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Epidermal necrolysis (EN) compromises a spectrum of life-threatening dermatoses (Stevens-Johnson Syndrome [SJS], overlap syndrome and toxic epidermal necrolysis [TEN]). Currently, no active therapeutic regimen with unequivocal benefit exists for SJS/TEN. SCORTEN is the widely-used prognostic scale specific for SJS/TEN. Nevertheless, a new prognostic scale, the ABCD-10, has been recently proposed. In this context, acute renal failure (ARF) seems to be an important comorbidity that could influence prognosis in SJS/TEN patients more than it is assumed by these two scales. Our objectives were to compare the accuracy of the SCORTEN and ABCD-10 scales in predicting the mortality in SJS/TEN, and to investigate the influence of renal failure on prognosis. The prognostic results of 18 patients with EN treated in two referral centers between 2013 and 2018 are presented. SCORTEN, ABCD-10 and renal function values were retrospectively collected for all patients. Out of the 18 patients who were analyzed, nine (50%) received only supportive therapy, four were treated with etanercept 50 mg in a single dose (22.2%) and five with corticosteroids (27.8%). Five patients developed ARF. Predicted mortality was 3.48 for SCORTEN and 2.33 for ABCD-10. Eventually, four patients died (22.2%), all had ARF and none of them received active treatment. Despite study limitations and in the absence of active treatment of choice, SCORTEN behaved as a reliable predictor of mortality in patients with EN, outperforming the newer ABCD-10. ARF was an early event associated with a poor prognosis, which could represent a prognostic marker to consider in the future.

Resumen: La función fisiológica que en la vida intrauterina tiene el cordón umbilical se pierde tras el nacimiento. Tras los cuidados iniciales del cordón en el periodo neonatal y una vez este se ha desprendido, poca es la importancia que recibe dicha región. Sin embargo, son múltiples las enfermedades que pueden afectar al área umbilical en pacientes pediátricos y resulta crucial reconocerlas, dada su importancia anatómica.El objetivo de este artículo es el de realizar una revisión de la anatomía de la región umbilical con el propósito de establecer una correlación con las lesiones que con frecuencia se manifiestan en ella. Abstract: The physiological function of the umbilical cord in intrauterine life is lost after birth. After the initial care of the cord in the neonatal period and once it has detached, little importance is attached to the region. However, there are many diseases that can affect the umbilical region in paediatric patients and it is crucial to know how to recognise them given its anatomical importance.The aim of this article was to review the anatomy of the umbilical region to establish a correlation with the lesions that frequently manifest in this location. Palabras clave: Cordón umbilical, Embriología, Anomalías estructurales, Uraco, Onfalomesentérico, Keywords: Umbilical cord, Embryology, Structural anomalies, Urachus, Omphalomesenteric

Resumen Introduccion Los trabajos que determinan la incidencia de cancer cutaneo no melanoma (CCNM) en la poblacion tratada con psoralenos orales + UVA son heterogeneos, dependen de la localizacion geografica de la poblacion estudiada y tienen periodos de seguimiento cortos. El objetivo del trabajo es determinar la seguridad a largo plazo de la PUVAterapia y en concreto determinar la incidencia de CCNM en los pacientes tratados con PUVAterapia oral en el area mediterranea. Material y metodo Se ha realizado un estudio longitudinal de seguimiento retrospectivo, recogiendo 234 pacientes tratados con PUVA sistemico entre 1982 y 1996 con un seguimiento hasta mayo de 2017. Se ha calculado la densidad de incidencia de CCNM bruta y ajustada por edad mediante estandarizacion directa. Resultados En 22 pacientes se diagnosticaron 50 neoplasias. La prevalencia de CCNM en pacientes tratados con fototerapia fue del 10,3%. El tiempo medio de seguimiento fue de 21 anos. la densidad de incidencia bruta-ajustada de CCNM fue de 554,4-183,9 casos/100.000 pacientes tratados-ano. La densidad de incidencia bruta-ajustada de carcinoma basocelular fue de 352,3-111,2 casos/100.000 pacientes y la de carcinoma epidermoide de 229-77,7 casos/100.000 pacientes. Conclusion La incidencia de cancer cutaneo en los pacientes tratados con PUVAterapia es superior a la descrita en la poblacion mediterranea.

