Dermatopathologic diagnosis of Lyme Borreliosis
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Abstract:
The goal of this study was to determine the general light microscopic findings which lend support to the histopathologic diagnosis of the main cutaneous manifestations of Lyme Borreliosis. Their diagnostic criteria are delineated and illustrated. In culminating lesions of erythema migrans and acrodermatitis chronica atrophicans, a peculiar connective tissue reaction includes an increase in the number of fibroblasts, proliferation of collagen fibers, and interstitial mucinous edema. The cellular infiltrates are patchy perivascular in erythema migrans and either patchy and/or band-like in acrodermatitis chronica atrophicans. They consist of lymphohistiocytic cells with a variable admixture of plasma cells. The damage to elastic (and even collagen) fibers occurs in early acrodermatitis chronica atrophicans and is reflected by the phenomenon of elastophagocytosis. Reduction or lack of pilosebaceous units is a constant finding. In advanced lesions of acrodermatitis chronica atrophicans, a thinning of the dermal breadth is noticed, resulting from a decrease in collagen and elastic fibers. Fibrous nodules and morphea-like conditions are characterized by excessive formation of collagen. Borrelial lymphocytoma exhibits two different patterns of infiltration, accompanied by dermal fibrosis and increased numbers of fibroblasts. Recent tick bites show a predominantly neutrophilic infiltrate. By applying the results of this synoptic study, histopathologic diagnosis of dermatoborrelioses should be possible without the absolute necessity of clinical correlation.Keywords:
Erythema migrans
Acrodermatitis
Morphea
Erythema
Infiltration (HVAC)
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Granuloma Annulare
Histopathology
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Rhinophyma may present with either of two distinct histopathologic appearances. The most common shows histopathologic features of rosacea. The second pattern shows telangiectasia, diffuse dermal fibrosis with abundant mucin, and a virtual absence of pilosebaceous structures. These histopathologic features of the lesser-known fibrous variant of rhinophyma mimic those of fibrous papule of the nose. We report an unusual case that histopathologically resembled the second variant. Immunohistochemically, the dermal fibroblasts in this case showed intense staining with Factor XIIIa in a pattern similar to that demonstrated in fibrous papules. S-100 protein failed to stain these dermal fibroblasts. The presence of Factor XIIIa+ dermal fibroblasts may portend the evolution of fibrosis in rosacea and other inflammatory dermatoses.
Rhinophyma
Factor XIIIa
Histopathology
Dermal papillae
Stain
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• Two women had malignant atrophic papulosis that affected the skin only. Immunologic studies showed normal amounts of serum immunoglobulins and complement and normal delayed hypersensitivity skin test results. Fibrinolysis autografts showed complete loss of fibrinolysis within the lesions but normal fibrinolysis in uninvolved skin. Histopathologic studies showed moderate inflammatory infiltration consisting of lymphocytes in addition to the typical cone-shaped regions of necrobiosis. Large amounts of mucin also were seen. Endovasculitis, thrombosis, and perivascular lymphocytic inflammation were noted in the subcutaneous arterioles of the lesions in only one of the patients. Enzyme histochemical studies showed oxidative enzymes, although in reduced amounts, in the epidermal and dermal cells of the affected skin. Electron microscopic studies of lesions showed paromyxovirus-like inclusions in endothelial cells and fibroblasts. The possibility of an unusual viral causation should be examined further. (Arch Dermatol112:357-363, 1976)
Infiltration (HVAC)
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Erythroderma
Acanthosis
Parakeratosis
Spongiosis
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We report on a 34-year-old patient suffering from erythema chronicum migrans, who developed clinically and histologically typical morphea, which was confined to the area previously involved by the erythema migrans. The patient's serum antibody level against borrelia burgdorferi spirochetes was significantly elevated. By means of the silver impregnation technique, we were able to identify spirochetal organisms both in the lower dermis and within the septa of the subcutaneous fatty tissue. In frozen sections, spirochetes were demonstrated by the immunoperoxidase method using specific anti-spirochetal antibodies. For a better assessment of the composition of the inflammatory infiltrate in morphea, we applied a panel of monoclonal antibodies to a sensitive two-stage immunoperoxidase technique. Helper-inducer T-cells and a large number of suppressor-cytotoxic T-cells were observed both in the perivascular infiltrate and between collagen fibers in close proximity to the HLA-DR reactive fibroblasts. A large number of mast cells were seen in the dermal infiltrate. The detection of spirochetal organisms in histological sections as well as the demonstration of closely associated helper-inducer T-cells, macrophages, and activated fibroblasts in the dermis strongly suggest that a cell-mediated immune response against borrelia may be the dominant pathogenetic event in this variant of scleroderma. Activated T-lymphocytes and various factors secreted either by activated lymphocytes or mast cells may cause proliferation of fibroblasts, which can lead to increased collagen synthesis and dermal fibrosis.
