Nodular scleroderma: Characterization of a distinct clinical phenotype

Area of Science:

• Rheumatology
• Dermatology
• Systemic Sclerosis Research

Background:

• Nodular scleroderma is a rare variant of systemic sclerosis (SSc).
• It is characterized by indurated skin nodules on extremities and trunk.
• Recently differentiated from keloid forms, it often co-occurs with other SSc manifestations.

Purpose of the Study:

• To review and characterize the clinical features of nodular scleroderma.
• To understand the presentation and associations of this SSc phenotype.
• To provide the first comprehensive review of nodular scleroderma.

Main Methods:

• Systematic literature review of all reported nodular scleroderma cases.
• Analysis of clinical features, serological markers, and organ involvement.
• Characterization of treatment responses.

Main Results:

• Nodular scleroderma is typically associated with diffuse SSc and early progressive skin involvement.
• Patients exhibit speckled/nucleolar antinuclear antibodies and SSc multiorgan involvement.
• Pulmonary hypertension is infrequent, and nodules are treatment-refractory.

Conclusions:

• Nodular scleroderma is a distinct SSc phenotype with specific clinical and serological features.
• The characteristic nodules show resistance to current immunosuppressive and antifibrotic therapies.
• Further research may be needed to identify effective treatments for nodular scleroderma.