Schnitzler's syndrome: A case report

Aceituno Caño, Alexandra María; Vogt Sánchez, Esteban Alessandro; León Ruiz, Laura

doi:10.1016/j.medcli.2020.06.046

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It was initially described by Liliane Schnitzler, a French dermatologist, who was the first clinician that recognized this particular combination.<a class="elsevierStyleCrossRef" href="#bib0030">1</a> This syndrome is under-diagnosed because many physicians do not know about it, the diagnostic criteria are well established, however, the pathogenesis remains unknown.<a class="elsevierStyleCrossRef" href="#bib0035">2</a> Schnitzler's syndrome is considered a potential autoinflammatory disorder, clinical researchers are focused on the role of proinflammatory cytokines, interleukin-1 (IL-1) in particular.The main clinical findings are chronic urticaria, periodic fever and musculoskeletal symptoms, the frequency of urticarial eruptions differs between patients, but in most cases, the rash is continuously present, sparing palms, soles, head and neck and it is histamine-independent, so antihistamines are not effective. Recurrent fever is the second most common symptom, the frequency of the episodes also differs between patients.We describe a 65-year-old man with arterial hypertension, who has been suffering, for twelve years, daily outbreaks of urticariform non pruritic lesions in chest and both limbs. He was evaluated by dermatologists being cataloged of chronic idiopathic urticaria, having only a partial remission of symptoms with high dose of corticosteroids. In 2014, he associated recurrent febrile episodes, generalized osteoarticular pain, neutrophilic leukocytosis and paraproteinemia in laboratory tests. A skin biopsy and a genetic study were made rulling out autoinflammatory diseases and vasculitis. He also was evaluated and followed by hematologists and rheumatologists. In 2017, he associated weight loss and arthralgias, being referred to Internal Medicine consultation.The physical examination highlighted anular erythematous and maculopapular non-confluent lesions in chest and upper limbs.In the blood tests there were leukocytosis with neutrophilia, C-reactive protein (CRP) of 12mg/dl and globular sedimentation rate (ESR) of 79mm/h. Autoimmunity, rheumatoid factor and vascular endothelial growth factor (VEGF) were negative, complement was normal. A monoclonal band in the gamma region, IgM 677mg/dl, was observed in the proteinogram. Bone sclerosis is seen on the dorsal-lumbar spine and pelvis radiographs and generalized subcentometric adenopathies were found in the thoracoabdominal scanner. The electroneurogram described a polyneuropathy. Bone marrow biopsy was performed with a negative cytogenetic study for lymphoproliferative diseases.The combination of chronic urticarial rash, monoclonal IgM gammopathy, recurrent fever, bone sclerosis and leukocytosis, allowed us to make the diagnosis of Schnitzler's syndrome.There are several pathologies that can occur with monoclonal gammopathy and chronic urticaria, including autoimmune, hematological, infectious or autoinflammatory diseases, so diagnostic tests are used to exclude these conditions.<a class="elsevierStyleCrossRef" href="#bib0040">3</a> There is no specific test for Schnitzler's syndrome, the diagnosis is made when patients meet Strasbourg criteria, which include: two obligate criteria and six minor criteria. The diagnosis is considered definite if the two obligate criteria (recurrent, nonpruritic urticaria and monoclonal gammopathy, either IgM or IgG) and, at least, two minor criteria (recurrent fever, abnormal bone remodeling, neutrophilic dermal infiltrate, and elevated protein C reactive (CRP) and/or leukocytosis) are met.<a class="elsevierStyleCrossRef" href="#bib0045">4</a>Hematological malignancy is the major complication, lymphoproliferative disorder can develop in about 20% of patients, so, this syndrome needs to be considered in the differential diagnosis of urticarial dermatoses.A wide range of drugs has been used in patients with Schnitzler's syndrome without achieving a complete remission of symptoms. All experts agree that Anakinra, an interleukin-1-neutralizing agent, is the only completely efficient treatment so it has been proposed to be added as a diagnostic criteria.<a class="elsevierStyleCrossRef" href="#bib0050">5</a>The patient started treatment with Anakinra at a dose of 100mg/24h, presenting a progressive decrease in the CRP and leukocyte count and an improvement of the skin lesions in 24h.Delay to diagnosis is still many years, in this case, it took thirteen years being assessed by different specialists.The aim of this report is to increase the knowledge of this rare and debilitating syndrome. An early treatment can improve the patient's quality of life and prognosis.FundingThis research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.Conflict of interestThe authors declare no conflict of interest." 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