An unusual case of granulomatous scleromyxedema

Scleromyxedema, also referred to as “generalized and sclerodermoid lichen myxedematosus” or “Arndt-Gottron disease,” is a progressive disease in the spectrum of primary cutaneous mucinoses.1 Given its rarity, existing knowledge is primarily derived from case reports and a few retrospective studies. Middle-aged adults are most commonly affected; however, there is no race or sex predominance.1 The pathogenesis has not been fully elucidated, but it is hypothesized to be triggered by dysregulated cytokine stimulation of glycosaminoglycan synthesis and fibroblast proliferation.