Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease that severely impairs patients' quality of life. It is characterized by recurrent painful nodules, abscesses and draining sinus ...tracts in primarily intertriginous areas. We aimed to review the most up‐to‐date information regarding the epidemiology, clinical presentation, diagnostic studies, pathogenesis, comorbidities and quality of life of patients with hidradenitis suppurativa. We performed a systematic search of Medline, Embase database (from inception to September 2019) and review of bibliographies without restrictions on year or language. HS has an estimated global prevalence of 0.00033–4.1% (but most likely 0.7–1.2% in the European‐US population). Patients still experience a significant diagnostic delay, up to several years. In the absence of pathognomonic tests, the diagnosis of HS is made from clinical observation and the disease narrative. Phenotypic variation renders diagnosis and severity assessment difficult. Ultrasound imaging is an emerging assessment tool for deep‐seated lesions. The Hurley Staging System is still widely used in severity rating. Follicular hyperkeratosis and dilatation, follicular rupture and chronic inflammation with architectural tissue changes have been implicated in the pathogenesis of HS. HS has been associated with metabolic syndrome and other risk factors for cardiovascular disease, diabetes mellitus type II, polycystic ovarian syndrome, depression, suicide and substance use disorders. It has been linked to other immune‐mediated diseases such as inflammatory bowel disease and spondyloarthropathy. Pain, pruritus, malodour, low self‐esteem, sleep and sexual dysfunctions, and poor mental health are chronic symptoms or consequences of uncontrolled disease. HS is an under‐diagnosed and under‐treated disease with a profound negative impact on patients' quality of life. In the light of its associated comorbidities, an interdisciplinary management approach may be needed to ensure the best outcomes.
Summary
During the 2011 International Pigment Cell Conference (IPCC), the Vitiligo European Taskforce (VETF) convened a consensus conference on issues of global importance for vitiligo clinical ...research. As suggested by an international panel of experts, the conference focused on four topics: classification and nomenclature; definition of stable disease; definition of Koebner’s phenomenon (KP); and ‘autoimmune vitiligo’. These topics were discussed in seven working groups representing different geographical regions. A consensus emerged that segmental vitiligo be classified separately from all other forms of vitiligo and that the term ‘vitiligo’ be used as an umbrella term for all non‐segmental forms of vitiligo, including ‘mixed vitiligo’ in which segmental and non‐segmental vitiligo are combined and which is considered a subgroup of vitiligo. Further, the conference recommends that disease stability be best assessed based on the stability of individual lesions rather than the overall stability of the disease as the latter is difficult to define precisely and reliably. The conference also endorsed the classification of KP for vitiligo as proposed by the VETF (history based, clinical observation based, or experimentally induced). Lastly, the conference agreed that ‘autoimmune vitiligo’ should not be used as a separate classification as published evidence indicates that the pathophysiology of all forms of vitiligo likely involves autoimmune or inflammatory mechanisms.
Summary
Background
Visible light (VL) induces multiple cutaneous effects. Sunscreen testing protocols recommended by regulatory bodies throughout the world require the use of solar simulators with ...spectral output in the ultraviolet (UV) domain only. However, sunlight contains VL and infrared radiation also.
Objectives
This study aimed to evaluate the contributions of VL and UVA on pigmentation and erythema, and optimize parameters for in vivo testing.
Methods
Ten subjects with Fitzpatrick skin phototype IV–VI were enrolled. Subjects were irradiated on their back with VL using two light sources: one containing pure VL and one containing VL with less than 0·5% UVA1 (VL+UVA1). Four different irradiances were administered to investigate reciprocity behaviour. Assessments, including photography, Investigator's Global Assessment, colorimetry and spectroscopy, were performed immediately, 24 h, 7 days and 14 days post‐irradiation.
Results
Pigmentation was observed with both light sources; however, pigment intensity was greater with VL+UVA1 than with pure VL. Reciprocity was observed in pure VL sites, but not VL+UVA1. Variation in spectral output had greater impact on pigment intensity than irradiance. Clinical erythema was observed on the VL+UVA1 side, but not on the pure VL side. A protocol for testing photoprotection product efficacy against VL‐induced effects has been proposed.
