Search

Aims: The current WHO classification of melanocytic tumours excludes neoplasms showing BRAF or NRAS mutations from the Spitz category. This study aimed to review and reclassify atypical melanocytic tumours with spitzoid morphological features diagnosed between 2009 and 2021 in our hospital after expanding the molecular profile, including BRAF and NRAS mutations in all cases. Methods and results: A total of 71 neoplasms showing spitzoid features (Spitz‐like) and atypia were included. The risk of progression of tumours was first studied by integrating the morphology, immunohistochemistry (p16, Ki67, HMB45 and PRAME) and fluorescence in‐situ hybridisation (FISH) results (melanoma multiprobe and 9p21). In a second step, after expanding the molecular study, including BRAF and NRAS mutational status, the neoplasms were finally classified into four subgroups: atypical Spitz tumour (AST, n = 45); BRAF‐mutated naevus/low‐grade melanocytoma with spitzoid morphology (BAMS, n = 2); Spitz melanoma (SM, n = 14); and BRAF or NRAS mutated melanoma with spitzoid features (MSF, n = 10). Follow‐up of patients revealed uneventful results for AST and BAMS. Only one SM presented lymph node metastasis after 134 months. Conversely, patients with MSF showed an unfavourable outcome: three developed lymph node metastases after a mean time of 22 months, with one patient presenting distant metastasis and dying of the disease 64 months from diagnosis. The progression‐free survival showed significant differences between the four groups of spitzoid tumours (P < 0.001) and between both melanoma subtypes (P = 0.012). Conclusions: The classification and prognostication of atypical neoplasms with spitzoid features requires the integration of histomorphology with the molecular investigation of tumours, which should include BRAF and NRAS mutational status. [ABSTRACT FROM AUTHOR]

Background: Microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) are the two major antineutrophil cytoplasmic antibody (ANCA)‐associated vasculitis (AAV). Objectives: To characterize a homogenous AAV cohort and to assess the impact of clinicopathological profiles and ANCA serotypes on clinical presentation and prognosis. Clinical differences in GPA patients according to ANCA serotype and the diagnostic yield for vasculitis of biopsies in different territories were also investigated. Results: This retrospective study (2000–2021) included 152 patients with AAV (77 MPA/75 GPA). MPA patients (96.1% myeloperoxidase [MPO]–ANCA and 2.6% proteinase 3 [PR3]–ANCA) presented more often with weight loss, myalgia, renal involvement, interstitial lung disease (ILD), cutaneous purpura, and peripheral nerve involvement. Patients with GPA (44% PR3–ANCA, 33.3% MPO, and 22.7% negative/atypical ANCA) presented more commonly with ear, nose, and throat and eye/orbital manifestations, more relapses, and higher survival than patients with MPA. GPA was the only independent risk factor for relapse. Poor survival predictors were older age at diagnosis and peripheral nerve involvement. ANCA serotypes differentiated clinical features in a lesser degree than clinical phenotypes. A mean of 1.5 biopsies were performed in 93.4% of patients in different territories. Overall, vasculitis was identified in 80.3% (97.3% in MPA and 61.8% in GPA) of patients. Conclusions: The identification of GPA presentations associated with MPO–ANCA and awareness of risk factors for relapse and mortality are important to guide proper therapeutic strategies in AAV patients. Biopsies of different affected territories should be pursued in difficult‐to‐diagnose patients based on their significant diagnostic yield. [ABSTRACT FROM AUTHOR]

Copyright of Latin American Policy is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Introduction: HLA sensitization is a growing problem in children awaiting kidney transplantation. In some cases, finding an immunologically compatible donor entails contemplating the option of an ABO incompatible transplant or paired transplant. Methods: Patient with genetic nephrotic syndrome and progressive chronic kidney disease, with a previous thrombosis of a first kidney transplant, resulting hypersensitized and remaining for a long‐time on hemodialysis. Despite a desensitization strategy, family members were incompatible and deceased donation options must be ruled out due to the presentation of donor‐specific antibodies (DSA). After 4 years, the possibility arises to perform a kidney paired transplant with a 62‐year‐old woman with an incompatible blood group. Although the current cytotoxicity‐ and cell‐based crossmatches were negative, history of DSA were recorded. Results: An intensive ABO and HLA desensitization protocol was performed in order to combat the isohemagglutinin antibodies and on the memory‐HLA, based on rituximab, apheresis sessions, and immunoglobulins. Despite the donor being older in terms of pediatric transplantation, the donor‐recipient weight difference, and immunological risk, the transplant was completed successfully. Maintenance of titration of up to 1/2 was confirmed after 3 weeks post‐transplant (IgM and IgG). Kidney biopsy at 2 weeks and 6 months without signs of rejection. The patient is currently 12 months post‐transplant and has not presented any signs of transplant rejection and has proper renal function. Conclusions: Kidney paired transplantation is an excellent solution for hypersensitized children, and ABO incompatibility can be considered to increase their options to find a good donor, without thereby obtaining worse results. [ABSTRACT FROM AUTHOR]

