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Background The "rainbow pattern" was initially described as a highly specific dermoscopic feature of Kaposi's sarcoma. Since then, it has been reported in many benign and malignant cutaneous tumors, including few melanomas. Aim To determine the frequency and presentation of this dermoscopic pattern in primary cutaneous melanomas in comparison to other cutaneous tumors. Methods The presence of "rainbow pattern" was evaluated in a sample of 1100 dermoscopic images of different melanocytic and non-melanocytic cutaneous neoplasms. Results Rainbow pattern was observed in 23/245 (9.4%) melanomas and 44/855 (5.1%) non-melanoma neoplasms. Melanomas presenting this feature were thicker than 1 mm (82.6%) and 2 mm (43.0%). Compared to non-melanomas, rainbow pattern was more commonly focal (82.7% vs 36.4%, p=0.001) and associated with more than two dermoscopic structures associated to melanoma (100% vs 9%, p=0.001) CONCLUSION: The "rainbow pattern" is a dermoscopic sign that can occasionally be observed in invasive melanomas. In melanomas, this feature is usually associated to other dermoscopic criteria of melanoma and located in a focal and eccentric area, as opposed to a diffuse and isolated presentation in non-melanoma neoplasms.

Background Many studies have corroborated the association of dipeptidyl peptidase-4 inhibitors (DPP4i) use with bullous pemphigoid (BP). It has been speculated that this drug-induced variant presents with a different clinical spectrum than conventional BP. Objective To determine the prevalence of DPP4i-induced cases of BP and to evaluate whether gliptin-related BP has specific clinicopathological and immunological features. Methods We conducted a retrospective, observational study of BP cases attended at our centre between January 2000 and June 2020. Epidemiological, clinical, histopathological and laboratory data were collected. Results A total of 257 cases of BP were collected; 51 (24.3%) were on treatment with DPP4i. When analysing DPP4i-induced BP cases, generalized BP was the predominant pattern and scalp/mucosal involvement was found in 13 patients. Gliptin-related BP cases were associated to a decrease in the eosinophilic infiltrate (p = 0.000) and both the detection rate and concentration of anti-BP180 IgG were lower (p = 0.004, p = 0.001, respectively) than non-DPP4i cases. Limitations Retrospective, single-centre study. Conclusion Our large DPP4i-induced BP case series has highlighted that DPP4i-induced BP is characterized by generalized lesions and scalp involvement. Lower titres of anti-BP180 antibodies and a decrease in eosinophils infiltrating into the skin may be distinct features of DPP4i-related BP.

T-cell prolymphocytic leukemia (T-PLL) is a T-cell lymphoproliferative disorder that frequently involves the skin. The objective was to describe two cases of T-PLL with cutaneous involvement and to present a review of the literature concerning the clinical characteristics, differential diagnosis and treatment of these patients.1) 79 year-old man, with a previous diagnosis of T-PLL based on a laboratory incidental finding. He had been treated with alemtuzumab, but it had to be interrupted due to recurrent infections. After interrupting the treatment, the patient developed a symmetrical rash on his extremities. The skin biopsy demonstrated TPLL infiltration. 2) 28 year-old man that presented with asthenia and lymphocytosis. He also showed a purpuric rash on his trunk and facial erythema. Histopathology of the skin and bone marrow confirmed the diagnosis of T-PLL with cutaneous involvement.T-cell prolymphocytic leukemia accounts for 2% of mature leukemias in adults. Skin involvement is reported in 20-50% of the patients. The characteristic features are facial involvement, purpuric lesions and symmetry of the rash, although there are atypical manifestations as well. Differential diagnosis includes other T-cell lymphoproliferative disorders with hematologic and skin involvement, such as Sézary syndrome. Patients with T-PLL may show cutaneous infiltration at the moment of debut or relapse of the disease. The skin is an accessible organ for taking samples to study and diagnose these patients.La leucemia prolinfocítica T (LPL-T) es una neoplasia hematológica del grupo de síndromes linfoproliferativos T que con frecuencia produce infiltración cutánea. Se presentan dos casos de LPL-T con afectación cutánea y se revisa la literatura en cuanto a características clínicas, diagnóstico diferencial y tratamiento de estos pacientes.1) Varón de 79 años diagnosticado de LPL-T tras un hallazgo analítico incidental. Tras suspender el tratamiento con alemtuzumab por infecciones recurrentes, comenzó con lesiones cutáneas maculopapulosas eritematopurpúricas que afectaban la raíz de las extremidades. La biopsia cutánea confirmó la infiltración por su enfermedad de base. 2) Varón de 28 años que debutó con astenia y hallazgos analíticos de leucocitosis. Había comenzado además con lesiones purpúricas en el tronco y eritema malar bilateral. El estudio de médula ósea y la biopsia cutánea confirmaron el diagnóstico de LPL-T con infiltración cutánea.La LPL-T corresponde al 2% de las leucemias linfocíticas maduras en los adultos. Entre el 20% y el 50% de los pacientes presentan afectación cutánea, con predominio en la región facial, y son característicos el eritema, la púrpura y la simetría, aunque existen manifestaciones atípicas. El diagnóstico diferencial incluye otros síndromes linfoproliferativos T con afectación cutánea y en sangre periférica, entre los que destaca el síndrome de Sézary. Los pacientes con LPL-T pueden presentar afectación cutánea en el debut o en una recidiva de la enfermedad. La piel representa un órgano accesible para la toma de muestras y para el diagnóstico y el estudio de estos pacientes.

