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The role of biologic treatment in special scenarios in hidradenitis suppurativa: Facial and nape phenotype, dissecting cellulitis of the scalp, and lymphedema

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Dermatologic Therapy
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Background: Hidradenitis suppurativa (HS) is a chronic inflammatory disorder affecting the hair follicle which typically affects the axilla, inguinal, sub-mammary and perianal areas. Atypical HS, such as facial HS, dissecting cellulitis of the scalp (DCS), nape HS and HS lymphedema has also been described. There is scarce evidence about the treatment of atypical HS. Aims: To describe the efficacy of biologic drugs and adjuvant therapeutic interventions used in patients with atypical HS who had an inadequate response to systemic antibiotics. Methods: Prospective case series study of patients with atypical HS (facial HS, DCS, nape HS and HS lymphedema) treated with biologic drugs in a HS Clinic setting. Disease activity indexes, socio-demographic, clinical and safety variables were collected. Results: Two patients met criteria for Facial HS, three met criteria for DCS / nape HS and three patients met criteria for HS lymphedema. Patients with facial HS achieved rapid improvement without requiring other therapies. Patients with DCS, nape HS and HS lymphedema showed variable response, with decrease of activity indexes, and requiring adjuvant treatments. Conclusions: Biologic drugs are useful in the management of special HS locations. In the case of facial HS, biologic therapy seems to be quite effective as monotherapy. In the cases of DCS, HS lymphedema and nape HS, treatment combination or intensification may be needed. This article is protected by copyright. All rights reserved.
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SHORT PAPER
The role of biologic treatment in special scenarios in
hidradenitis suppurativa: Facial and nape phenotype, dissecting
cellulitis of the scalp, and lymphedema
Manuel Sanchez-Diaz
1
| Antonio Martinez-Lopez
1,2
| Luis Salvador-Rodriguez
1
|
Trinidad Montero-Vilchez
1
| Salvador Arias-Santiago
1,2
| Alejandro Molina-Leyva
1,2,3
1
Hidradenitis Suppurativa Clinic, Department
of Dermatology, Hospital Universitario Virgen
de las Nieves, Granada, Spain
2
European Hidradenitis Suppurativa
Foundation (EHSF), Dessau-Roßlau, Germany
3
Instituto de Investigación Biosanitaria,
Granada, Spain
Correspondence
Salvador Arias-Santiago, Dermatology
Department, Hospital Universitario Virgen de
las Nieves, Avenida de las Fuerzas Armadas
2, Granada, Spain.
Email: salvadorarias@ugr.es
Abstract
Hidradenitis suppurativa (HS) is a chronic inflammatory disorder affecting the hair
follicle which typically affects the axilla, inguinal, submammary, and perianal areas.
Atypical HS, such as facial HS, dissecting cellulitis of the scalp (DCS), nape HS, and
HS lymphedema has also been described. There is scarce evidence about the treat-
ment of atypical HS. To describe the efficacy of biologic drugs and adjuvant thera-
peutic interventions used in patients with atypical HS who had an inadequate
response to systemic antibiotics. Prospective case series study of patients with atypi-
cal HS (facial HS, DCS, nape HS, and HS lymphedema) treated with biologic drugs in
a HS Clinic setting. Disease activity indexes, sociodemographic, clinical, and safety
variables were collected. Two patients met criteria for Facial HS, three met criteria
for DCS/nape HS and three patients met criteria for HS lymphedema. Patients with
facial HS achieved rapid improvement without requiring other therapies. Patients
with DCS, nape HS, and HS lymphedema showed variable response, with decrease of
activity indexes, and requiring adjuvant treatments. Biologic drugs are useful in the
management of special HS locations. In the case of facial HS, biologic therapy seems
to be quite effective as monotherapy. In the cases of DCS, HS lymphedema and nape
HS, treatment combination or intensification may be needed.
KEYWORDS
hidradenitis suppurativa, inflammatory disorders, pharmacology, therapy-systemic
1|BACKGROUND
Hidradenitis suppurativa (HS) is a chronic inflammatory disorder
affecting the hair follicle whose lesions cause pain, suppuration and
malodor and may significantly impair quality of life.
1
Different classifi-
cations have been proposed based on the phenotypes of the disease.
2
Dissecting cellulitis of the scalp (DCS), facial HS, nape HS, and HS
lymphedema are examples of atypical HS presentations. Its manage-
ment is generally complex, showing variable response to therapies,
with the need for biologic treatments in a high percentage of cases.
