Lege Artis Medicinae

[ERDHEIM-CHESTER DISEASE: A RARE FORM OF HISTIOCYTOSIS]

NÉMETH István, BOGÁTS Gábor, KAISER László, TISZLAVICZ László

MAY 16, 2007

Lege Artis Medicinae - 2007;17(04-05)

[INTRODUCTION - Erdheim-Chester disease is rare non-Langerhans cell histiocytosis with multisystem involvement. The histological hallmark of the disease is CD1a negative histiocyte proliferation. CASE REPORT - Histiocytosis presented in a 57- year-old Caucasian man with exophtalmus, which was after two years followed by progressive pericardial infiltration and effusion leading to cardiac compression and consequent functional disturbance. It was the histological analysis of a surgical sample removed during pericardial fenestration that revealed Erdheim- Chester disease. The patient died from multisystem involvement one year after the initial diagnosis. Postmortem examination showed long bone, orbital cavity and cranial cavity involvement as well as extensive retroperitoneal and mediastinal xanthogranulomatous infiltration. CONCLUSIONS - The clinical diagnosis of Erdheim-Chester disease is difficult because the symptoms are insidious and non-specific. The histological report is often only descriptive. The prognosis of the disease is poor due to the lack of targeted therapy and to the advanced stage at diagnosis.]

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[SEVERE GASTROINTESTINAL AND RENAL MANIFESTATIONS OF HENOCH-SCHÖNLEIN PURPURA]

GECSE Krisztina, ONDRIK Zoltán, KAIZER László, VARGA Erika, LONOVICS János, CZAKÓ László

[INTRODUCTION - Henoch-Schönlein purpura is a systemic small vessel vasculitis characterized by vascular and/or mesangial IgA deposits, primarily affecting the vasculature of the skin, joints, kidneys and gastrointestinal tract. Gastrointestinal findings of various severity occur in 50 to 85% of the cases. We report on a 70-year-old woman who developed ileocaecal invagination and upper gastrointestinal haemorrhage as manifestations of Henoch-Schönlein purpura. CASE REPORT - The patient presented with two days history of palpable purpuric rash localized on the lower extremities. Based on the result of the skin biopsy, which showed leukocytoclastic vasculitis, IgA and C3 deposits, Henoch-Schönlein purpura was suspected. On the second night after admission colicky abdominal pain, vomiting and diarrhoea developed. Radiological examination showed an ileocaecal invagination and since symptoms deteriorated caecum resection and ileo-ascendestomy was performed. On the third postoperative day the patient became oligo-anuric, which was attributed to her Henoch-Schönlein disease, and systemic steroid pulse therapy was given. One month after the admission the patient experienced haematochezia. Emergency upper endoscopy revealed petechiae, haemorrhagic erosions and mucosal oedema, primarily in the descending part of the duodenum. Since these lesions were also considered as presentations of Henoch-Schönlein disease, another bolus of parenteral steroid was administered. Upper endoscopy repeated 3 days later showed remarkable improvement of the gastric and intestinal lesions. CONCLUSION - Gastrointestinal symptoms are common manifestations of Henoch-Schönlein purpura, thus their recognition, in which endoscopy plays a crucial role, is of major importance. Methylprednisolone pulse therapy is an effective therapeutic option not only in the management of severe renal or joint symptoms, but also in the treatment of gastrointestinal manifestations.]

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