Cutaneous T-cell lymphoma in SHOC2 mutation-associated Noonan-like syndrome with loose anagen hair

Noonan-like syndrome with loose anagen hair (NS/LAH) is a newly described rare disorder caused by a germline mutation in SHOC2, which modulates the RAS/MAPK signaling pathway. NS/LAH has features overlapping with other RASopathies, genetic conditions caused by dysregulation in RAS/MAPK signaling, including structural cardiac defects, macrocephaly, central nervous system anomalies, and short stature with the addition of loose anagen hair and atopic dermatitis.1