X-linked ichthyosis


X-linked ichthyosis: A rare genetic skin disorder occurring in males only and resulting from an inborn error of metabolism (deficiency of the enzyme steroid sulfatase).


Dry skin, Scaling skin, Thick skin, Pruritus Enzyme steroid ,sulfatase deficiency, Increased blood cholesterol ,sulfate level Accumulation of cholesterol ,sulfate in the skin


Exfoliative dermatitis begins with rapidly developing generalized erythema. Desquamation with fine scales or thick sheets of all or most of the skin surface may cause life-threatening hypothermia. Other possible complications include cardiac output failure and septicemia. Systemic signs and symptoms include a low-grade fever, chills, malaise, lymphadenopathy, and gynecomastia.


Begin the history by asking how long the patient has had scaly skin and whether he has had it before. Where did it first appear? Did a lesion or skin eruption, such as erythema, precede it?