Actualmente existe controversia respecto a los beneficios de realizar linfadenectomía en pacientes de melanoma con una biopsia selectiva de ganglio centinela (BSGC) positiva. La carga tumoral1 mm se ha propuesto como el parámetro mas relevante asociado a una linfadenectomía positiva y un deterioro de la supervivencia libre de enfermedad.Se analizaron los datos de 119 pacientes de melanoma con BSGC positiva atendidos en el periodo entre Junio de 1997 y Junio de 2017. Los pacientes se clasificaron según la carga tumoral en dos grupos: ≤ 1 mm and1 mm.La linfadenectomía resultó positiva en sólo 6 (10%) pacientes con una carga tumoral ≤ 1 mm, y en 23 (37.7%) pacientes con carga tumoral1 mm (p0.001). En análisis univariante, la carga tumoral fue el único factor predictivo de linfadenectomía positiva (OR 5.24 (1.94-14.13)). En análisis multivariante, la carga tumoral fue la única variable independiente de supervivencia específica de melanoma (SEM).Aunque la realización de linfadenectomía debe individualizarse en cada caso, la carga tumoral1 mm puede ser un factor predictivo de la presencia de ganglios no centinelas positivos en piezas de linfadenectomía, y un factor pronostico independiente importante para la SEM.The benefits of complete lymph node dissection (CLND) in melanoma patients with a positive sentinel lymph node biopsy (SLNB) have been recently questioned. Sentinel node (SN) tumor burden1 mm has been proposed as the most reliable parameter associated with positive CLND and poorer disease-free survival.Between June 1997 and June 2017, data from 119 melanoma patients with positive SLNB were analyzed. Patients were classified by SN burden in two groups: ≤ 1 mm and1 mm.CLND was positive in 6 (10%) patients with SN tumor burden ≤ 1 mm and in 23 (37.7%) patients with1 mm (p0.001). In univariable analysis, SN tumor burden was the only predictive factor of positive CLND (OR 5.24 [1.94-14.13]). In multivariable analysis, SN tumor burden was the only independent factor of melanoma-specific survival (MSS).Although CLND should still be considered individually in patients with positive SLNB, SN tumor burden1 mm might be a good predictive factor of additional positive non-sentinel nodes and a strong independent prognostic factor in melanoma-specific survival.

TAMs constitute a large fraction of infiltrating immune cells in melanoma tissues, but their significance for clinical outcomes remains unclear. We explored diverse TAM parameters in clinically relevant primary cutaneous melanoma samples, including density, location, size, and polarization marker expression, in addition, because cytokine production is a hallmark of macrophages function, we measured CCL20, TNF, and VEGFA intracellular cytokines by single-cell multiparametric confocal microscopy. The Kaplan–Meier method was used to analyze correlation with melanoma-specific disease-free survival and overall survival. No significant correlations with clinical parameters were observed for TAM density, morphology, or location. Significantly, higher contents of the intracellular cytokines CCL20, TNF, and VEGFA were quantified in TAMs infiltrating metastasizing compared to non-metastasizing skin primary melanomas (p &lt, 0.001). To mechanistically explore cytokine up-regulation, we performed in vitro studies with melanoma-conditioned macrophages, using RNA-seq to explore involved pathways and specific inhibitors. We show that p53 and NF-κB coregulate CCL20, TNF, and VEGFA in melanoma-conditioned macrophages. These results delineate a clinically relevant pro-oncogenic cytokine profile of TAMs with prognostic significance in primary melanomas and point to the combined therapeutic targeting of NF-kB/p53 pathways to control the deviation of TAMs in melanoma.

