Cases reported "Carcinoma, Squamous Cell"

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21/28. Surgical correction of the hand in epidermolysis bullosa dystrophica.

    epidermolysis bullosa dystrophica (polydysplastic type) is a rare congenital skin anomaly which, in the hands, because they are exposed to repeated trauma, results in a severe "mitten"-like deformity. Functional benefit was obtained in three patients by separation of the digits and application of split-thickness grafts. Wolfe grafts or "split-off" (epidermis) grafts. arthrodesis of the interphalangeal joints and filleting of the little finger to provide a flap which could be turned in to the palm, resulted in an improvement in hand function.
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keywords = epidermolysis bullosa, epidermolysis, bullosa
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22/28. Management of squamous cell carcinoma in a patient with dominant-type epidermolysis bullosa dystrophica: a surgical challenge.

    epidermolysis bullosa dystrophica is a rare congenital skin disease inherited either as a recessive or dominant form, the latter form being less common and much less severe. Squamous cell carcinoma is a rare complication in the dominant form of the disease, only three such cases being reported before, making this case the fourth known case. Although the squamous cell carcinoma is well-differentiated by histopathology, it has a poor prognosis owing to its invasiveness, distant metastases, and multicentricity. Its management also poses a great challenge to the surgeon and personnel involved in patient care. A complete outline of the total management of such a case is described with some interesting observations not mentioned previously in the literature. All the previous three patients are deceased, and this is the only known surviving patient.
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ranking = 1
keywords = epidermolysis bullosa, epidermolysis, bullosa
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23/28. Squamous cell carcinoma in dominant type epidermolysis bullosa dystrophica.

    The unusual genetic disorder epidermolysis bullosa dystrophica has been reported in several patients in whom the chronic cutaneous scars led to the development of cutaneous squamous cell carcinoma. However, only one of these previously reported cases involved the autosomal dominant form of the disease; the remainder occurred in its recessive counterpart. We report the second and third patients with squamous cell carcinoma associated with the dominant form of epidermolysis bullosa dystrophica. In addition, we not only observed the previous electron microscopic findings of decreased numbers of anchoring fibrils beneath the basal lamina but have also noted marked disruption of the basal lamina itself.
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ranking = 1.4794151016142
keywords = epidermolysis bullosa, epidermolysis, bullosa
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24/28. Combined tissue expansion and free tissue transfer for reconstruction of the hand in epidermolysis bullosa-associated malignancy.

    The recessive form of dystrophic epidermolysis bullosa (RDEB) is associated with severe hand deformities characterized by cocooning pseudopolysyndactyly, recurrent ulceration, fibrosis, and adduction contracture of the thumb. In addition, RDEB is associated with an aggressive form of squamous carcinoma, notable for rapid metastases, appearance at an early age, and a relative resistance to radiation or chemotherapy. To date, reported reconstructive treatment has been limited to excision with skin grafting or amputation. We report the successful combined use of tissue expansion and microsurgical free tissue transplantation to reconstruct a patient with RDEB found to have malignancy of the hand. Donor skin was found to expand without blistering, and use of the expander allowed primary donor site closure after microsurgical transplantation of a scapular flap. We recommend wide tumor resection and the aggressive use of contemporary reconstructive methods to maintain hand function and associated quality of life in patients with RDEB who develop malignancy.
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ranking = 1.2328459180118
keywords = epidermolysis bullosa, epidermolysis, bullosa
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25/28. epidermolysis bullosa dystrophica of Hallopeau-Siemens and squamous-cell carcinoma: a case report.

    A 46-year-old patient suffering from Hallopeau-Siemens type epidermolysis bullosa dystrophica presented a large squamous-cell carcinoma of the left hand. No metastases were observed. The tumour was resected and the phalanxes were amputated. The wound was covered with autologous skin grafts. Squamous-cell carcinoma is extremely aggressive in these patients and surgery is the only effective therapy. This life-threatening complication should always be kept in mind and a biopsy taken of every chronic, non-healing ulceration and of each atypical lesion.
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ranking = 0.30146224596453
keywords = epidermolysis bullosa, epidermolysis, bullosa
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26/28. radiation therapy for squamous cell carcinoma in dystrophic epidermolysis bullosa: case reports and literature review.

