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Skin-nontumor / Clinical Dermatology

Other dermatoses

Scleroderma

Reviewer: Mowafak Hamodat, MB.CH.B, MSc., FRCPC, Eastern Health, St. Johns, Canada (see Reviewers page)
Revised: 2 August 2011, last major update August 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.

Definition
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● 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’s phenomenon, Borrelia burgdorferi infection

Clinical description
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● 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

Micro description
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● 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

Micro images
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Dermal sclerosis with atrophy of adnexae

Virtual slides
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Scleroderma

Negative stains
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● Immunoglobulins

Differential diagnosis
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● 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
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● eMedicine

End of Skin-nontumor / Clinical Dermatology > Other dermatoses > Scleroderma

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