1/5. Chronic granulomatous disease and acute neutrophilic dermatosis.Chronic granulomatous disease is an inherited disorder characterized by defective oxidative killing by neutrophils and other phagocytes. This results in susceptibility to persistent and life-threatening infections. We describe a 25-year-old man with chronic granulomatous disease who presented with an acute, febrile neutrophilic dermatosis. This indicates that normal neutrophil intracellular killing mechanisms are not essential in the pathogenesis of neutrophilic dermatoses.- - - - - - - - - - ranking = 1keywords = dermatosis (Clic here for more details about this article) |
2/5. The dermatosis of chronic granulomatous disease.A family with X-linked cytochrome-negative chronic granulomatous disease (CGD) involving three generations is reported. The diagnosis of CGD in both the latest male patient and the index male was confirmed by marked impairment in polymorphonuclear leucocyte oxidative burst activity in association with absence of both subunits of cytochrome b. The two female carriers have suffered from chronic inflammatory skin disorders characterized by slowly fluctuating erythematous plaques. The reported cases are discussed in the context of a literature review of the dermatosis of CGD.- - - - - - - - - - ranking = 1keywords = dermatosis (Clic here for more details about this article) |
3/5. Chronic granulomatous disease carrier geno-dermatosis (CGDCGD).A persistent eruption in light-exposed areas in two related carriers of X-linked chronic granulomatous disease is described. This eruption appears to be a separate entity, rather than a variant of cutaneous lupus erythematosus or Jessner's disease. Recognition may enable detection of carrier females prior to the birth of an affected son, so that genetic counselling and antenatal diagnosis can be instituted.- - - - - - - - - - ranking = 0.8keywords = dermatosis (Clic here for more details about this article) |
4/5. Lupus erythematosus-like oral mucosal and skin lesions in a carrier of chronic granulomatous disease. Chronic granulomatous disease carrier genodermatosis.We present a case of a young female patient who for 8 years was believed to have discoid lupus erythematosus of the skin and oral mucosae. Only after her infant son had a near-fatal pulmonary infection was the diagnosis of chronic granulomatous disease made and her lupus erythematosus-like mucocutaneous lesions recognized as manifestations of her carrier status for chronic granulomatous disease. The purpose of this report is to raise awareness of and better characterize the mucocutaneous manifestations of carriers of chronic granulomatous disease. Early identification of carriers permits genetic counseling and prenatal diagnosis and forewarns pediatricians so that they can provide better care for affected infants.- - - - - - - - - - ranking = 0.8keywords = dermatosis (Clic here for more details about this article) |
5/5. Chronic bullous disease of childhood and a paecilomyces lung infection in chronic granulomatous disease.A 12 year old boy suffering from p67-phox deficient chronic granulomatous disease presented with a bullous skin disease and a lung infection with paecilomyces species. The histopathology of a bullous lesion showed subepidermal blister formation and microabcesses containing eosinophils in the dermal papillae. By direct immunofluorescence, linear staining of IgA at the dermal-epidermal junction was detected which confirmed the clinical diagnosis of chronic bullous disease of childhood (linear IgA dermatosis).- - - - - - - - - - ranking = 0.2keywords = dermatosis (Clic here for more details about this article) |