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Biomedical subjects

P E LeBoit

Publications and source records attributed to P E LeBoit.

At least 19 recordsLinked to original sources

Isolation of Rochalimaea species from cutaneous and osseous lesions of bacillary angiomatosis.

BACKGROUND: Bacillary angiomatosis is characterized by vascular lesions, which occur usually in patients infected with the human immunodeficiency virus (HIV). A newly described gram-negative organism, Rochalimaea henselae, has been associated with cutaneous bacillary angiomatosis, but no organism has been isolated and cultivated directly from cutaneous tissue. METHODS: We used two methods to isolate the infecting bacterium from four HIV-infected patients with cutaneous lesions suggestive of bacillary angiomatosis: cultivation with eukaryotic tissue-culture monolayers and direct plating of homogenized tissue onto agar. The patients' blood was cultured with the lysis-centrifugation method. Isolates recovered from skin and blood were identified by sequencing all or part of the 16S ribosomal RNA gene amplified with the polymerase chain reaction. RESULTS: R. quintana, historically known as the agent of trench fever, was isolated from cutaneous lesions in three patients, after tissue homogenates were cultivated with endothelial-cell monolayers; R. henselae was isolated from a cutaneous lesion in one patient. In two patients, R. quintana was isolated from both cutaneous tissue and blood; in one patient it was also isolated from bone. CONCLUSIONS: In bacillary angiomatosis, either of two species of rochalimaea--R. quintana or R. henselae--can be isolated from cutaneous lesions or blood, providing an additional method of diagnosis.

AIDS-Related Opportunistic Infections

An eruptive vascular proliferation resembling acquired tufted angioma in the recipient of a liver transplant.

A 59-year-old man developed grouped erythematous papules on the skin of the right axilla and arm shortly after orthotopic liver transplantation. Histologic changes closely resembled those of acquired tufted angioma, with capillary lobules studding the dermis. The lesions spontaneously involuted over several months. Histopathologic and immunohistochemical findings excluded Kaposi's sarcoma and bacillary angiomatosis, the two vascular proliferations most frequently seen in the immunocompromised host. We believe that our case is one of acquired tufted angioma appearing as an eruptive condition similar to eruptive pyogenic granuloma, to which it seems closely related. The involution of lesions in this case is unexplained.

Arm

Dowling-Degos' disease mimicking chloracne.

We describe a patient with clinical features that resembled severe chloracne; however, histopathologic findings revealed a reticulated pigmented anomaly. Innumerable comedones, many cysts, acneiform scarring, and flexural and facial pigmentation were noted in this patient, who is a machinist. A serum test for polychlorinated biphenyl was negative, which eliminated a diagnosis of chloracne. The spectrum of clinical features of the histologically well-defined Dowling-Degos' disease is discussed; in this disease lesions can be flexural or acral and can appear as macules, comedones, or cysts.

Acne Vulgaris

Angiomatosis with luminal cryoprotein deposition.

BACKGROUND: Angioendotheliomatosis has recently been separated into two types. Malignant angioendotheliomatosis is a lymphoma in which the neoplastic cells accumulate in vascular lumens. Reactive angioendotheliomatosis is a proliferation of capillaries that is less well characterized. OBJECTIVE: Our purpose was to describe the clinical and histopathologic features of three cases of reactive angiomatosis in patients with cryoproteinemia. METHODS: Clinical and histologic data were correlated. Immunoperoxidase staining and electron microscopy were used in the histologic evaluation. RESULTS: All three cases showed tufts of capillaries with luminal deposits of cryoproteins. Immunohistochemical studies showed that the cellularity of the tufts was primarily caused by a proliferation of pericytes. CONCLUSION: Cryoproteinemia can cause angiomatosis that simulates a vascular neoplasm.

Aged

Chronic verrucous varicella-zoster virus infection in patients with the acquired immunodeficiency syndrome (AIDS). Histologic and molecular biologic findings.

