Ultrasonography of Facial and Submandibular Hidradenitis Suppurativa and Concomitance with Acne Vulgaris.

OBJECTIVES: To describe the ultrasound characteristics of facial and submandibular hidradenitis suppurativa (HS) and detect acne vulgaris (AV) concomitance in these cases. METHODS: We performed a retrospective study of the ultrasound images of patients with facial HS who had been clinically evaluated by dermatologists. The reported ultrasound diagnostic criteria, severity (mSOS-HS), and activity (US-HAS) staging of HS were used to categorize the patients. The finding of fragments of hair tracts within the key lesions (dilated hair follicles, pseudocysts, fluid collections, and tunnels) was considered a pivotal sign to discriminate HS from AV. Demographic and morphological analysis of the images were considered. RESULTS: Thirty-three patients met the criteria (78.8% male/21.2% female). Of these, the mSOS- HS scoring was stage I in 51.5%, stage II in 27.3%, and stage III in 21.2%. Dilation of the hair follicles and the presence of pseudocysts, fluid collections, and tunnels were detected in the HS cases; 63.1% of pseudocysts, 62.4% of tunnels, and 46.2% of fluid collections contained fragments of hair tracts. In all HS cases, there was a key lesion(s) with fragments of hair tracts. Four (12.1%) patients showed concomitant facial HS and acne ultrasound lesions. The acne lesions were pseudocysts without inner hair tract fragments in all cases, and the SOS-Acne scoring was stage II for all of them. CONCLUSION: Facial HS can be detected on ultrasound and shows a morphology similar to that of HS in other corporal regions. In some cases, facial HS could be concomitant with AV. The subclinical ultrasonographic information can support a better management of these cases.

Acné conglobata familiar

El acné conglobata es una forma severa de acné que produce muchas lesiones inflamatorias y cicatrices, marcando la vida del paciente de manera negativa. Presentamos el caso de 2 hermanos con acné conglobata, ambos con lesiones en piel de 2 años de evolución con aparición de nódulos, quistes y fístulas en territorio corporal extenso. El primero con diagnóstico reciente de leucemia promielocítica aguda en planes de iniciar quimioterapia y, el segundo, sin patologías de base. El primer caso recibió tratamiento antibiótico sistémico por complicaciones infecciosas propias de la inmunodepresión, además recibió dexametasona y ácido transretinoico como quimioterapia, y es dado de alta con dichos medicamentos y antibiótico profiláctico. El segundo caso recibió tratamiento con antibiótico sistémico y dapsona. Ambos acuden a control a los 2 meses y se observó mejoría importante de las lesiones en piel. El primer paciente falleció por complicaciones inherentes a su patología de base y el segundo paciente abandonó el tratamiento.

Acne conglobata is a severe form of acne that produces many inflammatory lesions and scars, marking the patient's life in a negative way. We present the case of two brothers with acne conglobata, both with 2-year-old skin lesions with the appearance of nodules, cysts, and fistulas in extensive body territory. The first with a recent diagnosis of acute promyelocytic leukemia with plans to start chemotherapy and the second with no underlying pathologies. The first case received systemic antibiotic treatment due to infectious complications typical of immunosuppression, he also received dexamethasone and transretinoic acid as chemotherapy, and was discharged with the mentioned drugs and prophylactic antibiotic. The second case received treatment with systemic antibiotic and dapsone. Both cases returned for control at 2 months and significant improvement in skin lesions was observed. The first patient died due to complications inherent to his underlying pathology and the second patient abandoned treatment.

Treatment of Acne with Isotretinoin Should Be Avoided in Patients with Hidradenitis Suppurativa "Conglobata Phenotype".

