ICD10 code of Cutis Laxa and ICD9 code

Cutis laxa is clinically classified under the ICD-10 code Q82.8 (Other specified congenital malformations of skin) and the ICD-9 code 757.39 (Other specified congenital anomalies of skin). These codes are used for administrative and billing purposes to identify the spectrum of connective tissue disorders characterized by inelastic, sagging skin.

What exactly is Cutis laxa?

Cutis laxa is a rare, heterogeneous group of connective tissue disorders characterized by skin that lacks elasticity and hangs in loose folds. Unlike other skin conditions, Cutis laxa often involves systemic manifestations, including pulmonary emphysema, inguinal hernias, and cardiovascular abnormalities due to the fragmentation of elastic fibers throughout the body.

How is Cutis laxa classified?

Because Cutis laxa can be inherited in various ways or occur sporadically, clinicians categorize it by the mode of transmission and the severity of systemic involvement. The clinical classification typically includes:

• Autosomal Dominant Cutis laxa: Often presents with milder skin symptoms and later onset.


• Autosomal Recessive Cutis laxa (Type I & II): Generally more severe, involving early-onset emphysema and joint laxity.


• X-linked Cutis laxa: Also known as Occipital Horn Syndrome, which includes skeletal abnormalities.

Is Cutis laxa hereditary?

Yes, Cutis laxa is primarily a genetic condition caused by mutations in genes such as ELN, FBLN5, or ATP7A. Because the condition is rare, genetic counseling is essential for families to understand the specific inheritance pattern—whether autosomal dominant, autosomal recessive, or X-linked—and the recurrence risk for future pregnancies.

Next steps

• Consult with a clinical geneticist to confirm the specific subtype of Cutis laxa through molecular testing.


• Schedule regular screenings with a cardiologist and pulmonologist to monitor potential systemic complications.


• Connect with the DiseaseMaps.org community to share experiences with others navigating the complexities of this rare diagnosis.

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 regarding a medical condition.

References

• Orphanet: Cutis laxa (ORPHA:211)


• NIH Genetic and Rare Diseases Information Center (GARD): Cutis laxa


• OMIM (Online Mendelian Inheritance in Man): Entry #219100 (Cutis Laxa, Autosomal Recessive)


• ICD-10 Data: Q82.8 - Other specified congenital malformations of skin