Localized Scleroderma (case Report of 23 Cases).

Area of Science:

• Dermatology
• Rheumatology
• Genetics

Background:

• Localized scleroderma is an uncommon connective tissue disorder with varied clinical presentations.
• Understanding its diverse manifestations is crucial for accurate diagnosis and management.
• Previous literature highlights the rarity of familial cases and concurrent autoimmune conditions.

Observation:

• This study presents clinical and histological data from 23 patients diagnosed with localized scleroderma.
• A rare familial occurrence of en coup de sabre, a subtype of localized scleroderma, was documented.
• Concurrent lesions of en coup de sabre and discoid lupus erythematosus were observed in a 16-year-old female.

Findings:

• The findings underscore the heterogeneity of localized scleroderma, encompassing rare familial patterns.
• The co-occurrence of en coup de sabre and discoid lupus erythematosus highlights potential overlaps in autoimmune pathogenesis.
• Distinctive facial hemiatrophy associated with en coup de sabre requires careful differentiation from Romberg's syndrome.

Implications:

• Accurate differentiation between localized scleroderma subtypes and other facial hemiatrophy conditions is essential for appropriate treatment.
• Further research into the genetic and immunologic factors underlying familial localized scleroderma and concurrent autoimmune diseases is warranted.
• This case series contributes to the understanding of rare presentations of localized scleroderma, aiding clinicians in diagnosis and patient care.