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Diffuse systemic sclerosis with bullous lesions without systemic manifestations* * Work carried out at the Pedro Ernesto University Hospital - State University of Rio de Janeiro (HUPE-UERJ) - Rio de Janeiro (RJ), Brazil.

Esclerose sistemica difusa bolhosa sem acometimentos sistemicos

Here, we describe an atypical case of systemic sclerosis in its diffuse cutaneous form with acute and rapid progression of the cutaneous condition, without any systemic manifestations and the infrequent formation of bullae, showing the importance of diagnosis and early treatment in such cases. This case also shows that special measures should be taken for bullous cutaneous lesions and ulcerations resulting from serious sclerosis, which are entry points and increase morbidity and risk of death. Other prognostic factors include age, ESR and renal and pulmonary involvement. Capillaroscopies can be useful predictors of greater severity of systemic scleroderma, revealing a greater link with systemic, rather than cutaneous, involvement.

Connective tissue diseases; Scleroderma, diffuse; Scleroderma, systemic; Skin diseases, vesiculobullous; Skin and connective tissue diseases


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