Ectodermal Dysplasia: Understanding the Genetic Disorder Affecting Skin, Hair, and Teeth

Ectodermal Dysplasia: Understanding the Genetic Disorder Affecting Skin, Hair, and Teeth

Question. Write briefly on ectodermal dysplasia.

Answer. The term ectodermal dysplasia is a group of inherited diseases in which two or more ectodermal structures fail to develop.

• Disease can be autosomal dominant or autosomal recessive, or it can be X­linked too.
• The most common ectodermal dysplasia is hereditary hypohidrotic ectodermal dysplasia.

“Understanding the role of ectodermal dysplasia in genetic disorders: Q&A explained”

Clinical Features Of Ectoacdermal Dysplasia

• Male predominance is seen.
• Patient is characterized by hypotrichosis, hypohydrosis, and at times anhydrosis.
• The patient has a characteristic saddle nose.
• Fine and sparse hair is seen on eyebrows and eyelashes
• Periocular skin has five wrinkles with hyperpigmentation.
• Frontal bossing is commonly present.
• Nails appear dystrophic and brittle.
• Skin of the patient remains dry and there is absence or partial presence of sweat glands. Due to this patient cannot perspire and his heat regulation is disturbed which leads to an increased temperature of patient.

“Importance of studying ectodermal dysplasia for better diagnostic outcomes: Questions explained”

Histopathology of skin reveals the following features:

• There is a reduction in several sweat glands, hair follicles, and sebaceous glands.
• The epidermis is thin and flattened.
• Eccrine sweat glands are few or poorly developed.
• In oral histopathology, salivary glands show ectasia of ducts and inflammatory changes.

“Common challenges in diagnosing ectodermal dysplasia effectively: FAQs provided”

Treatment Of Ectodermal Dysplasia

• The condition is genetic, so it is non­curable.
• Prosthetic rehabilitation of a patient should be done by complete dentures, RPDs or fixed dentures.
• Dental implants can also be considered.