Application of the free-style brachial artery perforator based propellar flap in the management of axillary hidradenitis suppurativa.

Hidradenitis suppurativa (HS) is a chronic and recurrent inflammatory disease often requiring surgical intervention in extensive lesions. Total of seven axillary lesion with HS was treated with free-style perforator based propeller flaps (innominate perforator originating from the brachial artery) after complete excision. The flap design mimicked the axis and design of the brachioplasty surgery. The flaps were elevated and after obliterating the defect, the remnant flap was discarded and donor site was primarily closed. All flaps were performed successfully and HS did not recur in any of the patients. In addition, the operated limb also showed a reduction of arm circumference of more than 5 cm. This is the first report using the free-style perforator based propeller flap to reconstruct the axillary HS. It is a simple and a reliable approach yielding good functional and aesthetic outcome with additional advantages of providing a brachioplasty.

Advances in surgical treatment of hidradenitis suppurative. / åŒ–è„“æ€§æ±—è ºç‚Žå¤–ç§‘æ²»ç–—ç ”ç©¶è¿›å±•.

Hidradenitis suppurative is a chronic, refractory and recurrent dermatological disease. The disease should be managed by targeted surgical intervention on the basis of medical treatment. Currently, the surgical treatment methods include local treatments like incision and drainage, unroofing, laser therapy, intense pulsed light therapy, photodynamic therapy, as well as complete lesion resection such as skin-tissue saving excision with electrosurgical peeling and extended excision. The clearance range, therapeutic effect, postoperative complications, and recurrence risk vary among the different treatment methods. Local treatments cause less damage, but have high recurrence rates, and are mainly for mild to moderate hidradenitis suppurative patients. Complete lesion resections have relatively low recurrence rates, but may bring more surgical injuries, and postoperative reconstructions are needed, which are mainly for moderate to severe hidradenitis suppurative patients. In this article, the surgical treatment principles and various surgical treatment methods of hidradenitis suppurative are reviewed, to provide a reference for the diagnosis and treatment of this disease in clinical practice.

Real-world evidence of biological agents in dermatology: A review of its applications, advantages, and limitations.

Randomized controlled trials (RCTs) are regarded as the gold standard of evidence-based medicine. However, the disadvantages of RCTs have been well-documented for a lengthy period of time. Due to the carefully controlled conditions, a small group of patients is studied, resulting in a lack of external validity and generalizability. To address this issue, real-world evidence (RWE) has grown in importance as a complement to randomized controlled trials (RCTs). We introduce and describe the databases used by RWE in this post. Applications of RWE are described in following aspects with examples of patients in the use of biological agents with skin diseases: (1) support the safety and efficacy outcomes of RCTs; (2) reveal real-world situations of adverse event management; (3) optimal treatment plans; (4) treatment's real-world cost burden. We also focus on its superiority of generalizability, external validity, and time and economic efficiencies as well as its deficiencies in data resources, study design, and statistical methodologies.

Clinical, microbiological, immunological and imaging characteristics of tunnels and fistulas in hidradenitis suppurativa and Crohn's disease.

Hidradenitis suppurativa (HS) tunnels and Crohn's disease (CD) fistulas are a challenge to treat. Although pathogenic similarities have been described between HS and CD, recent studies indicate that clinical, microbiological, immunological and imaging characteristics differ between these diseases. This review highlights the differences between HS tunnels and CD fistulas. Next-generation sequencing studies demonstrate a microbiome in HS tunnels dominated by Porphyromonas spp., Prevotella spp. whereas no specific bacteria have been associated with cutaneous CD. Immunologically, TNF has been found upregulated in HS tunnels along with various interleukins (IL-8, IL-16, IL-1α and IL-1ß). In CD fistulas, Th1, Th17, IL-17, IFN-ɤ, TNF and IL-23 are increased. US imaging is an important tool in HS. US of HS tunnels depict hypoechoic band-like structure across skin layers in the dermis and/or hypodermis connected to the base of a widened hair follicle. In CD, MR imaging of simple perianal fistulas illustrates a linear, non-branching inflammatory tract relating to an internal opening in the anus or low rectum and an external opening to the skin surface. An increased awareness of the immediate potential differences between HS tunnels and CD fistulas may optimize treatment regimens of these intractable skin manifestations.

The Development of Systemic Inflammatory Diseases in Hidradenitis Suppurativa.

