title: Histiocytoid Sweet Syndrome Is More Frequently Associated With Myelodysplastic Syndromes Than the Classical Neutrophilic Variant: A Comparative Series of 62 Patients.
abstract: Histiocytoid Sweet syndrome (H-SS) is a histological variant of Sweet syndrome (SS) differing from classical neutrophilic SS (N-SS) by a dermal infiltrate mainly composed of lymphocytes and histiocytoid myeloperoxidase-positive cells. We aimed to report a large series of H-SS and compare the frequency and type of hematological malignancies associated to H-SS and N-SS. We included 62 patients with a coding histopathologic diagnosis of SS prospectively registered between 2005 and 2014 in the database of our Department of Pathology. Overall, 22 (35.5%) and 40 (64.5%) patients had a histological diagnosis of H-SS and N-SS, respectively. Median age, sex ratio, and cutaneous lesions were similar in the 2 groups. The frequency of extra-cutaneous manifestations was similar (50% vs 37.5%, P = 0.42). Recurrent forms were significantly more frequent in H-SS than in N-SS patients (21% vs 2.5%, P = 0.01). A hematological malignancy was diagnosed in 22 patients, 12 (55.5%) with H-SS and 10 (25%) with N-SS (P = 0.019). Hematological malignancy was of myeloid origin in 8/22 (36.3%) H-SS and 5/40 (12.5%) N-SS patients (P = 0.02), and of lymphoid origin without myeloid component in 4/22 (18.1%) H-SS and 4/40 (10%) N-SS patients (P = 0.35), respectively. One N-SS patient had a hematological malignancy of mixed (myeloid and lymphoid) phenotype. A myelodysplastic syndrome (MDS) was diagnosed in 7/22 (31.8%) H-SS and 1/40 (2.5%) N-SS patients (P < 0.001). Hematological disease was diagnosed before (in 8 H-SS and 3 N-SS patients) or at the time of the occurrence of the cutaneous lesions (in 1 H-SS and 7 N-SS patients). However, in 3 H-SS patients, all with MDS, cutaneous lesions preceded the hematological disease by ≤6 months. In conclusion, H-SS was associated with MDS in one third of patients but also with lymphoid malignancies, and cutaneous lesions could precede the hematological diagnosis in patients with MDS. A complete hematological assessment is mandatory at diagnosis, and monitoring blood cell counts should be recommended for at least 6 months after the diagnosis of H-SS.
authors.affiliation: From the Department of Dermatology, Assistance-Publique-Hopitaux-de-Paris (AP-HP), Groupe Hospitalier Henri Mondor-Albert Chenevier, Créteil, France (LG, SI-HO, PW, OC); Université Paris-Est Créteil (UPEC) (NO, CH, PW, CC, OC); UMR-S 955, Faculté de Médecine, Hopital Henri Mondor, Créteil, France (NO); Department of Pathology, AP-HP, Groupe Hospitalier Henri Mondor-Albert Chenevier, Créteil, France (NO); Department of Hematology, AP-HP, Groupe Hospitalier Henri Mondor-Albert Chenevier, Créteil, France (WB, CP, FB, CR, CC, AT); Department of Dermatology, Centre Hospitalier René Dubos, Pontoise, France (EB); and Lymphoid Malignancies Unit, AP-HP, Groupe Hospitalier Henri Mondor-Albert Chenevier, Créteil, France (CH).
authors.forename: Lisa
authors.initials: L
authors.lastname: Ghoufi
authors.forename: Nicolas
authors.initials: N
authors.lastname: Ortonne
authors.forename: Saskia
authors.initials: S
authors.lastname: Ingen-Housz-Oro
authors.forename: Walid
authors.initials: W
authors.lastname: Barhoumi
authors.forename: Edouard
authors.initials: E
authors.lastname: Begon
authors.forename: Corinne
authors.initials: C
authors.lastname: Haioun
authors.forename: Cécile
authors.initials: C
authors.lastname: Pautas
authors.forename: Florence
authors.initials: F
authors.lastname: Beckerich
authors.forename: Christine
authors.initials: C
authors.lastname: Robin
authors.forename: Pierre
authors.initials: P
authors.lastname: Wolkenstein
authors.forename: Catherine
authors.initials: C
authors.lastname: Cordonnier
authors.forename: Olivier
authors.initials: O
authors.lastname: Chosidow
authors.forename: Andréa
authors.initials: A
authors.lastname: Toma
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D001772:Blood Cell Count; D005260:Female; D019337:Hematologic Neoplasms / Q000453:epidemiology; D006644:Histiocytes / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000453:epidemiology*; D009504:Neutrophils / Q000473:pathology*; D012008:Recurrence; D012189:Retrospective Studies; D012737:Sex Factors; D016463:Sweet Syndrome / Q000145:classification / Q000453:epidemiology* / Q000503:physiopathology*; D055815:Young Adult