Xp microdeletion syndrome characterized by pathognomonic linear skin defects on the head and neck.

We describe a new case of a rare syndrome characterized by ocular abnormalities and pathognomonic linear skin defects. This syndrome is the result of an unbalanced translocation resulting in a deletion of the distal end of the short arm of the X chromosome. We report the thirteenth case and review the clinical and cytogenetic aspects of this disorder.

Verrucous herpes virus infection in human immunodeficiency virus patients.

Objective: Two cases of varicella-zoster virus infection that were clinically and pathologically verrucous are reported. Although this phenomenon has previously been described in the dermatology literature, it has not, to our knowledge, been described in the pathology literature. It is important that pathologists are aware of these uncommon but histologically distinctive lesions.

Nerve sheath myxoma of the oral cavity: case report and review.

Nerve sheath myxoma is a benign peripheral nerve sheath tumor that rarely occurs in the oral cavity; approximately 12 cases have been reported to date. On histologic evaluation the lesions consist of closely aggregated fascicles that vary considerably in cellularity. Some of the lesions are predominantly myxoid with widely spaced, stellate-shaped cells.

Widespread cutaneous bacillary angiomatosis and a large fungating mass in an HIV-positive man.

Bacillary angiomatosis (BA), an infection caused by a gram-negative rod, can be a multiorgan disease. The usual causative organism, Bartonella (formerly Rochalimaea) hensalae, has only recently been identified. Bartonella quintana has also been shown to cause some cases of cutaneous BA.

Psoriasis: pathophysiology and oral manifestations.

Psoriasis is a chronic, remitting and relapsing inflammatory skin disorder with a strong genetic predisposition. Psoriasis affects 1-3% of the world's population in their early lives representing a disabling condition with significant social and economic impact. Despite a great deal of research on the etiology and tissue destruction mechanisms, the disease is not well understood.

Myxoid liposarcoma with cartilaginous differentiation.

Common histologic variants of liposarcoma are readily recognized. Histologic classification might be difficult, however, when liposarcoma demonstrates cartilaginous differentiation. Although this phenomenon has previously been noted, it has not, to our knowledge, been reported as a specific pathologic entity.

Epstein-Barr virus infection is an early event in gastric carcinogenesis and is independent of bcl-2 expression and p53 accumulation.

Ninety-five cases of adenocarcinoma of the stomach were evaluated for the presence of Epstein-Barr virus (EBV) using a sensitive in situ hybridization assay targeting Epstein-Barr virus-encoded RNA 1 (EBER1) transcripts. EBER1 was detected in 11 of 95 (12%) of cases. When present, the virus was localized to malignant epithelial cells and to dysplastic gastric epithelium, but was not seen in normal-appearing gastric epithelium or intestinal metaplasia.

Epithelioid glomus tumor.

Reported are five cases of an unusual histological variant of glomus tumor that we have designated epithelioid glomus tumor. Unlike conventional glomus tumors, which consist of small polygonal cells with dark round nuclei and scanty cytoplasm, the epithelioid lesions were composed of large polygonal to spindle-shaped cells with abundant eosinophilic cytoplasm and large, irregularly shaped nuclei: The cells had both epithelioid and myoid qualities. Two of the cases studied were predominantly epithelioid, with small remnants of conventional glomus tumor at the periphery.

Allelic imbalance in gastric cancer: an affected site on chromosome arm 3p.

In order to detect regions of DNA containing tumor suppressor genes involved in the development of gastric cancer, we performed an allelotype study on 78 gastric adenocarcinomas from a population composed largely of Texan Hispanics and Anglos, two ethnic groups that have a ratio of incidence rates of gastric cancer of approximately 2:1. In total, 42 microsatellite markers were employed, which detected at least one site per arm of each autosome in the human genome. These included several markers linked to known tumor suppressor genes (TP53, APC, DCC, RB1, and BRCA1).

Cutaneous involvement in chronic myelomonocytic leukemia.

Background: Chronic myelomonocytic leukemia has been associated with various nonspecific cutaneous manifestations. Rarely has the leukemia been reported to directly affect the skin.

