The Role of Nutrition in Immune-Mediated, Inflammatory Skin Disease: A Narrative Review.

Immune-mediated inflammatory skin diseases are characterized by a complex multifactorial etiology, in which genetic and environmental factors interact both in genesis and development of the disease. Nutrition is a complex and fascinating scenario, whose pivotal role in induction, exacerbation, or amelioration of several human diseases has already been well documented. However, owing to the complexity of immune-mediated skin disease clinical course and breadth and variability of human nutrition, their correlation still remains an open debate in literature. It is therefore important for dermatologists to be aware about the scientific basis linking nutrition to inflammatory skin diseases such as psoriasis, atopic dermatitis, hidradenitis suppurativa, bullous diseases, vitiligo, and alopecia areata, and whether changes in diet can influence the clinical course of these diseases. The purpose of this narrative review is to address the role of nutrition in immune-mediated inflammatory skin diseases, in light of the most recent and validate knowledge on this topic. Moreover, whether specific dietary modifications could provide meaningful implementation in planning a therapeutic strategy for patients is evaluated, in accordance with regenerative medicine precepts, a healing-oriented medicine that considers the whole person, including all aspects of the lifestyle.

Histologic Patterns and Clues to Autoinflammatory Diseases in Children: What a Cutaneous Biopsy Can Tell Us.

Autoinflammation is defined by aberrant, antigen-independent activation of the innate immune signaling pathways. This leads to increased, pro-inflammatory cytokine expression and subsequent inflammation. In contrast, autoimmune and allergic diseases are antigen-directed immune responses from activation of the adaptive immune system. The innate and adaptive immune signaling pathways are closely interconnected. The group of 'complex multigenic diseases' are a result of mutual dysregulation of both the autoinflammatory and autoimmune physiologic components. In contrast, monogenic autoinflammatory syndromes (MAIS) result from single mutations and are exclusively autoinflammatory in their pathogenesis. Studying the clinical and histopathological findings for the various MAIS explains the phenotypical correlates of their specific mutations. This review aims to group the histopathologic clues for autoinflammation into three recognizable patterns. The presence of these histologic patterns in a pediatric patient with recurrent fevers and systemic inflammation should raise suspicion of an autoinflammatory component in MAIS, or, more frequently, in a complex multigenic disease. The three major histopathological patterns seen in autoinflammation are as follows: (i) the 'neutrophilic' pattern, seen in urticarial neutrophilic dermatosis, pustular psoriasis, aseptic neutrophilic folliculitis, and Sweet's syndrome; (ii) the 'vasculitic' pattern seen in small vessel-vasculitis (including hypersensitivity/leukocytoclastic vasculitis, thrombosing microangiopathy and lymphocytic vasculitis), and intermediate-sized vessel vasculitis, mimicking polyarteritis nodosa; and (iii) the 'granulomatous' pattern. Beyond these three patterns, there are additional histopathologic clues, which are detailed below. It is important for a dermatopathologist to recognize the patterns of autoinflammation, so that a diagnosis of MAIS or complex multigenic diseases may be obtained. Finally, careful histopathologic analyses could contribute to a better understanding of the various clinical manifestations of autoinflammation.

Therapeutic Use of Botulinum Neurotoxins in Dermatology: Systematic Review.

Botulinum toxin is a superfamily of neurotoxins produced by the bacterium Clostridium Botulinum with well-established efficacy and safety profile in focal idiopathic hyperhidrosis. Recently, botulinum toxins have also been used in many other skin diseases, in off label regimen. The objective of this manuscript is to review and analyze the main therapeutic applications of botulinum toxins in skin diseases. A systematic review of the published data was conducted, following Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Botulinum toxins present several label and off-label indications of interest for dermatologists. The best-reported evidence concerns focal idiopathic hyperhidrosis, Raynaud phenomenon, suppurative hidradenitis, Hailey-Hailey disease, epidermolysis bullosa simplex Weber-Cockayne type, Darier's disease, pachyonychia congenita, aquagenic keratoderma, alopecia, psoriasis, notalgia paresthetica, facial erythema and flushing, and oily skin. Further clinical trials are still needed to better understand the real efficacy and safety of these applications and to standardize injection and doses protocols for off label applications.

