Deadly Dermatologic Diseases by David R. Crowe Michael Morgan Stephen Somach & Kara Trapp

Author:David R. Crowe, Michael Morgan, Stephen Somach & Kara Trapp
Language: eng
Format: epub
Publisher: Springer International Publishing, Cham

Figure 22.2.Scleromyxedema with extensive indurated plaques of the face resulting in leonine features.

Systemic features of scleromyxedema, other than associated monoclonal gammopathy, include neurologic, rheumatologic, and cardiac sequelae (Table 22.1). Peripheral neuropathy and proximal muscle weakness occur in 15 % and 27 % of patients, respectively, and a seronegative polyarthritis can cause further disability [7]. Less commonly, Raynaud’s phenomenon and carpal tunnel syndrome occur. Dysphagia related to esophageal dysmotility occurs in a significant portion of patients (32 %) and also occurs in the context of the so-called dermato-neuro syndrome. This includes fevers, convulsions, and occasional progression to coma in patients with scleromyxedema, and can also include acute psychosis, dysphagia, dysarthria, ataxia, and tremor. Thankfully, dermato-neuro syndrome is rare.Table 22.1.Systemic conditions associated with scleromyxedema

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