Verrucous skin lesions have been attributed to various herpes viruses in immunosuppressed patients, including those with human immunodeficiency virus infection (HIV). We examined such lesions from six HIV-infected patients to determine the range of microscopic findings present and to establish which herpesviruses were present. Verrucous epidermal hyperplasia, pseudocarcinomatous hyperplasia, and massive hyperkeratosis correlate with the warty clinical appearance of the lesions. Herpetic cytopathic changes, including multinucleated epidermal giant cells, steel-gray nuclei, necrotic acantholytic keratinocytes, and Cowdry type A nuclear inclusions were seen most prominently in the dells between papillations and in adnexal epithelium. In two cases, increased numbers of spindled cells were seen in the dermis. Immunoperoxidase staining with anti-type IV collagen antibodies demonstrated that these findings were not those of Kaposi's sarcoma, but represent a fibrotic reaction to the infection. Viral cultures of four of the cases demonstrated the presence of varicella-zoster virus, whose presence was detected by the polymerase chain reaction in paraffin-embedded lesional tissue from all six cases. Polymerase chain reaction did not show the presence of cytomegalovirus, herpes simplex, Epstein-Barr, or human papillomavirus. We conclude that these unusual verrucous lesions are a chronic manifestation of herpes zoster infection and that the reported presence of other agents in such lesions is probably coincidental.

Acquired Immunodeficiency Syndrome

Pagetoid intraepidermal spread in Merkel cell (primary neuroendocrine) carcinoma of the skin.

Pagetoid intraepidermal spread of neoplastic cells was noted in six cases of Merkel (primary neuroendocrine) cell carcinoma of the skin. In two cases, the volume of the intraepidermal portion of the neoplasm was either equal to or more extensive than the dermal component. The intraepidermal component in all six cases was remarkable because of the following findings: the presence of cells with scant cytoplasm arranged both individually and as nests, sometimes along the dermoepidermal junction; splaying of the apical portions of basal keratinocytes by solitary neoplastic cells; incomplete rims of compressed basal keratinocytes at the peripheries of some junctional nests; and occasional contiguity of neuroendocrine carcinoma cells with those of Bowen's disease or solar keratosis. These features can be used to distinguish these Merkel cell carcinomas from other lesions that have a pagetoid pattern, even in superficial biopsies, and immunohistochemistry can confirm the diagnosis or resolve problematic cases. The occurrence of cutaneous neuroendocrine carcinoma situated largely in the epidermis raises the possibility that some of these tumors may arise from intraepidermal Merkel cells.

Adult

Histologic features of cutaneous sinus histiocytosis (Rosai-Dorfman disease): study of cases both with and without systemic involvement.

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a systemic proliferation of cells that resemble the sinus histiocytes of lymph nodes. Whereas initial reports highlighted the often striking cervical adenopathy, more than 40% of patients have extranodal involvement. Cutaneous lesions are the most common form of extranodal disease, but cases that present as cutaneous disease without lymph node involvement are rare. We examined biopsies from three patients with cutaneous lesions of sinus histiocytosis, none of whom had detectable systemic involvement, and compared them to those of two patients whose lymph nodes were involved by the disease. The histology of skin lesions in all five cases showed dense nodular or diffuse infiltrates of "histiocytes," emperipolesis of lymphocytes, neutrophils, and plasma cells. "Histiocytes" were present in lumens of dilated lymphatics. At the peripheries of the lesions were lymphoid aggregates and thick-walled vessels surrounded by plasma cells. Staining with anti-S-100 protein antibody showed marked cytoplasmic positivity in the "histiocytes" in each case. The only features that differed among the two groups were the presence of pseudoepitheliomatous hyperplasia and infiltrates of eosinophils in one case in which the disease was limited to the skin. We believe that cutaneous sinus histiocytosis can be specifically diagnosed by skin biopsy. Because cutaneous sinus histiocytosis may be unfamiliar to general pathologists, dermatopathologists, and dermatologists, cases limited to the skin may be under-recognized.

Adult

Laser skin testing.

In order to accomplish more uniformity for standardization of allergy skin testing, a technique using a CO2 laser in place of a needle was developed. The dose of laser light was based on comparison of biopsies of punctures with Morrow-Brown Needles and varying combinations of laser energy parameters. The resulting laser settings were tested in twenty volunteers who had been referred for skin testing. Blinded tests were conducted with both procedures in parallel using histamine, saline, Dermatophagoides pteronyssinus, and Lolium perenne. Comparable whealing reactions were produced by laser skin testing and puncture testing with a Morrow-Brown Needle. These results suggest several potential advantages of laser skin testing over the existing test.

Adult

Dermatopathologic findings in patients infected with HIV.

