title: Association of hidradenitis suppurativa and familial Mediterranean fever: A case series of 6 patients.
abstract: OBJECTIVE
  Familial mediterranean fever (FMF) is the most common monogenic autoinflammatory disease. Hidradenitis suppurativa (HS) is an inflammatory cutaneous disease. Those diseases can occur simultaneously among the same individual. Our objective was to describe the features of patients displaying both FMF and HS.
  
  
  METHODS
  We screened the French adult FMF reference center for FMF patients with HS.
  
  
  RESULTS
  Six patients out of 151 (4%) with a median age of 36 years old were concerned. Among them, FMF was symptomatic at a median age of 11.5years old and colchicine was introduced at a median age of 20.5years old. HS was diagnosed at a median age of 31.5years old. An elderly patient displayed AA amyloidosis in the outcome of FMF, with a late diagnosis of HS, with response to anakinra. There was no temporal relation between FMF and HS attacks. Some patients had a persistent inflammatory syndrome under treatment.
  
  
  CONCLUSIONS
  FMF and HS are both inflammatory diseases involving young patients, with HS possibly being an autoinflammatory disease. Although their association seems to be fortuitous, both can induce an important inflammation state that could lead to AA amyloidosis and require a close monitoring of clinical signs and acute-phase reactants. Anakinra was successful in treating the only patient with both HS, FMF and amyloidosis.
authors.affiliation: Service de médecine interne, centre de référence des amyloses d'origine inflammatoire et de la fièvre méditerranéenne familiale, hôpital Tenon, Assistance publique-Hôpitaux de Paris (AP-HP), université Paris 6, Pierre-et-Marie-Curie (UPMC), 20, rue de la Chine, 75020 Paris, France.
authors.forename: Salam
authors.initials: S
authors.lastname: Abbara
authors.affiliation: Service de médecine interne, centre de référence des amyloses d'origine inflammatoire et de la fièvre méditerranéenne familiale, hôpital Tenon, Assistance publique-Hôpitaux de Paris (AP-HP), université Paris 6, Pierre-et-Marie-Curie (UPMC), 20, rue de la Chine, 75020 Paris, France; Inserm UMRS_933, hôpital Trousseau, AP-HP université Pierre-et Marie-Curie (UPMC)-Paris 6, Paris, France. Electronic address: sophie.georgin-lavialle@aphp.fr.
authors.forename: Sophie
authors.initials: S
authors.lastname: Georgin-Lavialle
authors.affiliation: Service de médecine interne, centre de référence des amyloses d'origine inflammatoire et de la fièvre méditerranéenne familiale, hôpital Tenon, Assistance publique-Hôpitaux de Paris (AP-HP), université Paris 6, Pierre-et-Marie-Curie (UPMC), 20, rue de la Chine, 75020 Paris, France.
authors.forename: Katia
authors.initials: K
authors.lastname: Stankovic Stojanovic
authors.affiliation: Service de médecine interne, centre de référence des amyloses d'origine inflammatoire et de la fièvre méditerranéenne familiale, hôpital Tenon, Assistance publique-Hôpitaux de Paris (AP-HP), université Paris 6, Pierre-et-Marie-Curie (UPMC), 20, rue de la Chine, 75020 Paris, France.
authors.forename: Claude
authors.initials: C
authors.lastname: Bachmeyer
authors.affiliation: Service de dermatologie, hôpital Tenon, AP-HP, UPMC, 20, rue de la Chine, 75020 Paris, France.
authors.forename: Patricia
authors.initials: P
authors.lastname: Senet
authors.affiliation: Laboratoire d'anatomie pathologique, hôpital Tenon, AP-HP, UPMC, 20, rue de la Chine, 75020 Paris, France.
authors.forename: David
authors.initials: D
authors.lastname: Buob
authors.affiliation: Service de médecine interne et immunologie clinique, Bocage Central, 14, rue Paul-Gaffarel, 21000 Dijon, France.
authors.forename: Sylvain
authors.initials: S
authors.lastname: Audia
authors.affiliation: Service de médecine interne, hôpital Lariboisière, AP-HP, 2, rue Ambroise-Paré, 75010 Paris, France.
authors.forename: Véronique
authors.initials: V
authors.lastname: Delcey
authors.affiliation: Laboratoire de biochimie, hôpital Tenon, 20 rue de la Chine, AP-HP, UPMC, 75020 Paris, France.
authors.forename: Soraya
authors.initials: S
authors.lastname: Fellahi
authors.affiliation: Laboratoire de biochimie, hôpital Tenon, 20 rue de la Chine, AP-HP, UPMC, 75020 Paris, France.
authors.forename: Jean-Philippe
authors.initials: JP
authors.lastname: Bastard
authors.affiliation: Inserm UMRS_933, hôpital Trousseau, AP-HP université Pierre-et Marie-Curie (UPMC)-Paris 6, Paris, France; Laboratoire de génétique, hôpital Trousseau, AP-HP, UPMC, 26, rue du Dr-Arnold-Netter, 75012 Paris, France.
authors.forename: Fawaz
authors.initials: F
authors.lastname: Awad
authors.affiliation: Laboratoire de génétique, hôpital Trousseau, AP-HP, UPMC, 26, rue du Dr-Arnold-Netter, 75012 Paris, France.
authors.forename: Marie
authors.initials: M
authors.lastname: Legendre
authors.affiliation: Inserm UMRS_933, hôpital Trousseau, AP-HP université Pierre-et Marie-Curie (UPMC)-Paris 6, Paris, France; Service de dermatologie, hôpital Tenon, AP-HP, UPMC, 20, rue de la Chine, 75020 Paris, France; Laboratoire de génétique, hôpital Trousseau, AP-HP, UPMC, 26, rue du Dr-Arnold-Netter, 75012 Paris, France.
authors.forename: Serge
authors.initials: S
authors.lastname: Amselem
authors.affiliation: Service de médecine interne, centre de référence des amyloses d'origine inflammatoire et de la fièvre méditerranéenne familiale, hôpital Tenon, Assistance publique-Hôpitaux de Paris (AP-HP), université Paris 6, Pierre-et-Marie-Curie (UPMC), 20, rue de la Chine, 75020 Paris, France; Inserm UMRS_933, hôpital Trousseau, AP-HP université Pierre-et Marie-Curie (UPMC)-Paris 6, Paris, France.
authors.forename: Gilles
authors.initials: G
authors.lastname: Grateau
mesh_terms: D000328:Adult; D000368:Aged; D010505:Familial Mediterranean Fever / Q000150:complications* / Q000175:diagnosis; D005260:Female; D017497:Hidradenitis Suppurativa / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D008297:Male; D008875:Middle Aged
keywords: Autoinflammatory syndrome; Colchicine; Familial Mediterranean fever; Hidradenitis suppurativa; Interleukin-1 inhibitors; MEFV