Nodular Scleroderma Revisited: Systemic Sclerosis Presenting as Annular Keloidal Sclerotic Plaques.

BACKGROUND: Nodular scleroderma, also known as keloidal scleroderma, is a rare variant of systemic sclerosis. PURPOSE: The clinical features, pathologic findings and postulated pathogenesis of nodular scleroderma are discussed. METHODS: A woman with previously undiagnosed systemic sclerosis who presented with nodular scleroderma is described. Using the PubMed database, a literature search was performed on keloidal scleroderma, nodular scleroderma, and systemic sclerosis. RESULTS: Nodular scleroderma is characterized by firm plaques or nodules, which can mimic a keloid, that are typically located on the anterior orposterior upper trunk and the arms; they show pathologic changes of scleroderma, keloid, or hypertrophic scar. Akeloidal response of inflamed skin that is involved in an active fibrotic process inherent to systemic sclerosis, in individuals who are genetically predisposed to keloid formation, is the hypothesized pathogenesis. CONCLUSION: Nodular scleroderma is rare. The authors' patient presented with diarrhea, dysphagia, fatigue, Raynaud's phenomenon, shortness of breath, and annular keloidal plaques of morphea whose biopsy showed features of hypertrophic scar; additional studies confirmed the diagnosis of the nodular scleroderma variant of systemic sclerosis. The possibility of systemic sclerosis should be entertained in patients who present with nodularor keloidal plaques that morphologically resemble morphea and have histologic findings of a scar or a keloid-especially if there are associated symptoms suggestive for systemic sclerosis.

Multiple poromas in a bone marrow transplant recipient: A case report.

A poroma is a benign epithelial neoplasm that most commonly presents as a solitary papule on the palm or sole. We report the case of a 25-year-old male, with a history of acute myelogenous leukemia, who developed multiple poromas on the feet. Poromatosis - the occurrence of multiple poromas - has been described in six adults and one child; it appears to be more prevalent in patients with a history of lymphoproliferative disorder or radiation exposure.

Signet ring-like cells: no longer a "signature" of glandular differentiation.

The term "signet ring" is used to describe a cell with an eccentric nucleus that is compressed to the periphery of the cell by a large cytoplasmic vacuole or by a substance accumulated within the cytoplasm. In pathology, these cells have been traditionally associated with adenocarcinomas. We describe two cases of cutaneous tumors that contained signet ring-like cells. Because of the morphology of the cells and the fact that one patient had a past history of lung cancer, the possibility of metastatic adenocarcinoma was considered. Upon further investigation, the tumors proved to be a primary cutaneous squamous cell carcinoma and a basal cell carcinoma; surgical management was curative. Because signet ring changes may pose a diagnostic challenge, we review the various benign and malignant cutaneous lesions that have been reported to contain signet ring cells and we explore the possible causative mechanisms of this phenomenon.

Multiple myeloma presenting as a novel mucocutaneous eruption.

A 71-year-old woman presented with exquisitely tender mucosal erosions, a diffuse polymorphous eruption, and night sweats. Workup revealed multiple myeloma with a monoclonal IgG-kappa paraprotein in the serum. Her severe oral involvement was suggestive of paraneoplastic pemphigus, but direct and indirect immunofluorescence tests were negative. A skin biopsy showed spongiosis and a sparse perivascular lymphocytic infiltrate, with occasional CD8-positive lymphocytes in the epidermis. Her lesions improved with intravenous immune globulin. Immunohistochemical staining on the formalin-fixed biopsy specimen was strongly positive for IgG and IgG-kappa in an epidermal "chicken-wire" pattern, but negative for IgG-lambda. Her pulmonary tissue stained negative for IgG-kappa, suggesting clinical relevance of the myeloma paraprotein in her epidermis. To our knowledge, this is the first report of a multiple myeloma patient with such an eruption.

Acrochordon-like basal cell carcinomas in patients with basal cell nevus syndrome.

Basal cell nevus syndrome is an autosomal dominant disorder characterized by multiple basal cell carcinomas, along with numerous other documented clinical features. Acrochordons (or skin tags) are common benign neoplasms that are appropriately left untreated in most patients. We describe two patients with known BCNS who were found to have multiple BCCs that clinically resembled acrochordons. Our findings support the biopsy of acrochordon-like growths in patients with basal cell nevus syndrome to rule out basal cell carcinoma.

Earlier detection of patients' second primary basal cell carcinomas: a quantitative analysis.

BACKGROUND: Basal cell carcinoma (BCC) is the most common occurring cancer in humans. No studies to date have compared the size of a patient's first and second primary BCC to determine whether we are detecting and treating second primary cancers at earlier stages of development. OBJECTIVE: The objective was to ascertain whether patients who have had a primary BCC are diagnosed and treated for subsequent BCC at an earlier clinical stage of development. METHODS AND MATERIALS: We performed a 4-year retrospective chart review of all patients presenting for Mohs micrographic surgery for two separate primary BCC. We compared the presenting clinical size of patients' first and second primary BCC. RESULTS: We found a statistically significant decrease in size of the second primary BCC when compared to the first primary BCC (p<.0001). The mean difference was 116.6 mm2 and the median difference was 28.3 mm2. CONCLUSION: Our results provide important quantitative data illustrating our effectiveness in detecting subsequent BCC at an earlier stage of development. Earlier detection of skin cancers can decrease the morbidity and chance of recurrence as well as lead to smaller scars and better cosmetic outcomes and may also allow for various treatment modalities and cost containment.