Τόμος 8 (1990) – Τεύχος 4 – Άρθρο 7 – Επιθεώρηση Κλινικής Φαρμακολογίας και Φαρμακοκινητικής-Ελληνική Έκδοση – Volume 8 (1990) – Issue 4 – Article 7 – Epitheorese Klinikes Farmakologias και Farmakokinetikes-Greek Edition

8: 238-248 (1990)

PHARMAKON-Press: Epitheorese Klin. Farmakol. Farmakokinet.

8: 238-248 (1990)

A case of a 16 year old girl with lymphomatoid granulomatosis involving the CNS only is reported. The main clinical manifestations were fever, convulsions, head­ache, hallucinations, loss of memory and maculopapular skin lesions. We stress the particularities of our case which were lack of respiratory tract involvement, olfactory hallu­cinations and leukocytoclastic vasculitis found on the skin biopsy. The diagnosis of the disease was established after temporal lobe biopsy (meninges and cortex). The patient was treated with steroids only, due to coexisting and unrelated reasons. She survived for 25 months; a cerebral hemorrhage was the cause of death. The fact that lymphomatoid granulomatosis is also described as polymorphic reticulosis or angiocentric immunoblastic disease or angio­centric lymphoreticular disease indicates the confusion regarding the nature of the disease, the immunologic disorders of which tend to malignancy. Treatment with cyclophosphamide, irradiation of lesions and use of extremely aggressive chemotherapy (e.g. MACOP-B) seem to prolong remissions of this fatal granulomatous vasculitis. Our case is the first case of this type of vasculitis described in Greece.

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