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Abstract Rarely, systemic lupus erythematosus (SLE) presents with bullous lesions due to severe edema and hydropic degeneration of the basal layer, or as a subepidermal blistering disease. Here, we describe two Mexican teenagers, one with SLE with blisters and another with bullous SLE. We also discuss the mechanisms and clinical implications of lesion formation in patients with SLE and bullae.

Background: Calcifying fibrous pseudotumor is an uncommon clinical and pathological disease, which was described by Rosenthal and Abdul-Karim in 1988. It has been reported previously in soft tissues and in the pleura. Peritoneal calcifying fibrous pseudotumors were found in adults. It is characterized by a predominantly lymphoplasmacytic infiltrate with abundant hyalinized collagen and psamomatous or dystrophic calcifications. The cause and pathogenesis are unclear. It has been postulated that CFP may represent a sclerosing end stage of inflammatory myofibroblastic tumor. Objective: To describe two cases of calcifying fibrous pseudotumor in visceral peritoneum. Methods: We describe two cases of calcifying fibrous pseudotumor in visceral peritoneum, one in an 11-year-old boy and the other in a 70-year-old man with immunohistochemical analysis. Results: Both cases presented similar histological findings to the ones described in the literature for the calcifying fibrous pseudotumor. These cases consisted of lesions covered by reactive mesothelium, not encapsulated, composed by dense bundles and sparse spindle collagen, vastly vascularized with sclerosis in the walls of the vessels evident with Masson stain, areas of dystrophic calcification (psamomatous), chronic inflammatory infiltrate with predominant lymphocytes and, in a lesser number, plasmatic cells. By immunohistochemistry both cases were positive for vimentin in fibroblast, blood vessels walls; the mesotelial cells were positive homogenous to the calretinina. CD 117 and CD 34 were negative and the proliferation index measure with Ki 67 were 0. [ABSTRACT FROM AUTHOR]

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We present two cases of type II glycogenosis diagnosed at autopsy in two Mexican boys 5 and 6 months of age. The diagnosis was based on characteristic tissue findings secondary to glycogen overload in the heart, skeletal muscle and central and peripheral neurons. Although clinical diagnosis and treatment are feasible, unfortunately this disease is usually identified at autopsy and hence its actual frequency is underestimated in our country. [ABSTRACT FROM AUTHOR]

Introduction: Mucopolisaccharidosis I (MPS I) is caused by deficiency of the enzyme α-L-iduronidasa which results in chronic lisosomal deposit of glicosaaminoglicanos (mucopolisacaridos) with cell, tissues and organic damage. It causes death by respiratory failure in patients between the 10 and 40 years of age. At present, the available enzymatic therapy relieves many of the signs and symptoms of this disease. Case report. A five-year-old girl, diagnosed with Type I mucopolysaccharidosis, confirmed by an a-L-iduronidase of 0.4 umol/L/h. (2.2 to 11.7) At three years of age she had ventilation tubes implanted in both timpanic membranes. Tonsillectomy was performed and ventilation tubes were implanted again at 4 years of age. This same year she was operated for correction of scoliosis. In order to perform a cerebral and cervical spine magnetic nuclear resonance she was anesthesized under oxygen (5 L/min) and 3% sevofluorane. Endotracheal intubation at 6 L/min by mask was carried out; she was given 200 mcg of atropine, 20 mcg of fentanyl, and 50 mg of propofol by vein which caused marked sinus bradycardia followed by cardiac arrest. A spontaneous 110/min heart rate took place whence a second irreversible cardiac arrest occurred. Autopsy showed abundant systemic glicosaaminoglicane deposits. Conclusion: There is great anesthetic risk for the patients with MPSI because of the shortness of their neck, infiltration of soft tissues of the upper airway, macroglosia, adenoamigdaline hypertrophy, and atlanto-occipital instability which make access to the airway route and intubation very difficult. These patients should only be put under general anesthesia in facilities where the anesthesiologists have experience in difficult airway intubation. [ABSTRACT FROM AUTHOR]

Background. Autopsy studies in patients who died with AIDS provide an insight on the occurrence and diversity of tissue changes directly resulting from HIV infection and of the infectious complications, often with opportunistic organisms, associated with the disease. Material and methods. This is a retrospective analysis of 34 autopsies performed in children dying with AIDS at the National Institute of Pediatrics in Mexico City from 1985 to 2004. The cases were classified according to the mechanisms of infection in maternal (prenatal and from breast feeding), transfusion related and from sexual contact. For comparative purposes, the cases were distributed in two decades based on the probable date of infection. Results. Involvement of the lymphoid tissue was the most frequent primary pathology (79%) followed by central nervous system disease in 24 cases (70%). Associated pathology included malnutrition (53%), nephropathy (11.7%), blood vessel involvement (5.8%) and hepatitis (2.9%). Secondary infection was present in every case. Infectious agents included bacteria (55.8%), Cytomegalovirus (44.15%), Pneumocystis (26.4%), Candida (23%), Herpes/Varicella (20%) and Histoplasma (14.7%). Conclusions. A wide variety of tissue changes directly related to HIV infection was found, some of them infrequent complications such as vasculitis. Histoplasmosis was an important cause of death and was not considered as a clinical diagnosis in any of our cases. Mycobacteria, probably non-tuberculous, were found in two patients. Pulmonary Pneumocystis infection is persistently frequent in spite of clinical awareness and prophylactic measures [ABSTRACT FROM AUTHOR]

