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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

The vascular calcification-cutaneous necrosis syndrome.

BACKGROUND: Although medial calcification of larger elastic arteries in chronic kidney failure and with advancing age is relatively common, calcification of the cutaneous vascular system is rare. OBJECTIVE: Our purpose was to describe three patients with the vascular calcification-cutaneous necrosis syndrome and review the cause, clinical and pathologic features, and treatment of this syndrome. METHODS: We describe three patients with ischemic necrotic ulcers and underlying cutaneous vascular calcification. The clinical setting was abnormal calcium metabolism from either chronic kidney failure or excessive vitamin D intake. RESULTS: The clinical findings in all patients consisted of multiple tender livedoid nodules and ulcerative plaques on the thighs and legs, which developed in the setting of abnormal calcium metabolism from either chronic kidney failure or excessive vitamin D intake. Histologic study demonstrated vascular calcification. Although this syndrome usually has a chronic course with significant morbidity and mortality, subtotal parathyroidectomy followed by kidney transplantation resulted in complete resolution in one of our patients. CONCLUSION: The clinical and histopathologic findings in the vascular calcification-cutaneous necrosis syndrome are unique. The pathogenesis is likely multifactorial. Treatment for the skin lesions is largely supportive.

Adult

Successful treatment response of granuloma annulare and carpal tunnel syndrome to chlorambucil.

We describe a 62-year-old woman in whom skin biopsies verified the clinical diagnosis of granuloma annulare and neurologic and electromyographic studies confirmed the neurologic diagnosis of carpal tunnel syndrome. Short-term treatment with a low dose of chlorambucil taken orally was prescribed. Within weeks, the granuloma annulare had disappeared, and the clinical symptoms of carpal tunnel syndrome had resolved. Electromyography showed variable improvement at the end of treatment and resolution at 9-month follow-up. Our case confirms that short-term treatment of granuloma annulare and associated carpal tunnel syndrome with low-dose chlorambucil is successful.

Carpal Tunnel Syndrome

Fatal systemic cytophagic histiocytic panniculitis: a histopathologic and immunohistochemical study of multiple organ sites.

The presence of hemocytophagic histiocytosis within subcutaneous tissue has been termed "cytophagic histiocytic panniculitis" (CHP). CHP may occur as a feature of hematopoietic malignancies or infections, particularly viral. In some patients with CHP, an underlying illness cannot be identified. We describe a young man with a rapidly fatal systemic illness characterized by CHP. Lymphoma was not present, and an infectious agent could not be identified. Immunohistochemical stains of tissue obtained at autopsy from multiple organ sites confirmed the presence of histiocytes and T lymphocytes within adipose tissue.

Adipose Tissue

Basal cell carcinoma on the lower extremity.

From 1987 to 1990, 21 of 648 patients with basal cell carcinomas (BCCs) had their lesion located on the leg. Basal cell carcinomas in this location are uncommon. Women outnumbered men by 1.6:1. The majority of patients had types I and II skin. Most reported residence in the southwestern United States for many years. Recreational sun exposure was extensive and dominated by activities in which exposure of the legs was common. Most lesions were recognized clinically and located on the anterior lower leg. The most frequent histologic description was superficial BCC. Treatment was with electrodesiccation and curettage or excision.

Aged

Treatment of lichen planus.

Lichen planus is an inflammatory pruritic dermatosis in which T lymphocytes attack the basal epidermis, producing characteristic clinical and histological lesions. Mild cases can be treated successfully with rest, topical corticosteroids with or without wet dressings, or occlusion. Severe generalised cases may require systemic corticosteroids. Chronic, aggressive lesions may need additional therapy ranging from psoralen plus ultraviolet A (PUVA) to retinoids or cyclosporin. Complications ranging from infectious, bullous disease or ulceration require special treatment considerations.

Adrenocorticotropic Hormone

[Granuloma annulare and carpal tunnel syndrome].

This is a report of 4 cases of cutaneous granuloma annulare associated with carpal-tunnel syndrome. Biopsies of skin lesions and carpal flexor synovialis both show a picture of palisading granuloma consistent with granuloma annulare.

Carpal Tunnel Syndrome

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