Immunofluorescence study of the placenta in a case of pemphigoid gestationis: Pathophysiological bases / Estudio de inmunofluorescencia de la placenta a propósito de un caso de penfigoide gestacional: bases fisiopatológicas

Introducción: El herpes gestationis (HG) es una de las principales dermatosis del embarazo que debe ser reconocida y tratada oportunamente ya que se relaciona con un empeoramiento del pronóstico fetal. Aunque se ha investigado la afectación cutánea, hay escasez de estudios morfológicos y funcionales de la placenta en esta patología. Principales síntomas o hallazgos clínicos: Erupción vesicular eritematosa a las 32+1 semanas de gestación. Diagnósticos principales: HG. Intervenciones terapéuticas y resultados: Inmunogammaglobulina en casos graves refractarios a los corticoides por vía oral con desaparición completa de las lesiones. Conclusión: Hasta donde sabemos, este es el primer caso que reporta un análisis detallado de los depósitos de IgG y C3 en la membrana basal de las vellosidades de la placenta mediante un estudio de inmunofluorescencia. Estos hallazgos podrían relacionarse con el ligero mal funcionamiento de la placenta que puede explicar los efectos neonatales adversos.(AU)

Introduction: Pemphigoid gestationis (PG) is one of the main dermatoses of pregnancy that must be recognized and treated promptly, since it is related to worsening of foetal prognosis. Although skin involvement has been investigated, there is a lack of morphological and functional studies of the placenta in this pathology. Main symptoms and/or clinical findings: Erythematous vesicular rash at 32+1 weeks of gestation. Main diagnoses: PG. Therapeutic interventions and results: Immunogammaglobulin in severe cases refractory to oral corticosteroids with complete disappearance of the lesions. Conclusion: To our knowledge, this is the first case to report a detailed analysis of IgG and C3 deposits in the basement membrane of the placental villi by means of an immunofluorescence study. These findings could be related to a slight malfunction of the placenta that may explain the adverse neonatal effects.(AU)

Diffuse hepatocutaneous hemangiomatosis: an unusual presentation. / Hemangiomatosis hepatocutánea difusa: presentación inusual.

INTRODUCTION: Infantile hemangiomas with multi-organ involvement are rare, and presentation in the form of uncontrollable bleeding is exceptional. CLINICAL CASE: 4-day-old newborn with multiple hepatocutaneous hemangiomas and a purplish vascular lesion in the third finger of the right hand. In the third week of life, the lesion became ulcerated and caused uncontrollable bleeding. Therefore, urgent amputation was required, with a histopathological result of GLUT-1 positive infantile hemangioma, and an architecture compatible with arteriovenous malformation in the deep portion. Imaging tests revealed it was a high-flow lesion. Genetic tests (MAP2KI, RASA 1, EPHB4, GNAQ, and GNA 11) were negative. Patient progression was good, with hepatocutaneous lesions receding and eventually disappearing. DISCUSSION: No explanation has been given yet as to why the same vascular lesion may behave differently in different patients. New mutations may be accountable for this.

INTRODUCCION: Los hemangiomas infantiles con afectación multivisceral son escasos y su presentación en forma de hemorragia incontrolable es excepcional. CASO CLINICO: Recién nacido de 4 días de vida que presentaba múltiples hemangiomas hepatocutáneos y una lesión vascular púrpura-violácea, que abarcaba el tercer dedo de la mano derecha. En la tercera semana de vida, la lesión presentó ulceración y un sangrado incoercible requiriendo amputación urgente, con un resultado histopatológico de hemangioma infantil GLUT-1 positivo, con arquitectura compatible con malformación arteriovenosa en la parte profunda. Las pruebas de imagen mostraron que se trataba de una lesión de alto flujo. La genética (MAP2KI, RASA 1, EPHB4, GNAQ y GNA 11) fue negativa. La evolución del paciente fue buena, con la involución de las lesiones hepatocutáneas hasta su desaparición. COMENTARIOS: La divergencia en el comportamiento de las mismas lesiones vasculares en diferentes pacientes aún no ha encontrado explicación. Es posible que nuevas mutaciones puedan darnos una respuesta.