3-6
The aim of this study is to identify those patients with atypical HS
who have an inadequate response to first-line antibiotics within a
cohort of patients with HS, and to describe the efficacy of the biologic
therapy and adjuvant therapeutic interventions used.
2|METHODS
A prospective case series study of consecutive patients with atypical
HS treated at the Hidradenitis Suppurativa Unit of the Virgen de las
Nieves University Hospital from December 2016 to March 2020. This
study was approved by the Institutional Review Board of the hospital.
Received: 15 November 2020 Revised: 21 January 2021 Accepted: 23 January 2021
DOI: 10.1111/dth.14829
Dermatologic Therapy. 2021;34:e14829. wileyonlinelibrary.com/journal/dth © 2021 Wiley Periodicals LLC. 1of9
https://doi.org/10.1111/dth.14829
... Adalimumab therapy has been depicted to be an effective treatment for DCS (PCAS) with appealing clinical outcomes [43]. A prompt and substantial improvement in clinical condition has emerged in several patients; some were also affected by other concurrent dermatological disorders [44][45][46][47][48][49][50][51][52][53][54][55][56][57]. Additionally, hair regrowth was successfully detected in a group of patients [46,55,56,[58][59][60]. ...
... Additionally, hair regrowth was successfully detected in a group of patients [46,55,56,[58][59][60]. Conversely, some patients exhibited no alteration in the progression of alopecia [48], did not manifest a favorable clinical response [38], experienced secondary treatment failure [53], and, in a few cases, even demonstrated an exacerbation of fibrosis and cicatrization, along with no discernible change in residual pathological structures [52]. The only reported AE was a reversible dose-dependent change in laboratory test results in one patient [56]. ...
... In the patient with secondary failure of adalimumab therapy, switching to infliximab stabled the disease after 16 weeks [53]. Two other patients receiving infliximab therapy for DCS (PCAS) had a similar clinical response. ...
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Dissecting cellulitis of the scalp (DCS) is a chronic inflammatory skin condition characterized by abscesses, nodules, fistulas, and scarring alopecia. Management of this oftentimes debilitating dermatosis can be challenging due to its recalcitrant nature. There is limited data regarding the efficacy of treatment options for DCS. The aim of this study was to conduct a systematic review of the literature to explore the efficacy and safety of reported DCS treatments. In October 2022, MEDLINE and EMBASE databases were searched for articles on treatments for DCS. Studies that contained outcome efficacy data for DCS treatments were included. Reviews, conference abstracts, meta-analyses, commentaries, non-relevant articles, and articles with no full-text available were excluded. Data extraction was performed by two independent reviewers. A total of 110 relevant articles with 417 patients were identified. A majority of studies (86.4%) were case reports or series. Treatment options included systemic antibiotics, oral retinoids, biologics, procedural treatments, combination agents, and topical treatments. Oral retinoids and photodynamic therapy were the most extensively studied medical and procedural interventions, respectively. Overall, randomized controlled trials are needed to evaluate various treatment regimens for DCS and provide patients with a robust, evidence-based approach to therapy.
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Hidradenitis suppurativa (HS) is a chronic, inflammatory skin condition that has been insufficiently studied in the pediatric population. Timely and effective medical treatments may improve quality of life, mitigate disease burden, and prevent the need for invasive procedural interventions such as surgical excisions. However, there is a paucity of research on the efficacy of medical management strategies for HS in children and adolescents. The aim of this study was to perform a systematic review of the literature on the efficacy and safety of medical treatments for HS in patients <18 years of age. In April 2022, MEDLINE and EMBASE databases were searched for articles on the efficacy of medical treatments for HS in the pediatric population. Between 1984 and 2022, 35 articles (101 patients) met the inclusion criteria. Most patients had Hurley Stage II disease (46.7%, 35/75) followed by Stage I (36%, 27/75), and Stage III (17.3%, 13/75). 100% (23/23) of patients responded to antibiotics, 100% (8/8) to finasteride, 93.9% (31/33) to biologics, 80% (4/5) to oral retinoids, and 50% (6/12) to metformin. Overall, this study demonstrates that medical treatment regimens can improve HS symptoms in pediatric patients, but the extent of improvement is unclear, and the results were largely based on case reports or case series. Prospective studies are warranted to better understand the efficacy and safety of medical treatments for pediatric HS. Clinical trials of HS therapies need to be inclusive of pediatric patients to help define the optimal timing of treatment initiation and guide patient selection.
