Ichthyosis Prematurity Syndrome (IPS)

Ichthyosis Prematurity Syndrome (IPS) is a rare skin disorder that mostly appears at birth. It affects the skin, making it scaly and sometimes causing respiratory problems.

Types:

There isn’t a well-defined list of types of IPS. However, IPS is a subtype of ichthyosis, a larger group of skin disorders. Some other ichthyosis types include:

  1. Ichthyosis vulgaris
  2. X-linked ichthyosis
  3. Harlequin ichthyosis
  4. Lamellar ichthyosis
  5. Congenital ichthyosiform erythroderma

Causes:

The exact causes of IPS are not entirely understood. Still, we know that:

  1. It’s genetic: This means it’s passed down from parents to children.
  2. Mutations in specific genes: Changes in the FATP4 gene are common.
  3. It’s autosomal recessive: Both parents must carry the gene mutation.
  4. It’s not due to external factors: Unlike sunburn, IPS isn’t caused by outside sources.
  5. Not related to diet.
  6. Not contagious.
  7. Not due to drugs or medications during pregnancy.
  8. It’s not gender-specific: Both males and females can have it.
  9. Not due to infections.
  10. Unaffected parents can have affected kids.
  11. Some families might have higher risk.
  12. Not caused by vaccinations.
  13. No environmental link established.
  14. Not due to allergies.
  15. Not linked to maternal age.
  16. Random mutations can happen.
  17. Consanguinity increases risk: Marriages within relatives might elevate the chances.
  18. Not influenced by altitude or climate.
  19. Not due to lifestyle.
  20. Not related to prenatal exposure.

Symptoms:

  1. Scaly skin: The most common sign.
  2. Reddish skin color.
  3. Respiratory problems: Breathing issues at birth.
  4. Skin feels tight.
  5. Dry skin.
  6. Itching.
  7. Eye problems: Sometimes eyelid issues.
  8. Eczema: Skin inflammation.
  9. Blistering: Especially at birth.
  10. Thickened skin.
  11. Hair loss or thinning.
  12. Ear anomalies.
  13. Difficulty regulating body temperature.
  14. Cracking or splitting skin.
  15. Nail abnormalities.
  16. Reduced sweating.
  17. Increased risk of infections.
  18. Swelling due to scratching.
  19. Sensitivity to sunlight.
  20. Ears might be underdeveloped.

Diagnostic Tests:

  1. Skin biopsy: A small skin sample is studied.
  2. Genetic testing: Identifies gene mutations.
  3. Blood tests: Check overall health.
  4. Prenatal testing: Before birth, checking for signs.
  5. Ultrasound: Checking fetus skin thickness.
  6. Eye examination: For eyelid issues.
  7. Skin hydration tests.
  8. Skin barrier function test.
  9. pH level testing of the skin.
  10. Microscopic examination of hair.
  11. Swab tests: For infections.
  12. Nail examination.
  13. Family history analysis.
  14. Dermoscopy: Detailed skin examination.
  15. Hearing test: Sometimes required.
  16. Skin culture: Identify infections.
  17. Electron microscopy: Detailed cell study.
  18. Sweat testing.
  19. Light microscopic study of skin.
  20. Enzyme level testing in the skin.

Treatments:

  1. Moisturizing: Keep skin hydrated.
  2. Topical steroids: Reduces inflammation.
  3. Antibiotics: If infections appear.
  4. Bathing routines: Helps remove scales.
  5. Keratolytics: Helps with scaling.
  6. Vitamin D analogs: For skin health.
  7. Sunscreen: Protects sensitive skin.
  8. Antihistamines: Reduces itching.
  9. Retinoids: Helps skin renewal.
  10. Phototherapy: Light treatment.
  11. Eye treatments: If eyelids are affected.
  12. Special shampoos: For scalp issues.
  13. Cool, humid environments: Helps with symptoms.
  14. Regular medical check-ups.
  15. Genetic counseling: Understand the risk of passing it on.
  16. Avoiding skin irritants.
  17. Gentle exfoliation.
  18. Wound care: For blisters or cuts.
  19. UVB therapy: Specific light treatment.
  20. Systemic retinoids: Oral medication.
  21. Biological therapies: Newer treatment.
  22. Immunosuppressants: Reduces skin’s immune response.
  23. Liquid bandage products.
  24. Counseling: Mental and emotional support.
  25. Surgery: Rarely, for severe complications.
  26. Dietary supplements: Supports skin health.
  27. Support groups: Emotional support.
  28. Acitretin: A specific retinoid.
  29. Physical therapy: For movement restriction.
  30. Laser treatments: For specific symptoms.

Drugs:

  1. Emollients: Skin moisturizers.
  2. Hydrocortisone: Mild steroid.
  3. Tetracyclines: Antibiotics.
  4. Tacrolimus: Reduces itching.
  5. Acitretin.
  6. Isotretinoin: Another retinoid.
  7. Methotrexate: Immunosuppressant.
  8. Cyclosporine.
  9. Mycophenolate mofetil.
  10. Prednisone: Steroid.
  11. Urea-containing creams.
  12. Coal tar: Reduces scaling.
  13. Salicylic acid.
  14. Lactic acid.
  15. Cetaphil: Gentle cleanser.
  16. Eucerin: For hydration.
  17. Aveeno: Oat-based moisturizer.
  18. Betamethasone: Steroid.
  19. Clarithromycin: Antibiotic.
  20. Azathioprine: Immunosuppressant.

