Ichthyosisis an infrequent clinical entity worldwide with an incidence of 1:600,000 births. It can be one of the two types: collodion baby and Harlequin fetus or malignant keratoma (most severe form). The clinical manifestations in either form are thick and hard skin with deep splits. Affected babies are born in a collodion membrane, a shiny waxy outer layer to the skin that is shed 10 - 14 day...

The ichthyoses comprise a group of keratinisation disorders characterised by generalised scaling of the skin. Lamellar ichthyosis (LI) is usually manifested at birth as a collodion baby with ectropion/eclabion. Collodion membrane means encased in a tight shiny covering. It is characterised afterwards by non-bullous scaling of the whole integument with variable erythroderma. LI is a major subtyp...

In our series, all patients had quick good-quality remissionwith amedianprogression-free survival of 6months. The rapidity of the response supports the role of vinblastine instead of spontaneous regression in these patientswithmultifocal lesions and a long-term history of refractory disease. In our series, no patient had to discontinue use of the drug because of infection or severe toxic effect...

A baby full term born of LSCS to a otherwise healthy mother was referred for ophthalmology department. On ophthalmological torch light examination, the patient had equal upper and lower lid bilateral ectropion. There missing eye lashes noted. The anterior segment evaluation found be within normal limit without evidence corneal opacities. Lamellar ichthyosis termed as Collodion is infrequent dis...

The association of Gaucher disease, the inherited deficiency of lysosomal glucocerebrosidase (EC 3.2.1.45), and congenital ichthyosis was first noted a decade ago. Subsequently, a null allele type 2 Gaucher mouse was generated that also exhibited ichthyotic skin, confirming that the skin disorder and enzyme deficiency were directly related. This paper details the clinical and molecular characte...

We assessed the clinical efficacy, tolerability and safety of acitretin in a patient with ichthyosis. A newborn infant with ichthyosis who presented at birth with collodion baby appearance, was treated with acitretin. A moderate response to acitretin therapy (1 mg/kg/day) administered for 6 months was observed, with improvement in cutaneous lesions. Clinical improvement was achieved shortly aft...