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- Presentation
Scleromyxedema Presenting With Diffuse Erythema, Skin Tightening, and Dermatoneuro Syndrome After Coma
Description
The speaker presented a challenging case of a 55-year-old woman who developed diffuse erythema, skin tightening, swelling of the hands and feet, and progressive joint stiffness after a hospitalization for seizure and coma, which she strongly believed was caused by a COVID-19 vaccine. Initial biopsies showed interstitial granulomatous dermatitis, and the differential included eosinophilic fasciitis, systemic sclerosis, and scleromyxedema. Extensive lab work was largely normal except for a monoclonal gammopathy on SPEP, and a later biopsy confirmed scleromyxedema with dermal mucin and increased fibroblasts. Key diagnostic features reviewed were waxy papules, monoclonal gammopathy, and absence of thyroid disease. The case also highlighted dermatoneuro syndrome, a medical emergency in which scleromyxedema is associated with fever, seizure, encephalopathy, and coma. The recommended first-line treatment was IVIG, and the patient improved with ongoing IVIG therapy over three years, though she remained unconvinced of the diagnosis. The talk emphasized careful clinical reasoning, the importance of biopsy and SPEP, and the need to consider misinformation while respecting patient beliefs.
View moreConclusions
- The patient’s eruption and skin tightening were ultimately most consistent with scleromyxedema rather than a vaccine allergy, scleroderma, or eosinophilic fasciitis.
- In this case, the key diagnostic clue was an M-spike on serum protein electrophoresis showing an IgG lambda monoclonal gammopathy.
- Skin biopsy findings of mucin deposition, fibroblast proliferation, and fibrosis confirmed lichen myxedematosus/scleromyxedema.
- First-line treatment for scleromyxedema is intravenous immunoglobulin, which can produce substantial clinical improvement and relapse may occur if it is stopped.
- Scleromyxedema should be recognized by its classic triad of waxy dome-shaped papules, monoclonal gammopathy, and absence of thyroid disease.
- Dermato-neuro syndrome is a rare but serious complication of scleromyxedema that can present with fever, seizure, and coma and may mimic infectious encephalitis.
- The case suggests that systemic or infectious triggers, including possibly COVID-related events, may precipitate or be associated with scleromyxedema manifestations in some patients.
- When patients present with confusing multisystem findings and multiple prior evaluations, returning to the history, exam, biopsy, and targeted labs is essential to reach the correct diagnosis.
- Rongioletti F et al. J Am Acad Dermatol. 2016 Jun;74(6):1194-200.
- Dermato-neuro syndrome after COVID-19 infection in a patient with scleromyxoedema: Previously successful treatment with intravenous immunoglobulins.
- Association of interstitial granulomatous dermatitis with messenger Rna-1273 Sars-Cov-2 vaccine.#10.1111/jdv.18490