Penicillamine-induced pseudoxanthoma elasticum-like skin changes requiring rhytidectomy.

A 42-year-old woman with pronounced skin laxity of her neck underwent a rhytidectomy and was found to have pseudoxanthoma elasticum-like changes of her skin. Her medical history was significant for Wilson's disease, requiring that she take penicillamine for 26 years. In patients on long-term penicillamine therapy, 20% to 33% will develop a dermatopathy. The drug has been used to alter scar formation in various surgical conditions. Penicillamine is known to alter cross-linking of elastin and collagen fibers. A review of the literature reveals other penicillamine-related dermatopathies that may present to the surgeon.

Pigmented basal cell carcinoma: investigation of 70 cases.

BACKGROUND: Pigmented basal cell carcinoma (PBCC) is a clinical and histologic variant of BCC. OBJECTIVE: Our purpose was to identify the histologic subtypes of BCC that were most often associated with pigment and to determine whether this correlated with outcome after excision. METHODS: A series of PBCC was identified and the histologic subtype noted. Margins of all excisions were examined for residual tumor. These results were then compared with a series of nonpigmented BCCs. RESULTS: In a series of 1039 consecutive BCCs, 70 (6.7%) contained pigment. The histologic growth pattern most frequently associated with pigment was the nodular/micronodular pattern (12.4%) followed by the nodular (7.7%), superficial (7.2%), micronodular (4.0%), and the nodular/micronodular/infiltrative (3.4%) patterns. Margins were examined for evidence of residual tumor in the 40 cases that were excised. In only one case (2.5%) was the margin positive for tumor. This was statistically significant (p less than 0.05) compared with 388 excisions of nonpigmented BCCs with comparable growth patterns in which 69 (17.7%) showed positive margins. CONCLUSION: PBCC, as a clinical variant, is more frequently excised with adequate margins than are tumors of comparable histologic subtypes that do not contain pigment.

Poorly differentiated primary orbital sarcoma (presumed malignant rhabdoid tumor). Radiologic and histopathologic correlation.

A 47-year-old man presented with acquired hyperopia and gaze-evoked visual loss. Computed tomography and magnetic resonance imaging demonstrated a well-circumscribed intraconal tumor. Histopathologic studies indicated that the tumor was a poorly differentiated primary orbital sarcoma compatible with malignant fibrous histiocytoma or malignant rhabdoid tumor. To our knowledge, no case of primary orbital malignant rhabdoid tumor has been previously described in an adult.

Cutaneous manifestations of multiple myeloma.

We report the cutaneous manifestations of multiple myeloma, using a retrospective review of 115 patients' records obtained from tumor registry files. Five patients were found to have biopsy-proved extramedullary plasmacytomas without extension from an underlying bony focus. Twelve patients had ecchymoses without thrombocytopenia; two of them had biopsy-proved amyloidosis. One patient presented with pyoderma gangrenosum and was subsequently diagnosed with multiple myeloma. The last patient initially presented with what clinically appeared to be leukocytoclastic vasculitis, and, in the course of a standard workup, he was diagnosed with multiple myeloma. These findings are discussed with regard to the current literature on the cutaneous manifestations of multiple myeloma.

HLA class I and class II antigen expression on squamous cell carcinoma of the head and neck.

We compared human major histocompatibility (HLA) class I and class II antigen expression on squamous cell carcinoma of the head and neck with that on normal mucosa. Frozen sections of a consecutive series of 30 squamous cell carcinomas were stained with the monoclonal antibodies W6/32 (class I) and anti-DR (class II) using an immunoperoxidase technique. Normal mucosa showed class I and class II expression in the basal layers only. Class I expression on tumors was diffuse in 87%, patchy in 10%, and scattered in 3%. Class II expression on tumors was diffuse in 20%, patchy in 53%, scattered in 20%, and absent in 7%. Patterns of expression did not correlate significantly with clinical parameters, including survival, except that class II diffuse and patchy patterns were found to correlate with more poorly differentiated tumors.

Skin involvement as the presenting sign of internal carcinoma. A retrospective study of 7316 cancer patients.

