Spitzoid proliferative nodules arising in a congenital melanocytic naevus: A case report with clinical, dermoscopic and histologic correlation.

Proliferative nodules (PNs) are benign nodular proliferation of melanocytes occurring within congenital melanocytic naevi (CMN). Differential diagnosis between PN and melanoma is challenging for clinicians and pathologists. We describe the case of a 9-month-old boy who developed multiple nodules arising in a medium-sized CMN. Clinically, pink papules were observed, with dotted vessels on dermoscopy, suggesting spitzoid PN. On histopathological examination, the dermoscopic findings correlated with the vertical vessels of a spitzoid PN. Dermoscopy could be a useful tool to differentiate PN from melanoma. However, further studies describing the dermoscopic features of the different PN subtypes are needed. Histopathology remains the gold standard for definitive diagnosis aided by ancillary molecular tests such as fluorescence in situ hybridization or comparative genomic hybridization.

New perspectives on the treatment of hidradenitis suppurativa.

Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease characterized by the presence of painful nodules, abscesses, chronically draining fistulas, and scarring in apocrine gland-bearing areas of the body. The exact pathogenesis of HS is not yet well understood, but there is a consensus in considering HS a multifactorial disease with a genetic predisposition, an inflammatory dysregulation, and an influence of environmental modifying factors. Therapeutic approach of HS is challenging due to the wide clinical manifestations of the disease and the complex pathogenesis. This review describes evidence for effectiveness of current and emerging HS therapies. Topical therapy, systemic treatments, biological agents, surgery, and light therapy have been used for HS with variable results. Adalimumab is the only US Food and Drug Administration (FDA) approved biologic agent for moderate-to-severe HS, but new therapeutic options are being studied, targeting different specific cytokines involved in HS pathogenesis. Comparing treatment outcomes between therapies is difficult due to the lack of randomized controlled trials. Treatment strategy should be selected in concordance to disease severity and requires combination of treatments in most cases.

Integra as Firstline Treatment for Scalp Reconstruction in Elderly Patients.

PURPOSE: Large scalp defects pose a reconstructive problem especially in elderly patients. The purpose of the study is to describe our experience of oncologic scalp reconstruction using a dermal matrix (Integra). MATERIAL AND METHODS: We conducted a retrospective cohort study (January 2007 to March 2021) of patients who had undergone scalp tumor excision and reconstruction using Integra and a split-thickness skin graft (STSG). The primary end point was Integra and STSG success (defined by ≥75% percent take) and the secondary end point was postoperative complications. Both end points were assessed by the surgeon during follow-up. Demographic data, tumor characteristics, average defect size, time between stages and full-thickness scalp defects were characterized using descriptive statistics. Univariate and multivariate logistic regression models were used to evaluate the association between variables and end points. RESULTS: The sample included 70 patients with a mean (SD) age of 83.3 (7.0) years, 75.7% men and 92.9% with comorbidities. Mean (SD) defect area was 23 (17.0) cm2 and the mean (SD) first-to-second phase interval was 30.6 (8.4) days. Sixty-four patients (91.4%) underwent outpatient surgery. Integra and STSG success rates were 87.1% (95% CI: 77.69 to 93.74%) and 100%, respectively. The complications rate was 18.6% (95% CI: 9 to 28%). Mean (SD) follow-up was 18 (16.7) months. Univariate and multivariate logistic regression analysis showed no association between variables and the primary and secondary end points. CONCLUSIONS: Reconstruction of oncologic scalp defects using Integra can be performed under sedation and local anesthesia. Integra should be considered as firstline treatment for the reconstruction of scalp defects in elderly patients with comorbidities, given the low postoperative major complications rate and Integra and STSG take success.

Vulvar hidradenitis suppurativa: Clinical cross-sectional study of 25 patients.