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Background: Hidradenitis suppurativa (HS) is a chronic inflammatory disease of the hair follicle which presents with painful nodules, abscesses, and fistulae in apocrine gland-bearing areas of the skin. Approved treatments include antibiotics and biologic drugs such as adalimumab. Despite these treatments, HS management is challenging. Acitretin is an oral retinoid used for its management as 3rd or 4th line therapy. There is little evidence regarding the effectiveness and safety of acitretin treatment for HS, and no reports have previously explored the potential clinical predictors associated with the response to the treatment. Methods: Retrospective cohort study to assess the effectiveness and safety of acitretin treatment in HS patients who failed to respond to topical therapies. Results: Sixty-two patients with moderate to severe HS were included. A significant decrease in the International HS Severity Scoring System (IHS4) score was found over time. Higher basal IHS4 score, family history of HS, follicular phenotype, and history of follicular plugging conditions were potential predictors of response. Most patients did not suffer any adverse events, and no severe side effects were observed. The main cause of discontinuation was lack of efficacy. Conclusion: Acitretin can be considered as a therapeutic option for patients with HS. The presence of follicular phenotype or a history of components of follicular occlusion syndrome is associated with better outcomes.
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Background Hidradenitis suppurativa (HS) is a chronic, inflammatory and recurrent skin disease. Different staging instruments have been suggested, but none has achieved universal acceptance. Despite the fact that the Hurley staging is one of the most widely applied HS disease severity staging instruments, it has not been validated. Objective To determine the inter‐ and intrarater reliability of the Hurley staging. Methods Fifteen raters (5 plastic surgeons, 5 general surgeons and 5 dermatologists) independently staged 30 photos of HS patients according to the Hurley staging at T1 and T2. Reliability was assessed using kappa statistics and multivariable logistic regressions were used to determine independent risk factors for photos with discordant staging. Results Interrater reliability was moderate for the three stages of HS (κ = 0·59; 95% CI, 0·48‐0·70). It was moderate for Hurley stage I (κ = 0·45; 95% CI, 0·32‐0·55) and stage II (κ = 0·51; 95% CI, 0·31‐0·71), and it was almost perfect for stage III (κ = 0·81; 95% CI, 0·62‐1·00). The intrarater reliability was substantial for all stages and all raters (κ = 0·65; 95% CI, 0·58‐0·72). For stage I it was moderate (κ = 0·50; 95% CI, 0·38‐0·62), for stage II it was substantial (κ = 0·62; 95% CI, 0·51‐0·73) and for stage III it was almost perfect (κ = 0·82; 95% CI, 0·77‐0·87). Hurley stage II and III were less likely to result in discordant staging compared to Hurley stage I (OR 0·47, 95% CI 0·29‐0·77 and OR 0·21, 95% CI 0·12‐0·38 respectively). The mean time spent on staging a photo was 14 seconds. Conclusions The Hurley staging is reliable for rapid severity assessment of HS, with a moderate interrater and substantial intrarater reliability for all stages. It is best for assessing Hurley stage III, which is an indication for surgery. This article is protected by copyright. All rights reserved.
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Hidradenitis suppurativa (HS)/acne inversa is a debilitating chronic disease that remains poorly understood and difficult to manage. Clinical practice is variable and there is a need for international, evidence‐based and easily applicable consensus on HS management. We report here the findings of a systematic literature review, which were subsequently used as a basis for the development of international consensus recommendations for the management of patients with HS. A systematic literature review was performed for each of nine clinical questions in HS (defined by an expert steering committee), covering comorbidity assessment, therapy (medical, surgical and combinations), and response to treatment. Included articles underwent data extraction and were graded according to the Oxford Centre for Evidence‐based Medicine criteria. Evidence‐based recommendations were then drafted, refined, and voted upon, using a modified Delphi process. Overall, 5,310 articles were screened, 171 articles analyzed, and 65 used to derive recommendations. These articles included six randomized controlled trials plus cohort studies and case series. The highest level of evidence concerned dosing recommendations for topical clindamycin in mild disease (with systemic tetracyclines for more frequent/widespread lesions) and biologic therapy (especially adalimumab) as second‐line agents (following conventional therapy failure). Good‐quality evidence was available for the hidradenitis suppurativa clinical response (HiSCR) as a dichotomous outcome measure in inflammatory areas under treatment. Lower‐level evidence supported recommendations for topical triclosan and oral zinc in mild‐to‐moderate HS, systemic clindamycin and rifampicin in moderate HS and intravenous ertapenem in selected patients with more severe disease. Intralesional or systemic steroids may also be considered. Local surgical excision is suggested for mild‐to‐moderate HS, with wide excision for more extensive disease. Despite a paucity of good quality data on management decisions in HS, this systematic review has enabled the development of robust and easily applicable clinical recommendations for international physicians based on graded evidence. This article is protected by copyright. All rights reserved.
