A variety of medications and chemical compounds have been associated with the development of a spectrum of scleroderma-like conditions. Some compounds induce sclerotic skin changes along with Raynaud phenomenon, pulmonary fibrosis and other internal organ involvement, whereas others induce circumscribed morphea-like plaques.
Causative medications include chemotherapeutic agents, analgesics, neurological drugs, appetite suppressants and other agents, including penicillamine, tryptophan, cocaine, and phytonadione (vitamin K).
Certain chemical compounds, such as plastics, solvents (paint removers and thinners, trichloroethane, xylene, benzene), vinyl chloride, contaminated rapeseed oil, and minerals can cause scleroderma-like disease. Long-term silica exposure can induce scleroderma that is difficult to distinguish from idiopathic scleroderma. This association is more common in males due to occupational exposure.
Drug-induced scleroderma may be distinguished from idiopathic scleroderma by the lack of positive autoantibodies. Furthermore, if there is cessation or reversibility of disease process upon discontinuation of the drug, this supports a drug-induced process.
The pathogenesis of drug-induced scleroderma is incompletely understood and likely varies by drug. Timing of symptom onset varies by drug and ranges from 2 days to 11 years, with the majority of drugs inducing disease between 3 weeks and 9 months. The clinical course is similarly variable and ranges from slow evolution to rapidly progressive disease. Complete resolution after discontinuation of offending agent is usual but not absolute.
Related topics: Scleroderma, Scleroderma of childhood, Raynaud phenomenon, Morphea, Pulmonary fibrosis, Silicosis
Drug-induced sclerodermoid reactions
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Synopsis
Codes
ICD10CM:
M34.2 – Systemic sclerosis induced by drug and chemical
SNOMEDCT:
201443009 – Systemic sclerosis induced by drugs and chemicals
M34.2 – Systemic sclerosis induced by drug and chemical
SNOMEDCT:
201443009 – Systemic sclerosis induced by drugs and chemicals
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
Drug-induced scleroderma shares clinical findings of localized and systemic sclerosis and thus a similar differential should be considered.
- CREST syndrome
- Generalized morphea – Asymmetric induration, no Raynaud phenomenon, no systemic involvement.
- Scleredema – ANA negative, no Raynaud phenomenon, no systemic involvement.
- Scleromyxedema – ANA and anticentromere negative, no Raynaud phenomenon, no sclerodactyly.
- Generalized myxedema
- Chronic graft-versus-host disease – ANA negative, vascular abnormalities such as Raynaud phenomenon absent.
- Eosinophilic fasciitis – ANA negative, no Raynaud phenomenon, no facial involvement.
- Nephrogenic systemic fibrosis – Assess for recent history of radiologic imaging with gadolinium-based intravenous contrast in patients with renal insufficiency or renal transplant patient; ANA negative, no sclerodactyly, no Raynaud phenomenon.
- Stiff-skin syndrome – Characteristic sparing of the hands and feet, develops during early childhood, systemic involvement rare.
- Porphyria cutanea tarda
- Phenylketonuria
- Carcinoid syndrome
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Therapy
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Drug Reaction Data
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.
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References
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Last Reviewed:09/06/2017
Last Updated:09/06/2017
Last Updated:09/06/2017