title: Erythema Nodosum Leprosum-Like Lesions Are a Histopathologic Pattern in Whipple's Disease and a Sign of the Immune Reconstitution Inflammatory Syndrome: A Case Series and Review of the Literature.
abstract: Inflammatory and subcutaneous nodules can arise in treated and untreated cases of Whipple disease (WD). The inflammatory immune reconstitution syndrome describes paradoxical clinical inflammatory worsening of a preexisting condition because of a return of immune function. Clinicopathologic examination of 4 patients with WD who presented with erythema nodosum leprosum (ENL)-like lesions and the findings of a systematic review of this phenomenon revealed that ENL-like lesions occurred in predominantly middle-aged male patients who suffered from WD, mostly on the legs. Patients showed a nonvasculitic, mostly septal panniculits with neutrophils, macrophages, and lymphocytes. Numerous bacteria-laden periodic acid-Schiff + macrophages and free bacilli were detected in the dermis, as well as subcutaneous septae and adipose lobules. These lesions occurred in both untreated and treated patients as part of inflammatory immune reconstitution syndrome. In conclusion, ENL-like lesions represent a characteristic histopathologic pattern associated with WD, which can occur in different contexts whenever there is a change in the immunological status of the patient. This change can be triggered by antimicrobial treatment, immunomodulatory and immunosuppressant therapy, or occur spontaneously, rarely.
authors.affiliation: *Dermatopathology Duisburg, Duisburg, Germany; †Department of Pathology, APHP Cochin Hospital, University Paris-Descartes, Paris, France; ‡Department of Dermatology, Saint Pierre-Brugmann and Children's University Hospitals, UniversiteLibre de Bruxelles, Brussels, Belgium; and §Divisions of Dermatopathology and Dermatology, Department of Pathology, Albany Medical College, Albany, NY.
authors.forename: Julia
authors.initials: J
authors.lastname: Liersch
authors.forename: Agnès
authors.initials: A
authors.lastname: Carlotti
authors.forename: Anne
authors.initials: A
authors.lastname: Theunis
authors.forename: Alexandra
authors.initials: A
authors.lastname: Leonard
authors.forename: Mary
authors.initials: M
authors.lastname: Barrett
authors.forename: John Andrew
authors.initials: JA
authors.lastname: Carlson
authors.forename: Jörg
authors.initials: J
authors.lastname: Schaller
mesh_terms: D004893:Erythema Nodosum / Q000382:microbiology* / Q000473:pathology; D006801:Humans; D054019:Immune Reconstitution Inflammatory Syndrome / Q000473:pathology*; D007166:Immunosuppressive Agents / Q000009:adverse effects; D008297:Male; D008875:Middle Aged; D008061:Whipple Disease / Q000188:drug therapy / Q000473:pathology*
chemical_list: D007166:Immunosuppressive Agents