Estudio de la prevalencia del síndrome metabólico en pacientes con hidradenitis supurativa. Estudio retrospectivo de los últimos 2 años en el Hospital Alejandro Posadas / Study of metabolic syndrome prevalence in pacients with suppurative hidradenitis. A retrospective study of the last 2 years in Hospital Alejandro Posadas

Antecedentes: La hidradenitis supurativa (HS) es una enfermedad inflamatoria crónica relacionada con el síndrome metabólico (SM), con un mayor riesgo de comorbilidades cardiovasculares; por lo tanto, se debe mantener un alto grado de sospecha clínica frente al hallazgo de alguno de sus componentes. Objetivo: Evaluar la prevalencia del SM en los pacientes con diagnóstico de HS en comparación con la población general. Diseño: Estudio retrospectivo descriptivo. Métodos: Se revisaron las historias clínicas y el archivo fotográfico de los pacientes con diagnóstico de HS que consultaron entre el 1/9/2017 y el 31/8/2019. Se analizaron las variables: sexo, edad, antecedentes familiares, tiempo de evolución, índice de masa corporal, obesidad abdominal, laboratorio (glucemia y perfil lipídico), presión arterial, tabaquismo, número y localización de las lesiones, gravedad clinicoecográfica, respuesta al tratamiento y evolución clínica. Resultados: Se evaluaron 30 pacientes con HS valorados en nuestro servicio en los últimos 2 años; 23 mujeres y 7 varones. Predominó el grupo etario de 31-40 años. De ellos, 19 pacientes (63,3%) cumplieron con los criterios necesarios para diagnosticar el SM, hallazgo que no se relacionó con el grado de severidad de la HS. Conclusiones: La prevalencia del SM en nuestros pacientes con HS fue del 63,3% con respecto al 27,5% de la población general. La importancia de este hallazgo radica en detectar de forma temprana y oportuna la presencia de este síndrome en los pacientes con HS para evitar el riesgo de comorbilidades cardiovasculares en el futuro. (AU)

Background: Suppurative hidradenitis (SH) is a chronic, inflammatory disease, closely related to metabolic syndrome (MS), with an increased risk of cardiovascular comorbidities; for that reason we should keep a high level of clinical suspicion when one of its components is found. Objective: To evaluate MS prevalence in patients with diagnosis of SH in comparison with general population. Design: Descriptive retrospective study. Methods: We reviewed clinical and photographical files of patients with diagnosis of suppurative hidradenitis who consulted from 1/9/2017 until 31/8/2019. Variables analyzed were: sex, age, familial history, time of evolution, body mass index, abdominal obesity, glycemia, lipid profile, blood pressure, smoking, number and location of lesions, clinicalechographic severity, response to treatment and clinical evolution. Results: Thirty patients with SH were evaluated, 23 females and 7 males. The age group between 31-40 years prevailed; of these, 19 patients (63.3%) meet necessary criteria for diagnosis of MS, this finding did not correlate to the severity degree of SH. Conclusions: The MS prevalence in our SH patients was 63.3%, compared to 27.5% in the general population. The importance of this finding lays on detecting in an early and opportune way, the presence of MS in patients with SH, and to avoid the risk of future cardiovascular comorbidities. (AU)

Clinical manifestations and quality of life in hidradenitis suppurativa patients: survey of participants from an internet support group

