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Biomedical subjects

L E Golitz

Publications and source records attributed to L E Golitz.

At least 19 recordsLinked to original sources

Cutaneous macroglobulinosis. A case report with unique ultrastructural findings.

BACKGROUND: Cutaneous macroglobulinosis is a rare cutaneous manifestation of Waldenström's macroglobulinemia. Lesions result from the direct deposition of macroglobulin in the skin and have been called IgM storage papules. A case of cutaneous macroglobulinosis with unique ultrastructural findings was studied. OBSERVATIONS: Cutaneous macroglobulinosis is characterized by multiple flesh-colored papules on extensor skin surfaces. Histologically, there are dermal collections of eosinophilic hyaline material, simulating amyloid. The material is positive on periodic acid-Schiff staining. Amyloid stains are negative or equivocal. Electron microscopy reveals thick, nonbranching, 56-nm-wide, linear material with cross striations at 12-nm intervals. These ultrastructural findings differ from the three previously reported cases. CONCLUSIONS: Cutaneous macroglobulinosis may be a rare presenting sign of Waldenström's macroglobulinemia. Deposits of macroglobulin in the skin result in a histologic picture that greatly resembles amyloid. Histochemical stains, direct immunofluorescence microscopy, and electron microscopy are useful tools that enable accurate diagnosis and help to distinguish cutaneous macroglobulinosis from other deposition disorders.

Humans

Papulonodular dermal mucinosis in lupus erythematosus.

We report two cases of lupus erythematosus (LE) in which a truncal papulonodular eruption predominated. Histologically the lesions were characterized by diffuse dermal mucin without the usual inflammatory or epidermal changes of LE. It is uncommon in LE for dermal mucin to be present in a sufficient quantity to produce a papulonodular eruption in the absence of typical epidermal changes.

Adult

Clinical, histologic, and immunofluorescent distinctions between subacute cutaneous lupus erythematosus and discoid lupus erythematosus.

Subacute cutaneous lupus erythematosus (SCLE) was originally described and distinguished from discoid lupus erythematosus (DLE) on the basis of clinical examination of the skin, but subsequent reports have questioned the concept of SCLE as a marker of a unique subset of LE patients. We classified 27 lupus patients, on the basis of cutaneous exam, as having discoid lupus skin lesions, subacute cutaneous skin lesions, or systemic lupus erythematosus (SLE) without DLE or SCLE lesions. Clinical features most characteristic of SCLE rather than DLE were superficial, non-indurated, non-scarring lesions, and photosensitivity, with lack of induration being the single most helpful finding. Histologic examination of lesional skin showed a relatively sparse, superficial infiltrate in SCLE and a denser, deeper infiltrate in DLE. A distinctive pattern of staining with direct immunofluorescence, particulate epidermal IgG deposition, was found in seven of seven SCLE patients (all anti-Ro/SSA positive) and none of the other patients. This distinctive pattern can be reproduced experimentally when anti-Ro/SSA autoantibodies are infused into human skin-grafted mice. Particulate dermal-epidermal junctional staining was the pattern seen in the patients who did not have SCLE. Clinically defining SCLE as a superficial inflammatory form of cutaneous lupus (i.e., considering lesions to be DLE if they are indurated) results in a meaningful segregation of SCLE and DLE patient groups. The epidermal IgG deposits unique to SCLE provide independent evidence that the clinical findings that were used to identify the patient groups actually identify distinctive cutaneous lupus subsets. The observation that antibodies are present in a different location in the skin in SCLE than in DLE indicates that SCLE and DLE are likely to have different pathomechanisms.

Antibodies, Antinuclear

Rheumatoid neutrophilic dermatitis.

Rheumatoid neutrophilic dermatitis is a rare entity reported to occur in association with severe rheumatoid arthritis. Three patients with seropositive rheumatoid arthritis and rheumatoid neutrophilic dermatitis are described. Clinically, the eruption is characterized by symmetric erythematous papules, plaques, and rarely vesicles on extensor skin surfaces. Light microscopy reveals a dermal neutrophilic infiltrate without vasculitis. Spongiotic intraepidermal blisters, subepidermal bullae, or papillary neutrophilic microabscesses may be seen. The histologic differential diagnosis includes the other dermatoses in which neutrophils predominate. Rheumatoid neutrophilic dermatitis is one of several neutrophilic dermatoses seen in association with rheumatoid arthritis, suggesting that it may be part of a spectrum of neutrophilic cutaneous reaction patterns in a predisposed host.