In simple terms, IPS is a skin condition present at birth. It causes scaly skin and sometimes breathing problems. It’s genetic, meaning it comes from the family line. There are tests to diagnose it, and many treatments and drugs can help manage the symptoms. Regular care and medical attention make living with IPS manageable.

 

Disclaimer: Each person’s journey is unique, always seek the advice of a medical professional before trying any treatments to ensure to find the best plan for you. This guide is for general information and educational purposes only. If you or someone are suffering from this disease condition bookmark this page or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. Thank you for giving your valuable time to read the article.

  1. https://medlineplus.gov/skinconditions.html
  2. https://www.aad.org/about/burden-of-skin-disease
  3. https://www.usa.gov/federal-agencies/national-institute-of-arthritis-musculoskeletal-and-skin-diseases
  4. https://www.cdc.gov/niosh/topics/skin/default.html
  5. https://www.skincancer.org/
  6. https://www.jaad.org/
  7. https://www.psoriasis.org/about-psoriasis/
  8. https://books.google.com/books?
  9. https://www.niams.nih.gov/health-topics/skin-diseases
  10. https://cms.centerwatch.com/directories/1067-fda-approved-drugs/topic/292-skin-infections-disorders
  11. https://www.fda.gov/files/drugs/published/Acute-Bacterial-Skin-and-Skin-Structure-Infections—Developing-Drugs-for-Treatment.pdf
  12. https://dermnetnz.org/topics
  13. https://www.aaaai.org/conditions-treatments/allergies/skin-allergy
  14. https://www.sciencedirect.com/topics/medicine-and-dentistry/occupational-skin-disease
  15. https://aafa.org/allergies/allergy-symptoms/skin-allergies/
  16. https://www.nibib.nih.gov/
  17. https://rxharun.com/rxharun/rxharun/article-types/skin-care-beauty/skin-diseases-types-symptoms-treatment/
  18. https://www.nei.nih.gov/
  19. https://en.wikipedia.org/wiki/List_of_skin_conditions
  20. https://en.wikipedia.org/?title=List_of_skin_diseases&redirect=no
  21. https://en.wikipedia.org/wiki/Skin_condition
  22. https://oxfordtreatment.com/
  23. https://www.nidcd.nih.gov/health/
  24. https://consumer.ftc.gov/articles/w
  25. https://www.nccih.nih.gov/health
  26. https://catalog.ninds.nih.gov/
  27. https://www.aarda.org/diseaselist/
  28. https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets
  29. https://www.nibib.nih.gov/
  30. https://www.nia.nih.gov/health/topics
  31. https://www.nichd.nih.gov/
  32. https://www.nimh.nih.gov/health/topics
  33. https://www.nichd.nih.gov/
  34. https://www.niehs.nih.gov
  35. https://www.nimhd.nih.gov/
  36. https://www.nhlbi.nih.gov/health-topics
  37. https://obssr.od.nih.gov/
  38. https://www.nichd.nih.gov/health/topics
  39. https://rarediseases.info.nih.gov/diseases
  40. https://beta.rarediseases.info.nih.gov/diseases
  41. https://orwh.od.nih.gov/

References
Amazon Best Seller
Bio-Oil Skincare Body Oil, Serum for Scars and Stretchmarks, Face Moisturizer Dry Skin, Non-Greasy, Dermatologist Recommended, Non-Comedogenic, For All Skin Types, with Vitamin A, E, 4.2 oz
Bio-Oil Skincare Body Oil, Serum for Scars and Stretchmarks, Face Moisturizer Dry Skin, Non-Greasy, Dermatologist Recommended, Non-Comedogenic, For All Skin Types, with Vitamin A, E, 4.2 oz
  • HELPS IMPROVES APPEARANCE OF SCARS AND STRETCH MARKS - Dermatologist recommended and clinically proven for scars, stretch marks, uneven skin tone and so much more
  • PACKED WITH NATUAL OILS - Vitamin E helps maintain healthy looking skin while natural Chamomile and Lavender Oil calm and soothe
  • LOCKS IN ESSENTIAL HYDRATION WITHOUT CLOGGING PORES - Bio-Oil Skincare Oil is a uniquely formulated, non-greasy body oil that hydrates skin and helps retain essential moisture
  • PLANET & ANIMAL FRIENDLY — Vegan friendly, paraben free, cruelty free, non-comedogenic, and 100% recyclable
  • FORMULATED FOR ALL SKIN TYPES - Helps soften skin for all types, tones, textures and safe for use on face and body and won't clog pores
$23.49 −$2.00 $21.49 Amazon Prime
Buy Now On Amazon.com Price incl. tax, excl. shipping