A combined digital follow-up and RCM strategy facilitates the recognition of thin malignant melanomas and reduces unnecessary excisions.15 In the lesions that were explored with RCM, 13 out of 15 lesions had a score that classified them as most probably melanoma. Dermoscopic evaluation of the lesion showed that most of the lesions lacked specific criteria for malignancy, and only 5 of the 20 patients had a TDS score of 5.45 or greater, which suggested a diagnosis of melanoma (Figures 1 and 2). The prognosis of melanoma depends largely on its early recognition and excision. Digital follow-up of high-risk patients allows early diagnosis of these lesions and in vivo confocal microscopy is particularly useful to confirm or suspect a diagnosis of melanoma. [Extracted from the article]

Summary: Carfilzomib has been associated with the development of thrombotic microangiopathy (TMA) in relapsed/refractory multiple myeloma patients, a severe disease with no currently available aetiological treatment. We evaluated the potential role of terminal complement pathway in four patients with carfilzomib‐induced TMA. Membrane attack complex (C5b‐9) deposition on endothelial cells in culture exposed to plasma from patients during the acute phase of the disease suggests complement overactivation as a mechanism of potential endothelial damage in three out of four patients. If confirmed in larger cohorts, C5b‐9 evaluation will allow early identification of patients who could benefit from complement blockade and treatment monitoring. [ABSTRACT FROM AUTHOR]

Background: Primary cutaneous follicular center‐cell lymphoma (PCFCL) is one of the most common types of cutaneous B‐cell lymphoma. Differences in immunohistochemical expression of BCL2 and CD10 antigens along with the presence of t(14:18) translocation in neoplastic cells have been postulated as relevant clues in differentiating PCFCL from cutaneous lesions secondary to a systemic follicular lymphoma (SCFL). The aim of this study is to evaluate the significance and usefulness of these parameters in a large series of patients. Methods: Patients with PCFCL and SCFL diagnosed at three university hospitals in Barcelona, from 2000 to 2015 were reviewed. Clinical, histopathological, immunophenotypical, genetic, and outcome parameters were analyzed. Results: Eighty‐one cases (59 PCFCL and 22 SCFL) were included. There were no significant differences between PCFCL and SCFL cases regarding clinical presentation, site of involvement, or predominant type of skin lesions. Most patients in both groups showed positivity for BCL2 and CD10, but strong expression of BCL2 and CD10 was associated with SCFL cases. Although more frequent in SCFL, a small proportion of PCFCL cases also showed the t(14:18) on FISH analysis. Conclusion: The intensity of BCL2 expression was found to be the single most valuable clue in differentiating PCFCL from SCFL cases on histopathological grounds. [ABSTRACT FROM AUTHOR]

Introduction Craniosynostosis and clavicular hypoplasia, delayed closure of the fontanelle, cranial defects, anal and genitourinary abnormalities, and skin (CDAGS), is an infrequent autosomal recessive entity with only 10 cases reported; no associated gene has been identified so far. Case Report The proband is a 2-year-old Mexican female with brachycephaly, cleft palate, anal malformation with rectovestibular fistula, and clinodactyly of the third toe overlapping the second. At 4 months of age, she developed a disseminated dermatosis with erythematous scaly nummular plaques, elevated keratotic sharp borders with thin to broad flaking, hematic crusts, and keratotic surface in others. The lesions were slightly pruritic and began at the lower limbs with posterior dissemination to the upper limbs, head, and trunk; palms and soles were unaffected. A skin biopsy showed hyperkeratosis, parakeratosis, acanthosis, and perivascular inflammatory infiltration in the upper reticular dermis among other alterations. She also presented mild bilateral neurosensory hypoacusia and enamel dysplasia. Her karyotype was normal. Treatment with topical hydrating creams partially improved the skin lesions at their center, while the sharply keratotic borders remained, giving a clinical resemblance to porokeratosis. Discussion The patient suffers from CDAGS syndrome but has normal development, and feet abnormality was described in only one other patient. The treatment with topical hydrating creams improved the skin lesions at their center, while porokeratotic characteristics persisted. CDAGS remains a diagnostic challenge; a comparison with previously reported cases is discussed. The timely detection of the syndrome will allow early treatment that may improve the condition of the patients. [ABSTRACT FROM AUTHOR]

Oedema of the right hand was also noticeable. gl Histopathology of the first biopsy showed a striking proliferation of dilated capillary vessels along the upper dermis (Figure 2a). On the right side, the second biopsy, after one week, showed less capillaries and a more obvious interstitial infiltrate of immature myeloid cells with a high proliferative index (Ki67), expressing ERG, CD15 and myeloperoxidase (MPO). gl Bone marrow study was subsequently performed. While ERG is a well-known marker of endothelial cells, it can be occasionally expressed in malignant myeloid proliferations.3 Remarkably, the density of the myeloid cellularity clearly increased in the second biopsy, enabling the MC diagnosis. [Extracted from the article]

The article presents a case study of a 49-year-old American male with a past medical history of Addison disease, autoimmune thyroiditis, type II diabetes mellitus and dyslipidemia who was referred to the Dermatology department to evaluate a pruritic nodule on his left nipple. His nipple showed a well-demarcated erythematous eroded nodule that was firm and slightly painful to palpation.. The case presents primary syphilis as a unilateral, solitary, erythematous nodule on the nipple.

