Foreign body granuloma reaction after endovascular therapy of an unruptured right frontal arteriovenous malformation.

Foreign body reactions after endovascular procedures for brain arteriovenous malformations are extremely rare. We report the case of a cerebral foreign body granuloma reaction after embolization of a frontal arteriovenous malformation with Onyx. A previously treated 36-year-old man underwent re-embolization of a residual and recurrent unruptured right frontal vascular malformation with Onyx. The post-procedural imaging revealed a right frontotemporal heterogeneously enhancing expansive lesion associated with a residual malformation. Following microsurgical resection, the histopathological examination of the expansive lesion revealed basophilic foreign body like deposits adjacent to multi-nucleated giant cells, highly compatible with cerebral foreign body granulomas reaction to Onyx. The clinical and radiological follow-up of the patient was favorable after complete resection of the lesions.

Regulatory T cells and IgE expression in duodenal mucosa of Strongyloides stercoralis and human T lymphotropic virus type 1 co-infected patients.

BACKGROUND: Strongyloides stercoralis is an intestinal nematode unique in its ability to replicate in the human host, allowing ongoing cycles of autoinfection, persisting for decades within the same host. Although usually asymptomatic, overwhelming infections can occur in Strongyloides and HTLV-1 co-infected individuals (SS/HTLV-1). Regulatory T cells (Tregs) are able to blunt specific Th2 responses necessary to control the parasite. We previously reported that peripheral blood Tregs are increased in SS/HTLV-1 and correlate with low Th2 responses. We hypothesized that Tregs are also increased at the site of infection in duodenal mucosa. METHODS: Paraffin embedded duodenal biopsies were obtained from 10 SS/HTLV-1 patients, 3 controls with non-parasitic chronic duodenitis, and 2 healthy controls. Immunohistochemistry was performed using monoclonal antibodies against human CD3, CD8, IgE and FoxP3. The number of cells were counted using a conventional light microscope. The number of CD3+, CD8+, FoxP3+ and IgE positive cells per 0.35 mm2 was measured using ImagePro Plus software comparing areas adjacent or distant from parasite material. RESULTS: In patients with SS/HTLV-1, T lymphocyte counts and CD8+ cells were lower in areas adjacent to the parasite compared to non-adjacent areas (CD3+: adjacent: 6.5 [Interquartile range (IQR: 2.8-12.3)]; non-adjacent: 24.5 [IQR: 20.9-34.4]; Mann-Whitney p = 0.0003; CD8+: adjacent: 4.5 [IQR: 2.3-11.8]; non-adjacent: 21 [IQR: 15.3-42.9]; Mann-Whitney p = 0.0011). Tregs cells in the intestines (FoxP3+ expressing cells) were increased in patients with SS/HTLV-1 compared with patients with chronic duodenitis (SS/HTLV-1: 1.5 [IQR: 0.7-2.3]; duodenitis controls: 0 [range 0-0.7]; healthy controls: 0; Mann-Whitney p = 0.034). There was also a trend towards fewer eosinophils adjacent to the parasites. Among SS/HTLV-1 patients the number of IgE expressing cells was increased for in areas not adjacent to the parasite compared to non-adjacent areas (ANOVA, p = 0.001). CONCLUSIONS: Our data shows increased Treg cell numbers localized adjacent to the parasites in the duodenum SS/HTLV-1 patients. In addition, other T lymphocytes and IgE expressing cells were decreased adjacent to the parasites, suggesting an important role for Tregs in down-regulating local parasite effector responses.

Pachyderma in Primary Cutaneous NK and T-Cell Lymphoma and Leukemia Cutis.

BACKGROUND: Pachyderma is defined as severely thickened skin with deep folds and is occasionally observed with primary cutaneous NK and T-cell lymphoma (pCNKTCL), primary cutaneous B-cell lymphoma (pCBCL), and leukemia cutis (LC). AIM: To describe the clinical, histological, and therapeutic particularities of a series of pCNKTCL, pCBCL, and LC patients with pachyderma. RESULTS: In a series of pCNKTCL (n = 70), pCBCL (n = 12), and LC (n = 2) patients followed up during 9 years, 6 cases of pachyderma were observed. Pachyderma occurred on the arms (n = 2), thighs (n = 1), forehead (n = 1), and face (n = 2). The mean age of the patients was 69 years (51-82). The stages were erythrodermic (T4) mycosis fungoides (MF) (n = 1), folliculotropic MF (FMF) (n = 2), classic (T2) MF (n = 2), and chronic myeloid leukemia (n = 1). The erythrodermic MF patient with acute pachyderma on the right arm responded rapidly to oral steroids. The other cases were indolent, appeared progressively, and were highly treatment resistant. Histology revealed dense dermal neoplastic infiltration. The immunohistological profile of the pachydermic lesions was similar to common MF and LC. CONCLUSION: Pachyderma is an atypical manifestation of MF and LC and may occur on the face (FMF) or the extremities (MF). The rapidly appearing pachyderma may be transitory and responds readily to oral steroids.

