[A girl with brownish grey macules on and around the lips]. / Een meisje met bruingrijze maculae op en rond de lippen.

A 14-year-old girl presented with brownish grey macules on and around the lips. Genetic testing revealed a mutation in the LKB1 tumor suppressor gene. The diagnosis made was Peutz-Jeghers syndrome, a rare inherited disease that is characterized by gastrointestinal polyps and an increased risk of cancer.

Surgical outcomes and the impact of major surgery on quality of life, activity impairment and sexual health in hidradenitis suppurativa patients: a prospective single centre study.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic debilitating skin disease, frequently located in the groin and anogenital area, leading to a substantial impact on quality of life and sexual health in patients with HS. Skin-tissue-sparing excision with electrosurgical peeling (STEEP) is a procedure with known low recurrence rates and high patient satisfaction in retrospective series. However, a prospective study to investigate the impact of any major surgery on specific aspects of the quality of life has not yet been performed. OBJECTIVE: To assess surgical outcomes and the effect of major surgery on the general quality of life, sexual health and activity impairment in patients with HS. MATERIALS AND METHODS: A single centre prospective survey study was conducted among 40 patients undergoing major surgery. Surveys were completed prior to the surgery and 2, 6, 12 and 26 weeks after surgery. Besides the objective parameters (time to wound closure and surface of the wound), patient-reported outcomes were reported. RESULTS: Thirty-nine patients with a total of 171 survey responses were included for analysis. Patients with Hurley stage I or II had a shorter time to wound closure (TTWC) compared to patients with Hurley stage III (P = 0.005). TTWC was significantly prolonged in patients treated with biologics (P < 0.001). Smoking did not significantly influence TTWC. For patient-reported outcomes, DLQI and ASEX scores did not significantly improve during the study period of 6 months. However, activity and overall work impairment showed considerable improvement after surgery. CONCLUSION: Time to wound closure is significantly prolonged by higher Hurley stage and treatment with biologics, contrastingly not by smoking. Major surgery improved the overall work and daily activity impairment.

Correlation of the refined Hurley classification for hidradenitis suppurativa with patient-reported quality of life and objective disease severity assessment.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic, debilitating, heterogeneous disease requiring different treatment approaches. Recently, we refined the classic Hurley classification into a seven-stage classification in order to guide these treatment choices. This new classification subdivides Hurley stage I and II into three substages, namely mild (A), moderate (B) and severe (C) HS disease. Hurley stage III is not subcategorized and is always severe. OBJECTIVES: To investigate the correlation between the given severity grades of Hurley I and Hurley II in the refined Hurley classification, and the patient-reported quality of life and physician-assessed objective severity score. MATERIALS AND METHODS: In this cross-sectional study, patients with HS participating in the observational cohorts of two Dutch tertiary referral centres were included before June 2017. The patient-reported Dermatology Life Quality Index (DLQI) and physician-assessed International HS Severity Score System (IHS4) scores were compared between the refined Hurley stages. RESULTS: In total, 433 patients were analysed. DLQI and IHS4 scores increased within Hurley stage I and II from A through C. There was a significant positive correlation of DLQI and IHS4 with increasing refined Hurley substages [refined Hurley stage I (A, B and C) to DLQI: rs = 0·259, P < 0·001 and refined Hurley stage II (A, B and C) to DLQI: rs = 0·185, P = 0·010; refined Hurley stage I (A, B and C) to IHS4: rs = 0·603, P < 0·001 and refined Hurley stage II (A, B and C) to IHS4: rs = 0·532, P < 0·001]. CONCLUSIONS: The refined Hurley classification accurately correlates with HS severity assessed by both patients and clinicians. Therefore, the refined Hurley classification is a useful tool for the quick assessment of severity in HS.

Sexual health and quality of life are impaired in hidradenitis suppurativa: a multicentre cross-sectional study.

BACKGROUND: Hidradenitis suppurativa (HS) has a major impact on patients' quality of life (QoL). Although it has commonly been assumed that HS impairs sexual health, only a single case-control study has been performed on sexual functioning in a small group of patients with HS. OBJECTIVES: To investigate the QoL with a particular focus on sexual health in a substantial population of patients with HS. METHODS: In total 916 patients with HS received an invitation to participate in this multicentre cross-sectional survey. RESULTS: Three hundred patients completed the questionnaires. This study showed a diminished QoL and sexual health in patients with HS (Female Sexual Function Index: 21·6 ± 9·6, International Index of Erectile Function: 49·7 ± 20·7, Arizona Sexual Experience Scale: 16·7 ± 5·3, Dermatology Life Quality Index: 12·5 ± 7·5). Sexual health was associated with QoL in women but not in men. Female sex and late onset of HS were associated with poor sexual function. Impairment of QoL was associated with anogenital involvement, early onset of HS, disease severity and disease activity. CONCLUSIONS: HS is associated with impaired sexual health and QoL. Physicians should not hesitate to ask patients with HS about their sexual function and, when needed, offer them psychological support.