Bullous lesions in scleroderma.
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.
Rencic
Adrienne
A
Department of Dermatology, of Johns Hopkins University, School of Medicine, Baltimore, MD, USA.
Goyal
Supriya
S
Mofid
Mona
M
Wigley
Frederick
F
Nousari
H Carlos
HC
D000328:Adult; D001768:Blister / Q000209:etiology* / Q000276:immunology / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012594:Scleroderma, Localized / Q000150:complications* / Q000276:immunology / Q000473:pathology*; D012595:Scleroderma, Systemic / Q000150:complications* / Q000276:immunology / Q000473:pathology*