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• W2509519671 abstract "To the Editor: Hidradenitis suppurativa (HS) occurs in approximately 1% of the general population.1Revuz J.E. Canoui-Poitrine F. Wolkenstein P. et al.Prevalence and factors associated with hidradenitis suppurativa: results from two case-control studies.J Am Acad Dermatol. 2008; 59: 596-601Abstract Full Text Full Text PDF PubMed Scopus (476) Google Scholar It is characterized by recurrent inflammatory nodules, sinus tracts, and scarring, most commonly in the axilla and groin. The origin is unknown, but is most likely related to inflammation and immune dysfunction, combined with extrinsic factors.2van der Zee H.H. Laman J.D. Boer J. Prens E.P. Hidradenitis suppurativa: viewpoint on clinical phenotyping, pathogenesis and novel treatments.Exp Dermatol. 2012; 21: 735-739Crossref PubMed Scopus (166) Google Scholar The relationship between HS and Down syndrome (DS) was noted in 1977 by Dvorak et al.3Dvorak V.C. Root R.K. MacGregor R.R. Host-defense mechanisms in hidradenitis suppurativa.Arch Dermatol. 1977; 113: 450-453Crossref PubMed Scopus (55) Google Scholar Since then, scattered case reports have been published but the mechanism underlying this association remains unidentified. Details regarding the relationship between HS and DS, such as effect on disease incidence and age of onset, are also lacking in the literature. To provide a better clinical characterization of patients with both HS and DS, we performed a retrospective chart review of all patients with HS seen by our dermatology service between January 1, 1992, and October 1, 2014. Patients were identified as having HS by billing records. Co-occurrence of HS and DS was confirmed by manual examination of the patient medical records. We collected data on sex, race, age of HS symptom onset (commonly recorded as a range), body mass index, disease location and severity, and family history of HS. We hypothesized that HS onset occurs earlier in DS, and to bias toward the null we used the oldest age in an age range when calculating mean/median age of onset in patients with DS. Disease severity, based on physician assessment and physical examination findings, was recorded using the Hurley criteria at the initial HS-specific visit.4Hurley H. Axillary hyperhidrosis, apocrine bromhidrosis, hidradenitis suppurativa, and familial benign pemphigus: surgical approach. In: Roenigk RK and Roenigk HH Jr, eds. Roenigk and Roenigk’s dermatologic surgery: principles and practice. 2nd ed. New York: Marcel Dekker Inc. 1996. p. 633-640.Google Scholar We identified a total of 667 patients with HS seen during this time, with 16 patients (2.4%) having both HS and DS (Table I). All portions of this study were conducted as a part of an institutional review board–approved study at Washington University School of Medicine.Table ICharacteristics of patients with both hidradenitis suppurativa and Down syndrome versus hidradenitis suppurativa onlyDown syndrome and HSHS onlyP valueSexn = 16n = 651 Male5 (31.2%)130 (20.0%).339∗Fisher exact test. Female11 (68.8%)521 (80.0%)Racen = 16n = 651 Non-Caucasian10 (62.5%)355 (54.5%).527†χ2 Test. Caucasian6 (37.5%)296 (45.5)BMI, kg/m2n = 15n = 527 Mean (SD)36.5 (11.3)34.4 (9.2).388‡Independent samples t test.Family history of HSn = 16n = 649 Positive2 (12.5%)84 (12.9%)1.000∗Fisher exact test.Age of HS symptom onset, yn = 8n = 335 Mean (SD)14.9 (4.6)23.3 (11.9).018§Mann-Whitney U test. Range9-245-68 Median14.519.0Age at presentation, yn = 16n = 645 Mean (SD)17.9 (4.9)33.9 (14.2)<.001§Mann-Whitney U test. Range11.7-29.39.7-71.7 Median17.232.1Disease severityn = 16n = 628 Mild10 (62.5%)312 (49.7%).420†χ2 Test. Moderate6 (37.5%)273 (43.5%) Severe0 (0.0%)43 (6.8%)Disease locationn = 16n = 651 Axilla12 (75.0%)361 (55.5%).120†χ2 Test. Buttocks3 (18.8%)84 (12.9%).452∗Fisher exact test. Inframammary0 (0.0%)86 (13.2%).246∗Fisher exact test. Inguinal/groin6 (37.5%)256 (39.3%).883†χ2 Test. Inner thighs3 (18.8%)92 (14.1%).487∗Fisher exact test. Pubic1 (6.3%)37 (5.7%).613∗Fisher exact test.Statistical analysis was completed using SPSS for Mac v22.0 (IBM Corp, Armonk, NY). Nonparametric analysis was used when examining age of onset to account for nonnormal distributions. Bold indicates statistical significance.BMI, Body mass index; HS, hidradenitis suppurativa.∗ Fisher exact test.† χ2 Test.‡ Independent samples t test.§ Mann-Whitney U test. Open table in a new tab Statistical analysis was completed using SPSS for Mac v22.0 (IBM Corp, Armonk, NY). Nonparametric analysis was used when examining age of onset to account for nonnormal distributions. Bold indicates statistical significance. BMI, Body mass index; HS, hidradenitis suppurativa. We found a younger age of HS onset in our patients with DS, and an overall increased prevalence of DS in our HS population. The mean age of HS symptom onset in those with DS was 14.9 years compared with 23.3 years (P = .018) for those without DS. We were able to obtain age of HS symptom onset in only 8 of 16 of the patients with DS. However, only 2 of 16 of patients with DS presented with HS after the age of 19 years–the median age of HS onset in general. The 2.4% prevalence of DS in our population of patients with HS was approximately 29 times higher than the 0.083% prevalence of DS in the general population.5Presson A.P. Partyka G. Jensen K.M. et al.Current estimate of Down syndrome population prevalence in the United States.J Pediatr. 2013; 163: 1163-1168Abstract Full Text Full Text PDF PubMed Scopus (234) Google Scholar Although this relationship may be falsely exaggerated as a result of referral bias, we believe that bias alone could not account for such a massive increase in prevalence. These results support the notion that DS is associated with the development of HS, particularly at a younger age. Further investigation into the nature of the relationship between these 2 disease entities is warranted." @default.
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• W2509519671 title "Hidradenitis suppurativa (HS) and Down syndrome (DS): Increased prevalence and a younger age of hidradenitis symptom onset" @default.
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