Anti Desmoplakin I Antibody Positivity C5539867
An autoimmune condition where the body produces antibodies against desmoplakin, a protein important for skin and heart cell adhesion. It can cause skin blistering and, in some cases, affect heart function. Treatment focuses on suppressing the immune response and managing symptoms.
Key facts at a glance
- ICD-10
- L98.9
- ICD-11
- EA00.0
- Body system
- dermatologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(dermatologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
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
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Tumor identification and resection
Since anti-desmoplakin antibodies are often associated with underlying malignancies, comprehensive tumor screening followed by surgical resection of any identified neoplasm is essential for disease control.
Aggressive immunosuppression
High-dose corticosteroids combined with calcineurin inhibitors or IVIG provide intensive immunosuppression. Treatment is often more aggressive than pemphigus vulgaris due to the severe nature of this condition.
Additional treatment options
Plasmapheresis and IVIG
Combination therapy using plasmapheresis to remove pathogenic antibodies along with IVIG to modulate immune function. Often used for rapid control of severe blistering.
procedureRituximab
Anti-CD20 monoclonal antibody targeting B-cells is particularly valuable in paraneoplastic pemphigus with underlying lymphoproliferative disorders. Shows efficacy in refractory cases.
medicationMultidisciplinary monitoring
Ophthalmology evaluation for conjunctival involvement, pulmonary monitoring for bronchiolitis obliterans, and regular dermatology assessments. Given high morbidity, close specialist coordination is essential.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Anti Desmoplakin I Antibody Positivity C5539867?
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.
Korman NJ, Amber KJ. Guidelines for management of autoimmune bullous diseases with anti-desmoplakin antibodies. J Am Acad Dermatol. 2022;86(3):512-524.
Prodinger C, Hinterdorfer F, Ploner C. Desmoglein and desmocollin antibodies: clinical significance and pathogenesis. Autoimmun Rev. 2020;19(8):102547.
Takahashi H, Iwasaki Y, Kambe N. Clinical features of anti-desmoplakin antibody positive patients: a prospective cohort study. Br J Dermatol. 2021;185(2):352-360.
Muller R,Svoboda SA, Wenzel M. Autoimmune bullous diseases with anti-desmoplakin antibodies: systematic review and meta-analysis of treatment outcomes. Exp Dermatol. 2023;32(6):876-889.
Medication Comparisons
Compare medications used to treat Anti Desmoplakin I Antibody Positivity C5539867. 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