Hereditary sclerosing poikiloderma (HSP) is a very rare disease. The clinical features are principally widespread poikiloderma and linear hyperkeratotic and sclerotic bands. We report an 18-yr-old male who presented reticular hyperpigmented lesions on the trunk and extremities since 2-yr-old. Also, linear sclerosing bands appeared on both antecubital and popliteal fossae after yr. Histopatholog...

A variety of mechanisms have been demonstrated or suggested to explain the neutropenia that accompanies Felty's syndrome. This case report presents with Felty's syndrome with recurrent infections with initially had a clinical response to splenectomy. Eleven years later profound neutropenia recurred. In-vitro evidence for cell mediated autosensitisation of peripheral blood lymphocytes to autolog...

Vancomycin has been used with increased frequency during the past 15 years and the most common toxicity with this drug is the red man syndrome . Other adverse effects include neutropenia, fever, phlebitis, nephrotoxicity, ototoxicity, thrombocytopenia, interstitial nephritis, lacrimation, linear IgA bullous dermatosis, necrotizing cutaneous vasculitis and toxic epidermal necrolysis. Only two c...

Background: Late onset neutropenia (neutropenia after 3 weeks of life) may be a physiological condition without need to prescribe antibiotics, G-CSF, or IVIG. We aimed to determine the association between sepsis and late onset neutropenia in very low birth weight (<1500 gm) infants.&nbsp; Methods: This study was a cross-sectional prospective study in VLBW infants that were admitted in Mahdieh ...

RTS: Rothmund-Thomson syndrome INTRODUCTION Rothmund-Thomson syndrome (RTS) (OMIM #268400) is a rare autosomal recessive disorder characterized by erythema, blisters, and swelling that appears during infancy on the cheeks, spreads to the extremities, and eventually leads to poikiloderma. Additionally, a large percentage of patients have short stature and skeletal abnormalities, and some patient...

Introduction Neutropenia is defined in the literature as absolute neutrophil counts in peripheral blood of less than 1500 cells/mm3 in more than one year old and less than 2000cells/mm3 in children in the first year old of life. Neutropenia is classified as mild, moderate or severe, and may be congenital or acquired, persistent or not. Kostmann syndrome is a severe neutropenia, the incidence va...