We present a case of a benign hybrid nerve sheath tumor showing features of both cellular schwannoma and perineurioma.The patient was a 33 year-old female who presented with a 6 month history of pain in the thorax-lumbar region.Axial CT and MIR images showed and homogeneously enhancing solid oval mass involving the left paravertebra region in contact with the vertebral foramina T9-T11. Morphologically the tumor presented a well formed capsule and showed a uniform highly spindle cell proliferation. The spindle cells were arranged in whorls and intersecting fascicles with focal intervening sclerosis and relatively uniform cellularity with a prominent perivascular lymphocytic infiltrate. No cytological atypia, necrosis or mitoses were present. A second cell component was present composed of spindle-shaped cells with inconspicuous bipolar, pale, eosinophilic cytoplasm with oval nuclei. By immunohistochemistry there was a diffuse staining for S-100, and the elongated bipolar cells were EMA, Glut-1 and Claudin-1. To our knowledge this is the first reported case of a hybrid cellular schwannoma/perineurioma.

Kikuchi-Fujimoto disease also known as histiocytic necrotizing lymphadenopaty (HNL) is a rare entity, originally described in Japanese population, although currently it has been described all over the world. It is more frequent in young women and it is usually located in cervical lymph nodes. We report 14 cases of HNL in Mexican population, their clinicopathological and immunohistochemical study as well as a comparative study with other necrotizing lymphadenopaties due to B or T-cell lymphomas, tuberculosis, Epstein Barr virus infection, and non-specific necrosis. In our study we found that there was more expression of the immunomarkers CD68, MPO, CD123 and antikerat in OSCAR in the cases of HNL in contrast with the lesser or even null expression of the same markers in the necrotized lymph nodes of the comparative study group.

Objective: To correlate the expression of p53 and BCl-2 with the clinical outcome and anatomic location of the gastrointestinal stromal tumours (GIST)., Background Data: The GIST are the most common nonepithelial neoplasm of the gastrointestinal tract. In spite of the existence of a wide range of predictive factors, their clinical outcome is unpredictable. There are several studies that correlate the expression of p53 and Bcl-2 with the clinical outcome and anatomic location of the GIST., Methods: We obtained 19 cases from the archives of the Department of Pathology of the ABC Medical Center, in Mexico City. GIST were classified into risk groups according to the Fletcher et al. classification. We performed an immunohistochemestry panel including CD117, CD34, actin, desmin, P-S100, p53 and BCl-2 and correlated their expression to the risk group and anatomical site of the tumors., Results: There was less expression of p53 in the gastric tumors (27%) than in small bowel tumors (100%). There was greater expression of p53 in the high-risk tumors than in the very low-risk ones, regardless of the anatomical site. Bcl-2 expression was more expressed in the small intestine tumors (100%) than in those located in the duodenum (50%) The high risk tumors showed slightly more expression of Bcl-2 than the low risk ones (89% vs. 100%), despite the anatomical location., Conclusions: Both, p53 and Bcl-2 are important markers to establish the anatomical site of GIST and are also helpful to predict the clinical behavior of these tumors.

The goal of this paper is to present a clinical case of a 4 year old boy, with hepatomegaly, splenomegaly and intestinal lipid infiltration due to a inborn error of lipid metabolism known as cholesterol ester storage disease. The main clinical manifestations were hepatomegaly, splenomegaly, hypertriglyceridemia, hypercholesterolemia. Duodenal endoscopy showed a yellow appearance of the mucous, and the histological study revealed the presence of macrophages with granular material. Liver biopsy showed steatosis infiltration at the hepatocytes, and macrophages with lipids. This disease is due to a lisosomal acid lipase partial deficiency, that is a glicoprotein that metabolize the hydrolysis of ester of cholesterol and triglycerides. The name of this pathology is cholesterol ester storage disease, but when the deficiency is total the name is Wolman's disease. We conclude that in all the children whit a clinical picture of hepatomegaly, splenomegaly, hypertriglyceridemia and hypercholesterolemia it is obligatory to rule out an inborn error of lipid metabolism like Wolman's disease or cholesterol ester storage disease.

We report the case of a 39 years old woman, with bilateral fibrocystic breast disease and several surgeries for this cause, including bilateral mammary implants. Nineteen months after the implants an angiosarcoma of the left breast was diagnosed. In the specimen from radical mastectomy infiltration of the adipose tissue was found but no metastases to the axillary lymph node resected. Prognostic factors were investigated in surgical specimen and immunophenotypic characterization was made, considering Ki-67 and p53 expression. The patient finished chemotherapic treatment and is well, without neoplastic disease relapse, 16 months after diagnosis. We comment the possible relation between silicona's implants and angiosarcoma's genesis, association not reported before in the literature. The adverse effects of silicone implants and angiosarcoma histogenesis were reviewed.