Table of Contents
Definition / general | Clinical features | Clinical images | Microscopic (histologic) description | Microscopic (histologic) images | Negative stains | Differential diagnosis | Additional referencesCite this page: Hamodat M. Scleroderma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skinnontumorscleroderma.html. Accessed March 6th, 2021.
Definition / general
- Either localized (morphea) or systemic (involving skin of face, upper trunk, hands and arms, esophagus, heart and lungs)
- May have visceral disease without skin disease
- May be associated with Raynaud phenomenon, Borrelia burgdorferi infection
Clinical features
- Usually presents on trunk or extremities as one or several indurated plaques with ivory center and violaceous border (the lilac ring)
- Also lesions confined to breast
- May have irregular areas of hyperpigmentation or hypopigmentation
- Occasionally more than one type is present
Clinical images
Microscopic (histologic) description
- Thickening and hyalinization of connective tissue of deep dermis, subcutaneous fat and muscular fascia, with perivascular and focal interstitial lymphocytic and plasma cell infiltrate in subcutaneous fat
- Frequent atrophy of adnexal structures, increased fibroblasts, thickening and luminal narrowing of small vessels, blunting of dermal-subcutis interface, diminished elastic tissue
- Often atrophy of epidermis, mucin deposition
- Variable dystrophic calcification of blood vessels
- Control biopsy from adjacent normal skin is helpful
Microscopic (histologic) images
Negative stains
- Immunoglobulins
Differential diagnosis
- Elastosis due to sunlight: normally on thick dermis of fingers and dorsum of hand
- Eosinophilic fasciitis: thickening of deep fascia only
- Lichen sclerosus et atrophicus, acrodermatitis chronic atrophicans
Additional references