Cases reported "Keratosis"

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1/10. Demonstration of antibody to 230 kDa bullous pemphigoid antigen in lichen planus-like keratosis.

    We describe a 67-year-old man with lichen planus-like keratosis associated with anti-230 kDa bullous pemphigoid antigen (BPAG1) autoantibody. The patient had noticed solitary dark brown macule more than 6 years previously on his left chest. Histological findings showed hypergranulosis, irregular acanthosis, liquefaction degeneration of basal cells, band-like infiltration of lymphocytes at the subepidermal portion, and a cleft at the basement membrane zone (BMZ), resulting in the formation of subepidermal blisters. Direct immunofluorescence findings of perilesional skin showed a linear deposition of IgG at BMZ. On indirect immunofluorescent study using normal human skin, circulating IgG autoantibody to BMZ was present in the patient's serum at a titer of 1:80. The antigen located on the epidermal site of normal skin split by 1M NaCl was reacted with the patient's serum. Immunoblot analysis using epidermal extracts demonstrated the presence of IgG antibody directed to BPAG1 in the patient's serum. These observations suggest that the presence of an antibody to BPAG1 could be caused by the damage of basal cells following lichen planus-like keratosis.
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2/10. Verrucous form of chilblain lupus erythematosus.

    A 45-year-old woman had symmetrical livid plaques with yellowish hyperkeratoses for 5 years, which progressed on to the fingers and toes and on the soles of the feet. Two years later creamy, whitish areas and maceration appeared on the buccal mucosa and the lips. A skin biopsy revealed massive collagen hyaline degeneration in the perivascular area, hyperkeratosis and hypergranulosis, small lymphocyte infiltrates with several melanophages and extravasates of erythrocytes in the upper corium in perivascular areas and hydropic degeneration of basal keratinocytes. The findings using direct immunofluorescence were compatible with lupus erythematosus (LE). Laboratory investigation showed a slight leucopenia and thrombopenia, a slightly elevated erythrocyte sedimentation rate, hypocomplementaemia C3 and C4, a high titre of rheumatoid factor and antinuclear antibodies positivity of extractable nuclear antigen. The results reflected probably the development of a systemic form of the disease. The patient was successfully managed by methylprednisolone and hydroxychloroquine. After 1 year of therapy, a new skin biopsy revealed a substantial reduction of hyperkeratosis and hyaline degeneration of collagen tissue in the perivascular areas. The combination of the extensive hyperkeratosis and hyalinization thus seems to be features of the long-lasting, untreated lesions in chilblain LE.
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3/10. Cutaneous undifferentiated small (Merkel) cell carcinoma, that developed synchronously with multiple actinic keratoses, squamous cell carcinomas and basal cell carcinoma.

    Merkel Cell Carcinoma (MCC) is an uncommon undifferentiated neuroendocrine tumor, arising in skin mainly on sun-exposed areas. We present an unusual case of primary cutaneous undifferentiated small cell carcinoma that co-existed with six other lesions; 2 actinic keratoses, 3 squamous-cell carcinomas and a basal-cell carcinoma. HE stained sections revealed MCC located in the mid-dermis, co-existing with severe actinic keratosis. Immunohistochemically, the tumor cells reacted to cytokeratin 20, epithelial membrane antigen, chromogranin and neuron specific enolase. This is an unusual case of cutaneous MCC co-existing with six other different lesions. The concurrent development of MCC, squamous-cell and basal-cell carcinoma in the same patient indicates the pluripotent epidermal stem cell origin of these tumors. Further research is needed to enlighten the factors inducing this divergent differentiation.
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4/10. Pigmented squamous cell carcinoma of the cheek skin probably arising from solar keratosis.

    We report a rare case of pigmented squamous cell carcinoma (SCC) of the cheek skin probably arising from solar keratosis. An 80-year-old man was referred to our clinic because of a black skin nodule in the right cheek. The nodular lesion was 1 cm in diameter, dome-shaped, hard, sharply demarcated, partially erosive and telangiectatic at the border. The lesion was completely excised under the clinical diagnosis of probable seborrheic keratosis. Microscopically, cutaneous horn and mildly atypical squamous epithelia suggestive of previous solar keratosis were present in the surface of the lesion. The lesion consisted of atypical squamous cells with keratinization and intercellular bridges, and it was regarded as SCC. The SCC cells were seen to invade lightly into the upper dermis, where lymphocytic infiltrations and melanophages were noted. Characteristically, heavy deposition of melanin pigment was recognized in the SCC cells as well as in proliferated dendritic and pigment blockade melanocytes that were scattered or colonized within the SCC cell nests. Masson-Fontana stain revealed numerous melanin granules in the SCC cells, as well as in dendritic and pigment blockade melanocytes. Immunohistochemically, the SCC cells were positive for cytokeratins and epithelial membrane antigen, and negative for S-100 protein and HMB45 antigen. Dendritic and pigment blockade melanocytes were negative for cytokeratins, epithelial membrane antigen, and HMB45 antigen, but positive for S-100 protein. The present case suggests that SCC cells of the skin may induce proliferation of melanocytes. The differential diagnosis and the histogenesis of pigmented SCC of the skin are discussed.
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5/10. Porokeratotic eccrine ostial and dermal duct nevus. An abnormally keratinizing epidermal invagination or a dilated, porokeratotically plugged acrosyringium and dermal duct?

    Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) has been said to represent a widely dilated, keratin-plugged acrosyringium and dermal duct. We have observed in a case of congenital PEODDN a normal-appearing, acrosyringium-like duct that traverses vertically the entire length of the parakeratotic column. Also, in its lower course, it stained positively for carcinoembryonic antigen, while the inner borders of the invagination from which the parakeratotic column arose stained negatively. This leads us to suggest that the epithelial structure in PEODDN is an abnormally keratinizing epidermal invagination through which an acrosyringium-like duct traverses, rather than an abnormally dilated, parakeratotically plugged acrosyringium and dermal duct.
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6/10. Bazex syndrome (acrokeratosis paraneoplastica). An analytic review.

    Bazex syndrome (acrokeratosis paraneoplastica) is characterized by a psoriasiform eruption that favors acral sites and has been associated with an underlying malignancy in all reported cases. Of the 93 patients in this series, 89 were male with a mean age of 60 /- 8.5 years. Squamous cell carcinomas of the head and neck and squamous cell tumors of unknown primary with cervical lymph node metastases were the most commonly associated neoplasms, suggesting that the factor(s) responsible for the development of the syndrome are relatively specific for tumors of the upper aerodigestive tract. The cutaneous lesions were erythematous to violaceous in color and had associated scale; the most frequently observed sites of involvement were the ears, nose, hands, and feet, including the nails. In 63% of the cases, the cutaneous lesions preceded the initial symptoms or diagnosis of the tumor by an average of 11 months (range, 1-72) and, in general, the eruption was resistant to a variety of topical treatments. Occasionally, a reappearance of the papulosquamous lesions signaled the recurrence of the tumor (6 cases) or the appearance of skin lesions coincided with the development of metastatic disease (3 cases). In 91% (64/70) of the patients, the skin eruption either improved significantly following treatment of the underlying malignancy or did not improve in the setting of persistent tumor. However, even when all of the skin lesions cleared, the nail dystrophy often persisted. Fifteen of the patients developed vesicles, bullae, and crusts in addition to papulosquamous lesions. Possible explanations include the formation of an epidermal-dermal split via a bullous lichen planus-like mechanism, or the coexistence of two diseases; i.e., acrokeratosis paraneoplastica plus either porphyria cutanea tarda, bullous pemphigoid, or epidermolysis bullosa acquisita. One possible explanation for the development of the characteristic cutaneous eruption is an immune reaction, humoral or cellular, directed against a common antigen present on the tumor and the normal skin. Alternatively, tumor production of a keratinocyte growth factor such as TGF-alpha may be involved in the induction of the psoriasiform skin lesions.
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7/10. Hyperkeratosis lenticularis perstans (Flegel's disease). In situ characterization of T cell subsets and Langerhans' cells.

    We report a patient with hyperkeratosis lenticularis perstans (HLP) manifesting as multiple reddish-brown hyperkeratotic papules on the lower extremities. Typical histologic features of HLP include hyperkeratosis, thinning or absence of the granular layer and a band-like infiltrate in the upper dermis underlying an atrophic epidermis. In order to determine the cellular composition of the infiltrate, skin biopsy specimens were studied immunohistochemically using a series of commercially available monoclonal antibodies. The dermal infiltrate consists predominantly of helper/inducer T cells (Leu-4 , Leu-3a ). Suppressor/cytotoxic T cells (Leu-2a ) were fewer at the periphery of the infiltrate. The majority of T cells were activated as they expressed HLA-DR-antigen. Large numbers of Leu-6 Langerhans' cells were observed at the dermo-epidermal interface. Few natural killer cells (Leu-11b ) were noted within the dermal infiltrate. These findings support the hypothesis than an active cellular immune reaction involving the epidermis is of pathogenic importance for HLP.
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8/10. vasculitis as the basis of cutaneous lesions in Reiter's disease.

