Hidradenitis Suppurativa in a Large Cohort of Italian Patients: Evaluation of the Burden of Disease.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic, inflammatory, recurrent, debilitating skin disease of the hair follicle that usually occurs after puberty with painful, deep-seated, inflamed nodules and sinus tracts in the apocrine gland-bearing areas of the body, most commonly the axillae and inguinal and anogenital regions, with a relevant impact on patients' quality of life (QoL). OBJECTIVE: To evaluate how the burden of HS disease impacts on patient well-being and working activities in a large Italian population over a period of 9 months. METHODS: A multicenter, prospective, epidemiologic cohort study was conducted in adult Italian patients with HS. HS severity was assessed through Hurley stage and HS Physician's Global Assessment (HS-PGA), clinical improvement by HS Clinical Response (HiSCR) and partial response, and disease burden through QoL questionnaires (HIDRAdisk, Skindex-16, Dermatology Life Quality Index [DLQI]), and Work Productivity and Activity Impairment - General Health (WPAI:GH). RESULTS: A total of 308 patients (56.2% women; mean age 35.2 ± 12.9 years) were enrolled in 27 dermatologic clinics. Men were older (37.4 years vs. 33.5), more smoking addicted (74.1% vs. 60.1%), and alcohol consumer (34.1% vs. 13.9%), while more women were obese (34.10% vs. 22.22%). At baseline, most patients had a Hurley severity stage of 2 (43.9%), a moderate HS-PGA score (57.1%), and poor QoL (HIDRAdisk: 65.7 ± 23.3, Skindex-16: 60.3 ± 26.9, and DLQI: 10.8 ± 8.1). Patients with more severe disease showed worse QoL. Mean values for the variables related to HS severity decreased during the study period. The achievement of HiSCR and partial response increased during the study. CONCLUSION: This study offers insight into the disease burden of HS in an Italian population. Our results underline the impact of QoL evaluation, also with the use of the HIDRAdisk, in clinical routine as a support to validated severity clinical and instrumental indexes for a "360-degree" assessment of HS patient's burden of disease.

Pediatric Hidradenitis Suppurativa: A Cross-Sectional Study on Clinical Features and Treatment Approaches.

BACKGROUND: Hidradenitis suppurativa is uncommon in patients of pediatric age, and differentiation with adult-onset disease is controversial. Treatment of pediatric hidradenitis suppurativa is scarcely standardized, and specific guidelines are lacking. OBJECTIVE: We report the clinical features, relevant risk-factors, comorbidity profile, and treatment patterns of a hospital-based cohort of pediatric hidradenitis suppurativa. METHODS: In a cross-sectional study data on patients' demographics, disease-specific characteristics, early/pre-pubertal onset of disease, comorbidities, and treatment management were retrieved. Reference population data and clinical data from the national hidradenitis suppurativa disease registry were used for comparison. RESULTS: From a database of 870 patients with hidradenitis, 71 (15 males and 56 females) patients aged <18 years (mean age: 15.3 years; range 8-17 years), with mild (Hurley I, 45.1%) and moderate-severe disease (Hurley II-III, 54.9%), were retrieved. Smoking (23.9%) and overweight/obese frequencies (59.2%) were higher than reference population standards. Patient's older age at baseline (OR 1.43, 95% CI: 1.01 to 2.02) and higher BMI (OR 1.26, 95% CI: 1.07-1.48) were the only factors associated with moderate-severe disease. Family history and early/pre-pubertal onset of disease were not associated with severity or extent of disease. Sebaceous-follicular comorbid conditions were associated with cigarette smoking (P = .002). Among 81 treatment courses, clindamycin-based and zinc-sulphate-based combination regimens were most frequently used (59.3%). Female preponderance, family history of disease and extensive involvement were significantly different from the general hidradenitis suppurativa population. CONCLUSIONS: Pediatric hidradenitis suppurativa presents a clinical spectrum comparable to adult-onset disease. Increased preventive measures should target obesity and smoking in this population.