Several inflammatory, infectious, and neoplastic conditions in HIV-infected patients are distinctive or require a biopsy for diagnosis. Some differ subtly from similar conditions seen in noninfected patients. The exanthem of acute HIV infection cannot be diagnosed specifically on biopsy as its histologic appearance is similar to that of other viral exanthemata. A condition that closely resembles seborrheic dermatitis occurs in HIV-infected patients. Plasma cells, necrotic keratinocytes, and leukocytoclasis may be present, in contrast to findings in sporadic seborrheic dermatitis. Psoriasis and Reiter's disease also occur in HIV-infected patients and can be specifically diagnosed as such. The category "psoriasiform dermatitis of AIDS" thus seems to include several distinct entities and not to be a single disease. Bacillary angiomatosis is a treatable infection caused by a rickettsialike organism similar to Rochalimaea quintana, the agent of trench fever. Cutaneous lesions are characterized by lobules of capillaries with protuberant endothelial cells, neutrophils and their debris, and purplish-staining clumps of organisms, which can be demonstrated with silver stains or electron microscopy. An unusual reaction to atypical mycobacterial infection, in which spindle-shaped macrophages are seen, resembles histoid leprosy. Viral skin diseases that may challenge the dermatopathologist include unusual verrucous reactions to chronic varicella-zoster infection and flat warts caused by the human papillomavirus associated with epidermodysplasia verruciformis. Keratinocytes with foamy basophilic cytoplasm may be a marker for one of these viruses, human papillomavirus type 5. Neoplastic complications of HIV disease include Kaposi's sarcoma and mycosis fungoides. The earliest lesions of the patch stage of Kaposi's sarcoma show a slightly increased number of cells with small ovoid nuclei around preexistent structures, accompanied, in some cases, by sparse infiltrates of lymphocytes and plasma cells. Staining with antisera to type IV collagen may highlight the vascular spaces in these early lesions. Later lesions that resemble hemangiomas may also prove challenging and require level sections to demonstrate the presence of spindle cells and eosinophilic globules. Although HIV is cytotoxic to helper T cells, neoplastic proliferations of them may be seen in HIV-infected patients. These cases of mycosis fungoides do not seem to differ from sporadically occurring ones and occur in patients who seem not to be infected by HTLV-I.

HIV Infections

Granulomatous vasculitis occurring after cutaneous herpes zoster despite absence of viral genome.

Granuloma annulare, sarcoidal and other granulomatous dermatitides, pseudolymphoma, lymphoplasmacytoid lymphoma, and Kaposi's sarcoma have been described as sequelae of herpes zoster. We report a new postzoster reaction, granulomatous vasculitis, that caused flat-topped papules restricted to the affected dermatome. Polymerase chain reaction failed to detect varicella-zoster virus in a biopsy specimen. These results suggest that granulomatous vasculitis occurs without persistence of the viral genome and, perhaps, is a reaction to minute amounts of viral proteins.

Adult

Circumscribed storiform collagenoma (sclerosing fibroma).

In the past several years we have examined eight dermal nodules that have morphologic features identical to the nodules described in patients with Cowden's disease. The patients in this series had no other clinical manifestations of Cowden's disease. In an attempt to better define this distinctive entity, we subjected tissue sections to a battery of histochemical and immunohistochemical stains and examined tissue from one of the nodules ultrastructurally. Although we found similarities between these nodules and other common dermal fibrotic lesions, we believe that they are distinctive architecturally (they are sharply circumscribed and have a strikingly uniform storiform pattern) and immunohistochemically (with uniformly scattered factor 13a-positive cells). Because of the unique histologic features, we propose that the term "circumscribed storiform collagenoma" be applied to these nodules.

Adult

Primitive polypoid granular-cell tumor and other cutaneous granular-cell neoplasms of apparent nonneural origin.

Most cutaneous and noncutaneous granular-cell tumors are currently thought to be of Schwann-cell derivation. We present seven unusual cutaneous granular-cell lesions in which Schwann-cell origin can be excluded or is inapparent. Four of these lesions are of a previously undescribed type, and, unlike conventional granular-cell tumors of the skin, show a polypoid configuration, numerous mitoses, cytologic atypia, and a primitive immunophenotype. We propose the term "primitive polypoid granular-cell tumor" for these lesions. One occurred in a child, and three in adults. There have been no metastases to date, with follow-up periods of 2, 4, 4, and 16 years, respectively, although one tumor recurred locally. Additional cases and longer follow-up may be required to rule out the possibility that primitive polypoid granular-cell tumor is a low-grade malignancy. Two other granular-cell lesions represent variants of leiomyosarcoma, one of which widely metastasized. The last case is a granular-cell form of nodular basal-cell carcinoma. Cutaneous granular-cell neoplasms can show varying differentiation and behavior. Pathologists should not equate the occurrence of cytoplasmic granularity in a cutaneous neoplasm with the diagnosis of granular-cell schwannoma.