Hemangiomatosis hepatocutánea difusa: presentación inusual / Diffuse hepatocutaneous hemangiomatosis: an unusual presentation

Introducción: Los hemangiomas infantiles con afectación multivisceral son escasos y su presentación en forma de hemorragia incon-trolable es excepcional.Caso clínico: Recién nacido de 4 días de vida que presentaba múltiples hemangiomas hepatocutáneos y una lesión vascular púrpura-violácea, que abarcaba el tercer dedo de la mano derecha. En la tercera semana de vida, la lesión presentó ulceración y un sangrado incoercible requiriendo amputación urgente, con un resultado histopatológico dehemangioma infantil GLUT-1 positivo, con arquitectura compatible con malformación arteriovenosa en la parte profunda. Las pruebas de imagen mostraron que se trataba de una lesión de alto flujo. La genética (MA-P2KI, RASA 1, EPHB4, GNAQ y GNA 11) fue negativa. La evolución del paciente fue buena, con la involución de las lesiones hepatocutáneas hasta su desaparición.Comentarios: La divergencia en el comportamiento de las mismaslesiones vasculares en diferentes pacientes aún no ha encontrado explicación. Es posible que nuevas mutaciones puedan darnos una respuesta.

Introduction: Infantile hemangiomas with multi-organ involve-ment are rare, and presentation in the form of uncontrollable bleedingis exceptional.Clinical case:4-day-old newborn with multiple hepatocutaneous he-mangiomas and a purplish vascular lesion in the third finger of the righthand. In the third week of life, the lesion became ulcerated and causeduncontrollable bleeding. Therefore, urgent amputation was required, witha histopathological result of GLUT-1 positive infantile emangioma,and an architecture compatible with arteriovenous malformation in thedeep portion. Imaging tests revealed it was a high-flow lesion. Genetic tests (MAP2KI, RASA 1, EPHB4, GNAQ, and GNA 11) were negative.Patient progression was good, with hepatocutaneous lesions recedingand eventually disappearing.Discussion: No explanation has been given yet as to why the samevascular lesion may behave differently in different patients. New muta-tions may be accountable for this.

Granulomas in Dermatopathology: Principal Diagnoses - Part 1. / Granulomas en dermatopatología: principales entidades. Parte I.

This series of 2 articles on dermatopathologic diagnoses reviews conditions in which granulomas form. Part 1 clarifies concepts, discusses the presentation of different types of granulomas and giant cells, and considers a large variety of noninfectious diseases. Some granulomatous diseases have a metabolic origin, as in necrobiosis lipoidica. Others, such as granulomatous mycosis fungoides, are related to lymphomas. Still others, such as rosacea, are so common that dermatologists see them nearly daily in clinical practice.

Granulomas in Dermatopathology: Principal Diagnoses - Part 2. / Granulomas en dermatopatología: principales entidades. Parte II.

Part 2 of this series on granulomatous diseases focuses on skin biopsy findings. Whereas the first part treated noninfectious conditions (metabolic disorders and tumors, among other conditions), this part mainly deals with various types of infectious disease along with other conditions seen fairly often by clinical dermatologists.

Clinical and Histopathologic Characteristics of the Main Causes of Vascular Occlusion - Part I: Thrombi. / Dermatopatología de la oclusión intraluminal vascular: parte I (trombos).

Vascular occlusion has multiple, diverse clinical manifestations, some of which can have grave consequences for patients. The causes of vascular occlusion are also highly variable, ranging from thrombi triggered by the uncontrolled activation of coagulation mechanisms, on the one hand, to endothelial dysfunction or occlusion by material extrinsic to the coagulation system on the other. In a 2-part review, we look at the main causes of vascular occlusion and the key clinical and histopathologic findings. In this first part, we focus on vascular occlusion involving thrombi.

Clinical and Histopathologic Characteristics of the Main Causes of Vascular Occusion - Part II: Coagulation Disorders, Emboli, and Other. / Dermatopatología de la oclusión intraluminal vascular: parteII (coagulopatías, émbolos y miscelánea).

