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Poikiloderma with neutropenia (PN) is a genodermatosis characterized by poikiloderma, permanent neutropenia, recurrent infections, nail abnormalities, and palmoplantar hyperkeratosis. We report the case of a Tunisian patient with PN. Skin lesions started from the face and spread to the extremities and trunk. Neutropenia was initially periodic and concomitant with infections periods. DNA analysis identified a novel homozygous deletion of a 1-bp (c.161delC, p.P54RfsX60) in the C16orf57gene, presumed to be causative. This report presents the variability of the clinical manifestations and evolution of PN and emphasizes the importance of studying other patients with PN to better delineate mutations profile among populations.

Kawasaki disease is a generalized systemic vasculitis, which primarily affects medium-sized arteries. Kawasaki disease shock syndrome is a rare but severe presentation of this disease. This report describes a case of delayed diagnosis of Kawasaki disease shock syndrome in a 13-year-old boy who presented with cervical adenophlegmon, persistent fever, injected conjunctiva, rash, and hypotension. Echocardiography revealed the presence of bilateral coronary aneurysms. Early recognition of Kawasaki disease shock syndrome can be difficult; however, delay in diagnosis and treatment can increase the risk of coronary artery disease.

Background: Plummer Vinson syndrome is a rare cause of dysphagia in children. It associates an oesophageal organic stenosis to a sideropenic anemia.Case Reports: Two Tunisian boys aged respectively 14 and 9 years-old were admitted to investigate dysphagia for solid foods. The adolescent patient was a black and he suffers from food blocking since 2 years ago. The assessment revealed a sideropenic anemia due to intestinal giardiasis. While the second patient was followed for a chronic anemia since he was 3 years old. For both of them, the swallow barium exploration showed an annular stenosis of the cervical esophagus mimicking "a thread knotted on a round tube" related to a circular web identified by fibroscopy. The iron supplementation and dilatation by the esophagoscope allowed the resolution of the clinical signs. Conclusion:Plummer Vinson syndrome exists in paediatric population. It should be evoked in any children presenting with upper dysphagia for solid foods in order to initiate iron supplementation and to watch for cancerous degeneration by regular followup.

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