Retrospective Multicentric Study of 25 Kimura Disease Patients: Emphasis on Therapeutics and Shared Features with Cutaneous IgG4-Related Disease.

Abstract

BACKGROUND:

Kimura disease (KD) is a rare lymphoproliferative inflammatory disease of unknown etiology. Data regarding therapeutic modalities and pathophysiology are scarce.

OBJECTIVES:

Analyze therapeutic and follow-up data and compare KD with cutaneous IgG4-related disease (IgG4-RD).

METHODS:

Multicentric retrospective study of 25 KD patients with analysis of treatment, follow-up and IgG4 immunostaining. Comparison with published cases of cutaneous IgG4-RD.

RESULTS:

Patients were mostly male (84%), median-aged 42 years with lymph node, lacrimal/salivary gland and kidney involvements in 45, 24 and 12%, respectively. Surgical excision had 100% complete response and 60% relapse. Oral corticosteroids had 100% response with 50% relapse. Thalidomide, cyclosporine or interferon-α had 100% response, but 100, 20 and 50% relapse, respectively. KD showed clinicopathological similarities with 27 published cases of cutaneous IgG4-RD.

CONCLUSION:

Surgery may be used in resectable KD cases, whereas cyclosporine or thalidomide may represent interesting alternatives to oral corticosteroids in other cases. KD shares features with cutaneous IgG4-RD.

• Authors: Diane Kottler, Stéphane Barète, Gaelle Quéreux, Saskia Ingen-Housz-Oro, Sylvie Fraitag, Nicolas Ortonne, Lydia Deschamps, Michel Rybojad, Béatrice Flageul, Béatrice Crickx, Anne Janin, Martine Bagot, Maxime Battistella
• Journal: Dermatology (Basel, Switzerland) (Dermatology) Vol. 231 Issue 4 Pg. 367-77 ( 2015) ISSN: 1421-9832 [Electronic] Switzerland
• PMID: 26452023 (Publication Type: Journal Article, Multicenter Study)
• Copyright: © 2015 S. Karger AG, Basel.

Chemical References

• Adrenal Cortex Hormones
• Immunoglobulin G
• Immunologic Factors
• Immunosuppressive Agents
• Interferon-alpha
• Immunoglobulin E
• Thalidomide
• Cyclosporine

Topics

• Adolescent
• Adrenal Cortex Hormones (therapeutic use)
• Adult
• Aged
• Aged, 80 and over
• Angiolymphoid Hyperplasia with Eosinophilia (immunology, pathology, therapy)
• Cyclosporine (therapeutic use)
• Dermatologic Surgical Procedures
• Eosinophilia (etiology)
• Female
• Humans
• Immunoglobulin E (blood)
• Immunoglobulin G (analysis)
• Immunologic Factors (therapeutic use)
• Immunosuppressive Agents (therapeutic use)
• Interferon-alpha (therapeutic use)
• Male
• Middle Aged
• Plasma Cells (chemistry)
• Recurrence
• Retrospective Studies
• Thalidomide (therapeutic use)
• Young Adult