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Chronic GVHD

Occurs in patients post-allogeneic stem cell transplant and can cause sclerotic skin changes, but the clear history of transplantation is the key differentiator.

Eosinophilia-myalgia syndrome

Linked to the ingestion of contaminated L-tryptophan, presenting with myalgias, peripheral eosinophilia, and skin thickening.

Eosinophilic fasciitis

Characterized by symmetrical, painful induration of the limbs, sparing the hands and feet, with a 'peau d'orange' appearance and peripheral eosinophilia.

Fibroblastic rheumatism

A rare paraneoplastic syndrome featuring skin nodules, polyarthritis, and Raynaud's phenomenon, but is associated with an underlying malignancy.

Generalized morphea

A form of localized scleroderma with widespread plaques of indurated skin, but crucially lacks the Raynaud's phenomenon and systemic organ involvement of systemic sclerosis.

Nephrogenic fibrosing dermopathy

Occurs in patients with renal failure, especially after exposure to gadolinium-based contrast agents, causing skin thickening and contractures.

POEMS syndrome

A paraneoplastic syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, Skin changes) where skin thickening is a feature, but the constellation of other signs is diagnostic.

Restrictive dermopathy

A lethal congenital disorder with extremely tight, thin, translucent skin, joint contractures, and a characteristic facial appearance at birth.

Scleredema

Presents as non-pitting, woody induration of the skin of the neck, upper back, and shoulders, often preceded by a streptococcal infection or associated with diabetes.

Scleromyxedema

Associated with a monoclonal gammopathy (paraprotein), characterized by waxy papules that coalesce into diffuse induration, especially on the face and hands.

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