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Background: It has been proposed that two main phenotypes of hidradenitis suppurativa (HS) exist. This proposal is based upon different elementary structures detected in the skin, namely follicular subtypes and inflammatory subtypes. Having an accurate definition of these two variants could help us to better identify patients who may require an early intervention with currently approved targeted immunomodulatory therapies. Objective: To define and distinguish between the epidemiological, clinical and analytic characteristics of these two HS phenotypes. Methods: An observational, descriptive, non-randomized and prospective study was conducted. Patients diagnosed with HS between May 2012 and April 2017 by a specialized unit were included. Ultrasound evaluation was performed in all cases. Results: About 197 patients were included, 100 women and 97 men, aged between 25 and 47 years. The mean age of onset was significantly different between phenotypes, ranging between 26.69 ± 9.05 in the inflammatory subtype and 17.62 ± 6.42 in the follicular subtype. Follicular subtype patients exhibited a significantly higher number of nodules combined with the presence of multiple commedons (5.65 ± 3.38 versus 0.89 ± 2.72). This contrasted with the higher count of abscesses and fistulas detected in the inflammatory subtype (respectively, 4 ± 2.74 and 3.11 ± 2.56 versus 0.56 ± 1.02 and 0.26 ± 0.56). IgA levels were significantly higher in the inflammatory subtype (497.71 ± 262.26 versus 232.38 ± 84.06). Mean IHS4 score evaluation was higher in the inflammatory subtype (21.04 ± 11.9) compared with the follicular phenotype (7.54 ± 4.66). The inflammatory subtype was found to be an independent risk factor for disease aggressiveness in the multivariate analysis (OR 0.034 [95% CI 0.015-0.072]). Limitations: Small sample size. Conclusion: Preliminary data suggest the existence of an inflammatory HS phenotype that is associated with higher aggressiveness and major risk of progression during natural history of the disease.
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Background: Patients with hidradenitis suppurativa (HS) suffer from symptoms such as pruritus and malodour which can significantly impair their quality of life. Objectives: (1) To analyse the impact of pruritus and malodour on the quality of life of patients with HS and (2) to explore the potential association between clinical features and the severity of these symptoms. Patients and methods: This is a cross-sectional study. The Numeric Rating Scale (NRS) was used to assess pruritus and malodour. Quality of life was assessed by means of the Dermatology Life Quality Index (DLQI). Results: Two hundred and thirty-three patients were included in the study. Both pruritus and malodour positively correlated with worse quality of life (p < 0.05). Pruritus intensity was associated with the number of regions affected by HS, female sex, the intensity of suppuration, and the presence of comorbid Crohn's disease. Statin use was associated with lower levels of pruritus. Malodour intensity was associated with higher body mass index, disease duration, the number of regions affected, Hurley stage, and intensity of suppuration. Conclusions: The results of our study show that pruritus and malodour are key symptoms in patients with HS which have a great impact on their quality of life. We have identified clinical features potentially associated with the intensity of these symptoms which could be useful to identify higher-risk patients and may influence treatment decisions.
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Lymphedema is the result of an alteration of the lymphatic drainage, and its most common worldwide cause is filariasis. In our practice usually is associated to neoplasic, inflammatory and granulomatous processes, radiotherapy, hydroelectrolytic disbalances, and idiopathic. It can affect any part of the body, including the penis and scrotum. The genital lymphedema is a rare presentation, it corresponds to 0.6% of lymphedema. However, causes serious functional, social and emotional limitations for the patient. Too often have pain, recurrent infections, sexual dysfunction, cosmetic deformity, sometimes it limits mobility and ambulation. Although there are several treatment options, both medical and surgical, it has not been found ideal for this disease. We present a 43 years old patient with penoscrotal lymphedema due to hidradenitis suppurativa, it limits his normal activity. The patient was referred to our center after unsuccessful medical treatment (doxycycline and clindamycin cycles). Surgical treatment consisted of total excision of the skin and subcutaneous tissue to Buck's fascia. Split thickness skin grafts were used to cover the defect. The result was satisfactory both functionally and aesthetically. Copyright: © 2019 Permanyer.
