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Biomedical subjects

R K Winkelmann

Publications and source records attributed to R K Winkelmann.

At least 19 recordsLinked to original sources

Necrobiotic xanthogranuloma with myocardial lesions and nodular transformation of the liver.

A 73-year-old woman with a history of necrobiotic xanthogranuloma (NXG) with paraproteinemia died suddenly while hospitalized for bronchitis and congestive heart failure. At postmortem examination, myocardial lesions histologically typical of NXG were found. This is the fifth reported autopsy of a patient with NXG. In all five autopsied cases, and in two additional surgical cases, necrobiotic granulomas of internal viscera were present, and in three cases the myocardium was involved. In addition, our patient had nodular transformation of the liver, a rare lesion that was also reported in one of the previous autopsy cases with NXG.

Aged

Clinicopathologic spectrum of specific cutaneous lesions of disseminated coccidioidomycosis.

BACKGROUND: Disseminated coccidioidomycosis merits greater attention because the number of persons living and traveling in endemic areas is increasing. OBJECTIVE: Our purpose was to study the clinical and histopathologic findings in patients with specific cutaneous disseminated coccidioidomycosis. METHODS: In six patients with specific skin lesions of disseminated coccidioidomycosis, the diagnosis was confirmed by identification of the organism in tissue or by positive results of tissue culturing. RESULTS: Clinical lesions included solitary granulomatous plaques in two patients and multiple papular, nodular, or pustular lesions in four patients, two of whom also had subcutaneous abscesses. Identifying organisms directly in tissue was possible in only 8 of 17 biopsy specimens and in five of six patients. The histopathologic features showed various degrees of three primary patterns: (1) abscess formation with necrosis, (2) epithelial hyperplasia and granuloma formation with microabscesses, and (3) vascular and perivascular proliferative and inflammatory cell reactions at times suggesting vasculitis. Tissue eosinophilia, present in all patients, was striking in two (eosinophilic abscess formation) and notable in another (vascular inflammation with eosinophilia). CONCLUSION: Cutaneous manifestations of disseminated coccidioidomycosis may be more common and varied than usually recognized.

Adolescent

Primary B-cell lymphoma with histologic features of a T-cell neoplasm.

A 58-year-old white man had dermatomyositis and primary cutaneous B-cell lymphoma. The cutaneous lymphoma was evidenced by a noduloulcerative disease of the lower extremities. Histologic results resembled a T-cell process with a diffuse, superficial infiltrate composed of small- and medium-sized lymphocytes with angioinvasion and epidermotropism. The infiltrate extended into the deep dermis and panniculus with scattered large lymphocytes and necrosis. With the help of gene-rearrangement analysis and immunophenotyping, the true B-cell lineage was discovered. The importance of gene-rearrangement analysis and immunophenotyping in the diagnosis of cutaneous lymphoma is emphasized.

Blotting, Southern

Granulomatous slack skin: a clinicopathological and immunohistochemical study of three cases.

Three cases of granulomatous slack skin (GSS), a rare variant of T-cell lymphoma, are reported. Immunohistochemical studies using a panel of 16 antibodies were carried out on both frozen tissue and tissue embedded in paraffin wax to characterize the infiltrate. A routine immunoperoxidase technique was used to identify T cells (UCHL1, CD45R0), B cells (L26, 4KB5 [CD45R]), S100 protein-positive cells, monocytes/macrophages (Mac-387, KP1 [CD68]), and dermal dendrocytes (factor XIIIa) in paraffin sections. A close association was found between UCHL1-positive T cells and KP1-positive giant cells. A number of S100-positive cells and factor XIIIa-positive cells were present in the infiltrate from all three patients. The lymphocytes in two of the patients were predominantly of the helper T-cell phenotype. Giant cells from all three patients stained with KP1 (CD68) and Leu M3 (CD14). These studies confirm that the infiltrate in GSS is predominantly a T-cell disorder associated with monocyte-derived cells rather than with resident dendritic macrophages.

