 activity was reported at 1%, leading to the conclusion that the etiology was hemolysis secondary to TTP.

References

1. Kremer Hovinga, J. A., & George, J. N. (2019). Hereditary Thrombotic Thrombocytopenic Purpura. N Eng J Med.
2. Reese JA, Muthurajah DS, Kremer Hovinga JA, et al. Niños y adultos con púrpura trombocitopénica trombótica asociada con deficiencia grave de Adamts13 adquirida: comparación de incidencia, características demográficas y clínicas. Pediatr Blood Cancer 2013.
3. Page EE, Kremer Hovinga JA, Terrell DR, et al. Púrpura trombocitopénica trombótica: criterios de diagnóstico, características clínicas y resultados a largo plazo desde 1995 hasta 2015. Blood Adv 2017.
4. Nokes T, George JN, Vesely SK, Awab A. Compromiso pulmonar en pacientes con púrpura trombocitopénica trombótica. Eur J Haematol 2014.
5. George J, Cuker A. Diagnosis of immune TTP. Consutado en UpToDate
6. Swisher K, Doan J, Vesely S, et-al. Pancreatitis preceding acute episodes of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: Report of five patients with a systematic review of published reports. Haematologica (2007).
7. Fujino, Y., Inoue, Y., Onodera, M. et al. Acute pancreatitis-induced thrombotic thrombocytopenic purpura with recurrent acute pancreatitis. Clin J Gastroenterol 9, 104–108 (2016).
8. Rock GA, Shumak KH, Buskard NA, et-al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. N Eng J Med, 325 (1991).

Disclosure

Of thrombotic microangiopathies, the most recognized are HUS and TTP, the latter being a rare clinical entity characterized by a pentad consisting of: microangiopathic anemia, thrombocytopenia, renal and neurological alterations, and fever. It is due to reduced activity of the ADAMTS13 protease that cleaves von Willebrand factor. Only a few cases of association with acute pancreatitis have been reported.
In 1924, TTP was described as a disease with high mortality, characterized by systemic microvascular thrombosis1. The incidence is approximately 3 per 1 million per year2. In its presentation, there is involvement of the CNS and/or gastrointestinal tract (69%), and renal impairment may be seen on biopsy. The classic pentad is infrequent (<5%) and anecdotal3.
There may also be pancreatic involvement, while pulmonary involvement is rare4. This pathology should be suspected when there are signs of microangiopathic hemolytic anemia and severe thrombocytopenia. Other findings include elevated LDH, indirect bilirubin, and negative Coombs test5. The activity of ADAMTS13 is important for diagnosis and should not be used in isolation1.
The association between TTP and pancreatitis has rarely been reported, described as either a cause or a consequence. In the literature, it is reported that 2% of patients develop pancreatic inflammation, although TTP secondary to pancreatitis is rare6. There are very few cases of this association described in the literature, and it is postulated that the etiology is due to an excess of cytokines, causing microvascular damage through increased thrombogenesis, associated or not with ADAMTS13 deficiency7. Plasmapheresis is the only treatment that has shown efficacy8. Rituximab may be effective in selected patients, especially in refractory or recurrent disease9.