From tumor registry data of 7316 cancer patients, we found 367 cases (5.0%) with skin involvement. Skin involvement was present at the time of presentation in 92 patients (1.3%), only 26 of whom had remote metastases. Skin involvement was the first sign of cancer in 59 patients (0.8%); 22 had direct extension of their tumor into the skin, 20 had local metastases, and 17 had distal metastases. Direct invasion was most common with breast cancer and second most common with oral cavity cancer. Local metastases were also most frequently caused by breast cancer but occurred in surgical scars in three women with pelvic cancer and in perianal abscesses in one patient with rectal carcinoma as well. Except for metastases from unknown primary sites, distant metastases were rare as presenting signs, and their origins were widely distributed. Our data show that internal cancer uncommonly presents with skin involvement. Nevertheless, an index of suspicion should be maintained and biopsy performed, particularly for nonhealing ulcers, persistent indurated erythema, and unexplained skin nodules.

Immunosuppression in men with bowenoid papulosis.

The immune status of four men with bowenoid papulosis was evaluated. Each case had been refractory to multiple methods of treatment. Three of the men had other infections and demonstrated a depletion of T4-helper cells. Two of these patients were anergic on skin testing, and the third showed weak reactivity. The fourth patient, who had no evidence of additional infections, had a normal T4 value and T4/T8 ratio, but was anergic on skin testing. All the men were serologically negative for human immunodeficiency virus antibodies. One of the immunosuppressed patients developed squamous cell carcinoma of the tongue, which, along with his bowenoid papulosis, contained human papillomavirus 16 DNA. We suggest that patients with persistent bowenoid papulosis be investigated for altered immune status and followed up as potential candidates for the development of epithelial malignant neoplasms.

Metaplastic synovial cyst after partial excision of nevus sebaceus.

The metaplastic synovial cyst (synovial metaplasia of the skin) is a recently described intradermal cyst lined by a membrane that resembles hyperplastic synovium. All of the reported cases thus far have occurred in adults after surgical incision. A case of metaplastic synovial cyst after partial excision of nevus sebaceus in a 7-year-old girl is herein reported. Pathogenesis and light-microscopic and immunohistochemical features of this case are discussed.

Metastatic phenotype in murine cells transfected with human DNA.

DNAs of cell lines derived from human metastatic tumors were transfected into tumorigenic, nonmetastasizing murine cells (BALB 3T12-3) to determine if the capacity to metastasize could be conferred by this transfer of portions of the human genome. Using the calcium phosphate method, whole cell DNA was cotransfected into the murine cells along with a neomycin-resistance gene. Recipient murine cells (10(6] which grew in neomycin, indicating successful transfection, were injected via tail vein into 4- to 6-week-old male athymic nude mice. Animals were sacrificed if they appeared ill or at times up to 24 weeks after injection if they remained healthy. Murine cells transfected with DNA from one cell line derived from a hepatic metastasis of a human pancreatic adenocarcinoma (NCI-ZRY) formed experimental pulmonary metastases in 11 of 13 animals injected. Neither murine cells (unmanipulated BALB 3T12-3 cells) nor murine cells transfected with DNA from the same cell type (BALB 3T12-3 cells transfected with BALB 3T12-3 DNA) produced experimental metastases when each cell type was injected into 20 and 10 animals, respectively (P2 less than 0.0001). The results are consistent with the hypothesis that expression of a structural or regulatory protein encoded on human DNA conferred the metastatic phenotype to the recipient murine cells.

Melanotic macules and melanoacanthomas of the lip. A comparative study with census of the basal melanocyte population.

Fifteen labial melanotic macules and three melanoacanthomas of the lip were studied with particular regard to their distinguishing histopathological features including a quantitative study of the basal melanocyte population. Both of these entities show an increased population of melanocytes arranged as single units along the junctional zone. These conditions differ from each other primarily by the presence of intraepithelial melanocytes in melanoacanthoma. In our opinion, the melanotic macule of the lip has numerous histological counterparts that occur on other mucosal and cutaneous surfaces. Oral mucosal analogies to the labial melanoacanthoma also exist.

Follicular lymphomatoid papulosis.

A patient with follicular lymphomatoid papulosis is reported. Only two cases of this rare variant have been previously reported in the literature. Lesions typical of lymphomatoid papulosis in this case were centered around disrupted hair follicles. They corresponded to the type A "histiocytic" variant of lymphomatoid papulosis. The histologic features and theories regarding histogenesis of this unusual disorder are discussed.