Conclusions
The findings suggest a synergistic relationship between VL and UVA1 and emphasize the need for developing means of photoprotection against VL.
What's already known about this topic?
Visible light (400–700 nm) (VL) induces dark and persistent pigmentation, erythema, DNA damage secondary to free radical production and exacerbation of photodermatoses.
In the U.S.A., sunscreens with critical wavelength (wavelength with 90% of area under the absorbance spectra when integrating from 290 nm to 400 nm) ≥ 370 nm can claim broad‐spectrum photoprotection.
Criteria in the European Union and Australia require the ratio of sun protection factor to ultraviolet A (UVA) protection factor to be ≤ 3 : 1.
What does this study add?
The findings demonstrate a synergistic relationship between VL and long‐wavelength UVA1 (370–700 nm).
These effects emphasize the need for photoprotection against this part of the solar spectrum.
A protocol for testing photoprotection product efficacy against VL‐induced effects has also been proposed.
Respond to this article
Plain language summary available online
Summary
Hidradenitis suppurativa (HS) is a chronic, inflammatory, recurrent and debilitating skin disease of the hair follicle unit that typically develops after puberty. The disorder is ...characterized by comedones, painful inflammatory nodules, abscesses, dermal tunnels and scarring, with a predilection for intertriginous areas of the body (axillae, inguinal and anogenital regions). Recruitment of neutrophils to HS lesion sites may play an essential role in the development of the painful inflammatory nodules and abscesses that characterize the disease. This is a review of the major mediators involved in the recruitment of neutrophils to sites of active inflammation, including bacterial components (endotoxins, exotoxins, capsule fragments, etc.), the complement pathway anaphylatoxins C3a and C5a, tumour necrosis factor‐alpha, interleukin (IL)‐17, IL‐8 (CXCL8), IL‐36, IL‐1, lipocalin‐2, leukotriene B4, platelet‐activating factor, kallikreins, matrix metalloproteinases, and myeloperoxidase inhibitors. Pharmacological manipulation of the various pathways involved in the process of neutrophil recruitment and activation could allow for successful control and stabilization of HS lesions and the remission of active, severe flares.
Summary
Background
There is no cure or firm clinical recommendations for the treatment of vitiligo. One of the main issues is the heterogeneity of outcome measures used in randomized controlled ...trials for vitiligo.
Objectives
To define successful repigmentation from the patients’ point of view and to propose how and when repigmentation should be evaluated in clinical trials in vitiligo.
Methods
We conducted three workshops with patients with vitiligo and their parents or caregivers. Workshop 1 was held at World Vitiligo Day (Detroit, MI), workshop 2 at the University of Texas Southwestern Medical Center and workshop 3 at the Vitiligo and Pigmentation Institute of Southern California, University of California.
Results
Seventy‐three participants were recruited. Consensus on the following questions was achieved unanimously: (i) the definition of ‘successful repigmentation’ was 80–100% of repigmentation of a target lesion and (ii) both an objective and a subjective scale to measure repigmentation should be used.
Conclusions
This was the largest patients’ outcomes workshop. We followed the guidance from the CSG‐COUSIN and the Vitiligo Global Issues Consensus Group. Our recommendations to use percentage of repigmentation quartiles (0–25%, 26–50%, 51–79%, 80–100%) and the Vitiligo Noticeability Scale are based on the best available current evidence. A limitation of the research is that the workshops were conducted only in the U.S.A., due to pre‐existing organisational support and the availability of funding.
What's already known about this topic?
There are no firm clinical recommendations for the treatment of vitiligo.
One of the main issues is the heterogeneity of outcome measures used in randomized controlled trials for vitiligo.
What does this study add?
Based on international consensus and the best available evidence, repigmentation should be assessed by measuring the percentage of repigmentation in quartiles (0–25%, 26–50%, 51–79%, 80–100%).
The cosmetic acceptability of results should also be assessed, for example using the Vitiligo Noticeability Scale.
Respond to this article
Linked Comment: Wolkerstorfer. Br J Dermatol 2019; 180:454–455.
Plain language summary available online
PDF
