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Table 1 Comparison of morphea ECDS and PHA

From: Progressive hemifacial atrophy: a review

 

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

  1. Female (F).
  2. Male (M).
  3. *Histopathologic features depend on disease activity.
  4. ^May extend below the forehead and involve the nose, medial cheek, and upper lip.
  5. ~Connective tissue fibrosis, adnexal/fat atrophy, and mononuclear cell infiltrates all seen ECDS > PHA although considerable overlap may be seen.