It is understood that the skin is a peripheral lymphoid tissue that defends against external environmental stimuli. Continuous activation from these factors, on the other hand, promotes persistent inflammation at the local location and, occasionally, tissue damage. Hidradenitis suppurativa (HS) is a typical inflammatory skin disease and becomes a source of numerous inflammatory cytokines due to the chronic intractable repeated inflamed tissues. Because inflammatory cells and cytokines circulate throughout the body from the inflamed organ, it has been hypothesized that HS-mediated skin inflammation impacts the systemic functioning of numerous organs. Recent updates to clinical and experimental investigations revealed that HS has a significant connection with systemic inflammatory disorders. We provide the details and comprehensive molecular mechanisms associated with systemic inflammatory illnesses due to HS.

Langerhans Cell Histiocytosis Presenting With Clinical Features of Hidradenitis Suppurativa.

Langerhans cell histiocytosis (LCH) is a rare neoplastic disease of myeloid dendritic cells with a widely variable presentation of organ system involvement and severity. In this case report, we share the details of a rare case of cutaneous LCH resembling hidradenitis suppurativa (HS).

Revision of the Failed Cleft Lift for Pilonidal Disease.

The cleft lift has been demonstrated to be one of the most successful operations for the treatment of pilonidal disease, however, there are times this procedure fails and further surgery is necessary. This article describes a reproducible and successful technique for the revision of a failed cleft lift. This procedure was performed on 76 consecutive patients who had previous cleft lift procedures. Failures were manifested by either a wound, sinus, abscess, dehiscence or fragile scar. The revision flattened the lower gluteal cleft with a rotation and advancement flap that placed the skin incision off-midline. Follow-up over the 10 years of this series was between six and 124 months with an average of 36 months. The revision was initially successful in 96.1% of patients; if the procedure was unsuccessful a repeat revision was subsequently curative. This procedure is proposed as an essential part of the treatment algorithm for patients with recurrent pilonidal disease after a cleft lift operation.

"The Terror of Death Began To Stalk Him": The Mysterious Fistula of Charles the Wise.

For 23 years, Charles V "The Wise" of France suffered from a mysterious fistula on his left arm that continuously drained pus. For all this time, he believed that as soon as the fistula ceased to weep, he would have a mere 15 days before death followed. His death in 1380, at the young age of 42, seemingly proved this assumption correct. This paper explores the possible explanations from arsenic poisoning at the hands of his longtime nemesis Charles II, as many of his contemporaries believed, to an undiagnosed case of hidradenitis suppurativa, to an underlying tuberculosis infection, to the possibility that his condition was entirely self-inflicted. While it is impossible to determine a definitive cause, it is highly unlikely that Charles II "The Bad" of Navarre had anything to do with his rival's strange condition.

A Controversial Relationship Between Crohn's Disease and Hidradenitis Suppurativa: A Case Series and Literature Review.

Crohn's disease (CD) is an inflammatory bowel disease (IBD) with major extraintestinal manifestations. Hidradenitis suppurativa (HS) is a chronic inflammatory skin condition that has previously been described to have a strong association with CD. Though the pathophysiology remains uncertain, this case series highlights the different aspects of disease presentation, similarities, severity, current treatment modalities, and the relational conflict between HS as a paradoxical side effect of biologic agents (BA) that is not well established. We identified a total of three patients with CD and HS and described their clinical presentation and management. A systematic search of the literature with PubMed and Ovid MEDLINE was done in 2021. Two patients were initially diagnosed with CD prior to developing skin manifestations. The third patient was diagnosed with HS first, then was found to have gastrointestinal symptoms. All patients had HS requiring surgical intervention. One patient failed a biological agent but responded to another. The second patient was treated with cytotoxic agents with acceptable results. The third patient was managed without the use of biologics. One of three patients' clinical courses may suggest a paradoxical side effect of BA.  The relationship between CD and HS is based on several case reports. A prospective study will help establish the relationship as well as shed light on the treatment of both conditions simultaneously. In addition, further evaluation of the causal relationship between BA, specifically adalimumab and infliximab as treatment for CD and HS are warranted to effectively manage Crohn's disease, evaluate paradoxical HS, and improve outcomes of both HS and CD. CD and HS impact a patient's quality of life and physicians should therefore have a high degree of awareness upon diagnosis.

Concomitant psoriasis and hidradenitis suppurativa responsive to adalimumab therapy: A case series.

