The Differential Diagnosis of Systemic Sclerosis

Alan Tyndall; Susanna Fistarol


Curr Opin Rheumatol. 2013;25(6):692-699. 

In This Article

Scleroedema Adultorum

Scleroedema adultorum is also called scleroedema of Buschke, who described a 46-year-old man with postinfluenza skin thickening in 1902. Despite the name, it is often seen in children and is considered to have three subgroups: associated with acute infections usually of the upper or lower respiratory tract (especially streptococcal), developing rapidly and resolving in a few months to 2 years; associated with paraprotein, slowly progressive and persisting for years and associated with insulin-dependent diabetes mellitus, with subtle onset and nonresolving.[16] This latter form is also referred to as scleroedema diabeticorum. Recently, other subgroups have been reported, for example, associated with infliximab therapy.[17]

The skin lesions are similar to SSc, but the distribution is different in that neck, back and shoulder girdle involvement are predominant, with a characteristic 'mattress' sign on the skin (Fig. 7) and with sparing of hands, fingers and feet. Rarely, systemic involvement of tongue, pharynx and upper oesophagus have been reported[18] and may cause difficulties with mouth opening and swallowing. The presence of paraproteinaemia should alert the clinician to a possible haematological malignancy. Raynaud's phenomenon, capillaroscopy changes and autoantibodies are rare. Histologically, the epidermis is normal. The reticular dermis is thickened with homogeneous, large collagen bundles separated from each other by clear spaces filled with mucin, resulting in fenestration of the dermis. The number of fibroblasts is normal to reduced.

Figure 7.

The back, shoulder girdle and upper arm involvement in scleroedema adultorum. The 'mattress' sign (arrow) is prominent.

The management of scleroedema is pragmatic with little evidence-based data. Various immunomodulatory strategies have been tried. Both psoralen + ultraviolet light A and UVA-1 phototherapy have been proven to be effective. The peri-infection form often resolves spontaneously over months to several years.