Methods: This case documents the progression of a patient who ultimately developed chronic myelomonocytic leukemia, by clinical examination, hematologic parameters, dermatopathology, and bone marrow pathology.

Multiple lymphomatous polyposis of the gut. A case with unusually widespread distribution.

Multiple lymphomatous polyposis (MLP) is a term used to describe malignant lymphomas of the gastrointestinal tract that manifest as polyposis. The cell population of MLP is identical to that in "mantle cell lymphoma." In most instances, relatively small segments of small and large intestine are affected.

Giant cell tumor of the larynx. Case report and review of the literature.

Giant cell tumor of bone is rare in the osteocartilaginous tissues of the larynx. We describe a 23-year-old man with a 6-month history of progressive hoarseness and an enlarging neck mass. Computed tomography demonstrated a 4-cm mass with central cystic change arising in the right ala of the thyroid cartilage.

Gastrointestinal tattoos.

Tattooing of the gastrointestinal tract is used to facilitate the relocation of biopsy sites or other sites of interest at the time of subsequent biopsy or surgery. Submucosal injection of sterile india ink produces a zone of blue-black coloration that is grossly visible from both the mucosal and serosal surfaces. The pathology of gastrointestinal tattoos has only been briefly mentioned previously in the medical literature.

Papulonecrotic tuberculid secondary to disseminated Mycobacterium avium complex.

Background: Papulonecrotic tuberculid is a rarely reported cutaneous reaction to the mycobacterium bacillus. It is most often encountered in association with tuberculosis. The clinical and histologic picture of the entity is a distinctive one, but the etiology of the disease process is uncertain.

p53 mutations in gastric and colorectal cancers in Texas Hispanics versus Anglos.

Gastric cancer is more than twice as common in Hispanics as in Anglos in Texas, while colorectal cancer is almost twice as common in Anglos as Hispanics. To test the hypothesis that mutations in the p53 tumour suppressor gene are involved in these differences, we examined 131 gastric and 138 colorectal cancers from Hispanic and Anglo patients from South Texas and Mexico using immunohistochemistry (IHC) as a screening assay for p53 mutations. The fraction of p53 positive cases was not significantly different in gastric cancers from Hispanics compared to Anglos (43% versus 61%, respectively, p = 0.

Transient acantholytic dermatosis induced by recombinant human interleukin 4.

Background: Interleukin 4 is one of many cytokines under investigation about its possible role in the pathogenesis and treatment of disease. Transient acantholytic dermatosis is of uncertain origin. It has not previously been linked to drug ingestion and is generally not believed to have an immunologic basis.

Compound (primordial) adnexal carcinoma arising in a systematized compound epithelial nevus.

A distinctive systematized epithelial nevus and associated multifocal carcinomas of the microcystic and sclerosing sweat duct type affected the lower extremities of a 9-year-old girl. The carcinomas were well differentiated and were both adenosyringomatous and microcystic. They qualify as microcystic and compound.

Histologic classification of the combined nevus. Analysis of the variable expression of melanocytic nevi.

The designation combined nevus gives recognition to mixed cytologic patterns. In the common variant, plump, pigmented spindle cells form fascicles among nests of ordinary nevus cells. In other variants, one or several cellular components that share cytologic features with either a blue nevus or a Spitz nevus are represented.

Granular cell tumor of the breast.

Granular cell tumors are rare tumors occasionally located in the breast. They are generally benign, but can mimic breast carcinoma thus complicating diagnosis and treatment. The etiology of these tumors remains obscure, but may be neurogenic in origin.

Cutaneous vascular calcification with ulceration in hyperparathyroidism.

We report a histologically distinctive form of small blood vessel calcification that led to necrosis and ulceration of the skin. Microscopically, wedge-shaped ulcers of the skin and superficial subcutis were observed. Calcified deposits were identified in the walls of small blood vessels at the ulcer bases.

Pigmented myomatous neurocristoma of the uterus.

A tumor of the myometrium composed of pigmented and nonpigmented melanocytes in a matrix of altered smooth-muscle cells is reported. Electron microscopy and immunohistochemical studies were used to identify the cellular constituents. The neurocristic components were arranged in patterns comparable with those in cellular blue nevus of the skin.