# Symptoms and phases of epidermolysis bullosa
Epidermolysis bullosa (EB) does not progress in clearly defined phases like some other diseases. Rather, it is an ongoing condition in which blistering and wound healing problems recur throughout life. However, there are differences in how severe the symptoms are and how they develop over time — particularly in the first years and later when complications occur. This description follows the disease course from early to late.
The first months and years (neonatal period and early childhood)
In many cases, EB is noticed when the newborn already has blisters or wounds, sometimes even in the womb. In others, symptoms appear in the first weeks or months of life.
**What happens:**
- **Blistering** on skin, mouth, lips and mucous membranes; these can occur spontaneously or after minor trauma (friction, pressure, temperature changes)
- **Bloody erosions and open wounds** that are painful and heal slowly
- **Infections** of the wounds, because the natural barrier of the skin is damaged
- **Pustules** on skin and sometimes also on mucous membranes
- **Feeding problems**: blisters in and around the mouth make drinking and eating difficult, which can lead to growth delay
- **Pain**, especially during diaper changes, washing or touching; many children cry intensely
- **Restlessness and sleep problems** due to discomfort and pain
**What this means for daily life: **
- Parents must handle the child carefully; even normal activities such as diaper changes, bathing and dressing require caution
- Feeding can be difficult; the child may drink more slowly or take in less
- Many hospitalizations for infection control and support
- Parents experience severe stress and uncertainty
**Figures on this period:**
The most severe forms (particularly severe recessive dystrophic EB and junctional EB with extensive involvement) can cause serious complications in the first weeks of life. Patients with the most extensive form have a higher mortality rate in the neonatal period and early childhood, mainly due to infections and malnutrition. However, exact survival rates for this stage are not consistently reported in the recent literature; they vary greatly depending on the EB type and severity.
Early and mid-childhood (approximately 1-12 years)
As the child grows older, the disease pattern often stabilizes somewhat, but symptoms remain considerable.
**What happens:**
- **Recurring blistering** especially on areas under pressure: feet, hands, knees, elbows, buttocks
- **Open wounds** that can last weeks to months before they heal
- **Scars and contractures** (skin shortening); especially on hands and feet, mobility can become limited
- **Nail problems**: nails grow deformed or are lost
- **Mouth and throat involvement**: blisters and erosions in the mouth can cause pain when eating; some children have difficulty swallowing
- **Gastrointestinal complaints**: constipation is very common
- **Infections** of wounds, sometimes with bacteria that are difficult to treat
- **Itching**, sometimes very intense
- **Hair loss** (alopecia) can occur, depending on the EB type
- **Dental problems**: tooth erosion and gum inflammation
- **Growth delay** due to chronic infections, inadequate nutrition and the disease burden itself
**What this means for daily life: **
- Children have difficulty playing, sports and normal physical activities
- Wound care is a daily task that can take hours
- School can be difficult; many children miss many lessons due to illness periods
- Social interaction can be strained by visible wounds and scars
- Parents must be constantly alert for infections and new blisters
- Nutrition and growth require extra attention
**Figures on this period:**
Most children with less severe forms of EB reach puberty. For children with recessive dystrophic EB (RDEB) and severe junctional forms: survival in childhood varies greatly. Patients with RDEB have, according to recent data, median life expectancies that can range from several years to adult life, depending on individual factors and type. Exact median life expectancy is not unambiguously reported in current guidelines.
Puberty and adolescence (12-18 years)
This is a turning point in the disease course for many people with EB.