Vascular occlusion has multiple, diverse clinical manifestations, some of which can have grave consequences for patients. It also has a wide variety of causes, including thrombi, which we recently addressed in partI of this review. In this second part, we look at additional causes of vascular occlusion.

Relapsing bullous amyloidosis of the oral mucosa and acquired cutis laxa in a patient with multiple myeloma: a rare triple association.

It is well known that primary systemic amyloidosis [light chain (AL) amyloidosis] is associated with hidden dyscrasia or multiple myeloma. Acquired cutis laxa (cutis laxa acquisita; CLA) has also been described in patients with plasma cell dyscrasias, including multiple myeloma. We report a case in which haemorrhagic oral bullae were the first sign of an undiagnosed primary systemic amyloidosis related to multiple myeloma IgG-λ and previously diagnosed CLA. There is only one report in literature of this rare triple association; however, in that case the patient did not have oral mucosal involvement or bullous amyloidosis.

Kaposi sarcoma secondary to endogenous adrenocorticotropic hormone-dependent Cushing syndrome.

Kaposi sarcoma (KS) is an angioproliferative tumour that develops as a result of an infection by human herpesvirus 8, which is considered a necessary cause but not sufficient. Other factors - genetic, immunological and environmental - might play a role in the development of the disease. We report a case of KS secondary to endogenous Cushing syndrome (ECS) due to a pituitary adenoma, an association that has been reported only once. We also conducted a search through the Medline and PubMed databases for cases involving KS and ECS, finding only three additional cases that shared common clinical and prognostic features with ours. ECS might favour the development of KS due to immunosuppression. Dermatologists and other clinicians should be aware of this association, as it might be an underdiagnosed condition. It also has an important impact on the management of KS, and based on this review it relies on a good prognosis when ECS is well controlled.

Xantoma verruciforme asociado a reactivación de virus de Epstein-Barr / Verruciform Xanthoma Associated with Reactivation of Epstein-Barr Virus

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Bilateral segmental neurofibromatosis diagnosed during pregnancy.

Bilateral segmental neurofibromatosis is a rare subtype of neurofibromatosis type 1 defined by lesions affecting a single segment of the body and crossing the midline, with no systemic involvement. We present a case diagnosed during pregnancy because of the characteristic increase in size of the lesions during this period.

Embolismos sépticos cutáneos tras angioplastia / Septic Emboli to the Skin Following Angioplasty

Los procedimientos vasculares invasivos son en el momento actual ampliamente utilizados para el diagnóstico y tratamiento de muchas patologías del sistema cardiovascular, con un buen perfil de seguridad y eficacia, aunque entrañan potenciales complicaciones que ocasionalmente pueden comprometer la vida del paciente. Presentamos un nuevo caso de pseudoaneurisma infeccioso postangioplastia complicado con embolismos sépticos cutáneos. Se trata de una entidad infrecuente caracterizada por bacteriemia persistente, sepsis sin foco aparente y embolismos sépticos regionales. El análisis histopatológico de las lesiones cutáneas habitualmente permite evidenciar la presencia de cocobacilos Gram positivos y de vasculitis séptica. Es una entidad con una importante morbimortalidad, por lo que el diagnóstico precoz resulta esencial. Por ello, ante lesiones cutáneas tras procedimientos vasculares invasivos deben considerarse en el diagnóstico diferencial no solo los embolismos de colesterol, sino también los embolismos sépticos (AU)

Invasive vascular procedures have good efficacy and safety profiles and are now widely used for the diagnosis and treatment of many cardiovascular disorders. However, they do have potential complications that can occasionally be life-threatening. We present a new case of infectious pseudoaneurysm following percutaneous transluminal coronary angioplasty and complicated by septic emboli to the skin. It is a rare condition characterized by persistent bacteremia, sepsis of unknown origin, and regional septic emboli. Histopathology of the skin lesions typically reveals gram-positive coccobacilli and septic vasculitis. The condition carries a significant morbidity and mortality, making early diagnosis essential. Both cholesterol and septic emboli should be considered in the differential diagnosis of skin lesions after invasive vascular procedures (AU)