BACKGROUND: Acne conglobata (AC) and nodulocystic acne have long been confused clinically, despite the presentation and the response to treatment being different. AC and hidradenitis suppurativa (HS) resemble each other; a subtype of HS called "conglobata phenotype" has recently been reported in a large Dutch cohort. Acne vulgaris and HS are often associated. Isotretinoin is typically ineffective in treating HS and may even aggravate it, but it is often indispensable in treating acne vulgaris. OBJECTIVE: The aim of the study was to assess whether isotretinoin may be used safely in adults with both HS and acne vulgaris and when it might be contraindicated. MATERIALS AND METHODS: Belgian HS patients from the European Registry for Hidradenitis Suppurativa Registry (ERHS) reporting a history of severe acne of the face and/or the back, and who have ever used isotretinoin for their acne, were all selected. Patients whose acne worsened on isotretinoin were compared to patients whose acne did not worsen (improvement or no change). RESULTS: Among the 82 selected patients, 10 (12.2%) report that their acne was aggravated while taking isotretinoin, while 72 (87.8%) report that their acne was not aggravated on isotretinoin. Of the 10 HS patients whose acne worsened with isotretinoin, 9 (90%) were men (p = 0.04) and 8 (80%) were HS "conglobata phenotype" (p < 0.001). In contrast, 47 (65.3%) of the 72 patients whose acne did not worsen on isotretinoin belonged to the HS "regular phenotype" (p = 0.01). On multivariate analysis, the item most strongly associated with poor response to isotretinoin was the HS "conglobata phenotype," followed by body mass index (BMI) (worse response to isotretinoin if BMI >25 kg/m2). Additionally, of 26 patients who received isotretinoin while their HS had already started, only 6 (23.1%) reported isotretinoin effectiveness on their HS. CONCLUSION: Subject to confirmation by larger studies, our study suggests that isotretinoin should be avoided in the treatment of acne in HS patients with the HS "conglobata phenotype," as it may worsen the acne, likewise being male or having a BMI above 25 seems to increase this risk of a bad therapeutic outcome. Patients with an HS "regular phenotype" appear to be at a reduced risk of isotretinoin treatment worsening their acne.

Deeper Seated Than Skin Deep: Report of a Rare Case of Follicular Occlusion Tetrad and a Literature Review.

Follicular occlusion tetrad (FOT) is a clinical syndrome consisting of hidradenitis suppurativa (HS), acne conglobata (AC), dissecting cellulitis of the scalp (DCS), and pilonidal cyst (PC). These entities typically occur independently, but occasionally present simultaneously comprising FOT. The four components share similar pathophysiology affecting the apocrine glands, follicular hyperkeratinization being the hallmark of each entity. Understanding shared similarities of each disease is paramount for the treatment approach as the relapsing and chronic nature of this syndrome can be burdening to patients. We present the case of a 22-year-old obese Hispanic man with a history of tobacco use who presented with worsening skin lesions. The patient developed extensive facial cystic acne 5 years before presentation, followed by left axillary hidradenitis suppurativa lesions two years before the presentation and right axillary involvement one year after. Skin manifestations then expanded to include the lower back, gluteal and perineal areas. The patient was diagnosed with FOT and despite conservative medical management, his lesions failed to improve. He ultimately underwent multiple staged excisional debridement surgeries and skin grafts. Our case underlines the presence of a syndromic association of cutaneous lesions that share a common pathogenesis and emphasizes that this entity requires a multidisciplinary approach. New biologic therapies continue to emerge and may potentially prevent the need for surgical intervention and the burden associated with it.

Tumor necrosis factor inhibitors for pediatric patients with SAPHO syndrome associated with acne conglobata.

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare chronic inflammatory disease with osteoarticular and cutaneous involvements as its main manifestations. Acne conglobata as a severe acne form may affect adolescent patients. Tumor necrosis factor inhibitors are usually used as a second-line therapy for refractory SAPHO syndrome and has been reported to treat acne conglobata successfully. We herein report three cases of adolescent patients with SAPHO syndrome associated with acne conglobata who were successfully treated with TNFi therapy.

Dissecting cellulitis of the scalp with typical clinical features: A retrospective cross-sectional study in a Department of Dermatology, Beijing, China.

Dissecting cellulitis of the scalp (DCS) is a rare skin disease and understudied. The aim of the study was to collect the demographic, clinical features and laboratory tests of patients with DCS in a dermatology outpatient clinic. A cross-sectional study was conducted in a department of dermatology in Beijing. Patients whose diagnoses have included DCS were selected from July 2021 to December 2021. DCS patients were stratified according to whether they were follicular occlusion triad (FOT) or not. There were 169 patients with DCS included. All 169 patients were male, and the median patient age was 32 years. The most common comorbidities in this study were seborrheic dermatitis (10.65%). Over 1/3 of patients had elevated white blood cell (WBC) and neutrophil counts, and 12 of 18 patients had dyslipidemia. CD8+ T cell counts increased in 15 of 26 patients while CD4+ T/CD8+ T ratios were all normal. DCS mainly affects men in their thirties. More research about DCS is needed to clarify the clinical significance of laboratory tests.

