Cutaneous Metastasis From Parotid Tumors: A Report of 2 Cases and Literature Review.

ABSTRACT: Cutaneous metastases from parotid tumors are uncommon and imply a poor prognosis. In this article, we report 2 new cutaneous metastasis cases from 2 different parotid malignancies and 42 additional cases from a literature review. Clinical manifestations, localization, and outcomes of skin metastasis from parotid tumors are described. Although infrequent, parotid neoplasms may develop skin metastasis many years after the initial diagnosis, mostly in the head and neck region. Therefore, long-term follow-up and periodic skin examination of these patients is mandatory. Dermatologists and surgeons must have a high index of suspicion when evaluating any skin lesion arising on these patients because cutaneous metastasis from parotid neoplasms generally implies a poor prognosis.

Case for diagnosis. Hyperpigmented and excoriated papules and nodules in a diabetic patient

Abstract Reactive perforating collagenosis is a rare perforating dermatosis clinically characterized by intensely pruritic hyperpigmented papules, plaques, and nodules with a central keratotic plug. Histopathology reveals transepidermal elimination of collagen fibers. Its pathophysiology is still under investigation, but the acquired form has been linked to systemic conditions such as diabetes mellitus and chronic kidney disease. However, it has also been described as a paraneoplastic syndrome. The authors present the case of a 65-year-old diabetic patient in which a myeloproliferative neoplasm was suspected.

Case for diagnosis. Hyperpigmented and excoriated papules and nodules in a diabetic patient.

Reactive perforating collagenosis is a rare perforating dermatosis clinically characterized by intensely pruritic hyperpigmented papules, plaques, and nodules with a central keratotic plug. Histopathology reveals transepidermal elimination of collagen fibers. Its pathophysiology is still under investigation, but the acquired form has been linked to systemic conditions such as diabetes mellitus and chronic kidney disease. However, it has also been described as a paraneoplastic syndrome. The authors present the case of a 65-year-old diabetic patient in which a myeloproliferative neoplasm was suspected.

Melanocytic BAP-1-Mutated Atypical Intradermal Tumor in Two Siblings: A Case Report.

Melanocytic BAP-1-mutated atypical intradermal tumor (MBAIT) is a tumor that appears early on life. It can be the first manifestation of a tumor predisposition syndrome. Prompt diagnosis will allow for the implementation of early screening techniques for associated malignancies. We present a case of 2 siblings with MBAITs and their future management.

[Differential diagnosis and minimally invasive surgery of an antenatal adrenal mass]. / Diagnóstico diferencial y cirugía mínimamente invasiva de un tumor suprarrenal de hallazgo antenatal.

INTRODUCTION: Adrenal masses are uncommon in newborns. The differential diagnosis includes be nign masses (adrenal hemorrhage, extralobar pulmonary sequestration) and malignant ones (neuro blastoma) that may be a finding during an obstetric ultrasound. The use of complementary imaging methods allows a better diagnosis approach during the postnatal period, with implications for the management of these patients. OBJECTIVE: To report the case of a female newborn with diagnosis of an adrenal mass, and to discuss differential diagnoses and management alternatives of adrenal lesions in newborns. CASE REPORT: Two-month-old female infant, referred for adrenal tumor study diagnosed at 22 weeks gestational age. Postnatal ultrasound showed a tumor compatible with neuroblastoma. The patient was asymptomatic, and the laboratory studies showed no relevant findings. The lesion was excised by laparoscopy. A histological study confirmed pulmonary sequestration. CONCLUSIONS: Extralobar pulmonary sequestration should be considered in the differential diagnosis of an adrenal mass in the newborn. Minimally invasive surgery should be the preferred surgical technique choice in these cases, given the technical feasibility and benefits in the recovery and cosmetic issues of the patient.