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We report eight cases of patients with severe acne who were treated with Isotretinoin and developed painful nodules in their axillae and groin consistent with Hidradenitis Suppurativa (HS). The pathogenesis of HS is still not completely understood; recent research from a study in 2011 of biopsies from HS lesions showed a reduction or absence of sebaceous glands compared to normal skin in HS patients and the author suggested this contributes to the pathogenesis of the disease. Interestingly the main effect of Isotretinoin is to decrease the size and action of sebaceous glands; so hypothetically, as Isotretinoin acts by reducing the sebaceous glands further it could potentially aggravate the condition. Our experience has instilled caution in our prescribing of Isotretinoin; patients are questioned about symptoms of HS prior to and during treatment, particularly patients with acne conglobata. This article is protected by copyright. All rights reserved.
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Some patients with hidradenitis suppurativa (HS) develop severe inflammatory lesions of the nape. Through a single‐center, cross‐sectional study with a total of 377 patients, we sought to compare patients with and without nape involvement, to determine whether disease severity is greater in these patients and to describe their clinical characteristics. Thirty patients (90% male) were identified as having nape involvement. Patients with versus without nape involvement had greater disease severity, earlier disease onset, a family history of HS, lower body mass, and higher Dermatological Life Quality Index and pain scores. Amongst them, involvement of the trunk and gluteal regions predominated. A significant univariate relationship existed between patients with nape involvement and male sex, early disease onset, family history, scalp and gluteal involvement, Canoui‐Poitrine phenotypes II and III, and Dowling–Degos disease. A significant multivariate relationship existed with early disease onset, Dowling–Degos disease, and phenotypes II and III.
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Background: Isotretinoin is the most potent treatment for acne but paradoxical flares can occur. HS lesions of the face may be mistaken for acne. We report on 4 patients in whom an "acne" flare on isotretinoin led to the correct diagnosis of HS. Patients and methods: Four young male patients aged 15 to 28 years were referred to us because of an acne flare on isotretinoin. Three of them had clinical features considered unusual in acne : involvement of the nape of the neck (2), retroauricular cysts (2), a rope-like pre-sternal lesion (1), a large bag-like sinus on the face (1), large deep depressed U-type scars on the back (3). Questioning revealed that all three had previously experienced several attacks of inflammatory nodules in the axillae and/or inguinal folds; they had not mentioned these lesions since they seemed so minor. The fourth patient had lesions typical of HS comprising nodules, sinus, rope-like hypertrophic scars on the face mistaken for acne, epidermal cysts on the scrotum and pubic folliculitis. Treatment with systemic antibiotics resulted in regression of lesions in all 4 patients. Discussion: An acne flare on isotretinoin requires investigations with a view to potential diagnosis of HS. Patients presenting "acne" and atypical features such as involvement of the neck, large U scars and cord-like structures should be questioned about the presence of nodules in the axillae and groin since patients with mild HS may not spontaneously acknowledge such typical symptoms.
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Background Lymphedema is a debilitating complication of hidradenitis suppurativa (HS) that can be therapeutically challenging. Systematic evaluation of the clinical presentation and management of this condition has yet to be performed. Methods OVID MEDLINE and EMBASE databases were searched along with a review of the references of searched articles for all English reports discussing lymphedema secondary to HS. Results Two case series and 15 case reports met inclusion criteria, representing 27 patients. On average, these patients had a history of HS for 18 years (range 4–30 years). Lymphedema was found in the following areas in order of frequency: scrotum (16 patients, 59%), penis (12 patients, 44%), labia majora (4 patients, 15%), perineum (3 patients, 11%), groin (3 patients, 11%), buttocks (2 patients, 7%), and abdomen (1 patient, 4%). In six patients (22%), lymphedema occurred in two or more of the aforementioned locations. Lymphedema most commonly presented not only as swelling with induration but also as verrucous papules or nodules. The majority of cases (14 patients, 52%) were treated with radical surgical excision combined with medical therapy and obtained favorable outcomes. Surgical defect was most frequently covered with split thickness skin grafts 7 patients, 54%) followed by skin flaps (3 patients, 11%) and healing by secondary intention (3 patients, 11%). Conclusions Lymphedema appears to be a relatively rare complication of chronic HS that most commonly affects the anogenital region. Based on the evidence available, dermatologists should pursue a collaborative approach with surgery in the management of this condition at early stages.