The Skin in EDS: Scars, Stretch Marks, and More

Ehlers-Danlos Syndrome (EDS) is a group of connective tissue disorders characterized by a wide range of symptoms, many of which prominently affect the skin. From fragile, easily bruised skin to distinctive scarring and hyperextensibility, the skin serves as a critical diagnostic feature in many EDS subtypes. These visible and tactile skin changes not only contribute to the diagnostic process but also play a significant role in the daily lives of individuals living with EDS.

Understanding the skin's behavior in EDS is essential for proper care and management. Skin features such as papyraceous scars, molluscoid pseudotumors, and hyperextensibility can vary greatly between EDS subtypes, and their presence may point to underlying connective tissue abnormalities. This guide explores the unique skin characteristics associated with EDS, offering insights into their clinical relevance, management strategies, and practical tips for individuals navigating life with this complex condition.

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Key Skin Features in EDS

EDS affects the skin in several notable ways, including:

1. Skin Hyperextensibility
   Hyperextensibility is a defining feature of EDS, referring to the ability of the skin to stretch beyond normal limits. When gently pulled, the skin can often stretch more than 1.5 cm, particularly on the volar forearm, before recoiling to its original state. This excessive elasticity is most pronounced in classical EDS (cEDS) but may also occur in other subtypes, albeit to a lesser degree.
2. Skin Fragility
   Fragile skin is a hallmark of many EDS subtypes, making it prone to splitting and tearing even with minimal trauma. This fragility is particularly evident in classical, vascular, and dermatosparaxis subtypes. Small tears, particularly on the knees, elbows, and forehead, are common and often accompanied by delayed healing.
3. Abnormal Scarring
   Individuals with EDS often develop abnormal scars that are thin, wide, sunken (atrophic), or papyraceous (resembling tissue paper). These scars tend to form on bony prominences and are more severe in classical EDS. Molluscoid pseudotumors, which are fleshy, fibrous nodules, and subcutaneous spheroids, which are small calcified lumps under the skin, can also develop at pressure points.
4. Easy Bruising
   Easy bruising is a common feature across many EDS subtypes. It occurs due to fragile blood vessels and reduced skin integrity, not clotting issues. In vascular EDS (vEDS), spontaneous bruising at unusual sites, such as the cheeks and back, is particularly notable and can indicate significant systemic fragility.
5. Unusual Skin Texture
   EDS skin often has a soft, velvety, or doughy texture. This characteristic is most apparent on the forearms and lower legs and is caused by alterations in collagen structure.
6. Translucent Skin
   In vascular EDS, the skin is thin and translucent, allowing veins to become visibly prominent, particularly over the chest, hands, and face. This feature, combined with fragile blood vessels, contributes to the unique presentation of vEDS.

Additional Skin Findings in EDS

Several unique dermatological findings are associated with EDS:

1. Piezogenic Papules
   These small, soft, skin-colored lumps appear on the heels due to fat herniation under pressure. They are common in hypermobile EDS and can occasionally cause pain.
2. Elastosis Perforans Serpiginosa (EPS)
   This rare condition involves the extrusion of damaged elastic fibers through the skin, presenting as raised, circular rashes. It is most often associated with vascular EDS.
3. Pretibial Plaques
   Seen in periodontal EDS, these pigmented plaques occur on the shins and overlap with features of vascular and classical EDS.
4. Subcutaneous Spheroids
   Hard, calcified lumps under the skin, often over bony areas like the tibia and ulna, caused by fat degeneration.

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EDS Subtypes and Their Skin Features

The type and severity of skin changes depend on the specific subtype of EDS.

Subtype & Primary Skin Features

• Classical EDS (cEDS):
  • Severe hyperextensibility
  • Atrophic scars
  • Soft velvety skin
  • Molluscoid pseudotumors
  • Easy bruising
• Hypermobile EDS (hEDS):
  • Mild hyperextensibility
  • Soft/doughy skin
  • Unexplained stretch marks
  • Piezogenic papules
• Vascular EDS (vEDS):
  • Thin, translucent skin
  • Prominent veins
  • Acrogeria
  • Easy bruising
  • Spontaneous hematomas
• Dermatosparaxis EDS:
  • Extreme skin fragility
  • Postnatal tears
  • Sagging skin folds
  • Doughy texture
  • Severe bruising
• Kyphoscoliotic EDS:
  • Hyperextensibility
  • Atrophic scars
  • Easy bruising
  • Keratosis pilaris
• Arthrochalasia EDS:
  • Hyperextensible skin
  • Atrophic scars
  • Bruising
  • Congenital hip dislocation

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Skin and UV Exposure in EDS

Individuals with EDS are especially susceptible to sun damage due to compromised skin integrity. UV radiation accelerates the breakdown of collagen and elastin, which are already weakened in EDS, leading to:

• Premature Aging: Thin skin, fine wrinkles, and loss of elasticity develop earlier in life.
• Delayed Healing: UV-induced damage heals poorly, often leaving hyperpigmentation or widened scars.
• Increased Fragility: Sun exposure can worsen skin tearing and bruising, particularly in cEDS and vEDS.