Morphea
Immunoperoxidase
Localized Scleroderma
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We obtained biopsies from early, fully developed, and late lesions of erythema elevatum diutinum (EED) in a 49-year-old man. The histologic and electron-microscopic findings were compared with those reported in the literature and three other cases from our files. Early lesions show leukocytoclastic vasculitis with capillary proliferation. Later lesions show vasculitis, dermal aggregates of neutrophils, fibrosis, and areas of granulation tissue. Newly formed vessels in granulation tissue may be more susceptible to damage by immune complexes, and the early formation of granulation tissue in EED may prevent an early resolution of vasculitis. Damage to dermal connective tissue in EED incites either scarring or, rarely, a fibrohistiocytic proliferation. Ultrastructural examination of one case showed histiocytes with myelin figures and intracellular lipid and cholesterol.
Granulation tissue
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Abstract: Lichen planus (LP) is a mucocutaneous inflammatory dermatitis of idiopathic origin that can involve the skin, mucous membranes, hair, and nails. LP has an associated set of characteristic histopathologic findings which include hyperkeratosis, vacuolization of the basal layer, Civatte bodies, wedge-shaped hypergranulosis, band-like lymphocytic infiltrate at the dermal epidermal junction, eosinophilic colloid bodies in the papillary dermis, and pigment incontinence. The infiltrate is usually composed of lymphocytes with few histiocytes, mast cells, and macrophages. The presence of plasma cell predominant infiltrate in LP has only been reported in four previous cases and 2 other cases of lichen nitidus. The authors report another 2 cases of LP with predominate plasma cell infiltrate in 2 female patients on the legs. The differential includes a drug-induced lichenoid reaction with predominate plasma cell infiltrate. However, there have been no case reports of that type of reaction. Because plasma cells are seen commonly in certain infectious diseases, malignancy, and macroglobulinemia, it is prudent to rule out those entities. Our patients responded well with a class 1 topical steroid, with improvement of their lower leg lesions within 1 month of treatment.
Acanthosis
Parakeratosis
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We report a case of inflammatory stage vulvar lichen sclerosus et atrophicans (LSA) with histopathologic features simulating both patch stage mycosis fungoides (MF) and extramammary Paget’s disease. The patient is a 79‐year‐old woman with an erythematous plaque on her vulva. A punch biopsy was performed that revealed mild psoriasiform hyperplasia of the epidermis with spongiosis, marked interface vacuolar alteration, scattered individual necrotic keratinocytes and Civatte bodies. A band‐like mixed inflammatory infiltrate was present in the papillary/upper dermis with prominent intraepidermal lymphocytes. Some intraepidermal lymphocytes showed atypical nuclear contours simulating the cells seen in MF. The presence of numerous intraepidermal epithelioid cells with a pagetoid distribution raised the possibility of extramammary Paget’s disease. A panel of immunohistochemical (IHC) stains showed a reactive lichenoid mixed population of lymphocytes with numerous intraepidermal Langerhans cells. This confirmed the diagnosis of the inflammatory stage of lichen sclerosus. The presence of prominent epithelioid Langerhans cells with activated small lymphocytes represents an immune response in LSA and could cause diagnostic confusion with neoplastic conditions. IHC staining can confirm cell type and rule out MF and extramammary Paget’s disease.
Pagetoid
Extramammary Paget's disease
Papillary dermis
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Erythema multiforme
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A 39-year-old Japanese woman presented with a pruritic infiltrated erythematous plaque on the right cheek. Histopathologic analysis of the erythema showed dermal edema, separation of collagen bundles, and nodular perivascular and periadnexal infiltration of lymphocytes in the whole dermis, without epidermal changes. Alcian blue staining intensity was elevated between the collagen bundles, indicating dermal mucinosis. The nodular infiltrates consisted of CD3+ T cell clusters and CD20+ B cell clusters (ratio, approximately 3:1) and included numerous CD123+ cells, indicative of plasmacytoid dendritic cells. Blood analysis revealed serum antinuclear antibody at a titer of 1:160 (homogeneous, speckled pattern). Lupus erythematosus tumidus with pseudolymphomatous infiltrates was diagnosed. Hydroxychloroquine treatment partially improved symptoms; however, the addition of prednisolone was required for complete resolution. Lupus erythematosus tumidus is sometimes accompanied by pseudolymphomatous infiltrates. Dermal mucinosis and the presence of numerous plasmacytoid dendritic cells are useful in differentiating lupus erythematosus tumidus from pseudolymphoma.
Pseudolymphoma
Erythema
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