Morphea en Coup de Sabre | Progressive Hemifacial Atrophy | |
---|---|---|
Average Age (years) | 10 | 13.6 |
Gender (F:M) | 2:1 – 3:1 | 3:1 |
Clinical Features | -Cutaneous induration/sclerosis | -Paramedian atrophy |
-Scalp to forehead^ | -No overlying skin induration | |
-Hyperpigmentation | -Atrophy may extend down entire face | |
-Alopecia (scalp/eyebrow) | ||
Histopathologic Features~ | -Dermal Sclerosis | -Dermal sclerosis |
-Adnexal atrophy | -Fat atrophy | |
-Mononuclear cell infiltrates* | -Decrease in adnexal structures | |
-Mononuclear cell infiltrates* | ||
Extracutaneous Associations | -Atrophy subcutaneous tissue, fat, muscle, and osteocartilaginous structures | |
-Atrophy and deformity of the tongue, teeth, and gingiva | ||
-Cranial neuropathies | ||
-Vision loss | ||
-Seizure disorder |