    Dystrophic epidermolysis bullosa (DEB) is a debilitating systemic disease frequently associated with biologically aggressive secondary squamous cell carcinomas arising from affected skin or mucosal surfaces. Treatment of these carcinomas with surgery, chemotherapy, or radiation is complicated by inherently poor wound healing. We report on two DEB patients treated with radiation therapy for locally advanced squamous cell carcinoma, and retrospectively analyze 10 DEB patients treated with radiation, reported in the literature. Of the 11 fully available and described case results from radiation therapy, six (54%) patients demonstrated a partial tumor response. All patients receiving > 4,500 cGy developed moist skin desquamation and delayed skin healing. radiation therapy may be of benefit in palliating DEB patients who have locally advanced carcinoma, but has been associated with enhanced normal tissue toxicity, suggesting a narrow or absent therapeutic index between irradiated carcinoma and skin.
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ranking = 1.2328459180118
keywords = epidermolysis bullosa, epidermolysis, bullosa
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27/28. Generalized atrophic benign epidermolysis bullosa in 2 siblings complicated by multiple squamous cell carcinomas.

    BACKGROUND: Generalized atrophic benign epidermolysis bullosa is a form of junctional epidermolysis bullosa characterized by skin fragility; atrophic alopecia; sparse eyebrows, eyelashes, and axillary and pubic hair; dystrophic fingernails and toenails; and enamel defects in decidual and permanent teeth. Substantial progress was recently made elucidating the genetic defects underlying this disorder. In affected persons, pathogenetic mutations were identified in the genes encoding the beta 3 chain of laminin 5 (LAMB3) or the 180-kd bullous pemphigoid antigen (BPAG2/COL17A1). OBSERVATIONS: Two brothers, aged 39 and 32 years, had characteristic clinical features of generalized atrophic benign epidermolysis bullosa. By electron microscopy, dermoepidermal separation was seen at the level of the lamina lucida, establishing a diagnosis of junctional epidermolysis bullosa. Lesional and clinically unaffected skin showed basal keratinocytes with hypoplastic hemidesmosomes, possibly indicating a defect of hemidesmosomal or associated proteins. Both patients presented with multiple fungating tumors on atrophic and scarred skin on their lower legs; 2 tumors in the older sibling and 4 tumors in the younger sibling were diagnosed as well-differentiated squamous cell carcinomas. Tumor staging elicited no evidence of regional lymph node involvement or systemic disease. Treatment was by microscopically controlled surgery. All wounds were allowed to heal by secondary intention. In both patients, wound healing was markedly delayed and characterized by the formation of abundant granulation tissue and poor re-epithelialization. CONCLUSIONS: In the absence of other apparent risk factors for the development of squamous cell carcinomas, chronic wounding resulting from recurrent skin blistering probably provided an important prerequisite for tumor promotion in these patients. The 2 cases presented herein provide evidence that the development of malignant skin tumors in patients with epidermolysis bullosa is not confined to the dystrophic forms but also may occur in some variants of junctional epidermolysis bullosa, such as generalized atrophic benign epidermolysis bullosa.
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ranking = 2.712261019626
keywords = epidermolysis bullosa, epidermolysis, bullosa
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28/28. Simultaneous manifestation of squamous cell carcinoma in identical twins with epidermolysis bullosa.

    We report a case of identical twins with recessive dystrophic epidermolysis bullosa, who developed squamous cell carcinoma in the hand at the latter half of their third decade. To our knowledge, this is the first case of manifestation of squamous cell carcinoma in identical twins and may contribute to the understanding of oncogenesis in such patients.
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ranking = 1.2328459180118
keywords = epidermolysis bullosa, epidermolysis, bullosa
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