Psoriasis and hidradenitis suppurativa are inflammatory dermatoses that have been associated with arthritis, metabolic syndrome, obesity, and smoking. They share common pathogenic mechanisms such as elevated levels of several proinflammatory cytokines including tumor necrosis factor (TNF), interleukin-17A, and impaired Notch pathway. Thus, treatments for both diseases are sometimes overlapping. Biological therapy such as adalimumab is effective for patients with hidradenitis suppurativa and psoriasis. Adalimumab is a monoclonal antibody that binds to TNF and inhibits the cytokine interaction with the TNF receptors, thus inhibiting the inflammatory cascade. Currently, data are lacking on the treatment for co-occurrence of psoriasis and hidradenitis suppurativa. This case series describes three patients with a diagnosis of concomitant psoriasis and hidradenitis suppurativa. In these cases, after 12 weeks of treatment with adalimumab 40 mg every other week, the average Psoriasis Area Severity Index score reduced from 21.4 to 2.9 for psoriasis, Hidradenitis Suppurativa-Physician's Global Assessment from 3.3 to 0.7, and pain Visual Analog Scale for hidradenitis suppurativa from 4.6 to 2. The results suggest that adalimumab is a treatment of choice for patients with concomitant hidradenitis suppurativa and psoriasis.

Factors affecting quality of life in patients with hidradenitis suppurativa.

Hidradenitis suppurativa (HS) has a substantial impact on patients' lives. We identified factors associated with decreased quality of life (QoL) in patients with HS. Consecutive newly referred patients with HS attending a tertiary referral centre for HS were evaluated with the dermatology life quality index (DLQI). Clinical evaluation was performed according to the Hurley stage. Furthermore, disease duration, number of boils in the past month, boil-associated pain score, overall disease-related distress score, smoking status, employment status and comorbidities were recorded. A total of 339 patients with a mean age of 39.4 years were included; 218 (64.3%) females and 121 (35.7%) males. Of these, 96 (28.3%) had Hurley stage I, whereas 195 (57.5%) and 48 (14.2%) had Hurley II and III, respectively. The mean BMI was 29.0 (SD 7.1) kg/m2 and 75.2% of patients were current or former smokers. The mean overall DLQI score was 11.9 (SD 7.6). After mutual adjustment for clinical characteristics a significant difference in mean overall DLQI score was observed between severity groups (8.6 vs. 12.6 vs. 16.1, adjusted p < 0.001, for Hurley I, II and III, respectively), age group (12.1 vs. 12.1 vs. 12.5 vs. 7.1, adjusted p = 0.002, for ≤ 20, 21-40, 41-60 and > 60 years, respectively), employment status (11.0 vs. 14.6, adjusted p = 0.003, for employed and unemployed, respectively), presence of boils in the preceding month (8.3 vs. 13.6, adjusted p = 0.001, for no boils and presence of boils, respectively), higher overall disease-related distress score (6.3 vs. 13.9, adjusted p < 0.001, for low and high score, respectively), involvement of the groins (8.7 vs. 13.0, adjusted p = 0.035 for no and involvement, respectively), high number of anatomical regions involved (9.8 vs. 12.4 vs. 14.5, adjusted p = 0.007 for 0-1, 2 and ≥ 3 anatomical regions involved, respectively) and diabetes (11.5 vs. 15.2, adjusted p = 0.043, for no and diabetes, respectively). All ten individual DLQI question scores increased significantly with increasing Hurley stage. Patients with HS referred for specialized hospital care report substantial impact on the quality of life. Disease severity (Hurley stage), younger age, diabetes, recent and increasing disease activity and specific anogenital localization are major aggravating factors.

Hidradenitis suppurativa is an autoinflammatory keratinization disease: A review of the clinical, histologic, and molecular evidence.