Real-Time Confocal Imaging for Hidradenitis Suppurativa: Description of Morphological Aspects and Focus on the Role of Follicular Ostia.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic, recurrent, inflammatory skin disease involving the follicular unit. Growing evidence suggests a crucial role of hyperkeratosis, infundibular plugging and perifolliculitis in its pathogenesis. OBJECTIVES: To characterize the microscopic morphology of open comedos using in vivo reflectance confocal microscopy (RCM) and define alterations occurring in HS normal-appearing perilesional skin compared to healthy skin of a control group, discussing therefore microscopic differences. MATERIALS AND METHODS: Twenty patients (11 males, 9 females; aged 19-51 years) affected by HS were recruited. RCM was performed on open comedos of axillary/inguinal regions and on normal-appearing skin areas distant at least 1.5 cm from HS lesions. Ten healthy individuals (6 males, 4 females) were included as control group. RESULTS: RCM aspects of 78 open comedos were explored, observing circular/ovoidal structures disclosing a hyperrefractive amorphous material (72/78, 92.3%) within the infundibular cavity surrounded with a bright (51/78, 65.4%) and regular (52/78, 66.2%) border. Follicular ostia of HS perilesional skin (n = 541) compared to follicular ostia of a control group (n = 321) were characterized by a larger infundibular diameter (183.4 vs. 85.8 µm) and diverged in terms of material collected inside infundibula (44.5 vs. 21%) and keratinization of the border (47.6 vs. 25.5%). CONCLUSION: In vivo characterization of open comedos and the recognition of microscopic subclinical alterations of HS normal-appearing skin, compared to healthy skin, could add further insights into the definition of biological events leading to HS manifestations, including the dysregulated process of keratinization.

Disease Severity Is Associated with Alexithymia in Patients with Atopic Dermatitis.

BACKGROUND: Atopic dermatitis (AD) is a chronic inflammatory skin disorder that is associated with higher rates of psychological disorders, but limited evidence supported the association with alexithymia, a psychoaffective dysfunction. OBJECTIVES: This study was aimed to investigate the occurrence of alexithymia in AD patients, compared to healthy subjects. METHODS: This cross-sectional study assessed AD severity by the Eczema Area and Severity Index (EASI) score, sleeplessness and itch by a numeric rating scale (NRS), and alexithymia by the 20-item Toronto Alexithymia Scale (TAS-20) score. The association between disease characteristics and alexithymia was evaluated through several logistic regression models. RESULTS: 202 AD patients and 240 healthy subjects were included in this study. The alexithymic personality trait (TAS-20 ≥51) was more frequently observed among AD patients compared to the control group (62.4% [126/202] vs. 29.2% [70/240], p < 0.0001). In particular, alexithymia (TAS-20 score ≥61) was detected in a significantly higher number of AD patients than in the controls (27.7% [56/202] vs. 7.5% [18/240]; p < 0.0001), whereas borderline alexithymia was detected in 34.6% (70/202) of AD patients compared to 21.7% of healthy controls. Alexithymia was more common among severe AD patients (43.6%) compared to mild AD patients (15.6%) and correlated with itch intensity and sleep disturbances. Among clinical variables, ordered logistic regression analyses revealed disease severity as predictor of alexithymia. Indeed, univariate analysis showed EASI score, sleep NRS, and itch NRS being significantly associated with alexithymia, while a multivariate model identified increased EASI score values as predicting factor. CONCLUSION: This study described alexithymia in AD patients correlating its occurrence with clinical AD severity markers (EASI score, itch, and sleeplessness) and identifying the increase in EASI score as predicting factor.

Clinical and Ultrasonographic Profile of Adalimumab-treated Hidradenitis Suppurativa Patients: A Real-life Monocentric Experience.

Ultrasonography has proven useful for diagnosis and treatment monitoring in patients with hidradenitis suppurativa. The aim of this study was to assess the clinical response to adalimumab using ultrasound findings. This prospective study collected data on demographic features, disease severity, and hidradenitis suppurativa findings from patients with hidradenitis suppurativa treated with adalimumab. Generalized estimating equations investigated relationships between disease severity measures and clinical/demographic variables. The study included a total of 41 patients with hidradenitis suppurativa who were treated with adalimumab for a mean period of 50.8 ± 32.2 weeks; range 6-108 weeks). Clinical improvement was observed during adalimumab therapy, with a progressively greater number of patients achieving HiSCR50 response (36.4% at week 52). Disease duration was identified as the most relevant clinical variable affecting disease severity and treatment response. Treatment response was also influenced by treatment duration, with a 4% greater likelihood of achieving HiSCR50 response at each time-point. At the ultrasound examination, subcutaneous involvement of hidradenitis suppurativa lesions was identified as a predictive negative factor for clinical response to adalimumab (HiSCR50 achievement).

Defining Fistular Patterns in Hidradenitis Suppurativa: Impact on the Management.