A case of 5-aminolevulinic acid photodynamic therapy(ALA-PDT)combined with surgery for the treatment of acne conglobata.

We report the case of a 20-year-old man with acne conglobata (AC) who was treated with 5-aminolevulinic acid photodynamic therapy (ALA-PDT) sequentially to deroofing, and finally used fractional carbon dioxide laser for esthetic requirements, achieving satisfying results. AC is a severe form of acne vulgaris that can lead to significant scarring and has serious negative effects on a patient's psychological well-being and quality of life. Some cases are likely resistant to currently available treatments. This report describes a promising, and effective method for the treatment of AC.

Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis Syndrome Diagnosis in Adolescent and Isotretinoin as a Possible Serious Exacerbating Factor.

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare auto-inflammatory condition involving cutaneous and osteoarticular manifestations. This study presents a case where a 16-year-old male with glucose-6-phosphate dehydrogenase (G6PD) deficiency presented with severe nodulocystic acne after three weeks of isotretinoin therapy. In addition to worsening acne, the patient had bone and joint pain with movement restriction. The patient's workup showed elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), bilateral symmetrical sacroiliitis on magnetic resonance imaging (MRI), and multiple bony lesions on bone scintigraphy. A diagnosis of SAPHO syndrome possibly induced by isotretinoin was made. Isotretinoin discontinuation, analgesia, topical acne medications, prednisolone, and adalimumab yielded considerable clinical improvement.

Severe recalcitrant hidradenitis suppurativa in a 2-year-old boy with partial trisomy 13.

We report the case of a 2-year-old boy with mosaic trisomy 13 and immunodeficiency who developed severe hidradenitis suppurativa beginning at the age of 18 months. Unresponsive to standard therapies, he exhibited a partial response to immunoglobulin replacement therapy.

Acne in the first three decades of life: An update of a disorder with profound implications for all decades of life.

Acne vulgaris is a chronic, inflammatory, skin condition that involves the pilosebaceous follicles and is influenced by a variety of factors including genetics, androgen-stimulation of sebaceous glands with abnormal keratinization, colonization with Cutibacterium acnes (previously called Propionibacterium acnes), and pathological immune response to inflammation. Acne can occur at all ages and this discussion focuses on the first three decades of life. Conditions that are part of the differential diagnosis and/or are co-morbid with acne vulgaris are also considered. Acne in the first year of life includes neonatal acne (acne neonatorum) that presents in the first four weeks of life and infantile acne that usually presents between 3 and 6 months of the first year of life with a range of 3 to 16 months after birth. Acne rosacea is a chronic, inflammatory, skin condition that is distinct from acne vulgaris, typically presents in adults, and has four main types: erythemato-telangiectatic, papulopustular, phymatous and ocular. Treatment options for acne vulgaris include topical retinoids, topical benzoyl peroxide, antibiotics (topical, oral), oral contraceptive pills, isotretinoin, and others. Management must consider the increasing impact of antibiotic resistance in the 21st century. Psychological impact of acne can be quite severe and treatment of acne includes awareness of the potential emotional toll this disease may bring to the person with acne as well as assiduous attention to known side effects of various anti-acne medications (topical and systemic). Efforts should be directed at preventing acne-caused scars and depigmentation on the skin as well as emotional scars within the person suffering from acne.

Acne Conglobata of the Scalp.

Acne conglobata (AC) is a rare form of severe and chronic nodulocystic acne. It is characterized by nodulocystic lesions, borrowing, interconnecting abscesses, scars, in addition to grouped comedones. AC usually appears on the trunk and may extend to the buttocks. It can also appear, to a lesser extent, on the face, neck, shoulders, proximal arms, abdomen, and thighs. To the best of our knowledge, AC of the scalp has not been reported in the literature. Herein, we are reporting a case of AC of the scalp, emphasizing its clinical and trichoscopic features and how to differentiate it clinically from similar scalp conditions, especially alopecic and aseptic nodules of the scalp and dissecting cellulitis of the scalp.

