2. ICHTHYOSES AND ICHTHYOSIFORM SYNDROMES Presented by Dr. Ibrahim Md. Sharaf HMO, Dept. of Skin & VD

7. Ichthyosis Vulgaris

12. X-linked Ichthyosis

14. Difference between I.V. and X-linked Ichthyosis (clinically) before 3 months of age in between 3-12 months of age Onset Coarser and darker Finer Scales Only male Both sexes Affected sex Palms and soles Flexures and face Spared area Absent Present Association with KP & AD Present Absent Eye involvement & hypogonadism Abdomen is more affected than back Affected area Moderate Mild Severity X-linked Ichthyosis Ichthyosis vulgaris Traits

15. Autosomal-Recessive Ichthyosis

18. Lamellar Ichthyosis

19. Lamellar Ichthyosis

20. Ectropion

22. Collodion baby

25. Harlequin

26. Harlequin Fetus

27. Harlequin Fetus

33. Features of different types of Ichthyosis Becomes less severe with age Severe Moderate Mild Severity All over body, bullae and hyperkeratotic lesions over knee, elbows; keratoderma All over body, very severe, involves flexure, neck, face, scalp, scaly palms and sole All over body Only men All over body Distribution Abnormal distribution of keratinocytes Transglutaminase 1 Steroid sulphatase enzyme Flaggrin protein Defect AD AR X-linked AD Inheritance EK Lamellar Ichthyosis X-linked Ichthyosis IV Features

34. Features of different types of Ichthyosis (contd.) Systemic and oral retinois + antibiotics Retinoids- acitretin Emollients Emollients Treatment Erythroderma Scales are large and quadrangular, ectropion and eclabium Scales are black and brown, eye involvement, cryptorchidism Fine scales, improves in summer Other features Birth- bullae, erythroderma. Birth- collodion baby Before 3 months of age 3-12 months of age Onset EK Lamellar Ichthyosis X-linked Ichthyosis IV Features Tends to become less severe with age Causes serious disability Good Good Prognosis None None Palms and soles Flexures and face Spared areas

37. Syndromes associated with Ichthyosis