The pathogenic model of hidradenitis suppurativa is in the midst of a paradigm shift away from a disorder of primary follicular occlusion to an autoinflammatory keratinization disease. Observational, experimental, and therapeutic evidence supports the concept of hidradenitis suppurativa as a primarily inflammatory disorder, a disorder of autoimmunity, or both, in contrast to the current prevailing paradigm of primary follicular occlusion. The lack of reliable and high-fidelity disease models has limited the available experimental and mechanistic evidence to support or refute one pathogenic model over another. This scholarly review synthesizes the existing clinical, histologic, and molecular data to evaluate the extant evidence supporting the autoinflammatory paradigm and further informing the molecular mechanisms of hidradenitis suppurativa pathogenesis. Follicular hyperkeratosis/occlusion and perifollicular inflammation coexist in histologic specimens, with interleukin 1α demonstrated to stimulate comedogenesis in the infundibulum. pH elevation in occluded body sites alters the microbiome and amplifies existing T-helper cell type 17 immunoresponses. Known metabolic comorbidities and smoking are known to upregulate interleukin 1α in follicular keratinocytes. Identified genetic variants may alter epidermal growth factor receptor signaling, leading to upregulated keratinocyte inflammatory responses. The process of follicular rupture and dermal tunnel formation can be explained as secondary responses to inflammatory activation of fibroblasts and epithelial-mesenchymal transition, with antibody production associated with inflammatory amplification in advanced disease. This review aims to reevaluate and integrate the current clinical, histologic, and molecular data into a pathogenic model of hidradenitis suppurativa. This is essential to advance our understanding of the disease and identify novel therapeutic targets and approaches.

Photodynamic therapy combined with surgery for hidradenitis suppurativa: A case report.

Hidradenitis Suppurativa (HS) is a distressing painful chronic inflammatory skin follicular condition. It is a challenge to achieve better therapeutic effect and lower recurrence rate. In this report, ALA-PDT combined with surgery for the treatment achieved ulcer healing, pain elimination, with no relapse during our follow-up.

Ustekinumab treatment for hidradenitis suppurativa.

Hidradenitis suppurativa (HS) is a follicular occlusive inflammatory skin disease that occurs in the axilla, groin, buttocks and vulval region. Control of the intractable inflammation is a primary goal of HS treatments. Benefit of anti-tumor necrosis factor (TNF) antibodies against HS have been reported, and adalimumab has been approved for HS in Europe, the USA and Japan. However, the alternative therapies for anti-TNF antibodies have not been established yet. We experienced a case of HS which developed during the infliximab treatment for Crohn's disease (CD) and was well managed by ustekinumab (UST). We reviewed the articles relating to ustekinumab treatments for HS. Twenty-four HS patients, 16 women and eight men, have been treated with ustekinumab. The average age was 35.7 ± 10.8 years (mean ± SD). All were of Hurley stage II or III. Ten (10/24, 41.6%) had received anti-TNF drugs including infliximab, adalimumab and etanercept prior to UST treatment for HS. Although the initial doses varied from 45 mg s.c. to 390 mg i.v., all cases were treated with 45 or 90 mg s.c. every 8 or 12 weeks at the regular dose, by following the regimen for psoriasis or CD. HS in most of the cases started to improve after 3-5 months of UST initiation, and some achieved complete remission. To our knowledge, our case is the first Asian HS patient improved by UST. Overall, UST is useful for HS and could be an alternative treatment if HS patients do not respond to other medications including anti-TNF drugs.

[Emotions and bodily experience in Hidradenitis Suppurative-Acne Inversa].

Hidradenitis Suppurative-Acne Inversa is one of the most debilitating chronic skin diseases. It seriously affects the emotional and relational life of the patient, it has a significant psychiatric comorbidity and it impairs the quality of life. We present the report of a clinical situation with onset of the illness in a young woman during pregnancy, a case characterized by particular evolution, severe systemic involvement, strong psycho-emotional impact on the patient and impairment of subjective well-being of the caregiver. The clinical evaluation highlights mainly the relevance of the bodily experience, that stands out as a central issue in the sufference of the patient. She feels an uncanny foreignness to herself and a lacerating wound of her identity, related to her desirability, her femininity and her motherhood. Specific personality factors, which are likely to have influenced and guided the experience of illness and the quality of the relationship with the treatment team, are also evaluated. This case exemplifies, at different levels, the clinical complexity of Hidradenitis Suppurative-Acne Inversa and its impact on individual subjectivity. This disease requires an integrated intervention by a multidisciplinary team, providing for the assessment, the treatment and the evaluation of outcomes. It is necessary an effective operational link between different competences, in order to promote the patient compliance and to activate and develop the best care and the right psychological support.

Hidradenitis supurativa crónica y su relación con el carcinoma epidermoide / Chronic suppurative hidradenitis and its relationship with squamous cell carcinoma

Se presenta un caso clínico con un cuadro de hidradenitis supurativa crónica que evolucionó a carcinoma epidermoide. Se realiza una revisión de la literatura de esta enfermedad. (AU)

A clinical case with a chronic suppurative hidradenitis syndrome that progressed to squamous cell carcinoma is presented. A review of the literature of this disease is made. (AU)