Sun Protection Strategies

To mitigate UV damage, individuals with EDS should:

• Use broad-spectrum sunscreen (SPF 30+), reapplying every two hours during prolonged exposure.
• Wear protective clothing, including hats, sunglasses, and UV-blocking fabrics.
• Avoid tanning beds and seek shade during peak sun hours.

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Wound Care and Surgical Management

Given the delayed wound healing and risk of scar widening in EDS, wound care requires special considerations:

• Deep Layered Sutures: Surgical wounds should be stitched in layers to minimize tension.
• Prolonged Stitch Retention: Superficial stitches should remain longer than standard timelines to prevent dehiscence.
• Adhesive Support: Steristrips or adhesive tapes can reduce the risk of wound stretching.
• Gentle Dressing Changes: Avoid harsh adhesives that can further damage fragile skin.

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Comparing EDS Skin to Normal Skin

A. Elasticity vs. Hyperextensibility

EDS skin stretches beyond normal limits due to hyperextensibility, which is most pronounced in classical EDS. This characteristic is distinct from the elasticity of healthy skin.

B. Fragility and Delayed Wound Healing

EDS-affected skin is more prone to tearing and bruising, with wounds taking longer to heal. This fragility leads to the development of distinctive scars like atrophic or papyraceous scars.

C. Stretch Marks in EDS

Stretch marks in EDS may appear without significant stretching events, such as weight changes or pregnancy. These marks often occur in areas of high skin tension, reflecting underlying connective tissue abnormalities.

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Daily Skin Care Tips for EDS

Managing EDS skin requires a gentle, preventative approach to avoid trauma and maintain hydration:

• Moisturize Regularly: Use hypoallergenic, fragrance-free moisturizers to reduce dryness and improve elasticity.
• Mild Cleansing: Avoid harsh soaps and scrubs; use gentle, pH-balanced cleansers.
• Protect Against Trauma: Soft padding on furniture and protective clothing can help reduce minor injuries.
• Monitor Wounds: Seek prompt care for cuts or bruises that show signs of infection, pain, or delayed healing.

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Conclusion

The skin manifestations of EDS are varied and significant, reflecting the underlying connective tissue defect. Hyperextensibility, fragility, easy bruising, and abnormal scarring are common across subtypes, though their severity differs. Environmental factors like UV exposure further exacerbate skin issues, requiring proactive care and prevention. By adopting tailored skincare routines, protective measures, and proper wound management, individuals with EDS can better maintain their skin health and reduce complications.

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FAQ

Skin Conditions and Scarring in EDS

• Does EDS cause skin issues?
  Yes, EDS can lead to fragile, stretchy, and bruised skin, as well as poor wound healing and distinctive scars.
• Does EDS cause skin rashes?
  While EDS itself doesn't cause rashes, associated conditions like Mast Cell Activation Syndrome (MCAS) may trigger them.
• What are papyraceous scars?
  These are thin, delicate scars resembling tissue paper, often seen in classical EDS.
• What are hemosideric scars?
  Hemosideric scars develop when blood leaks under the skin, leaving brownish discoloration due to iron deposits.
• Why do people with EDS bruise easily?
  Weak blood vessels and fragile skin lead to increased susceptibility to bruising, often with minimal trauma.
• What are cigarette-paper-like scars in EDS?
  Thin, wrinkled scars with a texture resembling cigarette paper, caused by inadequate wound healing.
• What are molluscoid pseudotumors?
  These are soft, fibrous lumps typically found over pressure points like elbows or knees, commonly in classical EDS.
• What are subcutaneous spheroids?
  Calcified nodules under the skin caused by fat degeneration, often found in pressure-prone areas.
• Are there skin disorders unique to EDS?
  Yes, features like molluscoid pseudotumors, papyraceous scars, and subcutaneous spheroids are specific to EDS.
• What are widened atrophic scars in EDS?
  Broad, sunken scars resulting from insufficient wound repair, a hallmark of classical EDS.
• What are elephant track signs in EDS?
  These refer to overlapping lines or streaks on the skin, often a pattern of scarring in EDS.
• What are hemosideric and papyraceous scars in EDS?
  Hemosideric scars involve discoloration from blood leakage; papyraceous scars are fragile and tissue-like.

