ABSTRACT
The disorder is characterized by the triad ichthyosiform dermatosis, hair shaft defects and atopic diathesis.
Ichthyosiform dermatosis Two phenotypes of ichthyosis maybe expressed:
Figure 5.1 ● Ichthyosis linearis circumflexa: slowly migrating, erythematous, scaling, serpiginous
lesions with a distinctive double-edged desquamation at the periphery (Figures 5.153)
Figure 5.3 ● Generalized erythroderma and erythematous collodion baby presentation at birth
(Figures 5.4 and 5.5)
Patients present a characteristic facies and, during adolescence and in adult life, flexural, malodorous lichenifications (Figures 5.6-5.10).
