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- Presentation
Case-Based Review of Scleromyxedema and Silicone-Induced Fibrosis
Description
The session presented two case-based reviews of unusual fibrosing skin disorders. The first case was a 49-year-old man with widespread monomorphic flesh-colored papules, indurated plaques, facial involvement, shiny skin, and a monoclonal gammopathy; biopsy showed dermal mucin, fibroblast proliferation, and fibrosis, leading to a diagnosis of scleromyxedema. The talk contrasted it with systemic sclerosis, scleroedema, and nephrogenic systemic fibrosis, emphasized that scleromyxedema can have serious systemic complications including myositis, neurologic disease, pulmonary and renal involvement, and noted that IVIG is the mainstay treatment, often requiring ongoing maintenance and producing marked skin and systemic improvement. The second case was a 37-year-old bodybuilder with waxy, shiny, indurated plaques on the chest, shoulders, and upper arms, initially considered for nephrogenic systemic fibrosis but ultimately found to have silicone-induced fibrosis after a more detailed history revealed injections of substances containing mineral oil and silicone. Histology showed vacuolated, Swiss-cheese-like changes consistent with silicone deposition. The presentation highlighted that silicone can cause fibrotic or granulomatous reactions and may be associated with hypercalcemia and renal failure, and stressed the importance of a broad, nonjudgmental exposure history when atypical sclerosing or granulomatous disease is suspected.
View moreConclusions
- Scleromyxedema presents as a rare mucin-deposition disorder with characteristic monomorphic papules and skin induration, often associated with an IgG lambda paraproteinemia.
- Because scleromyxedema can cause serious multi-system complications, patients should undergo a careful review of systems for neurologic, muscular, swallowing, pulmonary, cardiac, and renal involvement.
- IVIG appears to be the first-line and disease-modifying treatment for both cutaneous and systemic manifestations of scleromyxedema.
- Clinical improvement with IVIG may occur even without a major change in the underlying paraproteinemia, so treatment is aimed at disease control rather than cure.
- IVIG generally needs to be continued as maintenance therapy, since stopping treatment can lead to rebound disease.
- When fibrotic or granulomatous skin disease looks atypical, silicone injection or other exogenous material exposure should be considered in the differential diagnosis.
- A detailed, nonjudgmental exposure history is essential because patients may not initially disclose prior filler or injection use.
- Silicone-induced fibrosis or granulomatous reactions can mimic other sclerosing disorders and may also be associated with hypercalcemia and renal failure.
- The clinical timing of skin changes relative to kidney disease can help distinguish silicone-induced disease from nephrogenic systemic fibrosis.
- Recognizing the pattern of waxy plaques with vacuolated histology can prevent misdiagnosis and lead to the correct management approach.
- PMID: 28370513. High-dose IVIG for scleromyxedema: a prospective open-label clinical trial using an objective score of clinical evaluation system. 2017.