Adult

Erythema elevatum diutinum: a clinicopathological study.

Erythema elevatum diutinum is a syndrome of vasculitis in which lesions, typically over the extensor surfaces, showed a mixed inflammatory infiltrate on biopsy. We describe a series of 13 patients. The most common association in our series was with hypergammaglobulinemia; both mono and polyclonal. Chronic infection, not streptococcal, was a less frequent finding although two of three patients had a positive reaction to the intradermal injection of streptococcal antigen. Dapsone remains the initial treatment of choice.

Adolescent

Persistent painful plaque due to a brown recluse spider bite.

The bite of the arthropod Loxosceles is known to cause subdermal hemorrhage, dermal-epidermal separation, inflammatory infiltrates, as well as occlusion and necrosis of small arterioles. We report a case of a brown recluse spider bite that presented as a chronic painful skin plaque, with the unusual histologic findings of a cutaneous and deep subcutaneous hyalinizing panniculitis and myonecrosis.

Atrophy

Necrobiotic xanthogranuloma.

We reviewed 32 cases of necrobiotic xanthogranuloma and 16 cases from the world literature. Necrobiotic xanthogranuloma is a destructive dermal and subcutaneous xanthogranuloma that most frequently involves the face (periorbital region) and trunk. Monoclonal gammopathy is common (IgG-kappa in 23 cases and IgG-lambda in nine cases), and cryoglobulins have also been found. Low complement levels, anemia, and leukopenia are frequently present. Bone marrow examination shows plasma cell proliferation and, rarely, true myeloma. We describe mucosal, muscle, and systemic lesions, including xanthogranulomatous myocardial lesions. The clinical course is chronic and often progressive. Low-dose chlorambucil treatment is safe and effective, but individual patients have responded to treatment with corticosteroids, melphalan, local radiation, and plasma exchange.

Combined Modality Therapy

Epidermotropic eccrine porocarcinoma.

Three cases of epidermotropic eccrine porocarcinoma are summarized and compared with reported cases. All patients had a long-standing tumor on a lower extremity that rapidly metastasized to the skin and proximal lymph nodes. The histologic picture was consistent with an intraepidermal eccrine sweat gland carcinoma.

Adenocarcinoma

Cutaneous manifestations in patients with essential thrombocythemia.

In a retrospective study of 268 patients with essential thrombocythemia, related cutaneous manifestations were found in 58 (22%). In 27 cases (10%) the related skin lesions were present at the time of the primary diagnosis of essential thrombocythemia. Hematomas, ecchymoses, petechiae, or purpura occurred in 24 (41%) of the 58 patients. Fifteen patients (26%) had erythromelalgia, in 11 of whom it was the initial complaint. Livedo reticularis, recurrent superficial thrombophlebitis, ischemic complications with gangrene, leg ulcers, or ulcers on the toes were other manifestations. Urticaria occurred in two patients, and Raynaud's phenomenon and necrotizing vasculitis were each observed in one patient. Platelet function may be abnormal, and this was found in 13 of 19 patients studied, 9 of whom had spontaneous platelet aggregation. Knowledge of the cutaneous manifestations in essential thrombocythemia may lead to its earlier detection. Prompt diagnosis of essential thrombocythemia is important because treatment may prevent severe hemorrhagic or thrombotic events.

Adult

Histopathologic features of the L-tryptophan-related eosinophilia-myalgia (fasciitis) syndrome.