Skin Texture and Sensitivity

• Why is itchy skin common in EDS?
  Itchy skin may arise from dryness or hypersensitivity, both of which are common in EDS.
• Why does EDS skin feel damp or doughy?
  The abnormal collagen structure in EDS can cause skin to retain moisture, giving it a damp, soft texture.
• Can EDS cause dry skin?
  Yes, due to disrupted skin barrier function, individuals with EDS may experience dry, flaky skin.
• Does EDS cause skin sensitivity?
  Yes, EDS skin is more prone to irritation and sensitivity due to its fragility and underlying connective tissue issues.
• Why does translucent skin occur in vascular EDS?
  Thin skin and fragile vascular structures allow veins to appear more visible in vascular EDS.
• What is the connection between EDS and UV exposure?
  UV rays can exacerbate skin fragility and damage in EDS, making sun protection essential.
• Can EDS increase the risk of skin cancer?
  There is no direct link, but fragile skin may be more vulnerable to UV damage, which increases skin cancer risk.

Appearance and Fragility

• Why do people with EDS look younger?
  People with EDS often have soft, velvety skin due to connective tissue abnormalities, which may give the appearance of youthful skin. However, their skin is also fragile and prone to premature aging under environmental stressors like UV exposure.
• What is translucent skin, and why does it occur in EDS?
  Translucent skin is when veins are visible through the skin, caused by thin dermal layers in EDS.
• Can you have stretchy skin without having EDS?
  Yes, but stretchy skin without additional features (e.g., scarring, fragility) does not typically indicate EDS.
• Can EDS lead to stretch marks?
  Yes, stretch marks may develop even without significant stretching, reflecting underlying tissue abnormalities.
• Does EDS affect your hair?
  Indirectly, yes. Fragile scalp skin may affect hair density or growth, though this varies widely.
• Does EDS affect the scalp?
  Scalp skin may be prone to bruising or sensitivity, particularly during hairstyling or treatments.
• Why do I bruise when I scratch my skin?
  Bruising from scratching can occur due to fragile blood vessels under the skin. This is more common in individuals with conditions like Ehlers-Danlos Syndrome (EDS), aging skin, or medical conditions that affect blood clotting or skin integrity.
• Is bruising from scratching normal?
  Occasional bruising is not uncommon, especially if the skin or blood vessels are fragile. However, frequent or severe bruising should be evaluated by a healthcare professional.
• What causes fragile skin or blood vessels?
  Fragility can result from genetic conditions like EDS, aging, long-term use of corticosteroids, vitamin deficiencies (e.g., Vitamin C or K), or connective tissue disorders.
• Can allergies or itchy skin cause bruising when scratched?
  Yes, persistent itching from allergies or eczema, combined with vigorous scratching, can damage fragile blood vessels and cause bruising.
• Should I be concerned if I bruise easily from scratching?
  Occasional bruising isn’t usually concerning, but frequent or unexplained bruising warrants medical evaluation to rule out blood disorders, nutritional deficiencies, or connective tissue diseases.
• How can I prevent bruising when scratching?
  • Keep nails trimmed to avoid excessive damage.
  • Use gentle pressure instead of vigorous scratching.
  • Apply moisturizer or anti-itch creams to reduce the need to scratch.
  • Avoid known irritants or allergens that trigger itching.
• What treatments are available for easy bruising?
  Treatments depend on the underlying cause and may include:
  • Vitamin supplements (e.g., Vitamin C for collagen support).
  • Topical creams to strengthen the skin.
  • Medications to address underlying conditions, such as antihistamines for allergies

Skin Care Tips

• What is the best lotion for fragile EDS skin?
  Gentle, fragrance-free lotions with ceramides and hyaluronic acid are ideal for hydration and skin barrier support.
• What are the best ways to protect EDS skin?
  Use soft, protective clothing, avoid harsh adhesives, and apply sunscreen to prevent damage.
• Can people with EDS tan?
  Tanning is not recommended for people with EDS as UV exposure can exacerbate skin fragility, delay wound healing, and accelerate aging. Protective clothing, high-SPF sunscreen, and avoiding tanning beds are highly advised.

Tattoos and Piercings

• Can I get a tattoo if I have Ehlers-Danlos syndrome?
  Yes, tattoos are possible for individuals with EDS, but skin fragility, hyperextensibility, and delayed healing require extra care. Billie Eilish has hEDS and lots of tattoos. Many other regular folks with hEDS and celebrities alike have tattoos with hEDS.‍
• Are piercings safe for individuals with EDS?
  They can be, but special care and experienced professionals are necessary to reduce risks.‍
• Can you have EDS without stretchy skin?
  Yes, not all EDS subtypes involve noticeably stretchy skin, such as vascular or arthrochalasia EDS.

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References

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