Cases reported "Disease Progression"

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1/7. Pedunculated melanoma with pulmonary and bony metastases.

    We report a patient with pedunculated melanoma. Rounded and epithelioid-type melanoma cells had proliferated in the epidermis and massively into the deep dermis in the pedunculated nodule. At the base of the pedicle, lengtiginous-type melanoma cells formed nests of various sizes in the epidermis extending ten or more rete ridges beyond the site of invasion. Thus, this case shows that a pedunculated melanoma is not necessarily a variant of nodular melanoma. Six years after the primary operation, late recurrence was detected in regional lymph nodes, and nine years after the initial operation, the patient was found to have three large metastatic nodules, up to 8.5 cm in longest diameter, in her lung and bone. Although she died six months after the metastasectomy, prompt surgical excision of the primary tumor and metastasectomy can provide longer survival.
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keywords = epidermis
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2/7. cholesteatoma behind an intact tympanic membrane: histopathologic evidence for a tympanic membrane origin.

    BACKGROUND: Several theories have been proposed with respect to the origin and pathogenesis of cholesteatoma behind an intact tympanic membrane. CASE REPORT: The authors describe a case of cholesteatoma behind an intact tympanic membrane in a 71-year-old man with a history of tympanic membrane retraction fixed to the incus without evidence of a perforation. The membrane eventually became detached, and remnants of keratinizing squamous epithelium were found on the incus. DISCUSSION: Mechanisms such as metaplasia, ectopic epidermis rests, or ingrowth of meatal epidermis have been proposed to explain the pathogenesis of cholesteatoma behind an intact tympanic membrane. These findings, based on temporal bone histopathology, support the role of an acquired epidermal rest. CONCLUSIONS: This case report provides evidence that cholesteatoma behind an intact tympanic membrane can be established from a resolved retraction of the pars tensa of the tympanic membrane.
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keywords = epidermis
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3/7. erythema gyratum repens responding to cetirizine hydrochloride.

    We report a case of erythema gyratum repens (EGR) in a 59-year-old man with inoperable pancreatic cancer and liver metastasis. The patient had a widespread erythema with concentric marginal band spreading in waves over the trunk and extremities. Numerous vesicles were seen on the margin of the erythema. The skin lesions were severely pruritic, and his peripheral blood showed marked eosinophilia. He also had palmoplantar hyperkeratosis. A biopsy specimen of the erythema disclosed spongiosis, microvesicles filled with eosinophils, infiltration of eosinophils into the epidermis, and a perivascular infiltrate in the dermis. The skin lesions and pruritus cleared quickly after the administration of cetirizine hydrochloride.
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keywords = epidermis
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4/7. Distal phalangeal metastasis of extramammary Paget's disease.

    A rare case of phalangeal metastasis of extramammary Paget's disease in a 68-year-old man is described. The patient developed an erythematous, slightly elevated area in the pubic region. A biopsy specimen demonstrated numerous, large, rounded cells with ample pale-staining cytoplasm proliferating in the epidermis. With a diagnosis of extramammary Paget's disease, he underwent wide local excision and inguinal node dissection. Eleven months postoperatively, the patient developed a tender, red, swollen right ring finger. Bone X-ray showed that the distal phalanx of the ring finger had completely dissolved. Histopathological examination demonstrated proliferation of tumor cells in the adipose tissue. They had poorer and darker cytoplasm than the Paget's cells in the epidermis of the pubic region. Immunohistochemically, these cells showed the same staining pattern as did the Paget's cells at the primary site. Accordingly, the patient was diagnosed with distal phalangeal metastasis of extramammary Paget's disease. Two weeks after the appearance of the distal phalangeal metastasis, the patient died of cancerous pleurisy. It has been reported that patients with phalangeal metastasis have a very poor prognosis.
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keywords = epidermis
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5/7. Vesicular mycosis fungoides.

    A 62 years old patient presented with rapidly progressive mycosis fungoides. Lesions of the face, head, trunk and limbs exhibited numerous vesicles and erosions. Histopathology showed marked spongiosis and intraepidermal blisters, as well as invasion of the epidermis by atypical lymphocytes, which was confirmed by immunohistochemistry. The vesicular variant of mycosis fungoides is rare and associated with a poor prognosis.
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keywords = epidermis
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6/7. "Free-floating" desmosomes in lipoid proteinosis: an inherent defect in keratinocyte adhesion?

    The classic features of lipoid proteinosis - beadlike papules and hoarseness - result from the accumulation of hyaline material in the mucocutaneous dermis. However, the characteristic manifestation in children - erosive, crusted lesions that lead to scarring - is rarely discussed and poorly understood. Lipoid proteinosis results from mutations in extracellular matrix protein 1, but the function of this protein is largely unknown. We performed ultrastructural studies on lesional epidermis, cultured monolayer keratinocytes, and raft keratinocyte cultures from blistering lesions of a child with lipoid proteinosis. All sections showed the dissociation of relatively intact desmosomes from keratinocytes, with desmosomes that were "free-floating" in the intercellular spaces or attached by thin strands to the cell membrane. These changes were present in serial sections of both tissue and cultured keratinocytes, suggesting this observation to be an inherent feature of keratinocytes devoid of extracellular matrix protein 1, rather than an artifact. Although additional patients should be studied, the diminished appearance of the inner dense plaque - the region of attachment of keratin intermediate filaments to desmosomal proteins - provides preliminary evidence that extracellular matrix protein 1 may participate in attaching keratin intermediate filaments to desmosomal region protein(s).
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7/7. A case of sclerodermatous graft-versus-host disease following autologous peripheral blood stem cell transplantation.

    We report a 65-year-old woman with chronic graft-versus-host disease (GVHD) who developed severely sclerotic skin on the fingers, hand and trunk following autologous peripheral blood stem cell transplantation (APBSCT). The patient had suffered from breast cancer and been treated with surgery and chemotherapy. She showed pancytopenia and was treated with APBCST. Four years after APBSCT, she developed the severe sclerotic changes on the fingers, hands, extremities and trunk. The skin biopsy showed a flattened epidermis and a proliferation of collagen bundles in the dermis. No anti-nucleolar dna titers were detected in the serum. She was diagnosed with chronic GVHD. Despite treatment with oral prednisolone, the skin sclerotic change developed and the breast cancer recurred. She died due to pericarditis. This is a rare case of sclerodermatous GVHD following APBSCT. The serum interleukin (IL)-12 levels were examined during the treatment.
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