Chronic urticaria and macroglobulinemia (Schnitzler's syndrome): Report of two cases

Janier, M.; Bonvalet, Douceline; Blanc, Marie-France; Lemarchand, F; Cavelier, Bénédicte; Ribrioux, A.; Aguenier, Béatrice; Civatte, J

doi:10.1016/s0190-9622(89)70023-2

Cited by 61 publications

(30 citation statements)

References 15 publications

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“…[143][144][145] Skin findings consist of asymptomatic erythematous, edematous plaques with prominent borders, primarily found on the trunk and lower extremities. Lymphadenopathy, hepatomegaly, polyclonal lymphoplasmacytic infiltration of the bone marrow, and rarely, severe anemia and life-threatening thrombophilia have been reported.…”

Section: Schnitzler Syndromementioning

confidence: 99%

“…[154][155][156] Histology of lesional skin demonstrates a perivascular infiltrate consisting of lymphocytes, histiocytes, and neutrophils. 145 Immunofluorescence staining shows IgM deposits in the papillary dermis or at the basement membrane. 145 Daily administration of anakinra has provided long-term control.…”

Section: Schnitzler Syndromementioning

confidence: 99%

“…145 Immunofluorescence staining shows IgM deposits in the papillary dermis or at the basement membrane. 145 Daily administration of anakinra has provided long-term control. [157][158][159][160] Psoralen combined with ultraviolet A therapy and IL-6 blockade with tocilizumab may be effective, respectively.…”

Section: Schnitzler Syndromementioning

confidence: 99%

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Autoinflammation: From monogenic syndromes to common skin diseases

Nguyen

Cowen

Leslie

2013

Journal of the American Academy of Dermatology

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Autoinflammation is characterized by aberrant regulation of the innate immune system and often manifests as periodic fevers and systemic inflammation involving multiple organs, including the skin. Mutations leading to abnormal behavior or activity of the interleukin 1 beta (IL-1ß)-processing inflammasome complex have been found in several rare autoinflammatory syndromes, for which anticytokine therapy such as IL-1 or tumor necrosis factor-alfa inhibition may be effective. It is becoming clear that features of autoinflammation also affect common dermatoses, some of which were previously thought to be solely autoimmune in origin (eg, vitiligo, systemic lupus erythematosus). Recognizing the pathogenetic role of autoinflammation can open up new avenues for the targeted treatment of complex, inflammatory dermatoses.

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Section: Schnitzler Syndromementioning

confidence: 99%

Section: Schnitzler Syndromementioning

confidence: 99%

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Autoinflammation: From monogenic syndromes to common skin diseases

Nguyen

Cowen

Leslie

2013

Journal of the American Academy of Dermatology

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“…Bone pain and/or hyper ostosis occurs in half the cases as recently reviewed [3]. For the first time a double monocomponent of IgM was observed as well as a difference in the ultracentrifugation profile of immunoglobulins between the active and the quiescent states.…”

Section: Discussionmentioning

confidence: 88%

Urticaria with Macroglobulinaemia

Bonnetblanc

Drouet²,

Laplaud

et al. 1990

Dermatology

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A case of chronic urticaria-like lesions with macroglobulinaemia (Schnitzler syndrome) is described. This case is unique by its double IgM monocomponents and by the follow-up study which shows immunoglobulins modifications between cutaneous flare-up and remission. As 1 case had a lymphoma, we studied the mononuclear cells of the bone marrow of Schnitzler syndrome to raise a possible malignant evolution of that disorder, and we discovered a hypodiploid population, which we compared with similar studies of myeloma and monoclonal ‘benign’ gammopathies.

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“…Deposition of IgM and C3 within the granular basement membrane can also be seen. 10 Sometimes, the IgM is deposited at the junction between the dermis and epidermis, and there is evidence that these antibodies may target antigens present in the skin 31,32 and, possibly, induce an inflammatory response leading to the skin lesions and symptoms. Sometimes, patients with the syndrome undergo lymph node biopsy, but in the absence of lymphoma, for which these patients are at risk, the findings tend to be nonspecific.…”

mentioning

confidence: 99%

Schnitzler syndrome: clinical features and histopathology

Dingli¹,

Camilleri²

2015

PLMI

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Schnitzler syndrome is a rare and underrecognized syndrome characterized by chronic urticaria, a monoclonal protein, and a variety of other symptoms, including fever, bone pain, organomegaly, and evidence of an acute phase response. Biopsy of an involved area of the skin shows a neutrophilic infiltrate without evidence of vasculitis or hemorrhage. Although the etiology of the syndrome is unknown, current evidence suggests this is an autoinflammatory syndrome. Recognition of this syndrome is critical since it is highly responsive to anakinra.

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Chronic urticaria and macroglobulinemia (Schnitzler's syndrome): Report of two cases

Cited by 61 publications

References 15 publications

Autoinflammation: From monogenic syndromes to common skin diseases

Autoinflammation: From monogenic syndromes to common skin diseases

Urticaria with Macroglobulinaemia

Schnitzler syndrome: clinical features and histopathology

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