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Nodular Scleroderma Revisited: Systemic Sclerosis Presenting as Annular Keloidal Sclerotic Plaques
David N Lortscher1, Philip R Cohen1, Carolyn A Bangert2
1The Division of Dermatology, University of California San Diego, San Diego, California;
The Journal of Clinical and Aesthetic Dermatology
|July 8, 2016
Summary
Nodular scleroderma, a rare systemic sclerosis variant, presents as firm, keloid-like plaques. Early recognition is key, especially with systemic symptoms, for accurate diagnosis and management.
Area of Science:
- Dermatology
- Rheumatology
- Pathology
Background:
- Nodular scleroderma is an uncommon manifestation of systemic sclerosis.
- It is characterized by firm, keloid-like plaques or nodules.
Observation:
- A case study describes a patient with undiagnosed systemic sclerosis presenting with nodular scleroderma.
- Literature review included searches for keloidal scleroderma, nodular scleroderma, and systemic sclerosis.
Findings:
- Nodular scleroderma lesions mimic keloids and are typically found on the upper trunk and arms.
- Pathological examination reveals features of scleroderma, keloid, or hypertrophic scarring.
- Hypothesized pathogenesis involves a keloidal response in genetically predisposed individuals within the fibrotic process of systemic sclerosis.
Implications:
- Systemic sclerosis should be suspected in patients with nodular or keloidal plaques resembling morphea, particularly with concurrent systemic symptoms.
- Histological findings of scar or keloid in such lesions warrant further investigation for systemic sclerosis.
- Timely diagnosis of nodular scleroderma is crucial for managing associated systemic sclerosis complications.
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