BACKGROUND: Recent studies suggest that there are different fistular subtypes in hidradenitis suppurativa (HS) patients who are associated with variable therapeutic outcomes. OBJECTIVE: To describe clinical and ultrasound features that characterize the different fistular patterns in HS and to evaluate the response to medical therapies. METHODS: A retrospective study developed by a well-recognized center specialized in HS analyzing both clinical and ultrasound (US) aspects of fistular structures in HS patients was performed. Medical therapy response was evaluated through follow-up visits at Week 24. RESULTS: A total of 117 fistulas detected in the skin of 40 patients were evaluated. Four different types of fistulas were described: dermal fistula (Type A), dermoepidermal fistula (Type B), complex fistula (Type C), and subcutaneous fistula (Type D). Fistulas Type A and B showed a complete resolution after 6 months of different medical therapies in up to 95% and 65% of cases, respectively. Contrary to this, fistulas Type C and D showed no significant response after a medical intervention. CONCLUSION: The US evaluation seems to play an important role to define these important structures that will help the clinician in elaborating a personalized combined medical and surgical management of the HS patient.

Hidradenitis Suppurativa Associated with Down Syndrome Is Characterized by Early Age at Diagnosis.

BACKGROUND/AIMS: The association between hidradenitis suppurativa (HS) and multiple comorbidities has been widely investigated but data about the coexistence of Down syndrome (DS) are scarce. We sought to evaluate the prevalence of DS among a population of HS patients and assess their clinical features. METHODS: We collected demographic and clinical data of patients affected with HS referred to three Italian outpatient dermatology clinics. RESULTS: A total of 257 HS patients were enrolled, 62% females and 38% males (mean age [±SD]: 23.3 ± 10.7 years); 9 of the 257 patients (3.5%), 7 females and 2 males, had concomitant HS and DS. The patients with DS and HS had a significantly earlier age of onset (mean age: 14.3 ± 3.6 vs. 23.4 ± 12.31 years; p = 0.029), a significantly younger age at diagnosis (mean age: 21.1 ± 11.1 vs. 31.8 ± 13.5 years; p = 0.015), and were significantly younger (mean age: 23.3 ± 10.7 vs. 34.6 ± 13.07 years; p = 0.005). No significant differences about other clinical data were found between the two groups. CONCLUSION: The prevalence of DS in HS patients corresponds to a not negligible 3.5% of cases, who experienced an onset of HS at a younger age compared to patients with HS only.

The role of ovarian suspension in endometriosis surgery: a randomized controlled trial.

STUDY OBJECTIVE: A very high percentage of patients with severe pelvic endometriosis develop adhesions after laparoscopic surgery. The objective of this trial was to evaluate the role of ovarian suspension performed during surgery for severe endometriosis on ovarian adhesions and postoperative pelvic pain. DESIGN: A randomized controlled trial (Canadian Task Force classification I). SETTING: The tertiary care University Hospital of Bologna, Bologna, Italy. PATIENTS: Eighty patients with ovarian and posterior deep infiltrating endometriosis were included in the study. INTERVENTIONS: Patients underwent laparoscopic surgery for endometriosis and were randomized sequentially into 2 groups: transient ovarian suspension was performed in the treatment group (n = 40), whereas in the control group (n = 40) ovaries were left free in the pelvis. Symptom intensity (dysmenorrhea, chronic pelvic pain, dyspareunia, dyschezia, and dysuria) were ranked using a visual analog scale. Postsurgical ovarian adhesions were evaluated using transvaginal ultrasonographic scans performed by an ultrasound operator who was blinded to the details of the operative procedure and women's randomization allocation. Complications, lesion localization, endometrioma diameter, and surgery time were recorded. MEASUREMENTS AND MAIN RESULTS: At follow-up, a significantly lower rate of ultrasound-detectable ovarian adhesions with the uterus and the bowel was observed in the treatment group, respectively (46.7% vs 77.3%, p = .003 and 26.7% vs 68.2%, p < .0005). Patients in the control group showed a higher percentage of fixed ovaries with moderate and severe adhesions than the treatment group, respectively (56.8% vs 28.9%, p = .003 and 20.5% vs 8.9%, p = .110). No differences between the 2 groups were found regarding complications and pelvic pain. CONCLUSION: Ovarian suspension seems to be an additional effective surgical procedure associated with an increased ovarian mobility in women treated for severe endometriosis. Moreover, it is feasible, safe, simple, and fast. Hence, it should be routinely used during laparoscopic surgery for endometriosis.

Women with deep infiltrating endometriosis: sexual satisfaction, desire, orgasm, and pelvic problem interference with sex.