Introduccion Actualmente existe controversia respecto a los beneficios de realizar linfadenectomia en pacientes de melanoma con una biopsia selectiva de ganglio centinela (BSGC) positiva. La carga tumoral > 1 mm se ha propuesto como el parametro mas relevante asociado a una linfadenectomia positiva y un deterioro de la supervivencia libre de enfermedad. Material y metodos Se analizaron los datos de 119 pacientes de melanoma con BSGC positiva atendidos en el periodo entre Junio de 1997 y Junio de 2017. Los pacientes se clasificaron segun la carga tumoral en dos grupos: ≤ 1 mm and > 1 mm. Resultados La linfadenectomia resulto positiva en solo 6 (10%) pacientes con una carga tumoral ≤ 1 mm, y en 23 (37.7%) pacientes con carga tumoral > 1 mm (p Conclusion Aunque la realizacion de linfadenectomia debe individualizarse en cada caso, la carga tumoral > 1 mm puede ser un factor predictivo de la presencia de ganglios no centinelas positivos en piezas de linfadenectomia, y un factor pronostico independiente importante para la SEM. Background The benefits of complete lymph node dissection (CLND) in melanoma patients with a positive sentinel lymph node biopsy (SLNB) have been recently questioned. Sentinel node (SN) tumor burden > 1 mm has been proposed as the most reliable parameter associated with positive CLND and poorer disease-free survival. Material and methods Between June 1997 and June 2017, data from 119 melanoma patients with positive SLNB were analyzed. Patients were classified by SN burden in two groups: ≤ 1 mm and > 1 mm. Results CLND was positive in 6 (10%) patients with SN tumor burden ≤ 1 mm and in 23 (37.7%) patients with > 1 mm (p Conclusion Although CLND should still be considered individually in patients with positive SLNB, SN tumor burden >1 mm might be a good predictive factor of additional positive non-sentinel nodes and a strong independent prognostic factor in melanoma-specific survival.

C-ALCL is T-cell neoplasm composed of large cells with CD30 positivity and a variable expression of T-cell markers, with exclusive cutaneous onset and localization1 . Good prognosis and response to treatment characterized C-ALCL with a ten-year-disease-free survival of ~90% very different from that of the systemic forms of ALCL (S-ALCL)2 . S-ALCL are classified as ALK (anaplastic lymphoma kinase) positive (+) or ALK negative (-)2 .

Tumor cells attract and dynamically interact with monocytes/macrophages to subvert their differentiation into tumor-associated macrophages (TAMs), which mainly promote immune suppression and neoplastic progression, but the pathways and microenvironmental cues governing their protumoral deviation are not completely understood. To identify the molecular pathways responsible for TAM differentiation, we screened the biomarkers secreted during melanoma‒macrophage interactions using Quantibody microarrays and RNA sequencing of macrophages. We found that activin A, a member of the transforming GF family, plays an instrumental role in the cross-talk between melanoma cells and monocytes/macrophages, which results in the upregulation of distinct tumor-sustaining genes and the achievement of proinvasive and immunosuppressive functions of TAMs. Blockade of activin reduces the upregulation of part of these genes and prevents the acquisition of protumoral functions, facilitating human melanoma rejection by transferred human lymphocytes in a xenograft mouse model. Remarkably, screening of two independent cutaneous primary melanoma collections showed that activin A is enriched in TAMs and melanoma cells from patients with worse outcomes and constitutes a new and independent prognostic marker. Thus, we identify activin A as a key intermediary in the protumoral and immunosuppressive functions of TAMs, with significant potential as a disease biomarker as well as an immunotherapeutic target.

BACKGROUND T-cell prolymphocytic leukemia (T-PLL) is a T-cell lymphoproliferative disorder that frequently involves the skin. The objective was to describe two cases of T-PLL with cutaneous involvement and to present a review of the literature concerning the clinical characteristics, differential diagnosis and treatment of these patients. CASE REPORTS 1) 79 year-old man, with a previous diagnosis of T-PLL based on a laboratory incidental finding. He had been treated with alemtuzumab, but it had to be interrupted due to recurrent infections. After interrupting the treatment, the patient developed a symmetrical rash on his extremities. The skin biopsy demonstrated TPLL infiltration. 2) 28 year-old man that presented with asthenia and lymphocytosis. He also showed a purpuric rash on his trunk and facial erythema. Histopathology of the skin and bone marrow confirmed the diagnosis of T-PLL with cutaneous involvement. CONCLUSIONS T-cell prolymphocytic leukemia accounts for 2% of mature leukemias in adults. Skin involvement is reported in 20-50% of the patients. The characteristic features are facial involvement, purpuric lesions and symmetry of the rash, although there are atypical manifestations as well. Differential diagnosis includes other T-cell lymphoproliferative disorders with hematologic and skin involvement, such as Sezary syndrome. Patients with T-PLL may show cutaneous infiltration at the moment of debut or relapse of the disease. The skin is an accessible organ for taking samples to study and diagnose these patients.