Study of 18 biopsy specimens in 11 patients with L-tryptophan-related eosinophiliamyalgia (fasciitis) syndrome showed hyaline sclerodermoid changes. Dermal scleroderma was found in eight of nine punch biopsy specimens and eight of nine excisional biopsy specimens. Fascial scleroderma was found in eight excisional biopsy specimens. One specimen obtained by excision had no fascia present. Eleven biopsy specimens showed edema of the dermis, and 13 showed dilated lymphatic structures; thus, the clinical picture of edematous sclerosis was confirmed. Mucinous fasciitis was present in five excisional biopsy specimens, in conjunction with a large number of macrophages in four. Dermal mucinosis was present in 11 biopsy specimens. Lymphocytic and macrophage inflammation was minimal in 14 biopsy specimens and pronounced in only 4. Plasma cells were present in eight cases. Eosinophils were present in substantial numbers in three biopsy specimens and only occasionally in four. Eosinophilic spongiosis was observed in one patient. Lymphocytic inflammation was noted around a single muscle spindle and around large nerve trunks in three patients. No relationship was established between these pathologic features and the duration or dose of tryptophan, prednisone treatment, or duration of symptoms. Pathologic features of the L-tryptophan syndrome consist of hyaline sclerodermoid collagen in the dermis, the septa, and the fascia. Edema, focal mucinosis, and macrophage inflammation may be features that identify this event.

Collagen

The evolution of Hodgkin's disease and necrobiotic xanthogranuloma syndrome.

We describe a woman in whom hypogammaglobulinemia and severe granulomatous cutaneous lesions had developed during childhood; subsequently, Hodgkin's disease and necrobiotic xanthogranuloma were diagnosed. This case illustrates an apparent association with disease activity and raises the question of a direct relationship of necrobiotic xanthogranuloma with lymphoproliferative disease.

Adolescent

Adult lipophagic atrophic panniculitis.

We report two patients with chronic, recurrent, nodular panniculitis lesions that later developed areas of lipoatrophy. Histologically, there was a consistent lobular lipophagic replacement of fat cells with lipophagic giant cells. The clinical appearance was that of tender, erythematous, superficial or subcutaneous, symmetrical nodules and plaques of 1-2 weeks' duration. The lesions could occur with episodes of fever. One patient had hepatomegaly and the other had an increased sedimentation rate and leucocytosis. The histology and the clinical pattern of the panniculitus syndrome resembled those of lipophagic lipoatrophy of childhood. This is a panniculitis of unknown cause in which the principal inflammatory cell response in the subcutaneous tissue is the macrophage.

Adipose Tissue

Diffuse-plane normolipaemic xanthoma with aortic-valve xanthoma.

A patient with diffuse-plane normolipaemic xanthomatosis had mucous-membrane, conjunctival, and aortic-valve xanthomatosis. The presence of a monoclonal protein and hypocomplementaemia suggests that the xanthomatous lesions were probably not metabolic in origin but developed as secondary events in a histiocytosis of involved tissues. No heart-valve xanthomatization in like circumstances has previously been reported.

Aortic Valve

Destructive granuloma annulare of the skin and underlying soft tissues--report of two cases.

Two cases of extensive cutaneous granuloma annulare (GA) associated with severe deformity and impairment of function in the involved extremity are described. In the first patient a mutilating, destructive arthritic condition and infiltrative oedema of upper extremities developed over the years along with florid disseminated GA of the tendons and overlying skin. In the second patient disseminated GA of one lower extremity resulted in diffuse induration and chronic lymphoedema. In both cases, histology showed palisading granulomas typical of GA at all levels of the dermis, subcutaneous tissues, fascia, and synovium. The unique clinical features of GA in both presented cases emphasize the local destructive potential and, as yet, poorly understood progression of GA to a soft-tissue disease.

Aged

Lipophagic panniculitis in re-excision specimens.

