Bullous lesions in scleroderma

Adrienne Rencic, Supriya Goyal, Mona Mofid, Frederick Wigley, H. Carlos Nousari

Research output: Contribution to journalArticlepeer-review

31 Scopus citations


Background: The occurrence of bullous lesions in localized or systemic scleroderma is rare. Three histologic patterns have been reported: lichen sclerosus et atrophicus-like, lymphangiectatic blisters and autoimmune blistering diseases. Objective: To investigate the frequency, clinical, and immunopathologic features of patients with scleroderma and bullous eruptions and to review the literature regarding this rare condition. Methods: A retrospective study of 53 cases of scleroderma (localized, generalized, and systemic) in the dermatology and rheumatology clinics at one institution over an 8-year span. Clinical, serologic, and immunopathologic findings were analyzed in four cases. Results: Four of 53 patients exhibited bullous lesions in association with scleroderma. The first case illustrates lymphangioma-like clinical and pathologic presentation. The second case demonstrates bullous lichen sclerosus et atrophicus-like pattern. The other two cases exemplify a superimposed autoimmune skin disease, epidermolysis bullosa acquisita and penicillamine induced pemphigus foliaceus after treatment for systemic scleroderma. Conclusions: Of the 53 original patients, we have described four cases of bullous scleroderma (7.5%) illustrating several pathogenetic mechanisms of bulla formation. Inflammatory (lichen sclerosus et atrophicus), fibrotic/obstructive (lymphangiomatous), autoimmune (epidermolysis bullosa acquisita), and pemphigus foliaceus. The final case illustrates bullae as a complication of therapy for the underlying scleroderma.

Original languageEnglish (US)
Pages (from-to)335-339
Number of pages5
JournalInternational Journal of Dermatology
Issue number6
StatePublished - 2002

ASJC Scopus subject areas

  • Dermatology


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