Indianapolis – In a single-center retrospective study, episodes of recurrent reactive infectious mucocutaneous rash (RIME) were most common in men 11 to 12 years of age, more frequently than previously described young. In addition, 71% of patients with relapsing disease experienced 1-2 relapses—often mild and occurred at variable intervals.
These are the main findings of a study of 50 patients with RIME, presented by Catherina X. Pan at the annual meeting of the Society of Pediatric Dermatology.
Reactive infectious mucocutaneous rash (RIME) is a novel term covering a range of rare parainfectious mucositis disorders. Pan, a fourth-year medical student at Harvard Medical School in Boston. Formerly known as Mycoplasma pneumoniae -induced rash and mucositis (MIRM), common clinical features of RIME include less than 10% body surface accumulation and polymorphic skin lesions (vesicular lesions) bullous or target plaques)/papules); erosive oral, genital and/or ocular mucositis involving more than two sites, and evidence of prior infection including but not limited to upper respiratory tract infection, fever and cough.
M. Pneumonia , other pathogens are also implicated, she said. “While the underlying etiology of the disease is not fully understood, it is becoming increasingly clear that RIME tends to recur in a subset of patients.”
A cohort study of 13 RIME patients found that black Race, male sex, and age predominated in the 5 relapsed patients.
Estimated relapse rates range from 8% to 38%, but the clinical characteristics of patients with relapsed RIME tend to be
Ms. Pan with her mentor, Boston Children’s Hospital Dermatology A retrospective chart review was performed to describe the clinical history and course of patients diagnosed with recurrent RIME. They extracted data between January 2000 and March 2022 using the ICD-10 codes used by Boston Children’s Hospital board-certified dermatologists and RIME or MIRM text searches in dermatology notes. Patients were included if they had a RIME/MIRM diagnosis by a board-certified dermatologist and/or PCR/serological infection and mucositis with limited skin involvement.
The study population included 50 patients: 24 with recurrent RIME and 26 with solitary RIME. The majority (66%) were male, and the mean age at onset of RIME was between 11 and 12 years, two years younger 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 those with recurrent RIME and those with isolated RIME in terms of age, sex, or race.
Solitary RIME vs. Relapsed RIME
However, compared to patients with solitary RIME, a greater proportion of recurrent RIME RIME patients had a history of atopic disease (46% vs 23 %, respectively; P =.136), as well as a history of tonsillectomy and adenoidectomy (25 % vs 4%; P=.045). “This has not been observed before, but it may lead to a hypothesis that patients with a history of frequent infections as well as enhanced immune responses may be associated with disease relapse,” Ms Pan said.
Patients with recurrent RIME had an average of 3.5 episodes with a variable interval between episodes, an average of 10.2 months. Ms. Pan reported that 71% of patients with recurrent RIME experienced 1-2 episodes, although one patient experienced 9 episodes.
Clinically, all RIME patients had episodes characterized by infectious prodrome (69%), oral lesions (95%), ocular lesions (60%), genital lesions (41%) ) and skin lesions (40%). However, RIME recurrences were less severe and more atypical, with 49% involving only one mucosal surface and 29% involving both mucosal surfaces. Furthermore, with the exception of oral lesions, the incidence of infectious prodromal symptoms and other lesions was significantly lower in relapse compared with initial RIME.
“Notably, we found Mycoplasma pneumoniae to be the most common known cause of RIME, especially in the initial episode,” Ms Pan said. “However, 61% of recurrent RIME episodes had no known cause in terms of infectious etiology. And, consistent with previous studies, we also found a decrease in the severity of [RIME relapses], manifested in emergency department visits, hospitalizations, and length of hospital stay.”
In other findings, 33% of patients with recurrent disease and 22% of patients with isolated disease developed psychiatric complications such as anxiety and depression after RIME. In addition, the three most common treatments in all 50 patients were systemic steroids, topical steroids, and M. Pneumonia specific antibiotics.
“Although RIME is generally considered to have a lower mortality rate than Stevens-Johnson syndrome and toxic epidermal necrolysis, it can lead to serious complications including conjunctival atrophy, corneal ulcers and Scarring, blindness, and adhesions to the mouth, eyes, genitourinary organs,” Ms. Pan points out. “Increased use of corticosteroids and steroid-retaining agents (eg IVIG) was also observed. Multidisciplinary care in ophthalmology, urology and mental health services is critical.”
She acknowledged that there are some Limitations, including its retrospective, single-center design, and the potential to exclude milder cases due to lack of accurate diagnosis or referral.
Carrie C. Coughlin, MD, asked to comment on the findings, noted that nearly half (24) of the patients in the cohort experienced recurrent RIME. “This is a high percentage, which suggests that counseling about the likelihood of recurrence is more important than previously thought,” said Coughlin, chief of the Washington University/St. Louis Pediatric Dermatology Division. St. Louis Children’s Hospital.
“Fortunately, relapse cases tend to be less severe. However, many patients relapse more than once, which can be a challenge for affected patients.”
The researchers reported no financial disclosures. Coughlin is a board member of the Pediatric Dermatology Research Alliance (PeDRA) and the International Collaborative for Immunosuppression and Transplantation Skin Cancer.
This article originally appeared on MDedge.com, part of the Medscape Professional Network.