The article describes the case of a 21-year-old white, Spanish man with primary cutaneous anaplastic large cell lymphoma simulating an aggressive angiocentric T-cell lymphoma. The man has a three week history of asymptomatic nodules on his arms. Results of his histopathological examination showed diffuse dermal and subcutaneous infiltrate composed of large and pleomorphic cells. He was treated with low-dose methotrexate.

This article describes the case of Mexican female twins with congenital hypertrichosis universalis. It discusses the characteristics of congenital hypertrichosis, the patients' medical history, the results of the molecular cytogenetic analysis of the patients and the treatment given to the patients.

Dermoscopy has become an important tool for evaluating skin lesions in children. We report the dermoscopic findings in a case of Bean syndrome and emphasize the utility of this technique in vascular lesions. [ABSTRACT FROM AUTHOR]

The article presents the case study of a woman in her 30s with the history of high spiking fever, arthralgia, and sore throat. It mentions that she reported the transient erythematous linear lesions being associated with systemic symptoms. It mentions that the lesions appeared at scratching sites. The physical examination revealed linear erythematous urticarial lesions.

The article present a case study of a 54-year-old black man from Guinea-Bissau presented with a 3-month history of progressive nodular skin lesions. It mentions a skin biopsy showed a lymphoid infiltrate in the dermis with epidermotropism in some areas; and also mentions the patient treatment started with cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) plus zidovudine and interferon, but unfortunately died after the second cycle due to a bacterial pneumonia.

Magnetite (Fe3O4) and magnetic core‐shell mesoporous silica (Fe3O4‐mSiO2) nanoparticles are employed to coat cotton fibers using a room temperature water‐based direct electrostatic assembly method with polyelectrolytes. Core‐shell nanoparticles are fabricated by coating super‐paramagnetic magnetite clusters with a mesoporous silica layer (mSiO2) using a surfactant‐templating approach. The Fe3O4 clusters are initially synthesized by a co‐precipitation process. The cotton fibers are modified through a layer‐by‐layer technique using dipping cycles with PDDA and PSS polyelectrolytes solutions to enable further nanoparticles attachment. Transmission electron microscopy (TEM) and scanning electron microscopy (SEM) images provides morphologic information of the synthesized nanoparticles and the modified cotton fibers. Composition and physicochemical properties are studied by powder XRD, FTIR, and SEM‐EDX methods, and thermogravimetric analysis (TGA) are carried out to determine the amount of nanoparticles adsorbed onto cotton samples. The saturation magnetization (MS) of nanoparticles determined by a vibrating sample magnetometer (VSM) is greater than that of the cotton‐nanoparticles nano‐composites. Potential applications of these composite materials can include protective clothing and transdermal drug delivery systems. Cotton with magnetic personality: Cotton fibers are modified with magnetite (Fe3O4) and core‐shell mesoporous (Fe3O4‐mSiO2) nanoparticles using a room temperature water‐based direct electrostatic assembly method with polyelectrolytes. The layer‐by‐layer electrostatic assembly technique is carried out using dipping cycles with PDDA and PSS polyelectrolytes solutions to enable further nanoparticles attachment. [ABSTRACT FROM AUTHOR]

The article presents two case studies related to haemosiderotic dermatofibroma (DF), which revealed a green colour as a novel demoscopic finding. One case involves a 45-year-old woman who had a recurrent pigmented nodule, while another involved a 47-year-old woman who had a progressive enlargement and bleeding after trauma. The significant role of colours in dermoscopy is also discussed.

Eruptive vellus hair cysts are benign lesions that can be difficult to distinguish from other skin conditions, including molluscum contagiosum and acne vulgaris. Diagnosis can be corroborated with histopathology. We emphasize differing dermoscopy features to help distinguish eruptive vellus hair cysts from molluscum contagiosum or acne. [ABSTRACT FROM AUTHOR]

Keratitis-ichthyosis-deafness (KID syndrome) is a syndromes ichthyoses that is clinically and genetically heterogeneous requiring early and long-term multidisciplinary monitoring of affected individuals. A review of the clinical, etiopathogenic and therapeutic aspects is presented of this rare congenital ectodermal disorder., (© 2020 Wiley Periodicals LLC.)