This type of ichthyosis, also known as bullous ichthyosis was earlier known as bullous variety of ichthyosiform erythroderma. Just after birth the child shows bullous lesions and denuded areas with focal areas of hyperkeratosis.
As the child grows the bullous lesions subside, but he develops more hyperkeratosis. The lesions are warty and more pronounced in the flexural folds with secondary infection and foul smell.
An underlying erythroderma in some cases and palmo-plantar keratoderma may also be present. This disease is transmitted as an autosomal dominant trait, but extremely rare.
Histopathologically, the cells of the granular cell layer and the upper part of stratum spinosum develop intracytoplasmic vacuoles, an increased number and size of keratohyaline granules, along with massive hyperkeratosis and a perivascular round cell infiltrate in the upper dermis.
Treatment
Vesicular stage
- Local applications of one percent aqueous solutions of gentian violet or brilliant green or any other antibiotic agent.
- For widespread or heavily infected lesions, systemic antibiotics should be used.
Later stages
Local applications of emollients and keratolytic agents such as five percent salicylic acid in petrolatum or ten to 40 percent urea in glycerine may bring improvement.