Diagnóstico diferencial y cirugía mínimamente invasiva de un tumor suprarrenal de hallazgo antenatal / Differential diagnosis and minimally invasive surgery of an antenatal adrenal mass

Resumen: Introducción: Las masas suprarrenales en recién nacidos son infrecuentes. El diagnóstico diferen cial incluye masas benignas (hemorragia suprarrenal o secuestro pulmonar extralobar) y malignas (neuroblastoma), y pueden ser un hallazgo durante la ecografía obstétrica. El uso de imágenes com plementarias en el periodo postnatal permite una mejor aproximación diagnóstica, con implicancias en el manejo de estos pacientes. Objetivos: comunicar el caso de una recién nacida portadora de una masa suprarrenal, discutir los diagnósticos diferenciales y el manejo de lesiones suprarrenales en recién nacidos. Caso Clínico: Lactante de 2 meses de edad, derivada para estudio de tumor supra rrenal de diagnóstico antenatal a las 22 semanas de edad gestacional. El estudio imagenológico con ecografía postnatal mostró un tumor compatible con neuroblastoma. Paciente asintomática, estudios de laboratorios sin hallazgos relevantes. Se realizó resección laparoscópica de la lesión. El estudio histológico confirmó un secuestro pulmonar. Conclusión: El secuestro pulmonar extralobar debe ser considerado en el diagnóstico diferencial de una masa suprarenal del recién nacido. La cirugía mínimamente invasiva debiera considerarse como el abordaje de elección en casos como este, donde existe factibilidad técnica y beneficios en la recuperación y secuelas cosméticas del paciente.

Abstract: Introduction: Adrenal masses are uncommon in newborns. The differential diagnosis includes be nign masses (adrenal hemorrhage, extralobar pulmonary sequestration) and malignant ones (neuro blastoma) that may be a finding during an obstetric ultrasound. The use of complementary imaging methods allows a better diagnosis approach during the postnatal period, with implications for the management of these patients. Objective: To report the case of a female newborn with diagnosis of an adrenal mass, and to discuss differential diagnoses and management alternatives of adrenal lesions in newborns. Case report: Two-month-old female infant, referred for adrenal tumor study diagnosed at 22 weeks gestational age. Postnatal ultrasound showed a tumor compatible with neuroblastoma. The patient was asymptomatic, and the laboratory studies showed no relevant findings. The lesion was excised by laparoscopy. A histological study confirmed pulmonary sequestration. Conclusions: Extralobar pulmonary sequestration should be considered in the differential diagnosis of an adrenal mass in the newborn. Minimally invasive surgery should be the preferred surgical technique choice in these cases, given the technical feasibility and benefits in the recovery and cosmetic issues of the patient.

Very High- and High-Frequency Ultrasound Features of Cutaneous Larva Migrans.

Cutaneous larva migrans is a common infestation among travelers. Although the diagnosis may be suspected clinically, cases can show atypical presentations. We present the ultrasound features of 4 cases at 18 and 70 MHz. Small linear hyperechoic and hyper-refringent subepidermal and intrafollicular structures suggestive of fragments of larvae, hypoechoic dermal and hypodermal tunnels that match with dilatation of lymphatic ducts, and inflammatory dermal and hypodermal ultrasound signs can support the diagnosis. This work suggests that larvae can penetrate the cutaneous basement membrane through the ostia of the hair follicles and potentially disseminate through the dermal and hypodermal lymphatic network.

Autoimmune progesterone dermatitis manifesting as generalized fixed drug eruption

Abstract: Autoimmune progesterone dermatitis is an uncommon, poorly recognized and under-diagnosed catamenial dermatosis associated with hypersensitivity reactions to progestagens. Most cases manifest as urticaria, eczema or erythema multiforme-like. A 26-year-old woman developed violaceous plaques on the groin and abdomen, 4 days after a spontaneous abortion resolved with uterine curettage. The lesions recurred once monthly at the same sites, mimicking a fixed drug eruption. Although the histopathology was compatible with fixed drug eruption, positive intradermal testing and symptomatic improvement after using oral contraceptive pills gave us a clue to the diagnosis.

Autoimmune progesterone dermatitis manifesting as generalized fixed drug eruption.

Autoimmune progesterone dermatitis is an uncommon, poorly recognized and under-diagnosed catamenial dermatosis associated with hypersensitivity reactions to progestagens. Most cases manifest as urticaria, eczema or erythema multiforme-like. A 26-year-old woman developed violaceous plaques on the groin and abdomen, 4 days after a spontaneous abortion resolved with uterine curettage. The lesions recurred once monthly at the same sites, mimicking a fixed drug eruption. Although the histopathology was compatible with fixed drug eruption, positive intradermal testing and symptomatic improvement after using oral contraceptive pills gave us a clue to the diagnosis.

