By Disease Name > Congenital Ichthyosiform Erythroderma

Congenital Ichthyosiform Erythroderma

 Top  Previous  Next
•autosomal recessive; AKA non-bullous congenital ichthyosiform erythroderma

 

pathogenesis:

•unknown; increased epidermal turnover rate

 

clinical:

•newborn – collodion baby
•child/adult – generalized erythroderma with fine white scale, flexures involved
•± palmoplantar keratoderma
•scarring alopecia; ectropion

 

ddx:

•neutral lipid storage disease (NLSD), ichthyosis vulgaris, Netherton’s syndrome
•examine peripheral blood smear to rule out NLSD (lipid vacuoles in leukocytes and monocytes)
•skin biopsy: lipid vacuoles in basal epidermis in NLSD

 

 

 

see Ichthyoses