D3.265 - Gleish syndrome: A case report

Introduction: Gleich syndrome is a clinical entity which associates recurrent episodes of angioedema, hypereosinophilia and an elevation of immunoglobulins M. This syndrome described in 1984 by Gleich; it mainly affects young adults, it is characterized by the absence of organ damage, it has a good prognosis We report the first case of Gleich syndrome in Algeria.

Case description: we received a consultation with a 37-year-old woman, a professional anesthetist, married with 3 healthy living children, for recurrent episodes of angioedema especially of the face and both hands, with no family history of similar episodes. This was associated with a pruritic urticarial rush during the outbreaks. The symptoms began in 2022, associating erythematous papules of the upper part of the body, thighs, and lower limbs with a weight gain of 10 kilos in 2 months and a fever evolving in a fluctuating manner. The episodes occurred without a triggering factor every 4 weeks, lasting approximately 1 week, apart from taking an estrogen-progestogen pill. Apart from the seizures, the clinical examination was normal. Immunological investigations, in particular the dosage of serum complement C4, antigenic and functional inhibitor C1, returned without abnormalities. Parasitological examinations are normal. A blood count was performed which showed hypereosinophilia at 3500 cells/mm3, protein electrophoresis showed hypergammglobulinemia and hypergammaglobulinemia at 21.3 g/L with IgM increased to 6.02 g/L (N < 2, 3). All symptoms disappeared spontaneously within a few days. Resolution was spontaneous, accelerated by 5 to 7 days of systemic corticosteroid therapy. Pathological examination of an erythematous plaque revealed eosinophilic panniculitis. Apart from the seizures, the clinical examination was normal. No new clinical manifestations appeared after 1 year of follow-up

Discussion: Other causes of recurrent angioedema and hypereosinophilia had been eliminated. The search for visceral damage linked to hypereosinophilia. Mac Duffy vasculitis was ruled out (normal C1q). Gleich syndrome is a benign disease, even if it can be very uncomfortable. Its etiology remains unknown. The evolution of hypereosinophilia parallels the clinical evolution. It can reach 70,000/mm3 during attacks. General corticosteroid therapy helps control flare-ups but does not prevent them. Our observation illustrates the good general prognosis of Gleich syndrome.

Conclusion: Although Gleich syndrome is a diagnosis of exclusion; nevertheless this distinctive hypereosinophilic entity with a benign course, remains unexplored and underdiagnosed in the general population and in patients with chronic orofacial angioedema in particular, further studies are needed to evaluate the pathophysiology of this syndrome and lead to better therapeutic options.