| A child is described who had the signs of autoimmune lymphoproliferative syndrome from an early age and later developed a blistering dermatosis that was shown to be childhood linear IgA disease. ( info) |
742/3572. Epithelial and mesenchymal hamartomatous changes in a mature port-wine stain: Morphologic evidence for a multiple germ layer field defect. The port-wine stain (PWS) is a congenital cutaneous venulocapillary malformation of unknown pathogenesis. Many patients with facial PWS develop thickening with cobblestoning and nodularity during adult life. The histologic correlates of this maturational change are poorly documented and its mechanisms remain unclear. In this case study we present new histologic observations that may elucidate this phenomenon. An extensive PWS on the face of a 75-year-old man exhibited gross thickening with cobblestoning and nodularity. Histologic examination revealed not only the expected vascular abnormalities, but also a number of widely distributed epithelial, neural, and mesenchymal hamartomatous changes. Epithelial changes included epidermal nevus, sebaceous trichofolliculoma, and basaloid follicular hamartoma. Changes of connective tissue nevus, smooth-muscle hamartoma, neural hamartoma, and subcuticular hamartoma were also noted. The complex hamartomatous changes observed in the PWS of this patient involved multiple germ lines and were distributed in a widespread pattern. These changes not only offer an explanation for the skin thickening and nodularity of this patient, but also suggest a genetically determined, multilineage developmental field defect in the pathogenesis of this lesion. Further studies of other patients are necessary to understand the full implications of these findings in the late stage of PWS. ( info) |
743/3572. Seborrheic keratosis: an acantholytic variant. Two elderly women with seborrheic keratoses on the abdomen are reported on because of the peculiar histologic appearance characterized by prominent acantholysis. Although the basic histologic architecture was a papillomatous acanthoma corresponding to that of seborrheic keratosis, the upper portion of the lesions showed extensive acantholytic changes and dyskeratotic cells. Differentiation from seborrheic keratosis with focal acantholytic dyskeratosis, warty dyskeratoma, keratosis senilis and acantholytic squamous cell carcinoma could be made on the basis of clinical as well as histological features. Although the histogenesis is uncertain, it seems logical to propose that this is a rare variant of irritated seborrheic keratosis. ( info) |
744/3572. Post-transplant T-cell lymphoproliferative disorder/T-cell lymphoma: a report of three cases of T-anaplastic large-cell lymphoma with cutaneous presentation and a review of the literature. AIMS: To report the clinical, pathological and immunohistochemical features of three cases of post-transplant T-cell lymphoproliferative disorder (T-PTLD) T-cell lymphoma with primary cutaneous presentation. methods AND RESULTS: Three cases of primary cutaneous post-transplantation anaplastic large-cell lymphomas occurred in renal transplant recipients and were shown to display a T-cell immunophenotype; all were ALK 1 protein and EMA negative and two were Epstein-Barr virus positive using in-situ hybridization. Two displayed a CD4 phenotype, two were focally CD56 and all three were negative for the cytolytic enzyme granzyme B. In two cases monoclonality was established by T-cell receptor gene rearrangement study. All presented with nodular cutaneous involvement and all were ultimately fatal. CONCLUSION: T-PTLDs are uncommon histological subtypes both in a general context and associated with cutaneous presentation. Our findings suggest clinicopathological and immunophenotypic similarities to primary cutaneous anaplastic large-cell lymphoma but with a progressive clinical behaviour similar to previously reported T-PTLD and to systemic nodal ALK- anaplastic large-cell lymphoma. ( info) |
745/3572. Successful treatment of cutaneous langerhans cell histiocytosis with thalidomide. Langerhans cell histiocytosis (LCH) represents a group of rare histiocytic syndromes characterized by tissue infiltration with dendritic cells. The management of LCH is difficult as these disorders respond inconsistently to immunosuppressive and chemotherapeutic strategies. thalidomide (N-phtalimidoglutarimide), initially used as a tranquilizer, has recently been used in the management of several inflammatory skin diseases. We describe the case of a 38-year-old male with mucocutaneous LCH. A treatment course with 6 cycles of 2-chlorodeoxyadenosin (cladribine) was initiated. This was well tolerated but withdrawn after 6 months to prevent secondary malignancy. A partial remission was seen. Subsequently, a treatment course with thalidomide 200 mg daily was started. This therapy resulted in a significant improvement of the mucocutaneous lesions within 4 weeks and complete healing was achieved after 3 months. Treatment was then successfully continued with daily doses of 100 mg to prevent relapse. In conclusion, thalidomide monotherapy represents an effective, safe and well-tolerated treatment option that should be considered as first-line therapy for mucocutaneous LCH. ( info) |
746/3572. Skin disorders with prominent eosinophilic infiltration treated successfully with nicotine. Report of two cases. BACKGROUND: nicotine was effective in the treatment of skin disorders attributable to neutrophils such as pyoderma gangrenosum and orogenital ulcerations due to Behcet's disease. OBJECTIVE: We assessed the efficacy of nicotine for treating skin disorders with prominent eosinophilic infiltration. patients AND methods: A 67-year-old man with Kimura's disease was treated for 2 weeks with nicotine chewing gum (6 mg/day). A 50-year-old woman with erythema nodosum with eosinophilic infiltration was treated for 4 weeks with transdermal nicotine patches (5 mg/day). RESULTS: Both patients showed a dramatic clinical and histopathological improvement. CONCLUSION: Our results suggest that nicotine may be useful for treating skin disorders with eosinophilic infiltration as well as neutrophilic dermatoses. ( info) |
747/3572. Subgaleal hematoma and seven exchange transfusions. A 3 kg baby was delivered by cesarean section after prolonged labor. He had massive subgaleal hematoma. He developed anemia requiring packed cell transfusions and hyperbilirubinemia requiring a total of seven exchange transfusions and highly intensive phototherapy. There were no adverse complications of the hyperbilirubinemia or the exchange transfusion. ( info) |
748/3572. Imiquimod: potential risk of an immunostimulant. A 19-year-old woman with severe HLA B27 spondyloarthropathy whose disease was controlled on cyclosporin, methotrexate and prednisolone had human papillomavirus infection and developed cervical dysplasia and a large number of cutaneous and vulval warts. These were not responsive to cryotherapy, salicylic acid or cimetidine, so she was treated with topical imiquimod 5% cream. Two weeks after starting this treatment she had a significant flare of her spondyloarthropathy. She was so ill that she stopped using the imiquimod cream. She had full resolution of her warts after 3 weeks' treatment with imiquimod cream, but her spondyloarthropathy took more than 3 months to improve, despite significant augmentation of her immunosuppression. This case highlights the potential risk of using imiquimod cream (an immunostimulant) in a patient who has a condition requiring immunosuppression, such as autoimmune disease or an organ transplant. ( info) |
749/3572. Percutaneous management of a nephrocutaneous fistula due to a pyelocaliceal diverticular calculus. Nephrocutaneous fistulas arising from stone-containing pyelocaliceal diverticula are rare. We describe 2 patients who were treated by open drainage for perirenal abscesses. Subsequently, both patients presented with a nephrocutaneous fistula from a stone-containing pyelocaliceal diverticulum. These patients were successfully treated by percutaneous nephrostolithotomy with fulguration of the diverticula. ( info) |
750/3572. Onychotillomania: 2 case reports. Onychotillomania is an uncommon condition characterized by self-destruction of the fingernails and/or toenails by compulsive manipulation. We report 2 cases of onychotillomania with differing presentations in a young man and in an older man. Onychotillomania may be a form of obsessive-compulsive disorder (OCD), and we discuss the psychologic factors and current treatments for this condition. ( info) |