Introduction: Hand eczema is a frequent disease in adults. Diagnosing the cause of hand eczema is difficult due to different classifications. There is lack of evidence on hand eczema and its causes in children. Material and method: A total of 389 children between 0 and 16 years were identified between 1996 and 2016, from whom 42 (10.8%) with exclusively hand eczema were selected. In all cases a standard battery of epicutaneous patch tests was performed, as well as additional batteries depending on the clinical suspicion. The clinical and epidemiological features of these children were recorded and compared against children with eczema in other locations. Results: The 42 children with hand eczema included 25 (59.5%) girls, and 17 (40.5%) boys, with a mean age of 10.6 ± 3.9 years, and did not differ from that of children with eczema in other locations. The definitive diagnosis after patch-testing was Atopic Dermatitis in 15 cases, Allergic Contact Dermatitis in 14 patients, Endogenous Vesiculous Eczema in 6 cases, Endogenous Hyperkeratotic Eczema in 5 cases, and Irritant Contact Dermatitis in 2 cases. The most frequent allergens detected were thiomersal (9 cases), nickel (5 cases), mercury (5 cases), and cobalt (4 cases). Conclusion: Hand eczema is a common condition in children. The most common cause is atopic dermatitis, although cases of allergic contact dermatitis manifesting as hand eczema are not uncommon. Any child with eczema of hands in whom an allergic cause is suspected should be referred for patch- testing. Resumen: Introducción: El eccema de manos es una forma frecuente de eccema en adultos. Su diagnóstico en ocasiones es complejo debido a la existencia de diferentes clasificaciones diagnósticas. Existen pocos trabajos que estudien el eccema de manos y su clasificación en niños. Material y método: Se ha identificado a 389 niños entre 0 y 16 años remitidos a la Unidad de Alergia Cutánea de nuestro servicio para estudio con pruebas epicutáneas en el periodo 1996-2016. De entre todos los casos se han seleccionado 42 casos con dermatitis localizada exclusivamente en la mano (10,8% de todos los niños remitidos). En todos los casos se realizaron pruebas epicutáneas parchando la batería estándar, así como baterías adicionales en función de la sospecha clínica. Se recogieron datos epidemiológicos (edad, sexo, antecedentes de dermatitis atópica…), así como clínicos (localización de las lesiones). Resultados: De los 42 niños remitidos con dermatitis de la mano, 25 (59,5%) eran niñas y 17 (40,5%) niños. La edad media de los pacientes con dermatitis de la mano fue de 10,6 ± 3,9 años. El diagnóstico definitivo tras la realización de pruebas epicutáneas fue dermatitis atópica en 15 casos, dermatitis alérgica de contacto en 14 pacientes, eccema endógeno vesiculoso en 6 casos, eccema endógeno hiperqueratósico en 5 casos y dermatitis irritativa de contacto en 2 casos. Los alérgenos detectados más frecuentes fueron tiomersal (9 casos), niíquel (5 casos), mercurio (5 casos) y cobalto (4 casos). Conclusión: El eccema de manos es una entidad frecuente en niños. La causa más frecuente es la dermatitis atoípica, aunque no son infrecuentes los casos de dermatitis alérgica de contacto que se manifiestan como eccema de manos. Todo niño con eccema de manos en el que se sospeche una causa alérgica debe ser remitido para realización de pruebas epicutáneas.