Histiocitosis de células de Langerhans: el "camaleón" de la dermatopediatría / Langerhans cell histiocytosis

La Histiocitosis de Células de Langerhans (HCL) es una neoplasia mieloide de las células dendríticas Langerhans (CDL), caracterizada por acúmulos de células dendríticas mieloides CD207+. Corresponden a un espectro de enfermedades, desde sólo cutáneas a variantes multiorgánicas. El objetivo de este reporte es describir el caso clínico de un paciente pediátrico, con diagnóstico de Histiocitosis de Células de Langerhans, enfatizando el algoritmo clínico. Paciente masculino de 1 año y 5 meses, con antecedentes de otorrea persistente, refractaria a tratamiento, de un año de evolución. Consulta en policlínico de dermatología por "dermatitis severa" desde hace 1 año. Al examen físico se constatan lesiones tipo dermatitis seborreica generalizadas en tronco y cuero cabelludo, intertrigo fisurado, pápulas eritemato-costrosas plantares con petequias y pus en conducto auditivo externo bilateral. Presenta Hemoglobina 9,5 mg/dl, Hematocrito31,9%, leucocitos 12.400, linfocitos 33,3%, plaquetas 920.000, VHS 27. Subpoblaciones linfocitarias: CD3: 34,7%, C4: 22,7%, CD8: 9,7%, CD19:47,8%. HTLV negativo, VIH negativo. Acaro-test negativo. Dermatopatología: Denso infiltrado de células linfomonocíticas en dermis papilar, con ensanchamiento de estas y gran epidermotropismo, con abundante citoplasma eosinófilo con núcleos arriñonados, CD1a y langerina positivo. Recomendamos elevar la sospecha diagnóstica ante un cuadro de dermatitis seborreica generalizada que esta fuera del rango etario característico y en casos de dermatitis refractarias, donde a pesar de un adecuado tratamiento médico, el paciente persiste comprometido.

Langerhans Cell Histiocytosis (HCL) is a myeloid neoplasm of Langerhans dendritic cells (CDL), characterized by accumulations of myeloid dendritic cells CD207 +. They correspond to a spectrum of diseases, from cutaneous to multi-organ variants. The objective of this report is to describe the clinical case of a pediatric patient with diagnosis of, emphasizing the clinical algorithm. Male patient,1 year and 5 months old, with a history of refractory persistent otorrhea, consulted because of long term severe dermatitis. Physical examination revealed generalized seborrheic dermatitis lesions on the trunk and scalp, cleft intertrigo, plantar erythematous-crusted papules with petechiae, and pus in the external auditory canal. Laboratory findings showed: Hemoglobin 9.5 mg / dl, Hematocrit: 31.9%, leukocytes: 12,400, lymphocytes 33.3%, platelets: 920,000, HSV 27. Lymphocyte subpopulations: CD3: 34.7%, C4: 22.7%, CD8: 9.7%, CD19: 47.8%. HTLV negative, HIV negative. Scabies Negative. Dermatopathology: Dense infiltrate of lymphomonocytic cells in the papillary dermis with widening of the papilla and large epidermotropism, cells show abundant eosinophilic cytoplasm with "kidney nuclei", CD1a and langerin were positive. We recommend elevating the diagnostic suspicion in the face of a generalized seborrheic dermatitis that is outside the characteristic age range and in cases of refractory dermatitis, where the patient persists compromised.

[Paniculitis as manifestation of prolonged febrile syndrome: Case report]. / Paniculitis como manifestación de síndrome febril prolongado en pediatría: Caso clínico.