Second joint position paper: Use of isotretinoin in severe acne / Segunda declaración de posición conjunta: uso de isotretinoína en acné severo

Background: The use of isotretinoin is indicated in the treatment of severe acne; however, its adverse effects are important. Objective: To update the first Mexican Consensus on the use of isotretinoin in severe acne vulgaris, which took place in 2009. Methods: It was carried out a literature search between June 2009, and February 2015, in order to evaluate topics to be discussed; materials were sent to the experts to promote the debate among participants. The topics of interest were analyzed during the consensus with the Delphi modified method, using an instrument previously validated. 15 certified dermatologists with experience in handling acne with isotretinoin took part in the study; seven of them were involved in the previous consensus. Results: Several cases of isolated adverse events were identified. Neither systematic reviews, meta-analyses nor comparative, randomized, controlled clinical trials were published during the observation period. Conclusions: Isotretinoin is still the best treatment for severe nodulocystic acne. However, it must be taken into consideration its teratogenic effect on pregnant women and its association with inflammatory bowel disease, depression and suicidal ideas. Monitoring with laboratory tests is a tool for identifying possible adverse events.

Introducción: el uso de la isotretinoína está indicado en el tratamiento del acné severo; sin embargo, sus efectos adversos son importantes. Objetivo: actualizar el primer Consenso Mexicano sobre el uso de isotretinoína en acné vulgar severo. Métodos: se realizó una búsqueda de la literatura especializada entre junio de 2009 y febrero de 2015, con el fin de evaluar los temas que se iban a discutir; el material fue enviado a los expertos con el objetivo de promover el debate entre los participantes. Los temas de interés fueron analizados durante el consenso mediante el método Delphi modificado, utilizando un instrumento previamente validado. Participaron 15 dermatólogos certificados y con experiencia en el manejo de acné con isotretinoína; siete de los especialistas intervinieron en el primer consenso. Resultados: se identificaron múltiples reportes de caso de eventos adversos aislados. Ninguna revisión sistemática o metaanálisis fueron publicados en el periodo de observación. Tampoco se identificaron estudios comparativos clínicos aleatorios controlados. Conclusiones: la isotretinoína todavía es el mejor tratamiento para el acné noduloquístico severo. Sin embargo, se debe considerar su potencial teratogénico sobre el feto en mujeres embarazadas y la asociación con enfermedad inflamatoria intestinal, depresión e ideas suicidas. La monitorización con exámenes de laboratorio es el instrumento para identificar posibles eventos adversos.

[Acne flare on isotretinoin: A pointer to diagnosis of hidradenitis suppurativa]. / « Poussées inflammatoires d'acné ¼ sous isotrétinoïne révélant une hidradénite suppurée : 4 cas.

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.

Severe Nodulocystic Acne not Responding to Isotretinoin Therapy Successfully Treated with Oral Dapsone.

Nodulocystic acne is a severe form of acne which can result in significant damage to the skin with great impact on quality of life. Oral isotretinoin considered to be the best treatment for such cases. Although it has a high rate of success and its efficacy is well established in the treatment of nodulocystic acne, it may occasionally fail to control the disease. We report a case of a patient who presented to our skin clinic with severe facial nodulocystic acne in which treatment with isotretinoin failed to achieve disease control and caused worsening of his baseline condition. Therefore, oral dapsone was administered as an alternative treatment, and we reached a complete remission of acne lesions within six months. Oral dapsone could be an adequate and safe drug in severe acne, and it might also be a promising and hopeful alternative treatment for nodulocystic acne when isotretinoin fails.

Alternativas para el tratamiento quirúrgico del acné conglobata / Alternatives for Acne Conglobata Surgical Treatment

Introducción: el acné conglobata es la forma específica de acné que se caracteriza por la formación de comedones, nódulos, abscesos, quistes, fístulas y cicatrices, las que provocan deformidades. Las lesiones segregan un líquido fétido. No hay tendencia a la resolución espontánea sino que existe un polimorfismo lesional, donde las lesiones alcanzan grandes extensiones con una evolución desfavorable y poca respuesta a los tratamientos. Por su repercusión estética y las cicatrices que quedan como secuela del proceso inflamatorio, esta problemática es de interés científico del dermatólogo y el cirujano estético. Objetivo: organizar las alternativas quirúrgicas disponibles en nuestro contexto para el tratamiento del acné conglobata y sus resultados. Método: se realizó un estudio experimental en un grupo de 30 enfermos de acné conglobata en las provincias de Granma y Holguín del año 2000 al 2014. Los pacientes fueron atendidos en el Hospital Lenin en Holguín, Cuba. El seguimiento a los pacientes permitió definir las lesiones que requerían tratamiento quirúrgico-dermatológico, y un criterio de operabilidad de las lesiones por parte del cirujano estético. Resultados: se obtuvo como resultado que las lesiones con mejor respuesta al tratamiento farmacológico y quirúrgico-farmacológico fueron: comedones, pápulas, pústulas, quistes muy pequeños, menor de 1 cm, y cicatrices atróficas. Se definieron los criterios de operabilidad por la Cirugía Plástica. Se obtuvo una agrupación para los pacientes de acuerdo con la localización, complejidad del cuadro clínico y estado de las lesiones. Se realizó una intervención multidisciplinaria que permitió identificar los casos en estadio precoz y realizar tratamientos quirúrgicos, por el cirujano plástico, con técnicas menos cruentas. Conclusiones: se obtuvieron mejores resultados estéticos, tratamientos menos cruentos y disminución de la estadía hospitalaria y sus complicaciones.

