Plasmacitosis cutánea: una infrecuente enfermedad cutánea a tener en cuenta / Cutaneous Plasmacytosis: A Rare Diagnosis to Bear in Mind

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[Perifollicular xanthomatosis as a key histological finding in Fox-Fordyce disease]. / Xantomatosis perifolicular: hallazgo histológico clave en la enfermedad de Fox-Fordyce.

Fox-Fordyce disease is a rare skin condition characterized by the presence of multiple pruritic follicular papules in areas rich in apocrine glands, such as the axillae, mammary areolae, or genital regions. There is a high degree of variability in the histological findings seen in Fox-Fordyce disease. In addition to those described as typical of this entity, such as dilation of the infundibulum and hyperkeratosis and spongiosis of the infundibular epithelium, many other histological changes can be observed. We report the case of a 21-year-old woman with Fox-Fordyce disease and highlight the importance of perifollicular xanthomatosis as a key histological finding in the diagnosis of the disease.

Xantomatosis perifolicular: hallazgo histológico clave en la enfermedad de Fox-Fordyce / Perifollicular xanthomatosis as a key histological finding in Fox-Fordyce disease

La enfermedad de Fox-Fordyce es una rara dermatosis caracterizada por la presencia de múltiples pápulas foliculares pruriginosas en áreas corporales con riqueza de glándulas apocrinas como axilas, areolas mamarias o región genital. Los hallazgos histopatológicos que definen la enfermedad de Fox-Fordyce son muy variados. Además de los hallazgos descritos como típicos de esta entidad, como la dilatación del infundíbulo y la hiperqueratosis y espongiosis del epitelio infundibular, se pueden observar otros muchos hallazgos histológicos. Presentamos el caso de una mujer de 21 años de edad afectada por esta enfermedad y recalcamos la importancia de la xantomatosis perinfundibular como hallazgo histológico clave en el diagnóstico de esta entidad (AU)

Fox-Fordyce disease is a rare skin condition characterized by the presence of multiple pruritic follicular papules in areas rich in apocrine glands, such as the axillae, mammary areolae, or genital regions. There is a high degree of variability in the histological findings seen in Fox-Fordyce disease. In addition to those described as typical of this entity, such as dilation of the infundibulum and hyperkeratosis and spongiosis of the infundibular epithelium, many other histological changes can be observed. We report the case of a 21-year-old woman with Fox-Fordyce disease and highlight the importance of perifollicular xanthomatosis as a key histological finding in the diagnosis of the disease (AU)

[Protocol proposal for the histological report of the primary tumor in patients with cutaneous melanoma from the Task Force for Cutaneous Melanoma of the Valencian Community]. / Propuesta de protocolo para el informe histológico del tumor primario de los pacientes con un melanoma cutáneo del Grupo de Trabajo para el Melanoma Cutáneo de la Comunidad Valenciana.

This text compiles a histological protocol proposal for cutaneous melanoma agreed by the Task Force for Cutaneous Melanoma of the Valencian Community. It brings together the protocol itself and, in addition, includes a description of each of the proposed variables that has shown to have a greater prognostic impact in previous works.

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) treated with 2-chlorodeoxyadenosine.

Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease (RDD) is a rare but well-defined histiocytic proliferative disorder of unknown etiology that usually presents with cervical lymphadenopathy, fever, leukocytosis, and hypergammaglobulinemia in an otherwise healthy child. Although many patients undergo spontaneous remission, a subset of patients with systemic disease has a more serious course. For those patients with a poor outcome, steroids and chemotherapeutic agents such as etoposide or 6-mercaptopurine plus low dose methotrexate have been used. We present a child with a massive cervical lymphadenopathy treated with 2-chlorodeoxyadenosine (2-CdA, cladribine) after other approaches failed.

[Etiology of upper gastrointestinal bleeding of peptic origin: role of Helicobacter pylori and NSAIDs]. / Etiología de la hemorragia digestiva alta de origen péptico: papel de Helicobacter pylori y los antiinflamatorios no esteroideos.

INTRODUCTION: Upper gastrointestinal bleeding continues to be a severe and frequent complication in ulcerative disease. Etiologic diagnosis in these patients is highly important in order to initiate appropriate treatment and prevent bleeding recurrence. OBJECTIVE: 1. To investigate the prevalence of Helicobacter pylori infection and use of NSAIDs in patients with upper gastrointestinal hemorrhage of peptic origin. 2. To analyze the strategy used for the diagnosis of H. pylori in our previous work. PATIENTS AND MEHTODS: Seventy-three patients with endoscopically-diagnosed upper gastrointestinal bleeding of peptic origin were included in the study. The use of NSAIDs was investigated. H. pylori infection was diagnosed if one of the following tests was positive: urease test, histology, breath test. RESULTS: H. pylori infection was found in 92% of duodenal ulcers and in 88% of gastric ulcers. Fifty-six percent of the patients had taken NSAIDs. Excluding these patients resulted in an H. pylori infection rate of 96.7%. The diagnosis was based on urease test in 46%. In the remaining patients, breath test and histology were required. CONCLUSIONS: The main etiology in patients with upper gastrointestinal bleeding of peptic origin is H. pylori infection followed by the use of NSAIDs, and these two factors frequently coexist. The strategy of performing a urease test and, when this is negative, performing histological study and a breath test, is valid and allows a diagnosis of H. pylori infection to be made even if patients are receiving treatment that could make diagnosis difficult.

Insuficiencia hepática aguda en pacientes en tratamiento con metotrexato / Acute liver failure in a patient with methotrexate therapy

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Phyllodes tumor of the breast with actin inclusions in stromal cells: diagnosis by fine-needle aspiration cytology.

