title: Heterogeneity in presentation and treatment of catamenial anaphylaxis.
abstract: BACKGROUND
  Few reports have documented the uncommon association of the female menstrual cycle with anaphylaxis, an entity known as cyclic or catamenial anaphylaxis.
  
  
  OBJECTIVE
  To examine cases of perimenstrual anaphylaxis, focusing on differences in presentation and response to treatment, in the hopes of enriching the description of this rare entity.
  
  
  METHODS
  A cohort of 8 women with catamenial anaphylaxis were identified and retrospectively compared with regard to age at onset, organ involvement, diagnostic studies, and response to therapy.
  
  
  RESULTS
  The median age at onset was 34 years (range, 14-40 years), and the median number of perimenstrual anaphylactic episodes at presentation was 10 per patient (range, 4-24 per patient). Most had cutaneous and gastrointestinal symptoms. The results of extensive investigations for anaphylactic triggers were negative, and masquerading conditions, such as carcinoid syndrome, pheochromocytoma, and systemic mastocytosis, were ruled out in all patients. Skin test results for progesterone were negative in all but 1 of 4 patients tested. None had elevated total serum IgE levels. Response to suppressive treatments regimens varied considerably, but none treated with high-dose systemic steroids had improvement. Similarly, ketotifen, celecoxib, rofecoxib, and oral contraceptives failed to control the anaphylactic reactions. Although antihistamines failed in 7 patients, 1 had improvement. Others responded to leuprolide, medroxyprogesterone, or salpingo-oophorectomy.
  
  
  CONCLUSIONS
  Whether the mechanism causing cyclical anaphylaxis may involve hypersensitivity to progesterone or prostaglandins, the variable response to suppressive medications in these cases suggests that catamenial anaphylaxis is a heterogeneous disorder in which a number of mechanisms and mediators may play a role. It is an emergent and probably underrecognized entity in the medical literature.
authors.affiliation: Division of Allergy, Asthma, and Clinical Immunology, Department of Pediatrics, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, Wisconsin, USA. cindysbauer@gmail.com
authors.forename: Cindy S
authors.initials: CS
authors.lastname: Bauer
authors.forename: Thatchai
authors.initials: T
authors.lastname: Kampitak
authors.forename: Mary L
authors.initials: ML
authors.lastname: Messieh
authors.forename: Kevin J
authors.initials: KJ
authors.lastname: Kelly
authors.forename: Peter
authors.initials: P
authors.lastname: Vadas
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000707:Anaphylaxis / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008597:Menstrual Cycle / Q000502:physiology*; D012189:Retrospective Studies; D055815:Young Adult