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Bullous Ichthyosiform Erythroderma

Bullous Ichthyosiform Erythroderma is a rare autosomal dominant condition of keratinization produced by mutations in the genes for keratins 1 and 10 that, in its early stages, is associated with blistering. 

Within hours of delivery, extensive, flaccid blistering and skin fragility are visible. As a typical grey, waxy scale thickens, these clear up without leaving scars, blisters, or erythroderma in children. 

The flexures, scalp, anterior neck, abdominal region, and infragluteal areas are the regions of linear hyperkeratosis that are most noticeable. 

Abnormal skin develops erosions and recurrent infections. Many patients develop palmoplantar hyperkeratosis. 

With time, this ailment usually gets better.


Histology of skin shows characteristic changeso f epidermolytic hyperkeratosis. 

Electron microscopy reveals clumped keratin filamentsa round granular keratinocyte nuclei. 

Differential Diagnosis of Bullous Ichthyosiform Erythroderma

In the neonate: 

Epidermolysis bullosa

Staphylococcal scalded skin syndrome (SSSS) 

Herpetic infections

Incontinentia pigmenti

In older children:

other ichthyoses.

Treatment of Bullous Ichthyosiform Erythroderma

Emollients are of limited use, as the scaling is greasy, and keratolytics may be irritating. 

Topical antiseptics can reduce microbial overgrowth

Oral retinoids may help, although they increase skin fragility.

Prognosis of Bullous Ichthyosiform Erythroderma

At least half of the cases have no family history of the disease and are due to new keratin mutations, but parents of affected children should be examined carefully for focal keratotic lesions as these indicate that future siblings will be affected . 


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