Extramammary Paget’s disease (EMPD) represents an intraepithelial carcinoma whose diagnosis is often delayed as it can be easily misdiagnosed as a benign inflammatory dermatosis. Surgery is considered to be the standard treatment, but all surgical modalities are associated with high local recurrence rates. We give concern of this disease through the description of a case of primary EMPD in an 83-year-old lady treated with an alternative modality of treatment: the sequential use of topical imiquimod and photodynamic therapy. Topical treatments can be an alternative, and when used in combination, it allows increasing tolerance and, therefore, treatment compliance and satisfactory outcomes.

Intralesional methotrexate (il-MTX) has been reported as a useful therapy in keratoacanthoma (KA) and cutaneous squamous cell carcinoma (cSCC). However, the data available on the histological changes induced by this therapy are very scarce. We conducted a single center, prospective study that included 65 cases of cSCC treated with il-MTX before surgical treatment. Two histological studies were conducted in all patients: before intralesional treatment and after surgical removal. Lesions were assessed longitudinally both clinically and histologically. 60 patients (92.3%) responded to il-MTX treatment. There were no differences regarding aggressive histological features of the cSCC between responder and non-responder patients. All cases showed a chronic inflammatory infiltrate after il-MTX. Intratumoral necrosis areas were frequently observed. All cases showed local fibrosis with fine thickening of collagen bundles. Il-MTX induces a chronic lymphohistiocytic inflammatory reaction in both clinical responder and nonresponder patients. Tumor involution after il-MTX is followed by a fine fibrosis that explains the great cosmetic results and improves the accuracy of the follow-up.

Resumen Introduccion El leiomiosarcoma de piel es una neoplasia maligna de estirpe muscular cuya baja incidencia dificulta el desarrollo de protocolos especificos de diagnostico y manejo terapeutico. Objetivos Describir las caracteristicas clinicas e histopatologicas de una serie de leiomiosarcomas cutaneos primarios y secundarios, junto con su correlacion pronostica. Material y metodos Se realizo un estudio retrospectivo, descriptivo y observacional. Se seleccionaron 17 casos de leiomiosarcoma cutaneo en 12 pacientes, diagnosticados entre el 1 de enero de 2000 y el 31 de diciembre de 2015. Se recogieron sus datos demograficos, caracteristicas clinicas e histopatologicas, evolucion y respuesta al tratamiento. Resultados Se reclutaron 5 varones y 7 mujeres, todos ellos mayores de 50 anos al diagnostico. Se recogieron 4 leiomiosarcomas dermicos (4/17, 23%) en 4 pacientes, 2 leiomiosarcomas hipodermicos (2/17, 11,5%) en 2 pacientes, y 11 metastasis cutaneas de leiomiosarcoma (11/17, 65%) en 6 pacientes. Las localizaciones mas frecuentes fueron cuero cabelludo (7/17, 41%), miembros inferiores (3/17, 17%) y tronco (3/17, 17%). Durante el seguimiento, un 50% de leiomiosarcomas dermicos recidivaron, un 50% de leiomiosarcomas hipodermicos presentaron metastasis a distancia y 5/6 pacientes con metastasis cutaneas de leiomiosarcoma (83%) fallecieron a causa de su enfermedad. Limitaciones Este estudio es una revision retrospectiva de una serie de casos de tamano limitado en un centro unico. Conclusiones El leiomiosarcoma cutaneo es una neoplasia maligna poco frecuente. A la hora de adoptar una actitud diagnostico-terapeutica en estos pacientes debemos tener en cuenta la marcada heterogeneidad pronostica entre sus diferentes subtipos.

