Cases reported "Foot Dermatoses"

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1/5. Aplasia cutis congenita with epidermolysis bullosa: a case report.

    A child with epidermolysis bullosa and aplasia cutis congenita is presented. This is a very rare condition that represents an unusual manifestation of epidermolysis bullosa. Management of aplasia cutis congenita is controversial and may be conservative, surgical, or a combination of the two. In this patient, surgical treatment with split-thickness skin grafts failed, and conservative treatment using silver-sulfadiazine cream dressings was instituted.
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ranking = 1
keywords = epidermolysis bullosa, epidermolysis, bullosa
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2/5. A case of mixed bullous disease of epidermolysis bullosa acquisita and linear iga bullous dermatosis.

    A 75-year-old Japanese male visited us with bullous eruptions on the extremities. physical examination revealed large bullae on the hands, lower legs and feet. The oral mucosa was also involved. histology disclosed subepidermal blister with inflammatory cell infiltrates in the dermis. Direct immunofluorescence showed deposits of IgG and IgA at the cutaneous basement membrane zone. Indirect immunofluorescence on 1 M NaCl-split human skin sections demonstrated that the patient's IgG antibodies reacted with the dermal side of the split, while IgA antibodies reacted with the epidermal side. immunoblotting showed that the patient's serum reacted with the NC1 domain of type VII collagen (290-kDa epidermolysis bullosa acquisita antigen) as well as the 120-kDa linear iga bullous dermatosis antigen, LAD-1. Systemic prednisolone resulted in a favorable response. From the clinicopathological findings, the present case is not consistent with either epidermolysis bullosa acquisita or IgA bullous dermatosis. Therefore, we regarded the case as mixed bullous disease of epidermolysis bullosa acquisita and linear iga bullous dermatosis. Such a case has not been previously reported.
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ranking = 3.4343178186215
keywords = epidermolysis bullosa acquisita, epidermolysis bullosa, bullosa acquisita, epidermolysis, acquisita, bullosa
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3/5. 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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ranking = 0.49061683123164
keywords = epidermolysis bullosa acquisita, epidermolysis bullosa, bullosa acquisita, epidermolysis, acquisita, bullosa
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4/5. Acantholytic epidermolysis bullosa.

    BACKGROUND: We describe a new variant of inherited epidermolysis bullosa and elucidate the clinical, histologic, and ultrastructural features of this condition. OBSERVATIONS: This form of epidermolysis bullosa displays an autosomal dominant inheritance pattern, is characterized by acral bullae, and histologically demonstrates suprabasal clefting with acantholysis. Ultrastructural findings are nonspecific but reminiscent of those observed in benign familial pemphigus. CONCLUSION: Acantholytic epidermolysis bullosa is a rare but distinct clinicopathologic entity that warrants inclusion in the nosologic classification of epidermolysis bullosa.
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ranking = 1.3333333333333
keywords = epidermolysis bullosa, epidermolysis, bullosa
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5/5. epidermolysis bullosa acquisita occurring in association with systemic lupus erythematosus.

    A 77-year-old retired male physician with a 6-year history of systemic lupus erythematosus (SLE) developed a mechanobullous eruption, the features of which were clinically and immunopathologically consistent with a diagnosis of 'classical' epidermolysis bullosa acquisita (EBA). As EBA shares immunopathological findings with a number of cases reported as the 'bullous eruption of SLE', the clinical findings commonly recognized as 'classical EBA' may, in patients with SLE, represent a specific subset of the bullous eruption of SLE rather than a separate diagnostic entity. There are few reports in the literature describing classical EBA in patients with SLE. Findings in this patient add further support to the suggestion that EBA occurring in association with SLE, represents a subset of the bullous eruption of SLE, the clinical features of which may be modified by genetic susceptibility or disease activity.
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ranking = 0.82667526873127
keywords = epidermolysis bullosa acquisita, epidermolysis bullosa, bullosa acquisita, epidermolysis, acquisita, bullosa
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