INTRODUCTION: Endometriosis is a chronic and progressive condition of women of reproductive age. It is strongly associated with a significant reduction of quality of life (QOL) and sexual function. AIMS: This study aims to objectively evaluate sexual function in women with deep infiltrating endometriosis (DIE) and to study the impact of endometriosis symptoms and type of lesion on patient's sexual function. METHODS: This is a cross-sectional study in a tertiary care university hospital. It included 182 patients with preoperative clinical and ultrasound diagnosis of DIE who were referred to our center from 2008 to 2011. MAIN OUTCOME MEASURES: A sexual activity questionnaire, the Sexual Health Outcomes in Women Questionnaire (SHOW-Q) was used to collect data pertaining to satisfaction, orgasm, desire, and pelvic problem interference with sex. Short Form 36 (SF-36) was used to evaluate QOL. Demographic and clinical characteristics were assessed: age, body mass index, parity, ethnicity, postsecondary education, employment, smoking, history of surgical treatment, and hormonal contraception. Patients were asked about pain symptoms (dysmenorrhea, dyspareunia, dyschezia, chronic pelvic pain, and dysuria) using a visual analog scale. RESULTS: The mean values obtained on the different scales of the SHOW-Q showed poor sexual function (mean SHOW-Q total score 56.38 ± 22.74). Satisfaction was the dimension most affected (mean satisfaction score 55.66 ± 34.55), followed by orgasm (mean orgasm score 56.90 ± 33.77). We found a significant correlation between the SF-36 scores and the SHOW-Q scores (P < 0.0001). Sexual dysfunction and deterioration of QOL seem to be correlated. Analyzing the impact of symptoms and lesions on sexual function, we found that dyspareunia and vaginal DIE nodules significantly affect sexual activity (P < 0.05). CONCLUSION: The results of this study demonstrated that women with DIE have a sexual function impairment, correlated with the overall well-being decrease. Moreover, the presence of dyspareunia and vaginal endometriotic lesions seems to be involved in sexual dysfunction.

Similar Levels of Efficacy of Two Different Maintenance Doses of Adalimumab on Clinical Severity and Quality of Life of Patients with Hidradenitis Suppurativa.

Adalimumab is the only biologic agent approved for the treatment of moderate-to-severe hidradenitis suppurativa (HS) patients (i.e., with Hurley II or III), which is recommended in two different maintenance doses (i.e., 40 mg weekly or 80 mg every two weeks). We conducted a prospective multicentric study to measure outcomes related to the severity of disease and quality of life (QoL) of patients affected by moderate-to-severe HS, treated with adalimumab at a maintenance dosing of 40 mg or 80 mg. Assessments were performed at baseline (T0) and after 32 weeks of treatment (T32). We enrolled 85 moderate-to-severe HS Italian patients, 43 men (50.6%) and 42 women, aged between 16 and 62 years (median 31 years, interquartile range 24.4-43.8). Statistically significant improvements were observed for clinical status (with a mean reduction of 7.1 points for the International Hidradenitis Suppurativa Severity Score System (IHS4)), pain levels (3.1 mean decrease in VAS), and QoL (3.4 mean improvement in DLQI score). Patients with no comorbidities, and those with higher levels of perceived pain showed significantly greater improvement in QoL than their counterpart from T0 to T32. As for the proportion of patients who at follow-up reached the minimal clinical important difference (MCID) in QoL, significantly higher proportions of success were observed for age (patients in the 29-39 category), pain (patients with higher reported pain), and Hurley stage III. While both treatment regimen groups (i.e., 40 vs. 80 mg) improved significantly, no statistical differences were observed when comparing the two treatment dosages.

Apremilast for the treatment of hidradenitis suppurativa associated with psoriatic arthritis in multimorbid patients: Case report and review of literature.

INTRODUCTION: Hidradenitis suppurativa is a complex, chronic, difficult to treat condition belonging to the spectrum of cutaneous immune-mediated inflammatory diseases. Systemic treatment options for moderate-severe disease are limited to TNF-alpha antagonists and other biologic agents, with limited clinical evidence. PATIENT CONCERNS: We report two adult patients with severe hidradenitis suppurativa presenting concomitant psoriatic arthritis and multiple medical comorbidities. Both were ineligible or resistant to adalimumab, the only biologic drug approved for the treatment of hidradenitis. DIAGNOSIS: Both patients were diagnosed with severe Hurley III-stage disease and psoriatic arthritis, showing resistance to first-line systemic treatments and a complex comorbidity profile. INTERVENTIONS: Patients underwent treatment with apremilast, an oral phosphodiesterase-4 inhibitor, approved for the treatment of psoriatic arthritis. OUTCOMES: After 16 weeks of treatment, a clinically relevant improvement of inflammatory lesions, skin- and arthritis-related pain, and patient-reported outcomes was achieved in both patients. Apremilast was well tolerated and continued up to 48 weeks of treatment. CONCLUSION: We report the "real-life" use of apremilast in the treatment of multimorbid patients with hidradenitis suppurativa and review its potential role in the management of this severe condition.