Adult

L-tryptophan syndrome: histologic features of scleroderma-like skin changes.

The eosinophilia-myalgia syndrome (EMS) associated with the ingestion of L-tryptophan (LT) containing products has recently been recognized in the United States. We report the histologic features of the cutaneous scleroderma-like changes in four patients. All of the patients met the Center for Disease Control criteria for EMS and had a history of LT ingestion. Skin biopsies showed increased dermal mucin and dermal sclerosis, with trapping of adnexal structures. There are clinical and histologic similarities between EMS, scleroderma, the toxic oil syndrome, and fasciitis with eosinophils.

Adult

Absence of HTLV-I DNA sequences in cutaneous T-cell lymphoma/mycosis fungoides.

Recently, the presence of deleted forms of the HTLV-I provirus was described in 6 out of 6 cases of CTCL. We investigated whether the presence of these viral genomes could be verified in 20 patients with CTCL using the polymerase chain reaction (PCR) and Southern blot analysis. Only one of the 20 cases showed a band corresponding to the pX region of HTLV-I. These data indicate that in the majority of CTCL cases, sequences closely related to HTLV-I are not present, or their copy number is below the limit of detection employed in this study.

Base Sequence

Mononeuropathy multiplex associated with acute hepatitis B infection.

Immunologic syndromes are associated with hepatitis B viral (HBV) infection. However, mononeuropathy multiplex (MM), a syndrome in which immune factors may play a role, is rare in acute HBV infection. Few cases of MM associated with HBV infection have been reported. We report a case of acute HBV infection in which MM involving the median, ulnar and lateral femoral cutaneous nerves complicated the course of the acute illness. Skin biopsy demonstrated vasculitic changes which may accompany the immunologic factors in HBV infection.

Acute Disease

Cutaneous angiolipoleiomyoma.

We describe eight cases of cutaneous angiolipoleiomyoma, a rare tumor previously reported only once under the term cutaneous angiomyolipoma. Clinically, the tumors were acquired, solitary, asymptomatic nodules that were always acral in location. Patients' ages ranged from 33 to 77 years (median 52.6 years); the male/female ratio was 7:1. Signs of tuberous sclerosis or renal angiomyolipoma were absent in all cases. Histologically, the tumors were subcutaneous, well circumscribed, and composed of smooth muscle, vascular spaces, connective tissue, and mature fat. In some tumors the fat was the predominant component, and in others smooth muscle predominated. Elastic tissue stains revealed that some blood vessels had developed an elastic lamina whereas other blood vessels lacked it. Additional histologic features occasionally observed included vascular thrombi, glomus bodies, and focal mucin deposition.

Adult

Targetoid hemosiderotic hemangioma.

A case of targetoid hemosiderotic hemangioma on the buttock of a 20-year-old pregnant woman is presented. Eight cases of this newly-described entity were initially reported by Santa Cruz and Aronberg in 1988. It is important to distinguish these vascular lesions from Kaposi's sarcoma, epithelioid hemangioma, and progressive lymphangioma.

Adult

Hamartomas.

Hamartomas of the skin are tumor-like malformations of mature or nearly mature structures that are part of the normal structure of skin. The onset is usually at birth; however, it may be delayed until childhood or early adulthood. Hamartomas may occur on any part of the body and are sometimes linear and unilateral. Histologically, they may show an alteration of a single cell line or of multiple related cell lines. Some types of the hamartomas may be markers for underlying internal organ abnormalties, such as the epidermal nevus syndrome, the nevus comedonicus syndrome, or the organoid nevus syndrome. Some may be prone to develop various secondary benign or malignant tumors as in an organoid nevus. Hamartomas may occur as solitary, sporadic lesions unrelated to other conditions or as multiple lesions that are inherited as an autosomal trait. The latter are often associated with systemic abnormalites. Hamartomas such as Becker's pigmented hairy nevus appear to be inherited as an autosomal dominant trait, but the late onset and variable expression may be under hormonal influence. So far only tuberous sclerosis has been shown to be related to a specific chromosomal abnormality, mutant gene located on the long arm of chromosome 9.

