Unilateral linear pansclerotic morphea affecting face and limbs

Area of Science:

• Dermatology
• Rare diseases
• Connective tissue disorders

Background:

• Disabling pansclerotic morphea is a rare condition characterized by progressive atrophy and sclerosis of subcutaneous tissues, muscle, and bone.
• It typically affects one half of the face, potentially extending to limbs, causing hemiatrophy.

Observation:

• A case of a woman presenting with facial deformity and left upper and lower limb atrophy is described.
• The condition began as an indurated plaque on the left forehead at age 5, progressing to involve the left eye.

Findings:

• The patient exhibited extensive atrophic changes consistent with disabling pansclerotic morphea.
• Radiological evidence of hemiatrophy was noted in the affected limbs.

Implications:

• This case underscores the severe, disfiguring potential of disabling pansclerotic morphea.
• Reporting such rare occurrences aids in understanding the disease's progression and clinical spectrum.