Congenital Degos Disease: Case Report and Dermoscopic Findings.

Pediatric Degos disease is rare, with only 36 cases reported in the medical literature. Classically the diagnosis has been established according to pathognomonic histopathologic findings, but when these features are not present, there may be a delay in diagnosis. We report the second congenital case of Degos disease, highlighting the clinical and dermoscopic findings.

Compresión del plexo lumbosacro por plasmocitoma: mieloma múltiple de cadenas ligeras tipo lambda / Compression of the lumbosacral plexus by light chain lambda plasmacytoma: multiple mieloma

Se presenta el caso de una mujer de 51 años que desarrolló paraparesia y una masa abdominal palpable más pérdida de peso en el lapso de cuatro meses. La masa destruía la 5ª vértebra lumbar y se extendía a los tejidos blandos adyacentes. En suero se halló un patrón de gamapatía monoclonal por cadenas ligeras de tipo lambda. La punción aspiración de la masa reveló un plasmocitoma. El paciente recibió radiación y quimioterapia. Fue dada de alta con disminución de la paraparesia. (AU)

A 51 year-old woman developed paraparesis, a palpable abdominal mass and weight loss during the last four months. A mass that was destroying the 5th lumbar vertebra and invading the adjacent soft tissues was found. A monoclonal gamopathy pattern in serum due to light chain type lambda was shown. The patient received radiation plus chemotherapy. She was discharged with amelioration of paraparesis. (AU)

[Mucocutaneous leishmaniasis in a HIV infected patient. Case report]. / Leishmaniasis mucocutánea en un paciente con infección por VIH.

INTRODUCTION: Leishmaniasis is an endemic Andean vector-borne- tropical disease in Peru, whose mucocutaneous clinical presentation is rare. Leishmaniasis can occur in co-infections with HTLV-1 virus and HIV. We describe a case of L. mucocutaneous in a patient infected with HIV, with a history of cutaneous leishmaniasis with inadequate treatment 20 years ago. He was treated with stibogluconate with adequate response to treatment and regression of lesion after 4 weeks. Mucocutaneous leishmaniasis and HIV coinfection is rare and its clinical presentation may be atypically. It is important to consider it in patients coming from endemic areas and with a history of a previous cutaneous clinical presentation.

Leishmaniasis mucocutánea en un paciente con infección por VIH: Case report / Mucocutaneous leishmaniasis in a HIV infected patient

Introduction: Leishmaniasis is an endemic Andean vector-borne- tropical disease in Peru, whose mucocutaneous clinical presentation is rare. Leishmaniasis can occur in co-infections with HTLV-1 virus and HIV. We describe a case of L. mucocutaneous in a patient infected with HIV, with a history of cutaneous leishmaniasis with inadequate treatment 20 years ago. He was treated with stibogluconate with adequate response to treatment and regression of lesion after 4 weeks. Mucocutaneous leishmaniasis and HIV coinfection is rare and its clinical presentation may be atypically. It is important to consider it in patients coming from endemic areas and with a history of a previous cutaneous clinical presentation.

La leishmaniasis es una enfermedad metaxénica andino-tropical, considerada endémica en Perú. Su forma mucocutánea es poco frecuente. Puede presentarse en coinfección con los virus HTLV-1 y VIH. Se describe un caso de leishmaniasis mucocutánea en un paciente infectado con VIH, con antecedente de leishmaniasis cutánea con tratamiento incompleto 20 años atrás. Es tratado con estibogluconato sódico por 30 días, con adecuada respuesta y regresión de la lesión a las cuatro semanas. La coinfección de leishmaniasis mucocutánea y VIH no es frecuente. Las manifestaciones de leishmaniasis pueden no presentarse de forma típica en pacientes con VIH. Se debe considerar la procedencia de la zona endémica y/o el antecedente de haber presentado la forma cutánea previamente.

Fibrohistiocitoma maligno cutáneo con metástasis satélites / Malígnant fibrous histiocytoma with satellite metastasis

El fibrohistiocitoma maligno es un tumor subcutáneo de larga evolución y localizado principalmente en extremidades. Se presenta el caso de una paciente de 77 años con una tumoracián nodular de superficie ulcerada con múltiples pápulas perilesionales en su pierna derecha. Se realizó la confirmación histopatológica y de inmunohistoquimica compatible con Fibrohistiacitoma maligno asociado a metástasis cutáneas satélites.