Resumen: Introducción: El eccema de manos es una forma frecuente de eccema en adultos. Su diagnóstico en ocasiones es complejo debido a la existencia de diferentes clasificaciones diagnósticas. Existen pocos trabajos que estudien el eccema de manos y su clasificación en niños. Material y método: Se ha identificado a 389 niños entre 0 y 16 años remitidos a la Unidad de Alergia Cutánea de nuestro servicio para estudio con pruebas epicutáneas en el periodo 1996-2016. De entre todos los casos se han seleccionado 42 casos con dermatitis localizada exclusivamente en la mano (10,8% de todos los niños remitidos). En todos los casos se realizaron pruebas epicutáneas parchando la batería estándar, así como baterías adicionales en función de la sospecha clínica. Se recogieron datos epidemiológicos (edad, sexo, antecedentes de dermatitis atópica…), así como clínicos (localización de las lesiones). Resultados: De los 42 niños remitidos con dermatitis de la mano, 25 (60,5%) eran niñas y 17 (40,5%) niños. La edad media de los pacientes con dermatitis de la mano fue de 10,6 ± 3,9 años. El diagnóstico definitivo tras la realización de pruebas epicutáneas fue dermatitis atópica en 15 casos, dermatitis alérgica de contacto en 14 pacientes, eccema endógeno vesiculoso en 6 casos, eccema endógeno hiperqueratósico en 5 casos y dermatitis irritativa de contacto en 2 casos. Los alérgenos detectados más frecuentes fueron tiomersal (9 casos), níquel (5 casos), mercurio (5 casos) y cobalto (4 casos). Conclusión: El eccema de manos es una entidad frecuente en niños. La causa más frecuente es la dermatitis atópica, aunque no son infrecuentes los casos de dermatitis alérgica de contacto que se manifiestan como eccema de manos. Todo niño con eccema de manos en el que se sospeche una causa alérgica debe ser remitido para realización de pruebas epicutáneas. Abstract: Introduction: Hand eczema is a frequent disease in adults. Diagnosing the cause of hand eczema is difficult due to different classifications. There is lack of evidence on hand eczema and its causes in children. Material and method: A total of 389 children between 0 and 16 years were identified between 1996 and 2016, from whom 42 (10.8%) with exclusively hand eczema were selected. In all cases a standard battery of epicutaneous patch tests was performed, as well as additional batteries depending on the clinical suspicion. The clinical and epidemiological features of these children were recorded and compared against children with eczema in other locations. Results: The 42 children with hand eczema included 25 (60.5%) girls, and 17 (40.5%) boys, with a mean age of 10.6 +- 3.9 years, and did not differ from that of children with eczema in other locations. The definitive diagnosis after patch-testing was Atopic Dermatitis in 15 cases, Allergic Contact Dermatitis in 14 patients, Endogenous Vesiculous Eczema in 6 cases, Endogenous Hyperkeratotic Eczema in 5 cases, and Irritant Contact Dermatitis in 2 cases. The most frequent allergens detected were thiomersal (9 cases), nickel (5 cases), mercury (5 cases), and cobalt (4 cases). Conclusion: Hand eczema is a common condition in children. The most common cause is atopic dermatitis, although cases of allergic contact dermatitis manifesting as hand eczema are not uncommon. Any child with eczema of hands in whom an allergic cause is suspected should be referred for patch- testing.

The differential diagnosis of Grover disease (GD) includes pemphigus foliaceus. However, the focal nature of the histologic findings and negative direct immunofluorescent point to the diagnosis of GD. It has been associated with a variety of systemic and cutaneous diseases, but its association with other primary acantholytic disorders has been rarely documented. In this article, we describe the first case of GD in association with pemphigus foliaceus.

Pemphigus foliaceus is an autoimmune bullous disease with autoantibodies against desmoglein 1. Case reports of pemphigus after surgery have also been described, which may simulate an infection of the surgical wound, a contact dermatitis, or even a tumor recurrence. Cytoimmunofluorescence can help to establish a rapid diagnosis.

Eosinophilic fasciitis (EF) is an uncommon fibrosing disease of the fascia with characteristic cutaneous and hematologic manifestations. Although EF is most commonly treated with corticosteroids at the beginning, a considerable number of patients show an inadequate response and hence various therapeutic strategies have been tried, including extracorporeal photopheresis (ECP). We describe the case of a 66-year-old woman with steroid-resistant EF that improved significantly after ECP was added to her treatment regimen. To date, only six cases of this therapeutic strategy have been reported in English literature.

Background Melanocytic acral nevi have a series of distinguishing features, including their location, patient age at onset, clinical progression, and histological findings. In particular, histopathological analysis often reveals a melanocytic acral nevus with intraepidermal ascent of cells (MANIAC nevus), which in some cases can be mistaken for atypia or malignancy. Aim This study describes the clinicopathological characteristics of acral nevi in patients under 18 years old and contrasts the clinical and histological features between MANIAC vs non-MANIAC nevi. Methods This was a retrospective observational study, performed in our department in the decade between January 2007 and January 2017. We included patients younger than 18 years of age who were subjected to the removal of melanocytic acral nevi. Results A total of 70 patients were studied. 54.2% (38/70) were females and 45.8% (32/70) were males. With regard to the type of nevus, 34 were compound, 27 were junctional, and 9 were predominantly intradermal lesions. We identified a total of 41 MANIAC nevi and 29 non-MANIAC nevi. Statistically significant differences between these two groups were identified in nevus size (larger in MANIAC) and the frequency of compound nevi (higher in the MANIAC group), but not in the remainder of the histological parameters studied.

