Acrodermatitis enteropathica is a rare autosomal recessive genetic disorder characterised by periorificial dermatitis, alopecia and diarrhoea. It is caused by a mutation in the gene that codes for a membrane protein t...Acrodermatitis enteropathica is a rare autosomal recessive genetic disorder characterised by periorificial dermatitis, alopecia and diarrhoea. It is caused by a mutation in the gene that codes for a membrane protein that binds zinc. We report a case in a 7-month-old girl, admitted with altered general condition and scaly, pustular erythematous lesions, initially located in the occipital and cervical regions, and secondarily inguinal and on the knees. Management and outcome in this patient? Genetic assay was not available to confirm this rare genetic disease. A delay in establishing the diagnosis and a disastrous outcome did not save the patient despite the administration of zinc.展开更多
Background Gianotti-Crosti syndrome is characterized by an acute onset of a papular or papulovesicular eruption with a symmetrical distribution.Data sources A PubMed search was conducted using Clinical Queries with th...Background Gianotti-Crosti syndrome is characterized by an acute onset of a papular or papulovesicular eruption with a symmetrical distribution.Data sources A PubMed search was conducted using Clinical Queries with the key terms"Gianotti-Crosti syndrome"OR"papular acrodermatitis".The search strategy included meta-analyses,randomized controlled trials,clinical trials,observa-tional studies,and reviews.This paper is based on,but not limited to,the search results.Results The eruption of Gianotti-Crosti syndrome is found predominantly on the cheeks,extensor surfaces of the extremities,and buttocks.There is a sparing of antecubital and popliteal fossae as well as palms,soles,and mucosal surfaces.Although often asymptomatic,the lesions may be mildly to moderately pruritic.Gianotti-Crosti syndrome is most common in children between 1 and 6 years of age.The Epstein-Barr virus and the hepatitis B virus are the most common pathogens associated with Gianotti-Crosti syndrome.No treatment for Gianotti-Crosti syndrome is necessary because it is self-limited.In an era of vaccine hesitancy and refusal,Gianotti-Crosti syndrome may be important to mention to parents,because it can occur and trigger alarmism.Conclusions Gianotti-Crosti syndrome is mainly a disease of early childhood,characterized by an acute onset of a papular or papulovesicular eruption with a symmetrical distribution.With the advent of more universal vaccination against hepatitis B virus,Epstein-Barr virus has become the most common etiologic agent of Gianotti-Crosti syndrome.Few cases of post-vaccination Gianotti-Crosti syndrome have been reported.Currently,the emphasis should be placed on its self-limiting attribution.展开更多
文摘Acrodermatitis enteropathica is a rare autosomal recessive genetic disorder characterised by periorificial dermatitis, alopecia and diarrhoea. It is caused by a mutation in the gene that codes for a membrane protein that binds zinc. We report a case in a 7-month-old girl, admitted with altered general condition and scaly, pustular erythematous lesions, initially located in the occipital and cervical regions, and secondarily inguinal and on the knees. Management and outcome in this patient? Genetic assay was not available to confirm this rare genetic disease. A delay in establishing the diagnosis and a disastrous outcome did not save the patient despite the administration of zinc.
文摘Background Gianotti-Crosti syndrome is characterized by an acute onset of a papular or papulovesicular eruption with a symmetrical distribution.Data sources A PubMed search was conducted using Clinical Queries with the key terms"Gianotti-Crosti syndrome"OR"papular acrodermatitis".The search strategy included meta-analyses,randomized controlled trials,clinical trials,observa-tional studies,and reviews.This paper is based on,but not limited to,the search results.Results The eruption of Gianotti-Crosti syndrome is found predominantly on the cheeks,extensor surfaces of the extremities,and buttocks.There is a sparing of antecubital and popliteal fossae as well as palms,soles,and mucosal surfaces.Although often asymptomatic,the lesions may be mildly to moderately pruritic.Gianotti-Crosti syndrome is most common in children between 1 and 6 years of age.The Epstein-Barr virus and the hepatitis B virus are the most common pathogens associated with Gianotti-Crosti syndrome.No treatment for Gianotti-Crosti syndrome is necessary because it is self-limited.In an era of vaccine hesitancy and refusal,Gianotti-Crosti syndrome may be important to mention to parents,because it can occur and trigger alarmism.Conclusions Gianotti-Crosti syndrome is mainly a disease of early childhood,characterized by an acute onset of a papular or papulovesicular eruption with a symmetrical distribution.With the advent of more universal vaccination against hepatitis B virus,Epstein-Barr virus has become the most common etiologic agent of Gianotti-Crosti syndrome.Few cases of post-vaccination Gianotti-Crosti syndrome have been reported.Currently,the emphasis should be placed on its self-limiting attribution.
