has characteristic has characteristic Infantile onset hyaline fibromatosis syndrome infantile systemic hyalinosis Orphanet:2176 Infantile systemic hyalinosis (ISH) is a very rare disorder belonging to the heterogeneous group of genetic fibromatoses and is characterized by progressive joint contractures, skin abnormalities, severe chronic pain and widespread deposition of hyaline material in many tissues such as the skin, skeletal muscle, cardiac muscle, gastrointestinal tract, lymph nodes, spleen, thyroid, and adrenal glands. icd11.foundation:2089325724 MONDO:0016331 MEDGEN:1803732 UMLS:C5574678 SCTID:238867003 GARD:0006807 OMIM:236490