Sarcoidosis is an uncommon systemic granulomatous disease of unknown origin affecting lung, skin, liver, and other tissues. Noncaseating granulomas in the involved organs are the hallmark of this disease. An exaggerated immune response to an unknown antigenic stimulus could play a role in sarcoidosis development. Lung is one of the most frequently involved organs.1 Manifestations range from alveolitis to granulomatous infiltration of alveoli, bronchi, and blood vessels. The end stage of lung sarcoidosis is development of interstitial fibrosis with “honeycombing” of lung parenchyma. Interferon alfa in association with ribavirin is the treatment of choice for hepatitis C. Early treatment of acute hepatitis C with interferon alfa-2b will prevent the development of hepatic cirrhosis, but adverse effects are frequent and often can result in discontinuation of treatment.2 Most frequent adverse effects related to interferon are malaise, fever, arthralgia, and cough. Pulmonary events such as bronchial asthma, bronchiolitis obliterans, and interstitial pneumonitis have been reported. Interferon alfa is also used in the treatment of malignant melanoma, multiple myeloma, hairy-cell leukemia and HIV-associated Kaposi's sarcoma.3 Some cases of sarcoidosis after treatment with interferon alfa have been reported in the literature.4, 5, 6 Improvement of sarcoidosis has been reported with discontinuation of treatment, but in other cases an independent course of disease has been proposed, raising the belief that interferon discontinuation is unnecessary in mild-to-moderate cases of interferon-related sarcoidosis.7 We present the case of a patient with hepatitis C treated with interferon in whom pulmonary sarcoidosis developed. The sarcoidosis was initially believed to be a pulmonary neoplasm, but skin lesions developed that indicated the diagnosis of systemic sarcoidosis. The sarcoidosis finally resolved without discontinuation of interferon alfa.

We report the case of a 32-year-old man with measles in which skin biopsy helped to establish a definitive diagnosis. Follicular involvement is a common histopathologic feature of measles. Multinucleated epidermal and follicular cells are distinctive findings.

Molecular diagnostics are increasingly performed routinely in the diagnosis and management of patients with melanoma due to the development of novel therapies that target specific genetic mutations. The development of next-generation sequencing (NGS) technologies has enabled to sequence multiple cancer-driving genes in a single assay, with improved sensitivity in mutation detection. The main objective of this study was the design and implementation of a melanoma-specific sequencing panel, and the identification of the spectrum of somatic mutations in a series of primary melanoma samples. A custom panel was designed to cover the coding regions of 35 melanoma-related genes. Panel average coverage was 2,575.5 reads per amplicon, with 92,8% of targeted bases covered ≥500×. Deep coverage enabled sensitive discovery of mutations in as low as 0.5% mutant allele frequency. Eighty-five percent (85/100) of the melanomas had at least one somatic mutation. The most prevalent mutated genes were BRAF (50%;50/199), NRAS (15%;15/100), PREX2 (14%;14/100), GRIN2A (13%;13/100), and ERBB4 (12%;12/100). Turn-around-time and costs for NGS-based analysis was reduced in comparison to conventional molecular approaches. The results of this study demonstrate the cost-effectiveness and feasibility of a custom-designed targeted NGS panel, and suggest the implementation of targeted NGS into daily routine practice.

Background Mycosis fungoides (MF) is the most frequent type of cutaneous T cell lymphoma. Its clinicopathological spectrum is wide, and the resulting diversity makes it difficult to establish a differential diagnosis among pityriasis lichenoides (PL), lymphomatoid papulosis (LyP), and atypical MF. Objectives This study describes four patients with longstanding PL-like lesions, in whom clinicopathological correlations contributed towards the establishment of definitive diagnoses of MF. Methods The clinical histories of the four patients were reviewed. Skin biopsies were processed by hematoxylin and eosin staining and immunohistochemical techniques. Disease spread was studied according to laboratory tests, complete blood counts, biochemical parameters, lactate dehydrogenase, lymphocyte populations, and Sezary cells. Thoracoabdominopelvic computed tomography was performed. Results The four patients included two women and two men, aged 35–70 years, all of whom had chronic skin lesions located mainly on the trunk and extremities. In three patients, initial clinicopathological correlations led to the diagnosis of PL; further biopsies were required to diagnose MF. In all patients, the atypical lymphocytic infiltrate showed a lichenoid or perivascular distribution with focal epidermotropism and parakeratosis. All cases proved positive for CD4 and CD3, and negative for CD20, CD8, and CD30. Polymerase chain reaction showed monoclonal-type T cells in one and polyclonal infiltrates in three patients. All patients were initially treated with topical corticosteroids and photochemotherapy. None of the treatments proved completely successful, but subsequent tests have yielded no evidence of disease progression in any patient. Conclusions In patients with clinical features of longstanding PL and histological findings consistent with MF, differential diagnosis must include PL, LyP, and papular MF. As some forms of PL evolve towards MF and PL-like forms of MF, these patients should undergo regular follow-up and repeated biopsies in order to discard diagnoses of atypical forms of MF.