Malignant fibrous histiocytoma is a subcutaneous tumor of long evolution, more frequently located in the extremities. We present the case of a 77 year- old female with a nodular ulcerated tumor with multiple perilesional papules on her right lower leg. Histopathology and immunohistochemistry were consistent with cutaneous malignant fibrous histiocytoma associated to satellite metastasis.

Cutaneous gnathostomiasis: report of 6 cases with emphasis on histopathological demonstration of the larva.

BACKGROUND: Cutaneous gnathostomiasis is an emerging food-borne parasitic zoonosis. Histopathological demonstration of the larva on random biopsy specimen of erythematous plaques is infrequent because of its migrating nature. OBJECTIVE: We sought to determine whether medical treatment with albendazole or ivermectin increases the diagnostic yield of skin biopsy specimen. METHODS: A retrospective chart review was conducted in a private dermatology practice in Lima, Peru. Cases with a clinical diagnosis of nodular migratory panniculitis and pathological diagnosis of eosinophilic panniculitis or gnathostomiasis were reviewed. Only cases with definitive diagnosis confirmed by histopathology or parasite isolation were included in the study. RESULTS: A definitive diagnosis of gnathostomiasis was rendered in 6 of 55 reviewed cases. Histopathological or gross identification of the nematode's larva was made obtaining a biopsy specimen of papules or pseudofuruncles that developed after oral antiparasitic treatment. LIMITATIONS: This is a retrospective case series study and no serologic testing was available. CONCLUSION: Biopsy of a papule or pseudofuruncle subsequent to oral treatment increases the likelihood of demonstrating the larva on skin biopsy specimen, which allows definitive diagnosis and may have therapeutic benefit.

Sífilis nodular diseminada con fenómeno de prozona asociada a VIH/sida / Nodular syphilis spread associated with HIV/AIDS prozone phenomenon

Los cuadros de sífilis nodular diseminada que se presentan como un pseudolinfoma son muy raros, se ha descrito hasta la actualidad 11 casos publicados. La coinfección con el VIH puede alterar los resultados de las pruebas treponémicas y no treponémicas, lo que resulta en hallazgos falsos negativos y falsos positivos. Se estima que la coinfección sífilis y VIH está en aumento, por lo que se necesitaun diagnóstico acertado para evitar las graves consecuencias de un diagnóstico tardío. Presentamos un caso de sífilis nodular que sepresentó como un pseudolinfoma en un paciente con infección por VIH/sida que inicialmente mostró serología para sífilis negativaatribuida al fenómeno de prozona.

Disseminated nodular syphilis boxes presenting as a pseudo-lymphoma nodular are very rare, to date only 11 reported cases has been described. Co-infection with HIV may alter the results of the tests nontreponemal and treponemal not, resulting in false negative. It is estimated that co-infection HIV and syphilis is on the rise, so a correct diagnosis is needed to prevent the serious consequences of a late diagnosis. We present a case of nodular syphilis which was presented as a pseudolymphoma in a patient with HIV/AIDS infection that initially showed a serology for syphilis negative attributed to the prozone phenomenon.

Eritema Ab-igne / Erythema Ab-igne

El eritema ab-igne es una rara dermatosis asociada repetida a fuentes de calor; en el cual se presentan cambios epidérmicos y dérmicos que pueden ser difíciles de interpretar histológicamente. Sin embargo la mayor parte de veces el diagnóstico se establece mediante correlato clínico en pacientes con erupciones reticuladas eritematosas e hiperpigmentadas.

Erythema ab-igne is a rare dermatosis associated to repeated exposure to heat; it occurs with epidermal and dermal changes that could be of difficult histopathological. Nevertheless the diagnosis is established, in the majority of cases, through clinical correlation in patients with erythematous and hiperpigmented reticulated eruptions.

Acral junctional nevus versus acral lentiginous melanoma in situ: a differential diagnosis that should be based on clinicopathologic correlation.

CONTEXT: Acral lentiginous melanoma is the most prevalent clinical presentation of melanoma in ethnic groups other than whites and also occurs in significant numbers in North America and Europe. Despite a clear-cut clinical picture, histologic findings seen in partial biopsies may be too subtle and deceive pathologists dealing with such cases. OBJECTIVES: To make pathologists aware of the histologic findings during early phases of acral lentiginous melanoma (including the in situ phase), to compare those findings with what is seen in acral junctional nevus, and to highlight their similarities and differences. This review will also emphasize the important clinical and dermatoscopic findings to be considered when diagnosing acral lentiginous melanoma. DATA SOURCES: Review of published articles on the epidemiology; the clinical, dermatoscopic, and histopathologic findings; and the molecular biology of acral lentiginous melanoma as well as the personal experience of the authors when dealing with such cases. CONCLUSIONS: Acral lentiginous melanoma is a clinicopathologic entity with a clear-cut clinical picture: a diameter larger than 0.7 mm; ill-defined, darkly pigmented, flat lesion with irregular borders on acral locations; and the presence of mostly single-cell proliferations of melanocytes along the dermo-epidermal junction. Along with a few additional criteria, these findings should be sufficient to allow the pathologist to make the diagnosis and to recommend complete excision. Fluent communication between clinician and pathologist will facilitate a correct diagnosis.