Female

Sclerotic fibromas of the skin.

Eleven cases of a peculiar, hyalinized, hypocellular dermal fibroma are reported. The lesions are characterized by epidermal atrophy, a whorled appearance of sclerotic collagen bundles separated by clefts containing mucin, and sharp demarcation of the lesions from the surrounding normal skin. They are small white or flesh-colored waxy papules. Multiple lesions of this type have been reported in the multiple hamartoma syndrome (Cowden's disease). We believe that the solitary lesions of these eleven patients who did not have Cowden's disease represent an unrecognized form of the same fibrous hamartoma that occurs in Cowden's disease.

Adult

Papillary mesenchymal bodies: a histologic finding useful in differentiating trichoepitheliomas from basal cell carcinomas.

To distinguish a basal cell carcinoma (BCC) from a trichoepithelioma can be difficult even for an experienced dermatopathologist. Previously reported differentiating histologic features are relative criteria that may be shared by both tumors. In a review of 30 consecutive cases each of trichoepitheliomas, keratotic BCC, and routine BCC, classic criteria were compared with papillary mesenchymal body formation. Papillary mesenchymal bodies are distinct fibroblastic aggregations that represent abortive attempts to form the papillary mesenchyme responsible for hair induction. Papillary mesenchymal bodies were observed in 93% of all trichoepitheliomas, 7% of all keratotic BCC, and 0% of all routine BCC. Hair bulb formation was observed in 30% of trichoepitheliomas and in none of the BCC. We conclude that papillary mesenchymal body formation is an easily recognizable histologic criterion that is more reliable in differentiating these two tumors than standard criteria, including epidermal connections, keratinization, calcification, foreign body reaction, fibrosis, stromal retraction, tumor mucin, ulceration, frondlike epithelial pattern, and the inflammatory response.

Basal Cell Carcinoma

Mucinous carcinoma.

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Adenocarcinoma, Mucinous

An immunopathological study of herpes-associated erythema multiforme.

Any pathogenetic mechanism proposed for erythema multiforme (EM) must account for the prominent mononuclear cell infiltrate in the skin lesions. The purpose of this study was to characterize immunopathologically, with monoclonal antibodies to human leukocyte antigens, the inflammatory cells in early target lesions of recurrent herpes-associated EM. Cryostat sections of snap-frozen skin biopsies were studied by the avidin-biotin immunoperoxidase technique with use of the following monoclonal antibodies: anti-HLA-DR, anti-Leu M5, anti-Leu 4 + 5b, anti-Leu 3a + 3b, anti-Leu 2a, anti-Leu 14, and anti-Leu 6. The dermal mononuclear inflammatory infiltrate in the EM biopsies consisted of monocyte-macrophages and T-lymphocytes, with both helper and suppressor T cells present. Both the dermal inflammatory infiltrate and the overlying keratinocytes were strongly HLA-DR positive. No definite alteration of Langerhans cell number or distribution was noted. These findings are consistent with the characteristics seen in cell-mediated immune reactions in the skin and point to this as a likely immune mechanism for the tissue damage of EM.

Adult

Diffuse neonatal hemangiomatosis.

Diffuse neonatal hemangiomatosis is an often fatal disorder characterized by widespread capillary hemangiomas of the skin and visceral organs. Ultrasound and computed tomographic scans may be useful in determining the extent of visceral disease. The organs most commonly affected are the gastrointestinal tract, brain, liver, and lung. Complications include high-output cardiac failure, gastrointestinal bleeding, hydrocephalus, and consumption coagulopathy. Despite therapy with corticosteroids, the mortality rate is high.

Brain Neoplasms

The vasculitides and their significance in the pediatric age group.

The vasculitides in infants and children may involve small or large cutaneous vessels and may affect a variety of internal organs. With the exception of Henoch-Schönlein purpura, vasculitis is rare in the pediatric age group. Progress has been made in determining the pathogenesis of childhood vasculitis, and there has been a significant improvement in therapy.

Blood Vessels