Primary cutaneous neuroendocrine carcinoma (PCNC), previously known as Merkel cell carcinoma (MCC), is a rare tumor of the skin with aggressive behavior and poor prognosis. Typically, PCNC is positive for Cytokeratin-20 (CK20) and negative for Thyroid Transcription Factor-1 (TTF-1). Rarely, CK-20 negative and TTF-1 positive PCNC have been described. We present the case of two patients with skin lesions histologically compatible with MCCs and a behavior characteristic of this disease, but with expression of TTF-1 instead of CK-20. In conclusion, there are increasing reports of TTF1+ CK20− skin lesions without signs of systemic disease which behave clinically and prognostically like a PCNC. The origin of these TTF1 tumors are, to date, unknown.

Background According to the International Society for the study of vascular Anomalies, vascular anomalies are classified as vascular neoplasms and vascular malformations. In some vascular lesions, categorization as a neoplasm or malformation has not been established with confidence so far. In order to further clarify the nosology of verrucous hemangioma, we studied 13 cases. Objective To analyze immunohistochemical characteristics of verrucous hemangiomas in order to gain further insight in its histogenesis. Methods We carried out a retrospective review. Immunohistochemical expression for Wilms tumor 1 (WT1), Glut-1 and D2-40 was performed in 13 cases. Results Immunohistochemistry performed with Glut-1 and WT1 showed positive staining in all lesions. All verrucous hemangiomas lacked D2-40 immunostaining. Conclusions This is the first report in the literature investigating WT1 in verrucous hemangioma in order to further clarify the nosology of this vascular anomaly. Despite the clinical features of verrucous hemangioma, which are similar to those seen in vascular malformations, verrucous hemangioma exhibited an immunoprofile similar to vascular neoplasms, according to WT1 and Glut-1 positivity.

Superficial angiomyxoma is a distinctive cutaneous soft tissue neoplasm that is clinically variable, infrequent, and benign. However, this tumor has a high propensity for local recurrence. There is a known association of angiomyxomas with Carney complex. We report a case of superficial angiomyxoma in a 28-year-old woman who presented with an erythematous, mul-tilobulated nodule on the nipple. This tumor has a predilection for the trunk, head and neck, extremities, and genitalia. The present case, hence, is unusual, because the lesion developed on the nipple. The appearance of a solitary cutaneous angiomyx-oma warrants an examination to rule out the presence of Carney complex. After clinical examination, echocardiogram and en-docrine analyses, Carney complex was ruled out in our patient.

Dermatophytosis is a superficial fungal infection of keratinized tissues. Dermatophytes can cause discomfort but are not usually life threatening. However, the infection can spread and may lead to systemic fungal infections in immunocompromised patients. Currently available diagnostic methods include potassium hydroxide (KOH) testing and periodic acid-Schiff (PAS) staining. However, most diagnostic techniques cannot be performed rapidly; days to weeks may be required for conclusive results. Certain dermatophytes autofluoresce and can be observed under fluorescence microscopy. The authors examined a series of 24 cases of hematoxylin and eosin-stained dermatophytoses using fluorescence microscopy and compared the results with those obtained using PAS staining. The diagnostic performance of fluorescence microscopy was better than that of PAS staining. Fluorescence microscopy allowed the detection of all the cases that were detected using PAS staining. In addition, fluorescence microscopy facilitated the detection of weak fluorescence in 2 cases with ambiguous PAS results. These results support the integration into clinical practice of fluorescence microscopy as a simple and rapid diagnostic tool for evaluating cases of suspected dermatophytosis.

Resumen Introduccion La paniculitis pancreatica o necrosis grasa subcutanea es una dermatosis poco frecuente asociada a patologia pancreatica tanto benigna como maligna. Su histologia es muy sugestiva, siendo la biopsia cutanea diagnostica. Pacientes y metodo Presentamos 7 casos de paniculitis pancreatica, 6 asociados a patologia benigna, siendo la mas frecuente la pancreatitis aguda de etiologia biliar, y un caso asociado a adenocarcinoma pancreatico. En la mayoria de los pacientes la clinica digestiva fue paralela a la cutanea, excepto en el caso asociado a malignidad, en el que se diagnostico la neoplasia un ano despues de iniciar la clinica cutanea. Todos los casos fueron diagnosticados mediante biopsia cutanea. Discusion La importancia de esta entidad radica en que las lesiones cutaneas con frecuencia preceden a las manifestaciones de la afeccion pancreatica, que puede ser asintomatica en el momento del diagnostico. En caso de neoplasia ya diagnosticada nos puede hacer sospechar una recidiva o metastasis.