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Clinical Characteristics of Recurrent RIME Identified in Study

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INDIANAPOLIS. Recurrent reactive infectious mucocutaneous rashes (RIME) most commonly occurred in boys aged 11 to 12 years, younger than previously described, in a single-center retrospective study. In addition, 71% of patients with relapsing disease had 1-2 relapses—episodes that were usually milder and occurred at variable intervals.

These are some of the key findings from a study of 50 RIME patients presented by Katerina X. Pan at the annual meeting of the Society for Pediatric Dermatology.

Reactive infective mucocutaneous rash (RIME) is a new term covering a range of rare parainfectious mucosal disorders, said Pan, a fourth-year student at Harvard Medical School in Boston. Formerly known as Mycoplasma pneumoniae rash and mucositis (MIRM), the general clinical features of RIME include involvement of less than 10% of body surface area with polymorphic skin lesions (vesiculobullous or target-like macules/papules); erosive oral, genital, and/or ocular mucositis affecting more than two sites; and signs of a previous infection, including, but not limited to, upper respiratory tract infection, fever, and cough.

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In addition to M. pneumoniae, other pathogens have been implicated, she said. “Although the underlying etiology of the disease is not entirely clear, it is becoming increasingly known that RIME tends to recur in a subset of patients.”

A cohort study of 13 patients with RIME found that the five patients who relapsed were predominantly black, male, and older.

The estimated recurrence rate is between 8% and 38%, but the clinical characteristics of patients who develop RIME relapse are generally poorly understood, Ms Pan said.

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Together with her mentor Sadaf Hussain, MD, of the Department of Dermatology at Boston Children’s Hospital, Ms. Ban conducted a retrospective review of charts to characterize the medical history and course of the disease in patients diagnosed with recurrent RIME. They extracted data between January 2000 and March 2022 using the ICD-10 codes used by board certified dermatologists at Boston Children’s Hospital and a RIME or MIRM text search on dermatological notes. Patients were included if they had a RIME/MIRM diagnosis by a certified dermatologist and/or an infection by PCR/serology and mucositis with limited skin involvement.

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The study population included 50 patients: 24 with recurrent RIME and 26 with isolated RIME. The majority (66%) were male, and the median age at onset of RIME was 11 to 12 years, two years less than previously reported in a case series of 13 patients. The majority of study participants (79%) were white, but there were no significant differences between patients with relapsed RIME and those with isolated RIME by age, gender, or race.

Isolated and repetitive RIME

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However, compared with patients with isolated RIME, a greater proportion of patients with recurrent RIME had a history of atopic disease (46% vs. 23%, respectively; P = 0.136), as well as a history of tonsillectomy and adenoidectomy. 25% vs 4%; P = 0.045). “This has not been observed before, but it may raise the hypothesis that patients with a history of frequent infections as well as enhanced immune responses may be associated with disease recurrence,” Ms Pan said.

The median number of episodes among patients with recurrent RIME was 3.5, and the interval between episodes was variable, averaging 10.2 months. Ms. Pan reported that 71% of patients with recurrent RIME had 1-2 episodes, although one patient had 9 episodes.

Clinically, episodes among all RIME patients were characterized by infectious prodromal symptoms (69%), oral involvement (95%), ocular involvement (60%), genital involvement (41%), and skin lesions (40%). However, RIME relapses were less severe and more atypical, with 49% affecting only one mucosal surface and 29% affecting two mucosal surfaces. In addition, with the exception of oral lesions, the incidence of infectious prodromal symptoms and other lesions was significantly reduced among relapses compared to baseline RIME.

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“Notably, we found that M. pneumoniae was the most common known cause of RIME, especially among initial episodes,” Ms Pan said. “However, 61% of recurrent episodes of RIME had no known cause in terms of infectious etiology. And, consistent with previous studies, we also found a reduction in severity.” [of RIME recurrences] as indicated by a decrease in the frequency of emergency department visits, hospitalizations and duration of hospitalization.”

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In other data, psychiatric complications such as anxiety and depression followed the initiation of RIME in 33% of patients with relapsing disease and 22% of patients with isolated disease. In addition, the three most common treatments among all 50 patients were systemic steroids, topical steroids, and M. pneumoniae-specific antibiotics.

“While RIME is generally considered to be milder than Stevens-Johnson syndrome and toxic epidermal necrolysis with a low mortality rate, it can lead to serious complications, including conjunctival wrinkling, corneal ulceration and scarring, blindness, and oral, ocular, urogenital synechia,” says Ms. Pan remarked. “There has also been an increase in the use of corticosteroids and steroid-sparing drugs such as IVIG. Multidisciplinary care with ophthalmology, urology and mental health services is critical.”

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She acknowledged certain limitations of the study, including its retrospective single center design and the possibility that milder cases may have been excluded due to lack of a definitive diagnosis or referral.

Carrie S. Coughlin, MD, who was asked to comment on the results of the study, noted that nearly half (24) of the patients in the cohort experienced relapses of RIME. “This is a high proportion, suggesting relapse counseling is more important than previously thought,” said Coughlin, director of pediatric dermatology at Washington University/St. Louis. Ludovica Children’s Hospital.

“Fortunately, repeat cases have generally been less severe. However, many patients have had more than one recurrence, making it difficult for affected patients.”

The researchers said they do not disclose financial information. Coughlin serves on the boards of the Pediatric Dermatology Research Alliance (PeDRA) and the International Collaboration for Immunosuppression and Skin Cancer Transplantation.

This article originally appeared on MDedge.com, part of the Medscape professional network.

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