Scleromyxedema is a rare, generalized form of lichen myxedematosus, which may be associated with systemic involvement and can be fatal. The therapeutic options available provide partial or inconsistent response and are associated with significant adverse effects. We report a case of scleromyxedema with cardiac involvement, treated with low-dose intravenous immunoglobulin, with almost complete clearing of the skin lesions. The patient died after three cycles of treatment, possibly due to myocardial infarction.

Tuberous sclerosis is an autosomal dominant disease due to mutations in two genetic loci, characterized by hamartoma formation in the skin, nervous system, heart, kidney and other organs. Dyschromatosis universalis hereditaria is an autosomal dominant genodermatosis, characterized by small hyperpigmented and hypopigmented macules, uniformly distributed over the entire body. The face is rarely involved and the palms, soles and mucous membranes are usually spared. We report a case of tuberous sclerosis with dyschromatosis universalis hereditaria, with hyperpigmented and hypopigmented macules affecting the palms, soles and oral mucosa. To our knowledge, this is the first reported case of such an association.