Hidradenitis suppurativa (HS) involving the vulva is seldom reported in the gynecological or dermatological literature. The aim of this study was to describe the clinical characteristics of HS with vulvar affectation (VHS) and to compare it with patients without vulvar involvement. A cross-sectional study was conducted in a tertiary academic referral centre in Spain from May 1, 2015 to October 1, 2019. This study included 230 women with HS diagnosed in our hospital, 25 of them had vulvar involvement. In order to clinically characterize patients, demographic factors, comorbidities, clinical features, prescribed treatments and complications were recorded. The VHS group presented later median age of onset and lower body mass index (BMI) (P = 0.048), they mainly belonged to latent class 2 (LC2) and LC3 phenotypes involving groins, perineal and pubic area. A higher incidence of psychiatric disease was found in VHS (32% vs 10.7%). Significant positive association with fistula (P < 0.001), LC2 phenotype (P = 0.014), acne (P = 0.021) and thyroid disease (P = 0.006), and negative association with axillar lesions (P = 0.001) were noted. Ultrasonographical study of vulvar lesions demonstrated that most of them were fistulas with high Doppler signal suggestive of high inflammatory load. In conclusion, VHS is mostly seen in women with later onset and lower BMI and higher incidence of psychiatric disease compared to those without vulvar involvement. It is clinically characterized by the presence of fistulas and barely absent axillary involvement. Early diagnosis and treatment could be essential to prevent complications and quality of life impairment.

Hidradenitis Suppurativa: Proposal of Classification in Two Endotypes with Two-Step Cluster Analysis.

INTRODUCTION: Hidradenitis suppurativa (HS) is a chronic, inflammatory, recurrent disorder of the pilosebaceous unit. Currently, several attempts have been made to classify this disease according to its pathogenesis and clinical manifestations. We attempted at classifying 103 patients using two-step cluster analysis. METHODS: The final model included body mass index, C-reactive protein (CRP), and serum concentrations of IL-1, IL-6, IL-17, and IL-10 as continuous variables, and sex, later/early onset, anterior/posterior lesion sites, presence/absence of sinus tracts, nodules and abscesses, positive/negative history of pilonidal sinus, and presence/absence of mutations in gamma-secretase subunits (APH1A, APH1B, MEFV, NCSTN, PSEN1, PSEN2, PSENEN, PSTPIP1) as qualitative variables. RESULTS: The resultant model defined two groupings or clusters: cluster 1 (64.9% of patients) characterized by nonobese males, with nodular lesions in posterior sites, early-onset HS, higher IL-10, presence of gamma-secretase mutations, and history of pilonidal sinus; and cluster 2 (35.1% of patients) characterized by obese females or males, with lesions in anterior sites, more presence of sinus tracts and abscesses and less nodules, later-onset HS, and higher concentrations of IL-1, CRP, IL-17, and IL-6. Severity measures (Hurley, HS-PGA, and IHS4) and tobacco use were discarded because the analysis found them to be less relevant for clustering. CONCLUSION: Our resultant model confirms the clinical impression that HS is a disease spectrum with two pathogenic poles defining two clusters or endotypes. The probability of having severe disease was equally distributed in the two clusters. The variable with the highest predictive value for clustering was involvement of typical anterior sites (axillae, submammary) or atypical posterior sites (back, gluteal). Serum concentrations of interleukins, tobacco use, and sex had a lower predictive power for clustering.

Clinical characteristics of pediatric hidradenitis suppurativa: a cross-sectional multicenter study of 140 patients.

Hidradenitis suppurativa (HS) rarely affects pediatric patients. The literature on pediatric HS patients is scarce. This is a cross-sectional study based on case note review or interviews and clinical examination of 140 pediatric patients undergoing secondary or tertiary level care. Patients were predominantly female (75.5%, n = 105) with a median age of 16. 39% reported 1st-degree relative with HS. Median BMI percentile was 88, and 11% were smokers (n = 15). Median modified Sartorius score was 8.5. Notable comorbidities found were acne (32.8%, n = 45), hirsutism (19.3%, n = 27), and pilonidal cysts (16.4%, n = 23). Resorcinol (n = 27) and clindamycin (n = 25) were the most frequently used topical treatments. Patients were treated with tetracycline (n = 32), or oral clindamycin and rifampicin in combination (n = 29). Surgical excision was performed in 18 patients, deroofing in five and incision in seven patients. Obesity seemed to be prominent in the pediatric population and correlated to parent BMI, suggesting a potential for preventive measures for the family. Disease management appeared to be similar to that of adult HS, bearing in mind that the younger the patient, the milder the disease in majority of cases.

Hidradenitis suppurativa of the nape: Description of an atypical phenotype related to severe early-onset disease in men.

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.

Pleomorphic mastocytoma in an adult.