Lipophagic panniculitis consists of a macrophage infiltrate in the subcutaneous tissue. The macrophages transform into foam cells within the panniculus; they replace lipocytes and may form giant cells. Although those pathologic features have been described as diagnostic of Weber-Christian disease, we report the occurrence of lipophagic panniculitis in re-excision specimens. Among 252 re-excision specimens from previously biopsied skin tumors, 5 cases in which masses of lipophages were infiltrating and replacing the subcutaneous tissue were found. The infiltrate was localized to the deep dermis and superficial subcutaneous tissue below and beside the initial biopsy site. In 3 cases, suture or hair was detected within the tissue, and granulation tissue with foreign body giant cells was observed along the dermal suture line. In 4 cases there was evidence of phlebitis within or close to areas of infiltration. None of these patients developed symptomatic panniculitis. Lipophagia can be a normal response of wound healing in some patients.

Foam Cells

Septal granulomatous panniculitis: comparison of the pathology of erythema nodosum migrans (migratory panniculitis) and chronic erythema nodosum.

Fifty-eight cases of septal granulomatous panniculitis were reviewed; 14 cases were diagnosed as erythema nodosum migrans (migratory panniculitis) and 36 as chronic erythema nodosum on the basis of clinical and histopathologic features. Erythema nodosum migrans was characterized by markedly thickened and fibrotic septae, marked capillary proliferation (like granulation tissue), and massive granulomatous reaction (with giant cells) along the borders of the widened septa. Hemorrhage was rare, and phlebitis was not seen. Chronic erythema nodosum showed mild septal change, little fibrosis, and lymphohistiocytic perivascular inflammation with only focal granulomatous formation. Phlebitis and hemorrhage were common. The condition termed erythema nodosum migrans has many of the same clinical features as chronic erythema nodosum, and we think this term is preferable to migratory panniculitis. We believe that there are sufficient clinical and histopathologic features to justify considering erythema nodosum migrans as a unique clinicopathologic entity.

Adult

Mycosis fungoides in children and adolescents.

The clinical and histologic findings in five young patients with mycosis fungoides are reviewed. The skin specimen from a 16-year-old boy had an infiltrate predominantly Leu-1+, Leu-4+, Leu-3a + 3b+, OKT6+, Leu-2a-, Leu-8-, and Leu-9-, and rearrangements were noted in T cell receptor gene (beta chain [constant region probe] and gamma chain [J region probe]); no rearrangements were found in a histologically normal lymph node or peripheral blood. The skin specimen from an 11-year-old girl contained predominantly Leu-4+ and Leu-9+ lymphoid cells. No T cell receptor gene rearrangements were found in the skin or in an involved lymph node. The variations in clinical, histologic, and immunopathologic features suggest that mycosis fungoides in young patients represents a heterogeneous subgroup of cutaneous T cell lymphoma.

Adolescent

The heterogeneity of Jessner's lymphocytic infiltration of the skin. Immunohistochemical studies suggesting one form of perivascular lymphocytoma.

Seventeen patients (six women and 11 men) with Jessner's lymphocytic infiltration of the skin were studied. Nineteen biopsy specimens were analyzed with the following monoclonal antibodies: UCHL1 (T cells), 4KB5, L26 (B cells), LN1 (germinal center B cells), and BerH2 (Ki-1+ cells). Routine direct immunofluorescence was performed on 15 specimens. In 10 of 19 specimens both B and T cells were demonstrated; the B cells were in close proximity to small blood vessels. In 50% of these cases the B cell component expressed LN1 positivity. We use the term perivascular lymphocytoma to describe this pattern. Nine specimens showed T cells only. No Ki-1+ cells were detected. Routine immunofluorescence studies were performed on 15 specimens. In six of seven specimens in which there was a mixed cell population of B and T cells, results were completely negative. In contrast, six of the remaining eight specimens with a predominantly T cell population revealed weak, patchy, granular, positive labeling for immunocomponents, particularly IgM and C3 at the basement membrane. These findings suggest that Jessner's lymphocytic infiltration is a heterogeneous disorder with at least two separate immunophenotypes. The finding of perivascular follicular center differentiation in more than half the specimens suggests a possible relationship in these cases to other benign lymphoid hyperplasias (pseudolymphomas).

Adult