Xanthoma disseminatum: case report and literature review.

OBJECTIVE: To report a case of a young normolipidemic woman with mucocutaneous xanthomas who developed neurogenic diabetes insipidus and hyperprolactinemia because of an inflammatory pituitary stalk lesion. METHODS: The clinical features, laboratory results, magnetic resonance imaging, and pathology findings are presented. In addition, the pertinent literature is reviewed. RESULTS: A 23-year-old woman presented with a 9-month history of polydipsia, polyuria, galactorrhea, secondary amenorrhea, and weight gain. Her previous medical history included chronic anemia and widespread mucocutaneous xanthomas. Laboratory tests showed hyperprolactinemia, normal electrolytes, and a normal lipid profile. The results of a water deprivation test were compatible with neurogenic diabetes insipidus, and cerebral magnetic resonance imaging showed pituitary stalk thickening. Histologic findings on a skin biopsy specimen supported the diagnosis of non-Langerhans histiocytosis. Treatment was initiated with cabergoline, nasally administered desmopressin, radio-frequency ablation of facial skin lesions, and surgical excision of other accessible lesions. CONCLUSION: Xanthoma disseminatum is a rare, benign proliferative disorder characterized by extensive cutaneous and mucous membrane xanthomas in normolipidemic patients. Central nervous system involvement is rare and usually occurs in the systemic variety. Pituitary stalk disease commonly causes hyperprolactinemia, diabetes insipidus, and various degrees of hypopituitarism. The natural history of xanthoma disseminatum usually is benign, but lesions in critical anatomic sites may result in morbidity and mortality.

Verruga peruana en fase involutiva presentándose como pseudolinfoma / Involution phase peruvian wart presenting as pseudolymphoma

El espectro clinico de la infección por Bartonella bacilliformis varia ampliamente desde una infección subclínica hasta una enfermedad aguda fulminante, con hemólisis severa o desarrollo insidioso de tumores vasculares de la piel, con poca o ninguna sintomatología. Se reporta un caso enfermedad de Carrión presentándose como una tumoración única de aspecto angiomatoso, sin antecedente de enfermedad aguda previa, y con hallazgos histológicos compatibles con pseudolinfoma cutáneo, en la que la coloración de Warthin Starry nos permitió visualizar al agente infeccioso.

The clinical spectrum of Bartonella bacilliformis infection varies widely from subclinical infection to acute fulminant disease with severe hemolysis or insidious development of vascular tumors of the skin, with little or no symptoms. We report a case of Carrion's disease presenting as a single angiomatous tumor, with no history of previous symptoms, and histological findings consistent with cutaneous pseudolymphoma in which Warthin Starry staining allowed us to visualize the infectious agent.

Linfoma cutáneo primario de células grandes anaplásicas CD30+ / Cutaneous CD30+ Anaplastic Large-Cell Lymphoma

El linfoma primario cutáneo de células grandes anaplásicos, es un trastorno linfoproloiferativo que afecta principalmente a adultos, caracterizado por presentar tumores solitarios o localizados, que en la histopatología muestra un infiltrado de células CD30+ y tener buen pronóstico. Se presenta el caso de un paciente de 52 años con lesiones tumorales en miembro inferior izquierdo de 1 año de evolución.

Primary cutaneous lymphoma anaplastic large cell, is a lymphoproliferative disorder characterized by affect adult patients with solitary or localized tumors, the histopathology shows an infiltration of CD30 + cells and have a good prognosis. We report a male of 52 years with tumors in the left lower limb of 1 year of evolution.

Linfoma cutáneo de células T tipo hidroa / Cutaneous T-cell lymphoma type hydroa

El linfoma cutáneo tipo hidroa es un tipo de linfoma relacionado al virus Epstein Barr que afecta a niños y jóvenes, y se caracteriza por lesiones que semejan al hidroa vacciniforme. Se presenta el caso de una paciente adulta sin antecedentes patológicos con compromiso cutáneo facial.

The cutaneous T-cell lymphoma type hydroa is a type of lymphoma related to Epstein Barr virus that affects children and young people, and characterized by lesions that resemble the hydroa vacciniforme. A case of a female adult patient without pathological history and with skin lesions in face.