Adult

Lymphoepithelioma-like carcinoma of the skin with adnexal differentiation.

Lymphoepithelioma-like carcinoma of the skin (LELCS) is a recently-described tumor entity with a microscopic resemblance to undifferentiated carcinoma of the nasopharynx. Only five cases of this lesion have been reported heretofore. We document three additional examples of LELCS that were composed of clustered dermal arrays of cytologically malignant, mitotically active polygonal cells, without connections to the overlying epidermis or skin appendages. Chronic inflammatory cells were interspersed throughout each of the tumors. Two of them demonstrated multiple foci of trichilemmal-type keratinization among the neoplastic cells, whereas the other exhibited focal eccrine ductal or glandular differentiation. None of the neoplasms was found to contain nucleic acid of Epstein-Barr virus by in situ hybridization. It is postulated that LELCS represents a primitive cutaneous appendage tumor. Based on the results of a prior publication and those of the current assessment, this neoplasm is thought to have the potential for both sweat glandular and follicular differentiation.

Aged

Human immunodeficiency virus-associated eosinophilic folliculitis. A unique dermatosis associated with advanced human immunodeficiency virus infection.

We studied 13 patients with human immunodeficiency virus (HIV) infection and a chronic pruritic folliculitis that was unresponsive to systemic treatment with bactericidal anti-staphylococcal antibiotics. The skin eruption was characterized by multiple urticarial follicular papules scattered on the trunk (100%), the head and neck (85%), and the proximal aspect of the extremities (62%). Absolute peripheral eosinophil counts were increased in six of 13 patients; a relative peripheral eosinophilia was present in 10 of 13 patients. Serum IgE levels were elevated in all seven patients tested (range, 88 to 9050 IU). Histopathologic features included a folliculitis with eosinophils. Pathogenic bacteria were not consistently found by routine bacterial skin cultures, cultures of skin biopsy specimens, or histopathologic evaluation. CD4 counts were decreased in all of the 12 patients tested (less than 300 cells per cubic millimeter) and were below 250 cells per cubic millimeter in 10 patients. A clinical response was noted to astemizole, to ultraviolet light in the B range, and to topical clobetasol propionate. These observations demonstrate that HIV-associated eosinophilic folliculitis is a unique HIV-related cutaneous disorder that is characterized by a culture-negative, chronic, pruritic folliculitis and a characteristic histopathologic picture. Of special importance, because it is associated with CD4 counts of less than 250 to 300 cells per cubic millimeter, eosinophilic folliculitis appears to be an important clinical marker of HIV infection and, particularly, of patients at increased risk of developing opportunistic infections. We suggest that the term eosinophilic pustular folliculitis (Ofuji's disease), previously used to describe this dermatosis in HIV-infected patients, should be discarded.

Acquired Immunodeficiency Syndrome

Bacillary angiomatosis: a systemic opportunistic infection with prominent cutaneous manifestations.

Bacillary angiomatosis is an opportunistic infection with systemic manifestations. Although most cases have occurred in human immunodeficiency virus-positive patients, other immunosuppressed patients, and even seemingly immunocompetent individuals, can become infected. In addition to the well-characterized cutaneous manifestations, visceral involvement can occur and may be the only locus of infection. Lymphadenopathy, bone or soft-tissue masses, fever, and hepatosplenomegaly can be presenting signs. The causative bacterium is still unidentified, but resemblances to the rickettsiae, Rochilamea quintana, the recently identified cat-scratch disease bacillus, and Bartonella bacilliformis have been noted by various investigators. Systemic disease is treatable and can be cured with antibiotic therapy.

Angiomatosis, Bacillary

Bacillary angiomatosis: a newly characterized, pseudoneoplastic, infectious, cutaneous vascular disorder.

Bacillary angiomatosis (also called epithelioid angiomatosis) is a newly recognized disease most often characterized by a cutaneous infection with reddish papules of vascular origin. It is caused by a weakly reactive gram-negative bacillus, which can be easily demonstrated in tissue sections with the Warthin-Starry stain. Bacillary angiomatosis usually responds readily to treatment with oral erythromycin, 250 to 500 mg, taken four times a day for 2 weeks to 1 month. Because the skin is the most common site of involvement, it is important that the dermatologist recognize this unusual condition. It is essential that treatment be started as soon as possible because deaths may result from visceral and mucosal involvement. The clinical, histologic, and microbiologic aspects of bacillary angiomatosis are discussed and depicted in detail and speculations regarding the pathogenesis are rendered.

Angiomatosis