Cutaneous mastocytoma (CM) is a localized variant of mastocytosis, characterized by an over-accumulation of mast cells in the skin, without extra-cutaneous organ involvement. It is defined as the presence of up to 3 isolated mast-cell skin lesions and commonly develops in newborns and children. We report the case of a 35-year-old healthy Caucasian woman presenting with a 4-year history of a pruritic brown plaque on her left breast. Hematoxylin-eosin staining showed a dense dermal infiltrate of atypical mast cells extending to the subcutis. The cells presented a marked nuclear pleomorphism with bilobed and multilobed nuclei. Immunohistochemical studies revealed strongly expressed KIT (CD117) and CD25 proteins. Serum tryptase levels and bone marrow biopsy were normal. The diagnosis was a solitary cutaneous pleomorphic mastocytoma. This case can be added to 17 other cases of adult mastocytoma documented in the literature, although, unlike other reported cases, and as far as we are aware, this is the first case of pleomorphic mastocytoma in an adult.

Photodynamic therapy with intralesional methylene blue and a 635 nm light-emitting diode lamp in hidradenitis suppurativa: a retrospective follow-up study in 7 patients and a review of the literature.

Hidradenitis suppurativa is a chronic inflammatory skin disease which has an estimated prevalence of 1%. It is characterized by the formation of recurrent painful suppurative nodules and abscesses in the flexural areas of the body. It is believed that its pathogenesis involves an aberrant, genetically-determined activation of innate immunity against the bacterial commensal flora of intertriginous areas. It has been found that the formation of antibiotic-resistant bacterial biofilms is a common finding in hidradenitis lesions. Photodynamic therapy with different compounds and light sources has demonstrated its efficacy in a number of infectious diseases such as nail mycosis and chronic periodontitis. We retrospectively report our experience in the treatment of hidradenitis with photodynamic therapy using intralesional methylene blue and a 635 nm light-emitting diode lamp in 7 patients. Two patients received one session whereas 5 patients received two sessions. At one month follow-up good response was achieved in 6 patients. After 6 months, 5 patients (71%) maintained remission of the disease in the treated area. In view of the results and literature review, we regard methylene blue as an ideal photosensitizer for photodynamic therapy in this disease.

[Reemergence of measles in vaccinated patients: report of 6 cases and proposals for prevention]. / Reemergencia del sarampión en pacientes vacunados: descripción de seis casos y propuesta para su prevención.

EN: A 2014 measles outbreak in Catalonia affected 131 persons. We describe a series of 6 cases diagnosed in our emergency department. All the patients were under 31 years of age and complained of flu-like symptoms, including high fever and rash. Five had been properly vaccinated and one was a health care worker. A firm diagnosis of measles need not be made in the emergency department, but a high level of suspicion is important for ruling out complications, isolating the patient, and protecting health care staff at high risk for exposure. We found that 6% of the staff of our emergency department had a low level of immunity to measles. Given our findings, we suggest that preventive treatment is necessary when health care staff have been exposed to measles and their vaccination status is unknown.

ES: En el contexto de un brote de sarampión en Cataluña en 2014 que afectó a 131 personas, se presenta una serie de seis pacientes que fueron diagnosticados en nuestro servicio de urgencias (SU). Todos los pacientes eran menores de 31 años y consultaron por síndrome gripal con fiebre alta y exantema. Cinco de ellos habían sido correctamente vacunados y uno era personal sanitario. El diagnóstico de certeza en urgencias no es imprescindible, pero es importante su sospecha para descartar sus complicaciones, y para proceder al aislamiento del enfermo y a la protección del personal sanitario. El personal sanitario es un grupo de elevado riesgo de exposición. Se ha constatado que un 6% de los profesionales de nuestro SU presentó un mal estado inmunitario frente al sarampión. Ante estos resultados, planteamos la necesidad de realizar profilaxis postexposición en los profesionales en los que se desconozca el estado vacunal.

Intraoral Approach and Stereolithographic-guided Large Mandibular Reconstruction with Fibula Free Flap.

SUMMARY: The reconstruction of mandibular defects has always been of great concern, and it still represents a challenge for head-and-neck reconstructive surgeons. The mandible plays a major role in mastication, articulation, swallowing, respiration, and facial contour. Thus, when undertaking mandibular reconstruction, restoration of both function and cosmetics should be considered as the measure of success. Microsurgical reconstruction is the gold-standard method to repair a segmental mandibular defect. Reconstruction of sizeable defects often needs a large neck incision, leading to unsatisfactory cosmetic outcomes. Virtual surgical planning and stereolithographic modeling are new techniques that offer excellent results and can provide precise data for mandibular reconstruction and improve postoperative outcomes. We present a case of complete intraoral resection and reconstruction of a large ameloblastoma of the mandible.