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Patient Case: Cornification Disorder (Ichthyosis Vulgaris)

Patient Case: Cornification Disorder (Ichthyosis Vulgaris)

Discussion

A 10-year-old boy is brought to the dermatology clinic by his parents due to dry, rough, and scaly skin that has persisted since infancy. His parents noticed that the condition worsens during the winter months and improves in the summer. They report that his skin feels like sandpaper, particularly on his arms, legs, and back.

The child has no history of allergies or recent infections, but his mother mentions that she also has mildly dry skin. On examination, fine, white to grayish scales are present on the extensor surfaces of the arms and legs, sparing the flexural areas. There is no redness, inflammation, or itching. Examination of the palms reveals increased skin markings.

Based on the clinical findings, he is diagnosed with ichthyosis vulgaris, a genetic disorder of keratinization. He is prescribed emollients and keratolytic agents (e.g., urea or lactic acid creams) and advised to moisturize frequently to manage the dryness and scaling.

Questions
  1. What is the most likely diagnosis in this patient?
    a) Psoriasis
    b) Atopic dermatitis
    c) Ichthyosis vulgaris
    d) Tinea corporis
  2. Which of the following clinical features is characteristic of ichthyosis vulgaris?
    a) Erythematous plaques with silvery scales
    b) Thick, plate-like scales affecting the entire body
    c) Fine, white to grayish scales on extensor surfaces, sparing flexural areas
    d) Vesicular rash with intense itching
  3. Which of the following is the most effective first-line treatment for ichthyosis vulgaris?
    a) Oral antibiotics
    b) Systemic corticosteroids
    c) Moisturizers and keratolytic agents
    d) Antifungal creams
  4. What is a key distinguishing factor of ichthyosis vulgaris compared to other cornification disorders?
    a) It is acquired rather than genetic
    b) It worsens in warm, humid climates
    c) It is associated with atopy and improves with age
    d) It primarily affects the face and scalp
Reveal answers

Answers

  1. (c) Ichthyosis vulgaris
    • The patient’s dry, scaly skin on extensor surfaces, worsening in winter, and family history suggest ichthyosis vulgaris, a common disorder of cornification.
  2. (c) Fine, white to grayish scales on extensor surfaces, sparing flexural areas
    • Ichthyosis vulgaris presents with fine scales primarily on the arms and legs, while flexural areas remain unaffected. This distinguishes it from other scaling disorders.
  3. (c) Moisturizers and keratolytic agents
    • First-line treatment includes frequent moisturization and keratolytics (e.g., urea, lactic acid) to help exfoliate dead skin cells and retain hydration.
  4. (c) It is associated with atopy and improves with age
    • Ichthyosis vulgaris is linked to atopic conditions (e.g., eczema, asthma) and often becomes less severe with age. It also worsens in dry, cold climates.