Prevalence of Neuropathic Pain and Related Characteristics in Hidradenitis Suppurativa: A Cross-Sectional Study.

BACKGROUND: Pain is a core symptom of hidradenitis suppurativa (HS) and is of complex, multifactorial origin. HS patients frequently report typical neuropathic pain qualities, but its prevalence has been poorly described. METHODS: In this cross-sectional study, we examine the prevalence of neuropathic pain (NP) component and related pain-characteristics of a hospital-based cohort of patients with symptomatic HS. We administered the pain-DETECT tool (PDQ), a validated screening tool for NP, collecting clinical and patient-reported data on pain, pruritus and pain-management. We obtained 110 complete datasets from symptomatic HS patients (49.1% females; Hurley I (27.3%])-II (45.5%)-III (27.3%)). According to the PDQ tool, 30% of patients were classified with a high probability (>90%) of neuropathic pain (LNP). LNP status was significantly associated with increased pain severity, disease activity, pruritus intensity and use of pain medication. Regression analysis showed a significant impact of the PDQ score on patient-reported outcomes, including pain severity and the dimensions of activity and affective pain interference. HS patients may present a mixed chronic pain phenotype with a neuropathic component, thus requiring additional pain-assessments. A multi-modal approach to pain management, in combination with disease-specific treatment, should be implemented in future interventional studies.

Characterization of comorbid conditions burdening hidradenitis suppurativa: a multicentric observational study.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic, disabling, inflammatory skin disorder that primarily affects the hair follicle localized at the apocrine-gland-bearing areas of the body, including axillary, inguinal, buttocks, and anogenital areas, and it may be associated with a wide array of comorbid conditions. This study aimed to described comorbid conditions affecting HS patients and to detect any correlation with disease severity. METHODS: Analyzing clinic database, we included all charts of patients visited at the HS outpatient clinic of three University Dermatologic Departments in order to describe demographic data, anthropometric measures, disease features, personal habits, clinical history, and presence of comorbidities. RESULTS: Two hundred thirty-four patients, mostly females (62%), were enrolled in this study. Based on Hurley staging classification 41% of patients were classified as Hurley Stage I, 43.0% as Hurley II, and 16% Hurley III, with a mean mSartorius Score value of 24.7 (SD: ±19.39) and a mean AISI score value of 12.5 (SD: ±11.93). The most frequently observed comorbidities were: obesity (26.1%), polycystic ovary syndrome (PCOS) (13.8% of the overall study population and 22.3% of females), hypertension (11.9%), dyslipidemia (9.9%), type II diabetes (9.5%), thyroid disorders (9.1%), nervous system disorders (7.1%), acne (6.7%), metabolic syndrome (4.4%), and Crohn's disease (3.6%). Obesity represented a key-comorbid condition increasing the likelihood of having more severe HS and PCOS (odds ratio 3.35 and 3.74, respectively). CONCLUSIONS: HS is associated with a variety of comorbid conditions that should be considered to perform targeted routine screening and to improve HS management.

Alexithymia affects patients with hidradenitis suppurativa.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease that impairs patients' quality of life. Various psychiatric or psychological conditions have been associated with HS, however, no data are available on alexithymia, a psychological construct characterized by the inability to express, describe, and recognize feelings. OBJECTIVES: To assess the presence of alexithymia in HS patients. MATERIALS & METHODS: Demographic and clinical data from patients with HS were collected. Alexithymia was assessed using the Toronto Alexithymia Scale (TAS)-20 questionnaire to define non-alexithymic subjects (scoring 20-50), borderline (possible) alexithymia subjects (scoring 51-60), and alexithymic subjects (scoring ≥61). The alexithymic personality trait is identified based on TAS-20 score ≥51. RESULTS: This multicentre study included 86 HS patients, 100 obese individuals, and 85 healthy control subjects. The mean TAS-20 score was significantly higher in the HS patient cohort (55.37 ±13.42) than in the control group (40.96±10.47) (p<0.001). Compared to the healthy and obese control groups, the prevalence of alexithymic personality trait in HS patients was 61.6% versus 21.95% and 32%, respectively (p<0.001). Of the HS patients, 37.2% were classified as alexithymic and 24.4% as borderline alexithymia. CONCLUSIONS: This is the first study in which an association between HS and alexithymia has been reported, expanding the spectrum of psychological disorders associated with HS.