Fever of unknown origin (FUO) is defined as fever over 7 to 10 days without a diagnosis despite a complete initial study. The most frequent causes are infections, autoimmune and tumors. Even though most cases are self-limited there is a minority that has an underlying etiology with an ominous forecast, encouraging a systematized study. OBJECTIVE: To report a rare case of a boy who presented fever of unknown origin associated to panniculitis and was diagnosed of subcutaneous panniculitis-like-T cell lymphoma and to emphasis the importance of a sequential study of FUO, in order to reach a diagnosis in patients who need a timely intervention. CLINICAL CASE: A ten year old boy, previously healthy, presented subcutaneous nodular lesions of 2 month of evolution, located in abdominal region and extremities, given few symptoms, associated with prolonged fever. He was hospitalized for proper study, in first instance infectious and immune causes were discarded and through lesions biopsy the diagnose of subcutaneous panniculitis-like-T cell lymphoma was reached. CONCLUSION: When FUO is diagnosed, most prevalent causes must be discarded. Then, differential diagnosis, such as immune and neoplasic etiologies, have to be considered. If FUO is associated to elemental nodular lesions, biopsy must be indicated early, in order to find potential malignant cases, avoiding therapeutic delay.

Paniculitis como manifestación de síndrome febril prolongado en pediatría: Caso clínico / Paniculitis as manifestation of prolonged febrile syndrome: Case report

El síndrome febril prolongado (SFP) se define en pediatría como la presencia de un episodio febril de al menos 7-10 días de evolución sin diagnóstico etiológico pese a un estudio inicial completo. La etiología más frecuente es la infecciosa, seguida por causas inmunoreumatológicas y neoplásicas. En la mayoría de los casos la evolución suele ser benigna y autolimitada, sin embargo una minoría presenta una causa subyacente con pronóstico sombrío, determinando necesidad de un estudio sistematizado. Objetivo: Presentar caso clínico de un escolar con SFP asociado a paniculitis y enfatizar importancia de estudio secuencial de SFP para pesquisar pacientes que requieren intervención oportuna. Caso clínico: Escolar de sexo masculino, 10 años de edad, previamente sano, quien consultó por cuadro de 2 meses de evolución, caracterizado por aparición de lesiones nodulares en abdomen y extremidades, poco sintomáticas, asociado a fiebre prolongada. Se hospitalizó para estudio, descartando causa infecciosa y reumatológica. Se confirmó diagnóstico de linfoma paniculítico de células T mediante biopsia y análisis histológico e inmunohistoquímico de las lesiones. Conclusiones: Al diagnosticar SFP se debe descartar causas más prevalentes (infecciosa) y luego plantear como diagnóstico diferencial etiología reumatológica y neoplásica. Si SFP se asocia a lesiones elementales nodulares, plantear precozmente la biopsia de modo de pesquisar potencial causa maligna y evitar retraso terapéutico.

Fever of unknown origin (FUO) is defined as fever over 7 to 10 days without a diagnosis despite a complete initial study. The most frequent causes are infections, autoimmune and tumors. Even though most cases are self-limited there is a minority that has an underlying etiology with an ominous forecast, encouraging a systematized study. Objective: To report a rare case of a boy who presented fever of unknown origin associated to panniculitis and was diagnosed of subcutaneous panniculitis-like-T cell lymphoma and to emphasis the importance of a sequential study of FUO, in order to reach a diagnosis in patients who need a timely intervention. Clinical case: A ten year old boy, previously healthy, presented subcutaneous nodular lesions of 2 month of evolution, located in abdominal region and extremities, given few symptoms, associated with prolonged fever. He was hospitalized for proper study, in first instance infectious and immune causes were discarded and through lesions biopsy the diagnose of subcutaneous panniculitis-like-T cell lymphoma was reached. Conclusion: When FUO is diagnosed, most prevalent causes must be discarded. Then, differential diagnosis, such as immune and neoplasic etiologies, have to be considered. If FUO is associated to elemental nodular lesions, biopsy must be indicated early, in order to find potential malignant cases, avoiding therapeutic delay.

Breast Cancer Metastasis Misdiagnosed as an Angiokeratomatous Eruption. An Infrequent Presentation. Case Report.