Abstract: Background: Hidradenitis suppurativa is a complex and infrequent autoinflammatory disease that impacts on quality of life. Its pathogenesis is not fully understood, which limits the development of curative treatments. Objectives: To evaluate clinical and quality of life aspects of hidradenitis suppurativa patients from a social group on the Internet. Methods: A cross-sectional, Internet-based survey study among participants in a discussion group (Facebook) of individuals with hidradenitis suppurativa. Patients were asked to answer a questionnaire about clinical-demographic aspects and quality of life (DLQI-BRA). Results: A total of 390 individuals agreed to participate in the study, 82% of them female, median age (p25-p75), of 31 (25-37) years old, disease onset at 15 (13-23) years, family member affected in 20% of cases, overweight (BMI 29 [25-33]) kg/m2 and severe impact on quality of life (DLQI 20 [13-25]). Regarding Hurley's classification, the participants provided information that enabled classification into: I (19%), II (52%) and III (29%). More severe cases were associated with males (OR = 1.69), higher weight (BMI: OR = 1.03) non-white color (OR = 1.43) and higher frequency of other autoinflammatory diseases (OR = 1.37). Study limitations: Voluntary adherence survey with self-completion of the questionnaire by 390 from about 1600 group members. Conclusions: Hidradenitis suppurativa patients who participated in a social network group had onset of the disease after puberty, with a predominance in females and overweight people, with great impact on the quality of life.

[Verneuil's disease: a diagnosis that should be taken into consideration in patients with vulvar and perineal tumor-like lesion]. / Maladie de Verneuil: un diagnostic à ne pas méconnaitre devant une lésion tumorale vulvaire et périnéale.

We here report the case of a 58-year old nulliparous patient with a history of Niemann-Pick disease who presented with vulvoperineal, fluctuant, fistulized swelling measuring 10 cm associated with ulceration. The patient was febrile with impaired general state This mass caused some concern about necrotic and infected malignant tumor of the vulva associated with chronic dermatosis. Incision and purulent drainage associated with multiple biopsies excluded the presence of a neoplastic process. The diagnosis of suppurative hidradenitis or Verneuil's disease was based on the associations between lesions and their distributions. Then, the problem that arose was that of the choice between drug treatment and surgical procedures. This study, including a short review of the recent literature, aims to outline the diagnostic modalities, the complications and the therapeutic management of this disease.

Mucinous adenocarcinoma associated with chronic suppurative hidradenitis: Report of a case and review of the literature.

INTRODUCTION: Chronic suppurative hidradenitis (CSH) is a benign condition that can affect the perineal region and often leads to the formation of abscesses and fistulas. It is rare for CSH to undergo malignant degeneration into mucinous adenocarcinoma. PRESENTATION OF CASE: We report a case of a 55-year-old male patient with perineal CSH who suffered worsening long-term pain despite multiple surgical procedures to alleviate his symptoms. Pelvic magnetic resonance imaging (MRI) showed multiloculated cystic lesion on the left side wall of the distal rectum with gluteal extension. Pathological examination revealed mucinous adenocarcinoma. The patient underwent an abdominoperineal resection (APR) of the rectum with cutaneous muscle flap reconstruction. Although histopathological sections showed clear margins, the tumor recurred 6 months following surgery. DISCUSSION: Perineal mucinous adenocarcinoma arising in a patient with CSH is an extremely rare condition. This diagnosis is often difficult, due to the paucity of signs of malignant degeneration as well as the rarity of the disease itself. Surgical resection of the lesions is a well-established approach. In this case, diagnosing the tumor at such a late stage likely compromised his outcome. CONCLUSION: Malignant degeneration to mucinous adenocarcinoma must be suspected in patients with a history of long-term CSH. In such cases, local biopsies and a radiological examination, such as MRI can help in the diagnosis.

[Axillary hidrosadenitis due to Leishmania: Case report and literature review]. / Hidrosadenitis axilar por Leishmania: presentación de un caso y revisión de la bibliografía.