Resumen: Introducción: El tumor de Bednar es un sarcoma de bajo grado, infrecuente, considerado como la variante pigmen tada del dermatofibrosarcoma protuberans (DFSP). Objetivo: describir las características clínicas, histopatológicas, el tratamiento y la evolución de un caso pediátrico de esta infrecuente neoplasia. Caso Clínico: escolar de 9 años que consultó por una pápula indurada asintomática, de dos años de evolución en el dorso del cuarto dedo del pie izquierdo. La biopsia incisional de la lesión fue com patibles con un DFSP pigmentado. El estudio inmunohistoquímico mostró positividad intensa para CD34 en toda la lesión, con factor XIIIa negativo. Se complementó el estudio de la pieza histológica con citogenética molecular FISH para el gen PDGFB (22q13.1) el cual reflejó un patrón anómalo en las células tumorales, no así en los melanocitos ni en la piel peritumoral. Se realizó cirugía micrográfica de Mohs diferida con cobertura mediante sustituto dérmico, sin recidiva ni recurrencia tumoral a los 5 años de seguimiento. Conclusiones: El DFSP pigmentado es un sarcoma de bajo grado, que muy infrecuentemente se presenta en pacientes pediátricos. Las variantes clásica y pigmentada deben ser sospechadas ante una lesión papulonodular única, de crecimiento lento y progresivo, con presencia de células fusiformes con patrón estoriforme en la biopsia y con estudio inmunohistoquímico positi vo para CD34. Es una entidad con buen pronóstico, con escaso riesgo de recurrencia y metástasis, si se logra la realización de una extirpación completa.

Bednar tumor is a rare low-grade sarcoma considered the pigmented variant of dermatofibrosarco ma protuberans (DFSP).To describe the clinical and histopathological characteristics, treatment and evolution of this rare neoplasm.A 9-year old female presented with a 2-year history of an indurated, asymptomatic papule on the back of her fourth left toe. The incisio nal biopsy was compatible with pigmented DFSP. The immunohistochemical study showed intense positivity for CD34 throughout the lesion, with negative factor XIIIa. We complemented the study with molecular cytogenetics (FISH) for PDGFB gene (22q13.1) which showed an abnormal pattern in tumor cells, but not in the melanocytes or the peritumoral skin. Delayed Mohs surgery and skin substitute dressing were performed without neoplastic recurrence at 5 years of follow up. Conclu sion: Pigmented DFSP is a low-grade sarcoma that is very rare in pediatric patients. The classical and pigmented variants should be suspected in the presence of a single papulonodular lesion of slow and progressive growth, with presence of spindle cells with storiform pattern in the biopsy and positive immunohistochemical study for CD34. It is an entity with good prognosis, with little risk of recurren ce and metastasis, if complete excision is achieved.

Granulomatous dermatitis has been associated with hematologic disorders, including the myelodysplastic syndromes. We describe the first case of granulomatous dermatitis associated with polycythemia vera, presenting as large erythematous nodules mimicking panniculitis. We also present the seventh case associated with myelodysplasia, with erythematous plaques on the face and neck, similar to a neutrophilic dermatosis. We consider it particularly interesting for dermatologists to be aware of this dermatosis as a nonspecific manifestation of various hematologic disorders. We suggest performing additional tests (complete blood count) to exclude the possibility that the skin manifestations are the initial sign of hematologic disease. Furthermore, we propose using the broader term, granulomatous dermatitis, to refer to these disorders as, although there are more reports of interstitial forms, cases with a more nodular presentation have also been published, and the importance of the diagnosis derives not from the subtype but from the relationship with an underlying disease.

Resumen Las dermatitis de patron granulomatoso se han relacionado con trastornos hematologicos, entre ellos los sindromes mielodisplasicos. En este articulo se describe el primer caso de dermatitis granulomatosa asociado a policitemia vera, en forma de grandes nodulos eritematosos simulando paniculitis, y el septimo caso asociado a mielodisplasia, con placas eritematosas en cara y cuello que recordaban a una dermatosis neutrofilica. Consideramos de especial interes para el dermatologo conocer esta dermatosis como manifestacion no especifica de diferentes trastornos hematologicos, y sugerimos la realizacion de un estudio complementario (hemograma) en el caso de que la clinica cutanea sea el comienzo. Del mismo modo, proponemos el termino mas amplio de dermatitis granulomatosas para denominarlas, puesto que, si bien las formas intersticiales son mas numerosas en la literatura, tambien se han publicado casos mas nodulares, y la importancia de su diagnostico no radica en el subtipo sino en su relacion con un trastorno subyacente.

