Erythema multiforme associated with anti‐plakin antibodies: a multicentric retrospective case series

Background Erythema multiforme (EM) is a muco‐cutaneous inflammatory disease mainly triggered by herpes simplex virus (HSV) recurrences. Association of EM and circulating auto‐antibodies against plakins (anti‐PLK‐Abs [EM‐PLK+]) has been reported. However, little is known about this subset of EM. Obj...

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Published in:Journal of the European Academy of Dermatology and Venereology 2022-12, Vol.36 (12), p.2438-2442
Main Authors: Weill, A., Descamps, V., Chasset, F., Mahévas, T., Bourgault‐Villada, I., Wolkenstein, P., Chollet‐Martin, S., Ingen‐Housz‐Oro, S., Grootenboer‐Mignot, S.
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Language:English
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Summary:Background Erythema multiforme (EM) is a muco‐cutaneous inflammatory disease mainly triggered by herpes simplex virus (HSV) recurrences. Association of EM and circulating auto‐antibodies against plakins (anti‐PLK‐Abs [EM‐PLK+]) has been reported. However, little is known about this subset of EM. Objectives We aimed to describe the clinical and immunological features and response to treatment of EM‐PLK+. Methods We conducted a retrospective multicentric study of EM‐PLK+ selected from the database of the immunological laboratory of Bichat hospital, Paris, France, from January 2009 to December 2020. Anti‐PLK‐Abs were detected in ≥1 immunological tests: immunofluorescence assay, immunoblotting and/or ELISA. Patients with alternative diagnoses were excluded. Results We included 29 patients (16 women, median age 25 [range 2–58] years). EM‐PLK+ were mostly major (EM with ≥2 mucosal involvements; n = 24, 83%) and relapsing (≥2 flares; n = 23, 79%). Cutaneous lesions were target (n = 13, 54%) and target‐like lesions (n = 9, 38%) with usual topography (acral, n = 19, 79%; limbs, n = 21, 88%). Mucosal lesions affected the mouth (n = 27, 96%) and genitalia (n = 19, 68%), with a median of 2 [range 0–5] mucous membranes. EM‐PLK+ were suspected as certain or possible postherpetic (EM‐HSV) in 19 cases (65.5%); no triggering factors were detected in 9 (31%) patients. Desmoplakin‐I/II Abs were the most frequent anti‐PLK‐Abs (n = 20, 69%); envoplakin and periplakin Abs were detected in 11 and 9 cases. Relapsing EM‐PLK+ (n = 23) were still active (≥1 flare within 6 months) in 13 (57%) patients despite immunosuppressive therapy (n = 8, 62%). Antiviral drugs were ineffective in preventing relapse in 15/16 (94%) EM‐HSV. Conclusion The rationale for anti‐PLK‐Ab detection in EM is not elucidated. More systematic research of anti‐PLK‐Abs is warranted to better understand whether this association reflects humoral immune activity in a subset of EM or is fortuitous, related to an epitope spreading process. However, EM‐PLK+ seems to be associated with major and relapsing subtypes, and difficult‐to‐treat cases.
ISSN:0926-9959
1468-3083
DOI:10.1111/jdv.18259