**What happens:**
- **The same blistering pattern**, but hormonal changes can worsen itching and inflammatory reactions
- **Increasing scars and contractures**, especially if the disease has not been well controlled until now
- **Movement restrictions**: hands can become less flexible, feet can become stiffer
- **Sexual growth and sexual maturation** can be hindered or delayed
- **Psychological burden**: adolescents struggle with visible differences, chronic pain and limitations
- **Infections** continue to occur
- **Increased itching**
- **Skin and mucosal manifestations**: in some EB forms the skin can become thinner and more fragile
**What this means for daily life: **
- Social isolation; teenagers are ashamed of visible wounds and scars
- Difficulties with identity formation and self-acceptance
- School performance can suffer
- Relationships and friendships are affected
- Independence from parents is difficult because care is still intensively needed
- Future plans (education, work, career) must be assessed realistically
**Figures on this period:**
Adolescents with EB are at risk of depression and anxiety disorders. Recent studies on quality of life (such as the QoL-REB study from 2026) show that both children and adults with EB are significantly limited in activities and social participation, but exact survival figures for this phase are not separately reported.
Adulthood (18+ years)
Adults with EB who have reached puberty often have a more stable disease course, but continuous care remains necessary.
**What happens:**
- **Ongoing blistering** according to the same pattern as before
- **Increasing scarring and contractures** as the years pass; especially hands and feet can become rigid and stiff
- **Mobility can decline**: walking sometimes becomes difficult
- **Wounds that heal poorly**, sometimes persistently open
- **Chronic pain**
- **Infections** of wounds
- **Eye and dental problems** can occur
- **Psychological burden**: depression and social isolation are not uncommon
- **Oncological risk**: patients with certain EB forms (especially RDEB) have increased risk of skin cancer (squamous cell carcinoma) in adulthood
- **Nutritional problems** due to gastrointestinal difficulties; some adults need tube feeding
- **Pneumonia and airway complications** in severe cases
**What this means for daily life: **
- Adults can sometimes live independently, but many need continuous care
- Work is difficult for many; many adults with EB cannot work full-time
- Relationships and partnership are possible, but are hindered by the disease burden
- Parenthood is hindered by the disease burden and sometimes by genetic factors
- Financial burden from medical costs
- Social isolation remains an issue
**Figures on this period:**
Life expectancy for adults with EB depends greatly on the type and severity. Patients with less severe forms (such as simple EB) can have a normal life expectancy. For patients with recessive dystrophic EB the situation is more complex: historically it was thought that many did not live beyond the third or fourth decade of life, but with improved wound care and infection control, more patients reach older ages. However, current survival figures have not been recently published in the current literature.
Late stage and increasing complications
For patients who survive into adult life, serious complications can occur.
**What happens:**
- **Extensive scars and contractures** that severely limit mobility
- **Chronic wounds** that do not heal despite intensive care
- **Recurrent infections**, sometimes with antibiotic-resistant bacteria
- **Malnutrition** due to gastrointestinal complaints
- **Anemia** (blood deficiency) due to blood loss from wounds
- **Skin cancer**: this is an important risk, especially in RDEB patients in their 30s-40s and later
- **Lung problems**: narrowed by scars in the esophagus or by inhalation of harmful particles
- **Arthritis and joint stiffness**
- **Psychological problems**: chronic pain, social isolation and repeated hospital admissions take their toll
- **Organ damage**: in severe cases, kidneys, heart or other organs can be affected
**What this means for daily life: **
- Many patients are very limited in mobility
- Hospital admissions may be needed more frequently
- Wound care may require even more time and expertise
- Palliative care and symptom management become more important
- Quality of life is central
**Figures on this period:**
Few detailed figures are available on the late stage of EB. The course and complications vary greatly from person to person. The diagnosis of skin cancer brings its own disease trajectory with it.
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When to contact your doctor
Contact with your doctor is important if:
- **Sharp increase** in blister formation or wounds that do not heal
- **Signs of infection**: greater redness, warmth, pus, fever, or wounds that become more itchy and swollen
- **New symptoms**: for example problems with swallowing, nasal discharge, facial tingling
- **Severe pain** despite medication
- **Growth delay** or feeding problems (especially in children)
- **Extensive new scars** or contractures that limit movement
- **Psychological distress**: depression, anxiety, social isolation
_This information never replaces a doctor's judgment. Always discuss your situation with your own healthcare provider._