Intestinal metaplasia (IM) is a gastric cancer precursor lesion (GCPL) and an extremely high risk factor for progression to gastric cancer (GC). Clinical guidelines recommend that patients with extensive IM undergo a gastroscopy every 3 years. However, protein biomarkers that indicate a transition from IM to GC are lacking. Our group recently identified an interferon-alpha (IFN alpha)-responsive gene, Schlafen 4 (Slfn4), in immune cells that correlates with metaplastic changes in Helicobacter-infected mice. We therefore tested the hypothesis that a human homolog of Slfn4, namely, Schlafen 5 (SLFN5), correlates with progression of GCPL to GC. Jurkat T-lymphoid and HL-60 myeloid cell lines were treated with IFN alpha, and SLFN5 mRNA was quantified by quantitative PCR. SLFN5 protein expression in the inflamed gastric mucosa was co-localized to specific immune cell types by immunohistochemistry using CD20, CD2, and MAC2 antibodies. SLFN5 expression was also determined by immunohistochemistry in formalin-fixed paraffin-embedded samples from individuals with non-atrophic gastritis, atrophic gastritis, complete IM, incomplete IM, and GC, respectively. The IFN alpha treatment of Jurkat and HL-60 cells induced SLFN5 mRNA. SLFN5 protein was expressed mainly by T lymphocytes in inflamed gastric mucosa. The highest level of SLFN5 expression was observed in patients with IM that progressed to GC. Receiver operating characteristic curves demonstrated that correlating SLFN5 expression with the histologic diagnosis of IM significantly increased the probability of identifying patients who may progress to GC. In this study population, elevated SLFN5 protein expression in patients with IM correlated with progression to GC.

Papillary thyroid carcinoma is the most common malignant neoplasm of the thyroid gland, accounting for 50-89% of the cases. It usually presents as a well-differentiated, slow-growing, malignant disease with common spread to locoregional lymph nodes and high recurrence rates. In patients with limited cervical lymph node metastases who are not candidates for surgery or radioiodine therapy, percutaneous ethanol injection (PEI) guided by ultrasonography has proven to be a valuable treatment alternative. This article is protected by copyright. All rights reserved.

Introduction Cutaneous leiomyosarcoma is a malignant neoplasm derived from smooth muscle cells. Its low incidence hampers the development of specific protocols for diagnosis and treatment. Objectives To describe the clinical and histopathologic characteristics of a series of primary and secondary cutaneous leiomyosarcomas and to determine how these characteristics correlate with prognosis. Material and methods We performed an observational, descriptive, retrospective study based on 17 cutaneous leiomyosarcomas in 12 patients diagnosed between January 1, 2000 and December 31, 2015. We recorded demographic data, clinical and histopathologic characteristics, outcome, and response to treatment. Results We included 5 men and 7 women, all aged more than 50 years at diagnosis. There were 4 cutaneous leiomyosarcomas (23%) in 4 patients, 2 subcutaneous leiomyosarcomas (11.5%) in 2 patients, and 11 skin metastases of leiomyosarcoma (65%) in 6 patients. The most frequently affected sites were the scalp (41%), lower limbs (17%), and trunk (17%). During follow-up, 50% of the cutaneous leiomyosarcomas recurred, 50% of the subcutaneous leiomyosarcomas presented distant metastases, and 83% of the patients with skin metastases of leiomyosarcoma died of their disease. Limitations Ours was a retrospective review of a small case series at a single center. Conclusions Cutaneous leiomyosarcoma is an uncommon malignant neoplasm. Our approach to diagnosis and therapy must take into account the marked heterogeneity in the prognosis of the various subtypes.

Papular elastorrhexis (PE) is an uncommon elastic tissue disorder clinically characterized by the onset of asymptomatic flesh-colored to whitish papules over the trunk and upper limbs in children. Its etiology remains unknown, although isolated familial cases have suggested that acquired and inherited forms of the disease may exist. We present the case of a 13-year-old boy with human immunodeficiency virus who presented with such lesions after immunological recovery from very low CD4+ counts after introducing highly active antiretroviral therapy. Because neutrophils and macrophages are implicated in elastin metabolism and degradation pathways, immune dysregulation might be another etiologic factor to be considered in this rare disease.

In this edition of DeRmpath & Clinic, we discuss two paediatric patients who presented with a linear eruption.Case 1. A nine-year-old boy presented with a nine-month history of a slow-growing pruritic eruption, characterized by the presence of slightly erythematous flat-topped papules following a longitudinal linear distribution on his abdomen that improved with topical mometasone furoate.Case 2. A four-year-old boy presented with a three-month history of progressive, asymptomatic and centrifugal [...]