A 40-year-old female presented with gradually progressive thickening of the skin and multiple raised lesions over the body for 2 years. General and systemic examination were within normal limits. On cutaneous examination, there was a central depression over proximal interphalangeal joints surrounded by an elevated rim of infiltration (“doughnut sign”) [Figure 1a]. Multiple discrete erythematous waxy papules and confluent indurated plaques were present over the upper limbs, and trunk [Figure 1b], and face [Figure 1c]. Thyroid profile and ultrasound abdomen were normal. Histopathology showed fibroblastic proliferation along with mild increased thickness of collagen bundles and mucin deposition suggestive of the diagnosis of scleromyxedema [Figure 1d]. Masson trichrome stain highlighted the thickened collagen bundles [Figure 1e] and serum electrophoresis revealed monoclonal IgG gammopathy. Scleromyxedema is a rare variant of cutaneous mucinosis characterized by generalized papular and sclerodermoid eruptions and has found to be associated with paraproteinemia. Various treatment modalities including melphalan, systemic steroids, phototherapy, and intravenous immunoglobulin have been tried with variable success rates.
Figure 1.
(a) Induration and central depression over proximal interphalangeal joints (b) Multiple discrete skin-colored to slightly erythematous closely set firm waxy papules over trunk (c) Confluent indurated plaques present over face including forehead (inset) (d) Increased thickness of collagen bundles, proliferation of fibroblasts, and mucin deposition seen at high magnification (H and E, 40x) (e) Thickened collagen bundles highlighted (Masson trichrome 100x)
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