A 52-year-old man with bullous pemphigoid and bullous cystitis
Authors: Alison Launhardt, Jayantha Thiyanaratnam, Kristen Fernandez
Journal: Journal of the American Academy of Dermatology (2014)

To the Editor: A 52-year-old man with a medical history of psoriasis and bullous pemphigoid (BP) presented with a flare of his BP (Fig 1), which was first diagnosed in 2003 and confirmed by indirect immunofluorescence on salt-split skin. On examination, he had erosions and bullae on the bilateral lower extremities without evidence of oral or anogenital mucosal involvement. In addition, the patient stated that he recently had a cystoscopy with bladder biopsies for ongoing dysuria, frequency, and microhematuria, which started 10 months prior. On cystoscopy, the bladder was noted to have numerous bullae. Biopsy specimen was obtained of the perilesional epithelium in the bladder, which demonstrated numerous eosinophils and a subepithelial split (Fig 2). At the time of the cystoscopy, he was not taking any medications that could account for the bladder inflammation nor did his urinalysis or urine culture show evidence of infection. Because of previous resolution of his BP with Enbrel (as was initially prescribed for his psoriasis), the patient was restarted on Enbrel (50 mg) twice weekly for 3 months to be used in conjunction with clobetasol 0.05% ointment twice daily. At his follow-up appointment 4 months later, his BP was clear and his urologic symptoms had completely abated.Fig 2Bladder tissue from 52-year-old man with bullous pemphigoid and cystitis showing many eosinophils (broad arrow) and a subepithelial split (asterisk). (Hematoxylin-eosin stain; original magnification: ×100.)View Large Image Figure ViewerDownload Hi-res image Download (PPT) To our knowledge, symptomatic involvement of the bladder in BP has not been described to date; however, expression of BP180 has been demonstrated in the transitional epithelium of the bladder by indirect immunofluorescence.1Fairley J.A. Heintz P.W. Neuburg M. Diaz L.A. Giudice G.J. Expression pattern of the bullous pemhigoid-180 antigen in normal and neoplastic epithelia.Br J Dermatol. 1995; 133: 385-391Crossref PubMed Scopus (57) Google Scholar In addition, BP230 has been demonstrated by immunofluorescence in rat bladder tumor cell line.2Riddelle K.S. Green K.J. Jones J.C.R. Formation of hemidesmosomes in vitro by a rat bladder cell line.J Cell Biol. 1991; 112: 159-168Crossref PubMed Scopus (77) Google Scholar Despite this, the only bladder involvement in BP was demonstrated in asymptomatic patients based on histology alone. In a 2010 study,3Tsiriopoulos I. Kiorpelidou D. Gaitanis G. Sofikitis N. Bassukas I.D. Lower urinary tract involvement in patients with newly diagnosed autoimmune bullous dermatoses: an urethrocystoscopic study.Am J Med Sci. 2010; 340: 109-113Crossref PubMed Scopus (3) Google Scholar 14 patients with newly diagnosed autoimmune bullous dermatoses, including 4 individuals with BP, underwent cystoscopy; of these patients, 13 had lower urinary tract lesions. On pathology 2 patterns predominated: nonkeratinizing squamous metaplasia and mucosal inflammation of the bladder base. Among patients with BP, the pathologic pattern of mucosal inflammation prevailed (P = .003).3Tsiriopoulos I. Kiorpelidou D. Gaitanis G. Sofikitis N. Bassukas I.D. Lower urinary tract involvement in patients with newly diagnosed autoimmune bullous dermatoses: an urethrocystoscopic study.Am J Med Sci. 2010; 340: 109-113Crossref PubMed Scopus (3) Google Scholar Limitations of this case include that the subepithelial split on bladder biopsy specimen could have been caused by trauma from the biopsy; the biopsy specimen did however demonstrate numerous eosinophils, which would be consistent with BP. Direct immunofluorescence studies were not performed on the bladder tissue. In addition, a follow-up cystoscopy was not available to evaluate whether the bladder lesions had cleared. An interesting consideration is the atypical nature of this patient's BP. Given his age of onset (42 years), concomitant psoriasis, and response to Enbrel (as was initially used to treat his psoriasis, but also helped resolve his BP), it is possible that this is a variant of pemphigoid that is not yet described in the literature. Despite these limitations, given the cystoscopy findings of bullae by the urologist, pathology of the bladder epithelium on biopsy, and that resolution of the patient's urinary symptoms coincided with resolution of his cutaneous bullae, and typical immunofluorescence pattern on salt-split skin for BP, this case seems to be most consistent with symptomatic bladder involvement of BP.