Introduction: acnes conglobata, a specific form of acnes with comedons, nodules, abscess, cysts, fistulae and cicatrix. Lesions segregate a fetid fluid with espousal resolution and polymorph. They increase size during evolution and have no resolution capacity through the treatment. For aesthetics, the cicatrix and inflammation, are scientific matters of investigation for dermatologist and plastic surgeons. Objective: to organize surgical alternatives and their results, in the context of treatment of conglobata acnes. Method: an experimental study from 2000 to 2014 in Holguín and Granma provinces, where thirty patients with conglobata acnes were found. During their evolution we could realize the lesions that required plastic surgery, and the ones for dermatological treatment. Result: the lesions with positive to treatment were comedons, papules inflamed hyperthrophic, a little cystic (lest 1 cm) and atrophic scars. Plastic surgery criteria were determined. The patients with lesions were distributed. The ones with clinical complications and lesions stated. A multidisciplinary intervention to identify the cases early staid and surgery treatment for plastic surgery with lest bloody techniques was accomplished. Conclusion: more aesthetic results, surgery treatment with less aggressive techniques, a short time stay in the hospital and other complications.

Alternativas para el tratamiento quirúrgico del acné conglobata / Alternatives for Acne Conglobata Surgical Treatment

Introducción: el acné conglobata es la forma específica de acné que se caracteriza por la formación de comedones, nódulos, abscesos, quistes, fístulas y cicatrices, las que provocan deformidades. Las lesiones segregan un líquido fétido. No hay tendencia a la resolución espontánea sino que existe un polimorfismo lesional, donde las lesiones alcanzan grandes extensiones con una evolución desfavorable y poca respuesta a los tratamientos. Por su repercusión estética y las cicatrices que quedan como secuela del proceso inflamatorio, esta problemática es de interés científico del dermatólogo y el cirujano estético.Objetivo: organizar las alternativas quirúrgicas disponibles en nuestro contexto para el tratamiento del acné conglobata y sus resultados.Método: se realizó un estudio experimental en un grupo de 30 enfermos de acné conglobata en las provincias de Granma y Holguín del año 2000 al 2014. Los pacientes fueron atendidos en el Hospital Lenin en Holguín, Cuba. El seguimiento a los pacientes permitió definir las lesiones que requerían tratamiento quirúrgico-dermatológico, y un criterio de operabilidad de las lesiones por parte del cirujano estético.Resultados: se obtuvo como resultado que las lesiones con mejor respuesta al tratamiento farmacológico y quirúrgico-farmacológico fueron: comedones, pápulas, pústulas, quistes muy pequeños, menor de 1 cm, y cicatrices atróficas. Se definieron los criterios de operabilidad por la Cirugía Plástica. Se obtuvo una agrupación para los pacientes de acuerdo con la localización, complejidad del cuadro clínico y estado de las lesiones. Se realizó una intervención multidisciplinaria que permitió identificar los casos en estadio precoz y realizar tratamientos quirúrgicos, por el cirujano plástico, con técnicas menos cruentas.Conclusiones: se obtuvieron mejores resultados estéticos, tratamientos menos cruentos y disminución de la estadía hospitalaria y sus complicaciones.(AU)

