Table 1.

Comparison of Features of Patients with JHF and ISH

	Findings in Familya
Feature	JHF1	JHF2	ISH1	ISH2b
Consanguinity	+	+	+	−
Ethnic origin	Turkish	African American	Turkish	Swiss
Skin:
Multiple subcutaneous tumors	+	+	−	+
Thickened firm skin	−	−	+	+
Pearly nodules	+	+	−	+
Perianal granulomas	+	−	−	+
Gingiva:
Gingival hypertrophy	−	+	−	+
Gingival fibromatosis	+	?	−	?
Skeletal findings:
Joint contractures	+	−	+	+
Restricted movement of joints	+	−	+	+
Painful joints	+	−	+	+
Osteoporosis	+	?	+	+
Osteopenia	+	?	+	+
Growth:
Failure to thrive	−	−	+	+
Stunted growth	+	−	−	+
Facial features:
Coarse face	+	+	−	+
Narrow face	−	−	+	+
Large low-set dysplastic ears	−	−	+	+
Miscellaneous:
Early death	−	−	+	−
Recurrent infections	±	−	+	−
Histology/accumulation of material:
In skin	−	+	+	+
In articular soft tissues	?	−	?	+

^a+ = feature present; − = feature absent; ± = ambiguous finding; ? = unknown.

^bFamily ISH2 has been described elsewhere (Stucki et al. 2001).