Case report: A rare case of imiquimod-induced atypical pemphigus vulgaris.

Background: Pemphigus vulgaris is an autoimmune intraepithelial bullous disease involving the skin and the mucous membranes. Imiquimod, a topical therapy for skin basal cell carcinoma, is an amine that induces the production of tumor necrosis factor alfa, interleukin-1 and other cytokines. Pemphigus induced by drugs has been frequently reported, mostly after systemic therapy. Case presentation: We present the case of a 50-year-old man who developed skin, intraoral, and genital mucosae lesions 3 days after a treatment with Imiquimod for multiple superficial basal cell carcinoma of the trunk. Direct and indirect immunofluorescence results were compatible with the diagnosis of pemphigus vulgaris. Enzyme-linked immunosorbent assay was negative for desmoglein 1 and 3, but interestingly, by immunoblotting on keratinocyte extracts a band of 170 kDa was obtained by IgG. The patient, after interrupting Imiquimod application, started a treatment with prednisolone and in 4 weeks showed a complete remission. Conclusion: Topical Imiquimod therapy might induce atypical pemphigus vulgaris in some patients.

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.

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.

Pain as Defining Feature of Health Status and Prominent Therapeutic Target in Patients with Hidradenitis Suppurativa.

BACKGROUND: Pain is one of the main aspects of hidradenitis suppurativa that strongly affects the quality of life of patients. We explored the relationship between pain and clinical severity as well as its role in defining the health status in patients with HS. METHODS: Pain was defined by three measures: (a) question 1 ("my skin hurts") of the Skindex-17; (b) Bodily Pain (BP) scale of the SF-36; and (c) Visual Analog Scale (VAS). Clinical severity of HS was assessed by the Hurley staging, the Sartorius HS Score, and the International HS Severity Score System. RESULTS: The study population included 341 HS patients with complete data for the VAS pain, 316 for question 1 of the Skindex-17, and 294 for BP. Clinical severity was positively associated with pain. This result was observed for all three severity scores and all three pain evaluation methods. In addition, the number of fistulae, abscesses, and nodules were significantly associated with the three severity measures of pain, while the association with scars was not observed for question 1 of the Skindex-17 and BP. CONCLUSIONS: Pain may be a good proxy of clinical severity and efficacy of a treatment in HS and therefore a crucial hallmark of patients' health status.

Alexithymia, Psychological Distress, and Social Impairment in Patients with Hidradenitis Suppurativa.

BACKGROUND: Hidradenitis suppurativa (HS) is a rare, chronic, inflammatory skin disease characterized by deep-seated nodules, abscesses, and draining fistulas. HS has a substantial adverse impact on patients' lives. Only a few studies investigated the relationship between health-related quality of life, psychological distress, and emotional dysregulation in patients with HS. Alexithymia, namely the difficulty in describing or recognizing emotions, has been associated with various psychological disorders, such as anxiety, depression, and psychological distress. OBJECTIVE: The aim of this study was to examine the prevalence of alexithymia in patients with HS and its association with demographic and clinical variables, quality of life indices, and psychological distress. METHODS: Ninety outpatients with HS completed the 20-item Toronto Alexithymia Scale, the 12-item General Health Questionnaire (GHQ-12), the Dermatology Life Quality Index, the Skindex-17, and the 36-Item Short-Form Health Survey. Information on sociodemographic and clinical variables was retrieved from clinical records. RESULTS: Alexithymia or borderline alexithymia was observed in 44.4% of patients with HS, with a higher prevalence of the alexithymic trait in women than in men (51.7 vs. 31.2%). We did not find any association between alexithymia and clinical variables. Of the entire sample analyzed, 46.1% reported high psychological distress; among them, 78% reported alexithymia or borderline alexithymia compared to 16.7% among GHQ noncases. Furthermore, HS patients with alexithymia or borderline alexithymia showed significantly higher scores on the Skindex-17 psychosocial scale and the Dermatology Life Quality Index, and a lower score on the mental component of the 36-item Short-Form Health Survey, than nonalexithymic patients. CONCLUSIONS: Dermatologists should consider alexithymia in the diagnosis and treatment of HS patients, given its important role in psychological and psychosocial distress.

Age-specific physical and mental health status in Italian patients with hidradenitis suppurativa: a comparison with the general population.

Hidradenitis suppurativa (HS) has a considerable impact on quality of life. Despite comparisons of health status between HS patients and the general population have been done, there is a need to make more meaningful comparisons, such as age-specific ones. The objective of the study was to compare age-specific physical and mental health status in Italian patients with HS to that of a representative sample of the Italian general population. We conducted a cross-sectional, observational study on consecutive HS patients. Patients' health status was measured using the 36-item Short Form Health Survey (SF-36) and in the general population using the 12-item version (SF-12). The mean scores of the physical component summary (PCS) and the mental component summary (MCS) from the two instruments were graphically compared in different age categories according to sex and clinical severity. Data were complete for 298 HS patients. Young patients with HS reported a physical and mental health status similar to that of elderly people from the general population. Even HS patients with mild-to-moderate clinical severity had a consistently worse health status than the reference population. In conclusion, HS deeply affects patients' physical and mental status, starting from a very young age. An early intervention is advisable, not only with pharmacological treatment, but also with an appropriate discussion and interaction with the patient to focus on the aspects that would reduce the psychosocial impact of their condition.

Correlation between Depression, Quality of Life and Clinical Severity in Patients with Hidradenitis Suppurativa.

Depression is frequent in patients with hidradenitis suppurativa. However, its relationship with quality of life and clinical severity needs further investigation. In this cross-sectional study, 341 adult, consecutive patients with hidradenitis suppurativa completed the 12-item General Health Questionnaire (GHQ-12), which has been shown to be able to identify cases of major depressive disorder in dermatological patients. The frequency of depression in hidradenitis suppurativa patients was 29.0%. In patients with depression, severity (International Hidradenitis Suppurativa Severity Score System (IHS4)), quality of life (Skindex-17; Dermatology Life Quality Index (DLQI)), and health status (36-item Short Form Health Survey (SF-36)) were significantly worse compared with patients with no depression. The highest linear correlation was observed between GHQ-12 and the psychosocial scale of the Skindex-17 and the SF-36 mental scale. In contrast, correlation between GHQ-12 and clinical severity was poor. Depression is an important comorbidity in hidradenitis suppurativa, which is strongly associated with impairment in quality of life, but not linearly correlated with clinical severity.