Short answer · Medically reviewed summary · Last updated: 2026-05-08
Ullrich Congenital Muscular Dystrophy (UCMD) is classified under the ICD-10 code G71.09 (Other specified muscular dystrophies) and was historically categorized under ICD-9 code 359.1 (Hereditary progressive muscular dystrophy). Because UCMD is a rare condition, it does not currently have a unique, disease-specific ICD code, which is common for many rare collagen VI-related disorders. What is the clinical classification of Ullrich Congenital Muscular Dystrophy? Ullrich Congenital Muscular Dystrophy is a severe subtype of collagen VI-related dystrophy.
ICD10 code of Ullrich Congenital Muscular Dystrophy and ICD9 code
ICD-10 and ICD-9 codes for Ullrich Congenital Muscular Dystrophy, with classification details for clinicians, coders and patients.
Ullrich Congenital Muscular Dystrophy (UCMD) is classified under the ICD-10 code G71.09 (Other specified muscular dystrophies) and was historically categorized under ICD-9 code 359.1 (Hereditary progressive muscular dystrophy). Because UCMD is a rare condition, it does not currently have a unique, disease-specific ICD code, which is common for many rare collagen VI-related disorders.
What is the clinical classification of Ullrich Congenital Muscular Dystrophy?
Ullrich Congenital Muscular Dystrophy is a severe subtype of collagen VI-related dystrophy. Clinically, it is characterized by early-onset muscle weakness, joint hyperlaxity in the distal joints, and joint contractures in the proximal joints. Because the medical community classifies Ullrich Congenital Muscular Dystrophy as a rare genetic disorder, it is often grouped into broader diagnostic categories for billing and administrative purposes, necessitating the use of the G71.09 code in clinical practice.
How does the medical community track Ullrich Congenital Muscular Dystrophy?
While ICD codes are essential for insurance, clinicians often use the OMIM identifier #254090 to track the specific genetic mutations associated with Ullrich Congenital Muscular Dystrophy. Our community at DiseaseMaps.org currently supports 13 individuals living with this condition, highlighting the importance of patient-led data in understanding the real-world impact of Ullrich Congenital Muscular Dystrophy beyond standard billing codes.
What are the primary diagnostic features of this condition?
Diagnosis of Ullrich Congenital Muscular Dystrophy typically involves a combination of clinical evaluation and genetic testing. Key features include:
- Congenital muscle weakness and hypotonia (floppy infant syndrome).
- Proximal joint contractures (e.g., hips, knees).
- Distal joint hyperlaxity (e.g., fingers, toes).
- Respiratory insufficiency, which often requires early monitoring.
- Skin changes, such as follicular hyperkeratosis.
Next steps
- Consult with a neuromuscular specialist or a pediatric neurologist to confirm your diagnostic coding.
- Connect with the 13 community members at DiseaseMaps.org who share your experience with Ullrich Congenital Muscular Dystrophy.
- Request a referral to a genetic counselor to discuss family planning and inheritance patterns.
- Monitor respiratory function regularly, as this is a clinical priority for patients with Ullrich Congenital Muscular Dystrophy.
Medical disclaimer: This information is for educational purposes only and does not constitute professional medical advice, diagnosis, or treatment; always seek the advice of your physician with any questions regarding a medical condition.
References
- Orphanet: Collagen VI-related dystrophy (ORPHA:576)
- NIH GARD: Ullrich congenital muscular dystrophy
- OMIM: Ullrich Muscular Dystrophy 1 (#254090)
- Cure CMD: Information on Collagen VI-Related Dystrophies