    The cutaneous lesions of Reiter's disease (RD) and pustular psoriasis (PP) are said to be histologically similar and often indistinguishable. We encountered three cases of RD in which biopsy specimens of lesions clinically compatible with keratoderma blenorrhagicum showed a pustular psoriasiform tissue reaction in conjunction with a subjacent superficial leukocytoclastic vasculitis (LCV). In an attempt to ascertain if these changes were distinctive and unique to cutaneous RD, the incidence of such changes in lesions of PP was examined using light microscopy and immunohistochemistry. The role of chlamydial infection in the pathogenesis of the observed vascular changes also was explored by assessing for the presence or absence of vascular deposition of chlamydial antigen in cutaneous RD compared with that in a control group that included cases of LCV and PP. In addition to conventional light microscopic analysis, immunoperoxidase studies to identify immunoglobulin deposition were performed on formalin-fixed, paraffin-embedded tissue from two of three patients with RD and on skin biopsy specimens from 11 patients with PP. Direct immunofluorescence (DIF) studies with antibodies to immunoglobulin (Ig)G, IgM, IgA, C3, and chlamydial antigens were performed on frozen tissue from one patient with RD, two patients with PP, three patients with LCV, one patient with nonspecific dermatitis, and one patient with Behcet's disease, who had a high antichlamydia antibody titer. All three specimens of RD showed a pustular psoriasiform diathesis in conjunction with a subjacent superficial LCV that was of maximal intensity in the dermal papillae capillaries. Through an immunoperoxidase technique performed on formalin-fixed tissue, the RD cases for which tissue was available for study demonstrated Ig deposition in injured blood vessels; using the same technique one of 11 PP biopsy specimens showed vascular Ig deposition in concert with LCV. This patient's biopsy was from a lesion of drug-induced LCV. None of the other specimens showed either light microscopic or immunohistochemical evidence of vasculitis. In the one specimen of RD studied by DIF, vascular deposition of IgG, IgM, C3, chlamydia heat shock protein 60 (CHSP60), and chlamydia-specific lipopolysaccharide (LPS) was observed. In the two specimens of PP studied, vascular deposition of C3, fibrin, CHSP 60, and chlamydia-specific LPS was not observed. Two specimens of LCV and the one specimen of dermatitis with concomitant nonspecific vascular injury showed vascular Ig and C3 deposition; in contrast, no vascular deposition of CHSP 60 or chlamydia-specific LPS was observed.(ABSTRACT TRUNCATED AT 400 WORDS)
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9/10. Contact sensitization to cyanoacrylate adhesive as a cause of severe onychodystrophy.

    BACKGROUND: The technology used for artificial nails, the chemistry of cyanoacrylates and the reported reactions to these products are briefly reviewed. MATERIALS AND methods: We studied three patients with prominent fingernail dystrophy, nail bed hyperkeratosis, fingertip eczema, and onycholysis, two of whom also had eczematous lesions at distant sites, ostensibly from hand transfer. Patch testing was performed with a standard screening tray, dried cyanoacrylate adhesives, and, in one case, with fingernail clippings. RESULTS: All three patients were prominently patch-test positive to the polymerized 2-ethylcyanoacrylate, used as an adhesive on artificial (plastic) fingernails. One patient was also mildly formaldehyde sensitive, one was mildly reactive to two acrylate allergens, and one was extremely allergic to toluenesulfonamide formaldehyde resin. One patient had a positive patch test to her fingernail clippings 2 months after use of the adhesive was discontinued and an attempt was made to remove it. CONCLUSIONS: Reactions to cyanoacrylate adhesives used for artificial nails can cause both nail dystrophy and fingertip eczema, and may produce eczema elsewhere, probably from hand transfer. Persistence is likely due to retained polymer, which slowly degrades in the presence of water probably releasing antigens. Patch testing with the dried adhesive on an adhesive plaster seems to be reliable.
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10/10. Mixed Merkel cell carcinoma and squamous cell carcinoma of the skin.

    Four mixed Merkel cell and squamous cell carcinomas of the skin are described. The patients ranged in age from 74 to 90 years and demonstrated or had a history of previous ultraviolet or infrared damage to the skin, manifested by basal cell carcinoma, squamous cell carcinoma, actinic keratoses, solar elastosis, and erythema ab igne. light microscopic examination of all 4 cases revealed invasive neoplasms consisting of 2 distinct but admixed cell types. The predominant cell type was consistent with Merkel cell carcinoma and was characterized by scant cytoplasm, a small dark polygonal nucleus with granular chromatin, a high mitotic rate, and cytokeratin 20 positivity. In each case, the Merkel cell component merged with a cytokeratin 20 negative squamous component characterized by abundant eosinophilic cytoplasm, intercellular bridges, and keratinization with focal squamous pearl formation. Immunohistochemical staining patterns were consistent with the usual pattern for that cell type; transitional cells were not demonstrated. The intimate admixture of the 2 antigenically different neoplastic cell types, and common etiologic role of ultraviolet and possibly infrared damage, lend support to the theory that some Merkel cell carcinomas and squamous cell carcinomas may arise from a pluripotent epidermal stem cell.
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