The occurrence of hyaline inclusions in stromal cells in fibroepithelial tumors of the breast is very uncommon. These inclusions, characteristic of infantile digital fibromatosis, are comprised of actin filaments. This report illustrates a case of a benign phyllodes tumor of the breast with inclusion bodies, identified by fine-needle aspiration. Histologically, many of the stromal cells contained round intracytoplasmic inclusions, with positivity for smooth muscle actin.

Phyllodes tumor in ectopic breast tissue of the vulva.

Phyllodes tumor arising from ectopic breast tissue of the vulva is an extremely rare occurrence. A case is reported in a 34-year-old nulligravida woman, presenting as a nodule on the left labium majus. A simple excision was carried out, and no recurrence has been detected after 18 months of follow-up.

Displasia nodular pigmentada suprarrenal bilateral y síndrome de Carney. A propósito de un caso / Primary Pigmented Nodular Adrenocortical Disease and Carney syndrome. A case report

La displasia nodular pigmentada bilateral suprarrenal (DNPBS) es una forma peculiar e infrecuente de aparición del síndrome de Cushing ACTH-independiente. Se presenta con autonomía de las glándulas suprarrenales y displasia suprarrenal bilateral con características histológicas de benignidad. La suprarrenalectomía bilateral es el único tratamiento curativo para esta enfermedad. Se presenta el caso de una mujer de 31 años con DNPBS en la que destaca el inicio relativamente tardío de esta enfermedad y que justifica el aspecto micronodular en el estudio patológico, en contra de la clásica descripción de la DNPBS. Se estudia la posible asociación con el síndrome de Carney (mixomas, lesiones cutáneas hiperpigmentadas e hiperactividad endocrina), del cual la paciente presenta dos elementos: DNPBS y efélides, aunque el estudio familiar es negativo. Se revisa el diagnóstico diferencial de la enfermedad suprarrenal nodular, la DNPBS y el síndrome de Carney (AU)

Microadenoma hipofisario secretor de TSH: estudio morfológico tras tratamiento con lanreótido / TSH secreting pituitary microadenoma: Morphological study following treatment with lanreotide

Los adenomas hipofisarios secretores de TSH son muy infrecuentes. Los pacientes presentan concentraciones elevadas de TSH, clínica de hipertiroidismo y tumor hipofisario, habitualmente un macroadenoma. El presente trabajo describe el caso de un microadenoma hipofisario secretor de TSH tratado con análogos de somatostatina (lanreótido) y posteriormente con cirugía transesfenoidal. El estudio patológico confirmó un adenoma hipofisario con positividad inmunohistoquímica para TSH y subunidad alfa. Un hallazgo relevante fue la presencia de abundante fibrosis tumoral. La fibrosis es un fenómeno frecuente en los tirotropinomas, por lo que resulta difícil establecer su relación con el tratamiento con lanreótido (AU)

Comparison of diagnostic methods for Helicobacter pylori infection in patients with upper gastrointestinal bleeding.

BACKGROUND: Accuracy of the most frequently used tests for diagnosing Helicobacter pylori infection in patients with upper gastrointestinal bleeding of peptic origin is determined. METHODS: Seventy-eight patients with endoscopically-proven upper gastrointestinal bleeding of peptic origin were included. The presence of H. pylori was considered when observed from the histology or, if negative, when serology and breath test were both positive. Accuracy of the rapid urease test was estimated in accordance with results obtained with other diagnostic methods. RESULTS: Lesions causing gastrointestinal bleeding were 56 duodenal ulcers, 13 gastric ulcers, 7 pyloric channel ulcers, 13 acute lesions of the gastric mucosa and 16 erosive duodenitis. H. pylori infection was present in 68 patients (87.2%). Forty-four patients had received non-steroidal anti-inflammatory drugs. The sensitivity/specificity (%) of the diagnostic methods was 48.5/100 for the rapid urease test, 91/77.8 for the breath test, 89.5/80 for serology and 86.3/100 for histology. The prior consumption of proton-pump inhibitors and antibiotics induced false-negative results in the rapid urease test and breath test, with no effect on serology and histology. CONCLUSIONS: The prevalence of H. pylori infection in patients with upper gastrointestinal bleeding from peptic lesions is high. Sensitivity of the rapid urease test for diagnosing H. pylori is low in this setting. Cases with negative rapid urease test need the combination of two or more additional tests if diagnosis is to be achieved. Cases with positive rapid urease test do not need further investigation for diagnosis.

Amiloidosis laríngea localizada. Cirugía endoscópica con láser CO2 / Localized amyloidosis of the larynx. Endoscopic surgery with CO2 laser

La amiloidosis laríngea es una entidad poco frecuente. Dentro de las vías aerodigestivas, la localización más -común es el órgano laríngeo, en concreto la región supraglótica. Lo más frecuente es que la amiloidosis laríngea sea una forma primaria localizada, aunque en ocasiones puede presentarse dentro de una amiloidosis sistémica. Presentamos un nuevo caso de amiloidosis laríngea primaria localizada a nivel de la mucosa aritenoidea derecha en una mujer de 66 años que fue tratada mediante cirugía endoscópica láser C02 (AU)

Laryngeal amyloidosis is a very rare disease. In the upper airways, the most common localization is the laryngeal organ, particularly the supraglotic region. Usually laryngeal amyloidosis is a localized primary form, although it has occasionally been reported in conjunction with system amyloidosis. We are reporting a new case of localized primary laryngeal amyloidosis in the right arytenoid mucosa in a 66 years old woman who underwent CO2 laser endoscopic surgery (AU)