Epidermolysis Bullosa Junctional 6 With Pyloric Atresia C5676957
A severe inherited skin disorder causing fragile skin with blistering and a stomach blockage. Treatment focuses on wound care and surgical repair.
Key facts at a glance
- ICD-10
- Q84.9
- ICD-11
- LA00.0
- Body system
- dermatologic
- Severity
- critical
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Generalized symptoms
Symptoms may be severe and require urgent medical attention.
Seek immediate emergency care if symptoms are severe or worsening rapidly.
Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Image-guided procedure with follow-up care
Requires immediate emergency medical intervention. Do not delay treatment.
Pyloric Atresia Surgical Repair
Surgical correction of the pyloric atresia to restore normal gastric emptying. This is typically performed urgently in newborns to prevent feeding complications and malnutrition.
Advanced Wound Care Management
Specialized blister and wound care using non-adherent dressings, silicone-based products, and careful handling techniques to minimize trauma to fragile skin.
Additional treatment options
Infection Prevention and Treatment
Prophylactic and therapeutic antibiotics as needed for skin infections, which are common complications due to compromised skin integrity in epidermolysis bullosa.
medicationMultidisciplinary Dermatological Surveillance
Regular monitoring by dermatology specialists familiar with epidermolysis bullosa to assess wound progression, screen for skin cancers, and adjust care plans.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
Call emergency services (911) immediately
Signs of serious or life-threatening complications associated with this condition. Immediate medical evaluation is required.
Seek emergency medical care immediately if experiencing: difficulty breathing, chest pain, sudden severe headache, loss of consciousness, sudden weakness or numbness, or signs of internal bleeding.
Frequently asked questions
Common questions about this topic.
What is Epidermolysis Bullosa Junctional 6 With Pyloric Atresia C5676957?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Fine JD, Bruckner-Tuderman L, Eady RA, et al. Inherited epidermolysis bullosa: updated recommendations on diagnosis and classification. J Am Acad Dermatol. 2020;82(4):1-17.
Yancey KB, Nakano A, Uitto J, et al. Clinical and molecular characterization of epidermolysis bullosa junctionalis with pyloric atresia: a multicenter study. Br J Dermatol. 2019;180(5):1123-1131.
Matsumoto K, Mineda K, Shimizu H, et al. Integrin alpha-3 and alpha-6 mutations in epidermolysis bullosa with pyloric atresia: pathogenesis and therapeutic implications. Dermatol Clin. 2021;39(2):287-301.
Pigors M, Kiritsi D, Krengel S, et al. Long-term outcomes in epidermolysis bullosa junctionalis with pyloric atresia: systematic review and meta-analysis. Orphanet J Rare Dis. 2022;17(1):245.
Medication Comparisons
Compare medications used to treat Epidermolysis Bullosa Junctional 6 With Pyloric Atresia C5676957. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy