Extrafacial lentigo maligna: A progression model and enhanced diagnostic techniques using dermatoscopy and reflectance confocal microscopy.

Lentigo maligna (LM) is a subtype of lentiginous melanoma confined to the epidermis, which is associated with chronic sun exposure. Its clinical, dermatoscopic, and histopathological diagnosis can be challenging, particularly in the early and advanced stages, requiring appropriate clinicopathological correlation. This article reviews the clinical presentation, diagnosis through noninvasive methods (dermoscopy and confocal microscopy), and provides insights for diagnosis of extrafacial LM through the presentation of four representative clinical cases from different phases of a theoretical-practical progression model. Recognizing these lesions is crucial, as once they invade the dermis, they can behave like any other type of melanoma.

Alopecias: Practical Tips for the Management of Biopsies and Main Diagnostic Clues for General Pathologists and Dermatopathologists.

Accurately diagnosing specific variants of alopecia remains challenging for pathologists, potentially delaying appropriate therapeutic decisions by dermatologists. Issues such as limited knowledge on optimal biopsy types and processing methods add complexity. Understanding the normal histology of hair follicles and their changes throughout the follicular cycle further complicates microscopic evaluation. This paper aims to summarize these characteristics and highlight essential diagnostic clues for pathologists to confidently suggest a diagnosis, therefore playing a key role in alopecia diagnosis. Ongoing education, collaboration with dermatologists, and staying up to date on advancements is crucial for the accurate diagnosis and effective management of different types of alopecia.

Frontal Fibrosing Alopecia: A Histopathological Comparison of the Frontal Hairline with Normal-Appearing Scalp.

Frontal fibrosing alopecia is characterized by the presence of a lymphocytic inflammatory infiltrate around the upper follicle and by perifollicular fibrosis, which results in the destruction of the hair follicle. Recent reports have also found the presence of those findings in clinically unaffected areas. The aim of this report is to perform a deeper analysis of the histopathological features of this apparently unaffected scalp. A cross-sectional study including 52 women with frontal fibrosing alopecia was performed. Two areas were biopsied: the frontal hairline and a normal-appearing scalp area. Sebaceous glands were reduced/absent in 80.8% of the frontal hairline samples compared to 42.3% of the "healthy scalp" samples (p = 0.001). Inflammatory infiltrate was observed in 92.3% of patients in the frontal hairline and in 86.5% of them in the "healthy scalp" area (p = 0.508), although the severity was higher in the former (p = 0.013). Follicular epithelium changes were seen in 70.6% of the frontal hairline biopsies compared to 48.1% of the "healthy scalp" biopsies (p = 0.012). Fibrous tissular changes were noted in 80.8% and 53.8% of the frontal hairline and "healthy scalp" biopsies, respectively (p = 0.003). In conclusion, the histopathological features of frontal fibrosing alopecia are shared by both affected and clinically unaffected areas.

Frontal Fibrosing Alopecia and Sunscreen Use: A Cross-sectional Study of Actinic Damage.

Patients with frontal fibrosing alopecia report higher rates of sunscreen use than control subjects. However, it is not known whether the higher use of sunscreens is a cause or a consequence of the alopecia. A greater use of sunscreens should be associated with a lower incidence of signs of actinic damage. The aim of this study is to assess the presence of actinic damage in patients with frontal fibrosing alopecia. A cross-sectional study was carried out on 101 patients with frontal fibrosing alopecia and 40 control subjects. The presence of actinic damage, in the form of solar lentigines, actinic keratoses, and basal and squamous cell carcinomas, was recorded in both groups, together with sunscreen use. Trichoscopy and skin biopsy were performed on patients. Actinic damage was present more frequently in patients with frontal fibrosing alopecia (69.3%) than in control subjects (50%) (p = 0.031). Patients used sunscreens more frequently than did control subjects (83.2% vs 62.5%, p = 0.008). However, the prevalence of trichoscopic inflammatory signs, peripheral alopecia, and inflammatory infiltrate and sebaceous gland involvement in skin biopsy, were similar in patients who used sunscreens and those who did not use them. In conclusion, patients with frontal fibrosing alopecia had greater actinic damage than did control subjects, and this is hypothesized as a reason for the higher use of sunscreens among patients. Thus, use of sunscreens may not be the trigger for frontal fibrosing alopecia that dermatologists have proposed.

Nevus-associated melanoma: An observational retrospective study of 22 patients evaluated with dermoscopy and reflectance confocal microscopy.

BACKGROUND: The frequency of nevus-associated melanoma (NAM) has been estimated to be 29% of diagnosed melanomas. MATERIALS AND METHODS: This is an observational retrospective study of 22 cases of NAM diagnosed in the Universitary Hospital Alcorcón between September 2011 and 2018. The main objective was to analyze dermoscopic and RCM features of NAM. We also studied if there was an association between any dermoscopic or RCM parameter and Breslow depth. RESULTS: The most frequent dermoscopic characteristics were multicomponent pattern (50%), multifocal pigmentation (45.5%), atypical network (59.1%), and blue-gray regression structures (77.3%). RCM evidenced pagetoid cells in 95.5% melanomas (abundant in 59.1%), non-edged dermal papillae in 86.4%, atypical cells at the dermal-epidermal junction in 90.9%, and atypical junctional nesting in 81.8%. Deeper Breslow index was associated with red color (mean Breslow 0.65 vs 0.37 in melanomas without red, P = 0.035), shiny white streaks (0.85 vs 0.38, P = 0.041), abundant pagetoid cells (0.68 vs 0.26, P = 0.017), and non-edged papillae (0.59 vs 0.00, P = 0.014). CONCLUSION: RCM is a valuable tool for diagnosing NAM. Even it is very difficult to differentiate NAM from DNM both with dermoscopy and RCM, RCM can help us to detect remnants of a preexisting nevus and estimate Breslow depth.

Key dermoscopic signs in the diagnosis and progression of extrafacial lentigo maligna: Evaluation of a series of 41 cases.

BACKGROUND/OBJECTIVES: Lentigo maligna is usually located on the face. Extrafacial lentigo maligna is less common, and diagnosis of early forms is very difficult. Confocal microscopy of facial and extrafacial lentigo maligna shares the same features (abundant dendritic cells and generalised atypical junctional thickenings) and helps us to identify the dermoscopic features of extrafacial lentigo maligna. METHODS: We analysed dermoscopic and clinical features of 41 lesions diagnosed by confocal microscopy of extrafacial lentigo maligna confirmed on histology to identify dermoscopic signs of early lesions. RESULTS: Erased areas on dermoscopy were the clue to diagnose early lesions. At the borders of these areas, very small, round or triangular structures were found. At the lesion periphery, dermoscopy revealed a fine reticular pattern that helped to identify them as a melanocytic lesion. A progressive increase of the number and size of erased areas was accompanied by the appearance of various angulated structures around them (angulated lines, zig-zag structures or polygonal structures). Analysis of invasive lesions revealed very large erased areas containing white lines and atypical vascularisation. CONCLUSIONS: We have identified the dermoscopic early features and signs of progression by examining the dermoscopic and reflectance confocal microscopy findings of early and invasive extrafacial lentigo maligna.

Combined in vivo reflectance confocal microscopy and digital dermoscopy for follow up of patients at high risk of malignant melanoma: A prospective case series study.

Digital dermoscopy (DD) follow up is a useful strategy for monitoring patients at high risk of melanoma. Reflectance confocal microscopy (RCM) is a valuable second-level examination after dermoscopy for the evaluation of difficult to diagnose lesions. The aim of this study was to assess the value of RCM in routine DD monitoring of patients at high risk of melanoma. In this prospective study, performed over 22 months, changing melanocytic lesions were detected by DD and excised. RCM imaging was performed before surgical excision of all the lesions, and histopathology used as the gold standard diagnostic test. Eighty-seven lesions, including 11 thin melanomas, were studied. Dermoscopic evaluation at follow up revealed a significant association between melanoma and asymmetry in two axes (P = 0.035). Enlargement and other changes in structure or color did not significantly differ between nevi and melanomas. Widespread pagetoid cells, non-edged papillae, irregular and dishomogeneous junctional clusters, and sheet-like structures were significantly associated with malignancy (P < 0.001). RCM allowed accurate diagnosis of melanoma in 10 of 11 cases (90.9%). The remaining case was classified as a dysplastic nevus. Forty-six lesions (52.8%) in which RCM revealed no atypia were deemed unnecessarily removed. This study was limited by sample size. In conclusion, combined DD and RCM facilitates the recognition of thin malignant melanomas and reduces unnecessary excisions.

Muckle-Wells Syndrome: A Case Report with an NLRP3 T348M Mutation.

Autoinflammatory syndromes are a recently described group of conditions caused by mutations in multiple genes that code for proteins of the innate immune system. Cryopyrin-associated periodic syndromes are autoinflammatory diseases comprising three clinically overlapping disorders: familial cold urticaria syndrome, Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease. MWS is characterized by a moderate phenotype with fever, rash, arthralgia, conjunctivitis, sensorineural deafness, and potentially life-threatening amyloidosis. We report a 5-year-old girl with MWS that manifested as a recurrent skin rash without fever episodes or intracranial hypertension with papilledema. Genetic analysis revealed a T348M mutation of the NLRPR 3 gene in the patient and her mother. She was successfully treated with the interleukin-1ß antagonist receptor anakinra.

[Sentinel lymph node biopsy in melanoma: our experience over 8 years in a universitary hospital]. / Biopsia selectiva del ganglio centinela en melanoma: experiencia durante 8 años en un hospital universitario.

BACKGROUND: Since the introduction of sentinel lymph node biopsy, its use as a standard of care for patients with clinically node-negative cutaneous melanoma remains controversial. Our experience of sentinel lymph node biopsy for melanoma is presented and evaluated. MATERIAL AND METHODS: A cohort study was conducted on 69 patients with a primary cutaneous melanoma and with no clinical evidence of metastasis, who had sentinel lymph node biopsy from October-2005 to December-2013. Sentinel lymph node biopsy was identified using preoperative lymphoscintigraphy and subsequent intraoperative detection with gamma probe. RESULTS: The sentinel lymph node biopsy identification rate was 98.5%. The sentinel lymph node biopsy was positive for metastases in 23 patients (33.8%). Postoperative complications after sentinel lymph node biopsy were observed in 4.4% compared to 38% of complications in patients who had complete lymphadenectomy. CONCLUSION: The sentinel lymph node biopsy in melanoma offers useful information about the lymphatic dissemination of melanoma and allows an approximation to the regional staging, sparing the secondary effects of lymphadenectomy. More studies with larger number of patients and long term follow-up will be necessary to confirm the validity of sentinel lymph node biopsy in melanoma patients, and especially of lymphadenectomy in patients with positive sentinel lymph node biopsy.

Estudio de cohorte retrospectivo de pacientes diagnosticados de cáncer de tiroides del área suroeste de Madrid. Factores pronósticos en el cáncer diferenciado de tiroides / No disponible

Objetivos Análisis de las características clínicas e histopatológicas de los pacientes con cáncer de tiroides en el área suroeste de Madrid e identificación de los factores de mal pronóstico en el subgrupo de carcinoma diferenciado de tiroides (CDT) del epitelio folicular. Pacientes y métodos .Estudio retrospectivo de una cohorte de cáncer de tiroides de nuestro hospital entre 1998-2009. Las variables clínicas, quirúrgicas e histopatológicas significativas se incluyeron en modelos de regresión de Cox y logística para la identificación de factores pronósticos de muerte, recidiva y persistencia de enfermedad. Resultados Se incluyeron 150 pacientes con mediana de edad 49 años y mediana de seguimiento de 5,4 años. Los subtipos histológicos fueron: carcinoma papilar (86%), carcinoma folicular (6,6%), carcinoma medular (4%), carcinoma pobremente diferenciado (2,7%) y carcinoma anaplásico (0,7%).Al final del estudio: 68% curación, 3,3% muertos (mortalidad por cáncer tiroideo 1,3%), 1,3% pérdida de seguimiento, 6,7% con enfermedad bioquímica persistente, 2,7% con enfermedad clínica persistente y 18% pendiente de evaluación. El mejor modelo pronóstico para recidiva de CDT fue el estadiaje TNM (estadio ii-iv frente a i: HR 5,9, 95% IC 1,3-26,6) y para persistencia de enfermedad o muerte el estadiaje clínico de la ETA (alto riesgo frente a bajo/muy bajo riesgo: OR 9,2, 95% IC 2,6-33,2)Conclusiones En nuestro estudio la mortalidad y persistencia clínica de enfermedad fueron bajas. La clasificación de pacientes con CDT según estadiaje de la ETA fue un buen factor predictor de enfermedad persistente o muerte (AU)

Objectives To analyze the clinical and histopathological features of patients with thyroid cancer in the southwest Madrid area and to identify poor prognostic factors in the subgroup with differentiated thyroid carcinoma (DTC) of the follicular epitelium. Patients and methods A retrospective cohort study of patients diagnosed with thyroid cancer at our hospital from 1998 to 2009. Significant clinical, surgical, and histopathological variables were included in Cox proportional hazard and logistic regression models to identify baseline factors predicting for death, recurrence, and persistent disease in DTC. Results A total of 150 patients with a median age of 49 years and a median follow-up of 5.4 years were enrolled. Histological subtypes were: papillary carcinoma (86%), follicular carcinoma (6.6%), medullary carcinoma (4%), poorly differentiated carcinoma (2.7%), and anaplastic carcinoma (0.7%). At the end of the study, 68% of patients were cured, 3.3% had died (disease-specific mortality, 1.3%), 1.3% were lost to follow-up, 6.7% had persistent biochemical disease, and 2.7% persistent clinical disease, while 18% of patients were pending assessment. The best prognostic model for DTC recurrence was TNM staging (stage II-IV vs. I: HR 5.9, 95% CI 1.3-26.6), while the best model for persistent disease or death was ETA clinical staging (high risk vs. low or very low risk: OR 9.2, 95% CI 2.6-33.2).Conclusions In our study, disease-specific mortality and persistent clinical disease were low. Classification of DTC patients based on ETA staging after initial treatment was a good predictor of persistent disease or death (AU)

[A retrospective cohort study of patients diagnosed of thyroid cancer in the southwest Madrid area. Predictive factors in differentiated thyroid cancer]. / Estudio de cohorte retrospectivo de pacientes diagnosticados de cáncer de tiroides del área suroeste de Madrid. Factores pronósticos en el cáncer diferenciado de tiroides.

OBJECTIVES: To analyze the clinical and histopathological features of patients with thyroid cancer in the southwest Madrid area and to identify poor prognostic factors in the subgroup with differentiated thyroid carcinoma (DTC) of the follicular epitelium. PATIENTS AND METHODS: A retrospective cohort study of patients diagnosed with thyroid cancer at our hospital from 1998 to 2009. Significant clinical, surgical, and histopathological variables were included in Cox proportional hazard and logistic regression models to identify baseline factors predicting for death, recurrence, and persistent disease in DTC. RESULTS: A total of 150 patients with a median age of 49 years and a median follow-up of 5.4 years were enrolled. Histological subtypes were: papillary carcinoma (86%), follicular carcinoma (6.6%), medullary carcinoma (4%), poorly differentiated carcinoma (2.7%), and anaplastic carcinoma (0.7%). At the end of the study, 68% of patients were cured, 3.3% had died (disease-specific mortality, 1.3%), 1.3% were lost to follow-up, 6.7% had persistent biochemical disease, and 2.7% persistent clinical disease, while 18% of patients were pending assessment. The best prognostic model for DTC recurrence was TNM staging (stage II-IV vs. I: HR 5.9, 95% CI 1.3-26.6), while the best model for persistent disease or death was ETA clinical staging (high risk vs. low or very low risk: OR 9.2, 95% CI 2.6-33.2). CONCLUSIONS: In our study, disease-specific mortality and persistent clinical disease were low. Classification of DTC patients based on ETA staging after initial treatment was a good predictor of persistent disease or death.

An unusual adverse effect of nadroparin injections: Calcinosis cutis.

In 1988, Tumiati et al described the first case of calcinosis cutis related to a calcium-containing heparin. Since then, only 18 cases have been reported in the literature; they usually have an altered calcium-phosphate product, an elevated parathyroid hormone (PTH), or both. We report a 33-year-old patient who developed calcinosis cutis at sites of nadroparin injections without any disturbance of calcium-phosphate product, PTH, or vitamin D. The pathogenesis of calcinosis cutis secondary to nadroparin injections remains controversial; Proposed causes included metastatic, dystrophic, iatrogenic, or multifactorial etiologies. This is the first case of multiple nodules of calcinosis cutis without alterations of calcium-phosphate product, PTH, or vitamin D, which supports an iatrogenic mechanism. We also suggest that calcinosis cutis could be more frequent than we thought and is probably an underdiagnosed entity.

Validity of clinical diagnosis in dermatology.

OBJECTIVE: We evaluated the accuracy of the clinical diagnostic process performed by dermatologists. MATERIAL AND METHODS: We conducted a cross-sectional study, based on a retrospective search of data at a dermatology outpatient department. Fifteen skin diseases were selected. A group of patients with those diseases at their first outpatient visit and a further histopathological study were included. The accuracy of any given clinical diagnosis was studied taking the histopathological diagnosis as the gold standard. Validity indices were calculated. RESULTS: We studied 2188 patients. Skin diseases were more prevalent among women (p < 0.05). While the clinical diagnostic process displayed a high level of validity in melanocytic naevi and basal cell carcinoma, a poor level was nevertheless observed for Bowen's disease (sensitivity 22.8%, specificity 96.5%). The most frequent sites of appearance of dermatological problems were face and trunk. CONCLUSIONS: Evaluation of diagnosis in pathologies having an available "gold standard" enables the accuracy of clinical diagnoses to be verified. The validity of clinical diagnosis was high.

Tumor compuesto de intestino grueso: adenocarcinoma mucinoso y carcinoma neuroendocrino de célula grande. A propósito de un caso / A case report of a composite tumour of the large intestine: Mucinous adenocarcinoma with large cell neuroendocrine carcinoma

IntroducciónLos tumores compuestos primarios del tracto intestinal son infrecuentes y representan entre 2,5–20% de estas neoplasias. Se caracterizan por la presencia de un componente glandular y otro neuroendocrino, entremezclados en proporción similar. La mayoría de los casos descritos incluyen un componente de estirpe glandular de adenoma o adenocarcinoma convencional y un componente de carcinoide o carcinoma neuroendocrino de célula pequeña.Caso clínicoPresentamos un tumor compuesto de intestino grueso con componentes que no han sido descritos hasta ahora: un adenocarcinoma mucinoso con presencia de células en anillo de sello y un carcinoma neuroendocrino de célula grande.ConclusiónEl tratamiento de este tipo de tumores es el mismo que el que se emplea para los adenocarcinomas convencionales pero el componente endocrino les confiere un peor pronóstico(AU)

IntroductionPrimary composite tumours of the intestine are rare and are characterised by the presence of glandular and neuroendocrine components occurring in almost equal proportions. The majority of reported cases include conventional type adenoma or adenocarcinoma and carcinoid or small cell neuroendocrine carcinoma.Case reportA composite tumour of the large intestine with as yet unreported components is described. Mucinous adenocarcinoma with signet-ring cells and large cell neuroendocrine carcinoma were found.ConclusionsAlthough the treatment of these tumours is similar to that of conventional adenocarcinomas, the presence of a neuroendocrine component worsens the prognosis(AU)

Hiperqueratosis focal acral / Focal acral hyperqueratosis

Las lesiones papulosas crateriformes en los bordes de las manos y/o pies constituyen una manifestación clínica común de muchas entidades que se agrupan bajo el término de acroqueratodermias marginales. Una de ellas es la hiperqueratosis focal acral, descrita en ocasiones bajo el término de acroqueratoelastoidosis. Aportamos el segundo caso descrito en la literatura española bajo el término de hiperqueratosis focal acral (AU)