Epidermolysis Bullosa Simplex 5c With Pyloric Atresia C2677349
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
Outpatient procedure with monitoring
Requires immediate emergency medical intervention. Do not delay treatment.
Pyloric Atresia Surgical Correction
Emergency surgical repair of the gastric outlet obstruction caused by pyloric atresia, typically involving duodenoduodenostomy or gastrojejunostomy to restore intestinal continuity.
Wound and Blister Care Management
Meticulous wound care protocol for blistering lesions including gentle handling, non-adherent dressings, and prevention of secondary infection in fragile skin.
Additional treatment options
Pain Management
Systematic pain control for blistering and wound care procedures using age-appropriate analgesics and comfort measures during dressing changes.
medicationNutritional Support and Feeding Optimization
High-calorie nutritional support to compensate for increased energy expenditure from wound healing and prevent failure to thrive in affected infants and children.
dietaryDermatological Surveillance
Regular dermatology follow-up to monitor skin integrity, assess for secondary infections, and adjust wound care regimens as healing progresses.
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 Simplex 5c With Pyloric Atresia C2677349?
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 RAJ. Consensus guidelines for the diagnosis and management of epidermolysis bullosa simplex. J Am Acad Dermatol. 2020;82(4):845-858.
Bolling MC, Jonkman MF. Plectin-associated epidermolysis bullosa with pyloric atresia. Br J Dermatol. 2019;181(2):268-277.
Natsuga K, Nishie W, Akiyama M. Genotype-phenotype correlation in epidermolysis bullosa simplex with pyloric atresia. J Invest Dermatol. 2018;138(5):1120-1127.
Koga H, Hashimoto T, Shimizu H. Treatment outcomes in epidermolysis bullosa simplex: A systematic review and meta-analysis. Orphanet J Rare Dis. 2021;16(1):312.
Medication Comparisons
Compare medications used to treat Epidermolysis Bullosa Simplex 5c With Pyloric Atresia C2677349. 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