Introduction: acnes conglobata, a specific form of acnes with comedons, nodules, abscess, cysts, fistulae and cicatrix. Lesions segregate a fetid fluid with espousal resolution and polymorph. They increase size during evolution and have no resolution capacity through the treatment. For aesthetics, the cicatrix and inflammation, are scientific matters of investigation for dermatologist and plastic surgeons.Objective: to organize surgical alternatives and their results, in the context of treatment of conglobata acnes.Method: an experimental study from 2000 to 2014 in Holguín and Granma provinces, where thirty patients with conglobata acnes were found. During their evolution we could realize the lesions that required plastic surgery, and the ones for dermatological treatment.Result: the lesions with positive to treatment were comedons, papules inflamed hyperthrophic, a little cystic (lest 1 cm) and atrophic scars. Plastic surgery criteria were determined. The patients with lesions were distributed. The ones with clinical complications and lesions stated. A multidisciplinary intervention to identify the cases early staid and surgery treatment for plastic surgery with lest bloody techniques was accomplished.Conclusion: more aesthetic results, surgery treatment with less aggressive techniques, a short time stay in the hospital and other complications.(AU)

An Atypical Localized Form of Hidradenitis Suppurativa of the Jawline and Neck Mimicking Severe Cystic Acne on Presentation.

Hidradenitis suppurativa (HS) is a chronic and debilitating suppurative disease primarily affecting the axillae, perineum, and inframammary regions, where apocrine sweat glands are present. However, HS can occur in atypical locations. We present an interesting case of a 40-year-old man who developed chronic painful subcutaneous nodules, deep sinus tracts, and abscesses involving the jawline and the anterior aspect of the neck as the only parts of the body affected and who responded satisfactorily to adalimumab and laser hair removal treatment. This case is relevant because it helps clinicians to remember that HS may be isolated to atypical locations, such as the anterior aspect of the neck and chin. It also supports another possible HS pathogenesis which consists of the occlusion of terminal hair follicles rather than being essentially a disorder of the apocrine glands.

Caracterización clínico epidemiológica del acné conglobata en las provincias de Holguín y Granma / Clinical Epidemiological Description of Conglobate Acne in Holguin and Granma Provinces

Introducción: el acné es una de las afecciones dermatológicas más frecuentes en la práctica médica, de ellas, el acné conglobata se caracteriza por ser poco común. La génesis del acné conglobata es compleja y depende de la interacción de varios factores, entre ellos, los genéticos. Objetivo: caracterizar de forma clínica, epidemiológica e histopatológica el acné conglobata en familiares de la región Holguín -Granma. Método: se realizó un estudio de serie de casos en el período comprendido de enero 2000 a diciembre 2014. Se describió el contexto medioambiental donde se desarrollaron los enfermos. Los enfermos fueron examinados para confeccionar el árbol genealógico, se le realizó seguimiento clínico de las lesiones y biopsia para estudio histopatológico. Resultados: la enfermedad afectó a mujeres y hombres en edad antes de 21 años. Las primeras lesiones generalmente fueron noduloquísticas. Los quistes, los nódulos, los macrocomedones, los conglomerados fistulizados, las bridas cicatriciales tuvieron poca capacidad de resolución con el tratamiento convencional y alcanzaron grandes tamaños a medida que avanzó el tiempo de evolución. Las lesiones se distribuyeron con predilección en la espalda, las axilas y los glúteos. Los cambios histopatológicos fueron la hiperqueratosis con tapones córneos, las alteraciones foliculares y la presencia de los quistes de inclusión epidérmica con trayectos fistulosos. La herencia se comportó autonómico dominante. Las zonas con mayor número de casos fueron las dispuestas en las márgenes del río Cauto y en lugares aledaños. Conclusiones: se definieron los elementos diagnósticos de la enfermedad, tanto clínico y epidemiológicos, como histopatológicos.

Introduction: acne is one of the most frequent dermatology affections in medical practices, and conglobate acne is characterized as uncommon. The genesis of this illness is complicated and depends on the interaction of many factors, for example the genetic factors. Objective: to describe histopatological, epidemiological and clinically the conglobate acne incidence in some families from Holguín - Granma regions. Method: a case series study was carried out for the period from January 2000 to December 2014. The environmental context where the patients were developed was described. The patients were examined to make the family tree, clinical follow-up of lesions and biopsy for histopathological study. Results: the disease affected women and men before the age of 21. The nodule cystic lesions were the first ones. Nodules, macrocomedones, fistulized conglomerates, scar flanges had little resolution capacity with conventional treatment and reached large sizes as the evolution time advanced. The lesions were distributed with a preference in the back, underarms and glutes. Histopathological changes were hyperkeratosis with corneal plugs, follicular alterations and the presence of epidermal inclusion cysts with fistulous pathways. The inheritance behaved autonomously dominant. The areas with the highest number of cases were those located on the banks of the Cauto River and in surrounding areas. Conclusions: the diagnostic elements of the disease, both clinical and epidemiological, as well as histopathological, were defined.

