Question
Regarding the skin condition shown in the image,which of the following statements is true:
A. May be caused by underlying Systemic disease
B. May be transmitted as Autosomal Dominant disease
C. Affected children may also suffer from Atopy
D. All of the above
Show Answer
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Correct Answer » D Explanation |
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Ans:D. All of the above.
The Skin condition shown in the image is Icthyosis Vulgaris.
Ichthyosis vulgaris
- It is a skin disorder causing dry, scaly skin.
- The term ichthyosis is derived from the ancient Greek root ichthys, meaning fish.
Types:
- Hereditary ichthyosis vulgaris is an autosomal dominant genetic disorder . It is the most common form of ichthyosis, accounting for more than 95% of ichthyosis cases. It is often caused by altered profilaggrin expression leading to scaling and desquamation.Hereditary ichthyosis is also associated with atopy.Hereditary ichthyosis vulgaris typically is absent at birth. It appears in most patients during the first year of life and in the vast majority by age 5 years
- Acquired ichthyosis, usually appearing for the first time in adulthood, is a nonhereditary condition associated with internal disease like:
- Nutritional disorders, chronic renal failure, bone marrow transplantation, and HIV infection.
- Autoimmune diseases, including systemic lupus erythematosus and dermatomyositis
sarcoidosis, leprosy, thyroid disease, hyperparathyroidism
- Cancers :Hodgkin disease, non-Hodgkin lymphoma (including mycosis fungoides), myeloma, Kaposi sarcoma, leiomyosarcoma, and carcinomas of the lung, breast, ovary, and cervix.
- Drugs: nicotinic acid, triparanol, butyrophenones, dixyrazine, cimetidine, and clofazimine
Pathophysiology
- Ichthyosis vulgaris is classified as a retention hyperkeratosis.
Clinical Features:
- Ichthyosis vulgaris (both hereditary and acquired) is characterized by symmetrical scaling of the skin, which varies from barely visible roughness and dryness to strong horny plates..
- Scaling tends to be most prominent on the extensor surfaces of the extremities and absent on the flexor surfaces.
- Scales are small, fine, irregular, and polygonal in shape, often curling up at the edges to give the skin a rough feel.
- The lower extremities generally are more affected than the upper extremities.
- Secondary infections at fissure sites are common.
- Pruritus can be caused by dry skin.
Treatment:
- The main approach to treatment of both conditions includes hydration of the skin and application of an ointment to prevent evaporation.
- Alpha-hydroxy acids (eg, lactic, glycolic, or pyruvic acids) are effective for hydrating the skin.
- Topical retinoids are helpful for some patients.
- Removal of scales can be aided by keratolytics (eg, salicylic acid).
- Moisturizers containing urea in lower strengths