INTRODUCTION: Cutaneous metastases represent 2% of all metastases. Breast cancer is the most common cause of skin metastases. Schwartz described 8 types of cutaneous breast metastases; one uncommon form is carcinoma telangiectodes (CT), which may resemble angiosarcoma or lymphangioma circumscriptum. However, there are no reports of CT misdiagnosed as eruptive angiokeratomas. CASE REPORT: The authors describe the clinical, histopathologic, and immunohistochemical features of a unique case of CT. Clinical examination revealed an itchy, dark blue-reddish scaly papular eruption that resembled angiokeratomas. The papules were grouped in a zosteriform pattern on the right chest. A biopsy of the papules revealed intravascular thrombi of neoplastic cells and erythrocytes. The neoplastic cells were pleomorphic and with mitotic figures. Immunohistochemical analysis revealed expression of HER-2-neu in neoplastic cells but negative for estrogen and progesterone receptors. The involved vessels were positive for CD31 and negative for podoplanin. This immunoprofile demonstrated intravascular spread of aggressive breast carcinoma. CONCLUSION: CT is an uncommon form of cutaneous metastasis from breast carcinoma with less than 10 cases described in the literature. There are no previous reported cases of CT mimicking angiokeratomas, although lymphangioma circumscriptum-like lesion may occasionally resemble angiokeratomas. A unique clinical presentation of CT is described.

Vasculitis periflexual como manifestación de primoinfección por parvovirus B19: a propósito de un caso / Periplexual vasculitis as a manifestation of primary infection by parvovirus B19: about a case

La infección por parvovirus B19 es común, siendo más recurrente en la edad escolar, invierno y primavera, y su seroprevalencia alcanza hasta el 85% en adultos mayores. La presentación típica de la infección por este virus se conoce como eritema infeccioso (o "quinta enfermedad", destacando la afectación facial característica y la erupción reticulada), sin embargo, se han reportado múltiples manifestaciones dermatológicas de la infección por el virus. Paciente de 17 años, con antecedente de migraña, que desarrolló máculas y pápulas eritematoso-violáceas confluentes con petequias, predominando en pliegues antecubital, poplíteo e inguinal y cara lateral del tórax, con menor compromiso abdominal, espalda y muslos. La biopsia de la piel y los exámenes de laboratorio fueron compatibles con vasculitis leucocitoclástica secundaria a infección por parvovirus B19. Se han descrito diferentes patrones de la manifestación cutánea de la infección por parvovirus B19. La paciente del caso clínico expuesto desarrolló un rash cutáneo que constituyó una superposición entre el patrón vasculítico y el periflexural, presentación poco descrita en la literatura.

Parvovirus B19 infection is common, being more typical among school-age children, with seasonal changes. Infectious erythema (or 'fifth disease') is the classic manifestation of this viral infection; however, multiple dermatological manifestations of the virus infection have been reported. An otherwise healthy 17-year-old female patient, developed confluent erythematous-violaceous macules and papules with petechiae. They mainly involved the antecubital, popliteal and inguinal crease and lateral thorax. The lower abdomen, back and thighs were less involved. The skin biopsy and laboratory workup showed leukocytoclastic vasculitis and positive reaction for parvovirus B19, respectively. A variety of patterns have been described related to the cutaneous manifestation of parvovirus B19 infection. In the present case, the patient developed a cutaneous rash that constituted an overlap between the vasculitic and the periflexural patterns. This variant of parvovirus B19 infection has not been frequently described in literature.

[Radiation organizing pneumonia: report of one case]. / Neumonía en organización por radiación en el paciente con cáncer de mama: caso clínico.

We report a 64-years-old woman who underwent sparing mastectomy with adjuvant radiotherapy for breast cancer. One month after the end of radiotherapy, she presented with malaise, fever, fatigue, cough and migratory bilateral pulmonary infiltrates on serial radiological images. The microbiological studies of broncha alveolar lavage were negative. The patient under went a trans bronchial biopsy and the pathological diagnosis was compatible with an organizing pneumonia presumably associated with radiotherapy. Systemic steroid treatment was successful with rapid and complete resolution of clinical and radiographic manifestations.

Neumonía en organización por radiación en el paciente con cáncer de mama: Caso clínico / Radiation organizing pneumonia: Report of one case

We report a 64 years-old woman who underwent sparing mastectomy with adjuvant radiotherapy for breast cancer. One month after the end of radiotherapy, she presented with malaise, fever, fatigue, cough and migratory bilateral pulmonary infiltrates on serial radiological images. The microbiological studies of broncha alveolar lavage were negative. The patient under went a trans bronchial biopsy and the pathological diagnosis was compatible with an organizing pneumonia presumably associated with radiotherapy. Systemic steroid treatment was successful with rapid and complete resolution ofclinical and radiographic manifestations.