Scleroderma - Nail and Distal Digit
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Synopsis
Scleroderma, or systemic sclerosis, is an autoimmune connective tissue disease that involves sclerotic changes of the skin and internal organs. While the etiology remains unknown, the disease is characterized by autoantibody production, collagen deposition, and vascular dysfunction. The disease is observed in all ages and is slightly more common in individuals of African descent and 3-4 times more common in women. The age of onset is usually between 30 and 50 years.
Scleroderma can affect the connective tissue of any organ, including the skin, gastrointestinal tract, lungs, kidneys, joints, muscles, heart, and blood vessels. Pulmonary disease is the leading cause of mortality. Additional common clinical features include esophageal fibrosis and dysmotility, arthralgias, and Raynaud phenomenon. Less common manifestations include hypertensive renal crisis, pulmonary hypertension and interstitial lung disease, and cardiomyopathy.
The three major clinical subsets of scleroderma are limited cutaneous systemic sclerosis, diffuse cutaneous systemic sclerosis, and systemic sclerosis sine scleroderma.
A number of nail findings are seen in scleroderma. Examination of the proximal nail fold reveals capillary enlargement and loss of some capillaries in a majority of scleroderma patients. Other common nail changes include the following:
- Trachyonychia (roughness; "sandpaper nails")
- Scleronychia (hardened, inelastic, opaque)
- Brachyonychia (short nail, with width exceeding length; "racquet nail")
- Splinter hemorrhages
- Nail thickening
- Parrot beaking (over-curvature of the fingernail free edge; hooked nail deformity)
- Pterygium inversus (distal expansion of the hyponychium, which anchors to the undersurface of the nail plate, ultimately resulting in the obliteration of the distal nail groove)
- Cuticle changes
Codes
ICD10CM:M34.9 – Systemic sclerosis, unspecified
SNOMEDCT:
89155008 – Scleroderma
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Differential Diagnosis & Pitfalls
- Lichen planus
- Systemic lupus erythematosus
- Chronic phase of graft-versus-host disease
- Dermatomyositis
- CREST syndrome (calcinosis cutis, Raynaud phenomena, esophageal dysfunction, sclerodactyly, and telangiectasia)
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Last Reviewed:07/25/2017
Last Updated:07/08/2020
Last Updated:07/08/2020
Scleroderma - Nail and Distal Digit
See also in: Overview