BACKGROUND: Hidradenitis is a disorder where abscesses appear after the infection of the apocrine sweat glands. It is located normally in the axillae, groin, perineal region, and the scalp. CLINICAL CASE: A 37 year old male was referred by his GP to the General Surgery Department with axillary hidradenitis which had evolved over the years. The physical examination shows signs of hidradenitis in both axillae, with a noticeable suppurative hidradenitis in the right armpit. En bloc extirpation was performed to remove the whole affected area. The pathological examination revealed a cutaneous leishmaniasis. Subsequently, fucidin was administered topically, as well as local infiltrations of one millilitre of Glucantime™. DISCUSSION: Hidradenitis normally appears in intertriginous areas and its manifestation is accompanied by recurrent subcutaneous nodules. The incidence rate in females is three times higher than in males. The isolated Hidradenitis caused by Leishmania is a rare condition presented only in endemic areas or in immunocompromised patients, such as HIV-infected patients. Clinical manifestations can be different and the diagnosis can be confirmed through haematoxylin-eosin. The main pattern displays a disorganised granuloma without necrosis. Systemic or topical treatment can be applied. Immunotherapy treatment is the most common. CONCLUSIONS: Hidradenitis caused by Leishmania in HIV-negative patients is a rare condition. Therefore it is important to perform a good histological diagnosis and to administer the right treatment.

[Ultrasound in the management of inflammatory dermatosis]. / Ecografía aplicada al manejo de las dermatosis inflamatorias.

Cutaneous ultrasound is a dermatological diagnostic imaging technique based on the interaction of high-frequency ultrasounds with the skin. Because it is non-invasive, rapid and accessible, it has increasingly wide clinical applications. This article reviews its use in the management of inflammatory dermatological diseases.

Hidradenite supurativa crônica perianal e glútea: tratamento cirúrgico com ressecção ampla e rotação de retalho dermogorduroso / Perianal and gluteal hidradenitis suppurative chronic: surgical treatment with a great resection and rotation of skin flaps

OBJETIVO: Avaliar a utilização de retalho dermogorduroso de vizinhança, numa única etapa, na reparação da área resultante da ressecção de lesões de hidradenite supurativa. PACIENTE E MÉTODOS: Estudo epidemiológico transversal e retrospectivo de prontuários de pacientes com hidradenite supurativa crônica extensa em regiões perianal e glútea submetidos à ressecção ampla e rotação de retalho cutâneo de vizinhança, no período de janeiro de 2000 a novembro de 2008. RESULTADOS: O retalho dermogorduroso permitiu, em única etapa, a cobertura total da área ressecada em oito pacientes. Não houve necrose ou infecção em nenhum dos casos. No seguimento, seis pacientes compareceram a todas as consultas ambulatoriais agendadas. Todos se mostraram satisfeitos com o resultado. CONCLUSÃO: O tratamento da hidroadenite supurtiva perianal e/ou glútea deve ser individualizado segundo a extensão, gravidade e grau de interferência na qualidade de vida. A ressecção cirúrgica com procedimentos plásticos como a rotação de retalho de vizinhança em única etapa é segura e traz resultados satisfatórios e deve ser sempre considerada nos casos crônicos, extensos e refratários ao tratamento clínico e com grandes áreas ressecadas.

OBJECTIVE: To evaluate the use of skin flaps neighborhood in a single step in the repair of the area from the resection of lesions of Suppurative Hidradenitis. METHODS: Retrospective cross-sectional epidemiological study of patients with chronic extensive Suppurative Hidradenitis in perianal and gluteal regions underwent extensive resection and skin flap rotation of the neighborhood, from January 2000 to November 2008. RESULTS: The skin flaps allowed in single step, the total coverage of the area resected in eight patients. There was no necrosis or infection in any cases. Following, six patients attended all scheduled outpatient consultations. All were satisfied with the result. CONCLUSIONS: The treatment of suppurative perianal hidradenitis and / or gluteal should be individualized according to the extent, severity and degree of interference in the quality of life. Surgical resection with plastic procedures such as rotation of retail in the neighborhood in one step is safe and has satisfactory results and should be considered for chronic cases, extensive and refractory to medical treatment and with large areas resected.