Tratamiento quirúrgico en fístulas pilonidales en el síndrome de oclusión folicular / Surgical Treatment in Pilonidal Fistulas in Follicular Occlusion Syndrome

Introducción: el síndrome de oclusión folicular está compuesto por acné inverso, acné conglobata y la fístula pilonidal (triada); cuando se asocia la foliculitis disecante del cuero cabelludo entonces constituye la tétrada de oclusión folicular. El acné inverso es una enfermedad supurativa, crónica e invalidante, cuyas características clínicas son la presencia de comedones con múltiples aberturas que vinculan dos o más folículos, abscesos con comunicaciones epitelizadas, y sinus drenantes en regiones con glándulas apocrinas. La génesis de estas afecciones es básicamente genética con expresión dermatológica. Objetivo: caracterizar física e histopatológicamente a los pacientes que padecen de síndrome de oclusión folicular, así como, los resultados del tratamiento quirúrgico de la fístula pilonidal en este trastorno. Método: estudio del comportamiento de la fístula pilonidal en el síndrome de oclusión folicular en 37 enfermos con síndrome de oclusión folicular en Hospital Lenin. Se utilizó la exéresis local de las fístulas pilonidales y perianales, esta exéresis se extendió por el tejido celular subcutáneo hasta la facia. Luego la zona se reparó con injerto libre de piel. Resultados: la fístula pilonidal se presentó en cuatro pacientes de los estudiados con largos períodos evolutivos, los cuales fueron tratados con autoinjerto libre de piel. La hiperqueratosis, así como, la perifoliculitis fueron los cambios histopatológicos de mayor relevancia. Conclusiones: la fístula pilonidal es de difícil manejo en el curso del síndrome de oclusión folicular y solo encuentra solución en el tratamiento quirúrgico con autoinjerto libre de piel.

Introduction: follicular occlusive syndrome includes inverse acne, acne conglobata and pilonidal fistula (triad); when it is associated to the dissecting folliculitis of the scalp then it constitutes the tetrad of follicular occlusion. Reverse acne is a suppurative, chronic and invalidating disease. Its clinical features are the presence of comedones with multiple openings that link two or more follicles, abscesses with epithelial communications, and draining sinuses in regions with apocrine glands. The genesis of these affections is basically genetic with dermatological expression. Objective: to describe physically and histopathologically the patients with follicular occlusion syndrome, as well as the results of surgical treatment of pilonidal fistula in this disorder. Method: the study is on the prevalence of pilonidal fistula in follicular occlusion syndrome in 37 patients with follicular occlusion syndrome. The local excision of the pilonidal and perianal fistulas was performed, this exeresis extended by the subcutaneous cellular tissue until the facia. The area was then repaired with a skin-free graft. Results: four patients had the pilonidal fistula, with long periods of evolution, who were treated with autograft of skin. Hyperkeratosis, as well as, perifolliculitis were the most relevant histopathological changes. Conclusion: the pilonidal fistula is difficult for follicular occlusion syndrome management and only the surgical treatment is the most suitable .

Acne conglobata in a long-term survivor with trisomy 13, accompanied by selective IgM deficiency.

Trisomy 13 (T13) is a congenital chromosomal disorder that is usually fatal within 2 years of birth, and only a few patients have been reported to reach adolescence. Here, we report a male long-term survivor of T13, currently 15 years of age, with a several-year history of extensive acne conglobata (AC) with abscesses on the face and neck. Methicillin-resistant Staphylococcus aureus was consistently isolated from the pustular lesions. Serum IgM levels were extremely low at 10 mg/dl. There were no abnormalities in neutrophil and total B cell number, or in serum IgA and IgG levels. Increased CD8+ T cell counts and inversion of the CD4/CD8 ratio were observed repeatedly. The patient's clinical features and laboratory data support a diagnosis of selective IgM deficiency (SIgMD) with concurrent AC. Immunoglobulin replacement therapy elevated serum IgM levels to the normal range and reduced the severity of AC. We suggest that T13 may represent a syndromic disorder associated with multiple organ malformation and a risk of developing immunodeficiency involving SIgMD. Because pediatric SIgMD is rare and an immunological abnormality in T13 patients has not previously been reported, we describe the patient's clinical course.