Nonbullous Pemphigoid Unrecognized Cause of Pruritus in Nursing Homes

By Marilynn Larkin

November 19, 2019

NEW YORK (Reuters Health) - Nonbullous pemphigoid can be more frequent among nursing home residents than bullous pemphigoid and may be an unrecognized cause of pruritus, a cross-sectional study suggests.

"The recently recognized nonbullous variant of pemphigoid contradicts the clinical image of bullous pemphigoid that clinicians have," Dr. Joost Meijer of the University of Groningen told Reuters Health by email. "The often elderly patients with nonbullous pemphigoid may have complaints of chronic itch that could be misdiagnosed as eczema, with a potential long delay until the (correct) diagnosis is made."

"Because of the strong associations of the disease with aging and neurodegenerative disorders - mainly dementia - we hypothesized that nursing home residents could be a high-risk population that is overlooked," he said.

The study included 125 nursing home residents in the Netherlands with a mean age of 84; 94 (75%) had a history of neurodegenerative disease.

As reported online November 6 in JAMA Dermatology, pruritus was present in 59 of 125 participants (47%) and was chronic (>6 weeks) in 48 (81%).

Seven participants had a pemphigoid diagnosis, yielding an overall prevalence of 6%.

"Remarkably, we found more patients with the nonbullous variant (four) than with bullous pemphigoid (three)," Dr. Meijer said. "The patients with nonbullous pemphigoid had complaints of often severe itch of chronic duration."

Nonbullous skin manifestations included erythematous papules and/or nodules, urticarial plaques, and excoriations, mostly on the back and extremities.

Nonspecific serological findings in participants without a pemphigoid diagnosis were single detection of IgA by indirect immunofluorescence on salt-split skin (two cases) and low titers of IgG autoantibodies by BP180 NC16A (10 cases) or BP230 enzyme-linked immunosorbent assays (three cases).

Dr. Meijer said, "Recognition of the disease is essential, because it requires more intensive treatment with either whole-body application of corticosteroid cream or immunosuppressive agents."

"We recommend including pemphigoid in the workup of elderly patients with chronic itch," he advised. "The diagnosis can be confirmed by a blood sample for serological testing or a skin biopsy for direct immunofluorescence. The high burden of chronic itch and this potentially underrecognized disease merits attention from clinicians."

"These findings seem feasible," Dr. Lindsey Bordone, a dermatologist at ColumbiaDoctors in New York City, commented in an email to Reuters Health. "There are times when an elderly patient will have pruritus far ahead of their bullous lesions appearing and I think this could be an important addition to a clinician's work-up for pruritus of unknown etiology."

"Most dermatologists become suspicious of potential bullous pemphigoid if an elderly patient has urticarial (hive-like) lesions that are new in onset and itchy," she said. "However, without obvious lesions, it is often thought that medications could be causing pruritus as a side effect, or something more severe like lymphoma could be the cause of their itch."

"It is relatively easy to draw blood to run indirect immunofluorescent tests," she said, though cost might be a barrier.

"I think most dermatologists would naturally run through the most common causes of pruritus and rule those out as a possibility before considering less common conditions," she added. "This article will help remind clinicians to include this possibility in their differential diagnosis."


JAMA Dermatol 2019.