Myopathy associated with sclerodermal facial hemiatrophy

Area of Science:

• Dermatology
• Pathology
• Neurology

Background:

• Linear scleroderma is a rare autoimmune condition characterized by skin hardening and connective tissue inflammation.
• Facial hemiatrophy and tongue atrophy are uncommon but severe manifestations that can arise from scleroderma.
• Understanding the underlying pathophysiology is crucial for effective patient management.

Observation:

• A patient presented with linear scleroderma accompanied by ipsilateral tongue and facial hemiatrophy.
• Biopsy of the affected skin plaque revealed characteristic scleroderma changes.
• Fascial and muscle tissues beneath the plaque showed significant pathological alterations.

Findings:

• The fascia exhibited plasma cell fasciitis, a notable inflammatory response with abundant plasma cells.
• Histochemical analysis of the temporalis muscle demonstrated severe, localized atrophy of both type 1 and type 2 muscle fibers.
• These muscle changes mirrored previously documented findings in similar localized scleroderma cases.

Implications:

• This case highlights the potential for scleroderma to cause profound muscle and fascial atrophy, leading to facial disfigurement.
• The findings underscore the importance of considering deeper tissue involvement in localized scleroderma.
• Further research into the mechanisms driving scleroderma-associated muscle atrophy may inform therapeutic strategies.