An unusual clinical presentation of merkel cell carcinoma: a case report.

Introduction. Merkel cell carcinoma is a rare, aggressive neuroendocrine cell carcinoma arising in the sun-exposed skin of elderly patients. Most of these tumors are located in the dermis. An unusual clinical presentation of such a tumor in the subcutis, if not biopsied, may be easily mistaken as a benign lesion. Case Presentation. An 83-year-old white woman presented with a several-month history of a painless 7 mm subcutaneous mass that was initially thought to be a lipoma. A conservative follow-up was planned. At the insistence of the patient, an excisional biopsy of the mass was performed revealing a subcutaneous Merkel cell carcinoma. The tumor cells stained positively for CK 20, chromogranin, and synaptophysin. No other primary or metastatic tumors found after a thorough work-up. The patient was treated with local irradiation. She remains disease free at her six-month follow-up visit. Conclusion. When a new growth is encountered in the sun-exposed skin of elderly patients, a biopsy is warranted even if the lesion clinically appears benign.

Purpura annularis telangiectodes of Majocchi: case report and review of the literature.

BACKGROUND: Purpura annularis telangiectodes of Majocchi is an uncommon form of pigmented purpuric dermatosis which may present a therapeutic challenge. Given the rare nature of this condition, there is limited anecdotal information available regarding optimal therapy. Although pigmented purpuric dermatoses are generally innocuous, in some cases they may cause patients significant distress, and there is a need to exclude cutaneous T-cell lymphoma. METHODS: We reviewed the literature on the treatment of pigmented purpuric dermatoses and managed a 69-year-old woman who presented with purpuric annular patches on the legs. RESULTS: Three separate biopsies demonstrated an interstitial to perivascular lymphocytic infiltrate with erythrocyte extravasation, consistent with pigmented purpuric dermatosis. The patient's condition proved refractory to many of the previously reported modes of management, but markedly improved with methotrexate. Treatment alternatives for pigmented purpuric dermatosis are reviewed, and a treatment algorithm is proposed. CONCLUSION: This is the first reported case regarding the successful use of methotrexate for pigmented purpuric dermatosis. Methotrexate may offer a therapeutic alternative to patients with highly symptomatic pigmented purpuric dermatosis refractory to other, more conservative, treatment modalities.

Poroma of the hip and buttock.

Poroma is a benign adnexal skin tumor seen in middle aged individuals with no sex predilection. The acral sites are the most commonly affected regions. Hip or buttock as a location of origin has rarely been reported. We report two cases of poroma, one located on the hip of a 75-year-old man and the other on the buttock of a 60-year-old man.

Angiosarcoma of the scalp mimicking a sebaceous cyst.

A 58-year-old Caucasian woman presented with a cystic lump behind the right ear that was clinically diagnosed as an infected sebaceous cyst. The lesion was treated with incision and drainage followed by antibiotics for 3 months. Because there was no resolution, a biopsy was performed that revealed a high grade angiosarcoma. She expired 2 months later. Cutaneous angiosarcoma is an extremely aggressive tumor. Therefore early diagnosis and management is crucial in providing better patient care.

Possible implantation carcinoma of the scalp following craniotomy for metastatic renal cell carcinoma.

We report a case of a 50-year-old man who developed a possible implantation carcinoma of the scalp within the craniotomy scar 19 months after a metastatic renal cell tumor nodule was removed from the brain. The English literature on the implantation carcinoma is briefly reviewed.

Desmoplastic trichoepithelioma: report of a case illustrating its natural history.

First described more than 30 years ago, desmoplastic trichoepithelioma is a rare but benign adnexal neoplasm. Most often identified in middle-aged individuals and females, desmoplastic trichoepithelioma usually is a solitary annular plaque. Though the tumors are benign, the possibility of malignant neoplasm may spark both clinical and histologic concern. A full-thickness skin biopsy is advisable when desmoplastic trichoepithelioma is suspected. A patient's clinical history may provide some clues to help guide diagnosis, as the tumors may be present for years and slow growth is commonly reported. We present a patient with desmoplastic trichoepithelioma that uniquely documents and supports the typical natural history of this tumor, as demonstrated by annual school photographs.

Mycobacterium mucogenicum: report of a skin infection associated with etanercept.

Mycobacterium mucogenicum is a recently characterized organism that rarely may cause human infections. This rapidly growing mycobacterium is commonly identified in tap water. Both immunosuppressed and immunocompetent patients may develop infections from Mycobacterium mucogenicum. Some patients have experienced lethal disease, including sepsis. Infections occurring in the skin and soft tissues have been described only after a preceding injury. We present the first case of infection with Mycobacterium mucogenicum occurring in a patient on the TNF-alpha antagonist etanercept and without any prior soft tissue injury.

Plasmapheresis for refractory urticarial vasculitis in a patient with B-cell chronic lymphocytic leukemia.

BACKGROUND: Urticarial vasculitis is a form of cutaneous leukocytoclastic vasculitis clinically characterized by persistent and often painful urticarial lesions. Numerous systemic diseases have been associated with urticarial vasculitis, including certain hematologic disorders. This distinctive form of cutaneous necrotizing vasculitis can be resistant to standard therapeutic modalities, necessitating more aggressive intervention. METHODS: We report a case of refractory urticarial vasculitis developing in association with B-cell chronic lymphocytic leukemia in a 46-year-old man. We also reviewed the literature to identify other cases of urticarial vasculitis managed with this therapeutic modality. RESULTS: The disease progressively improved during 6 treatments with plasmapheresis (plasma exchange). In the additional cases identified in the literature, plasmapheresis was generally effective and well tolerated. CONCLUSION: On the basis of these findings, we propose that plasmapheresis be considered a treatment option for refractory urticarial vasculitis.

Angiosarcoma arising in a lymphedematous abdominal pannus with histologic features reminiscent of Kaposi's sarcoma: report of a case and review of the literature.

BACKGROUND: Despite common endothelial origins, angiosarcoma and Kaposi's sarcoma are clinically and histologically distinct vascular proliferations. The development of angiosarcoma in a chronically edematous abdominal pannus is extremely uncommon. Similarly, tumors with the combined histologic features of angiosarcoma and Kaposi's sarcoma have rarely been described. METHODS: We reviewed the literature on angiosarcoma arising in a lymphedematous abdominal pannus and evaluated an 81-year-old morbidly obese woman who had profound, long-standing edema of the lower abdominal wall in which an aggressive vascular tumor developed. RESULTS: Three clinically similar cases were identified in the literature. All patients were women who generally experienced rapid disease progression. In addition, in our patient, sequential cutaneous sampling from different lesional sites demonstrated disparate histologic changes, ranging from those of classic Kaposi's sarcoma to high-grade angiosarcoma, to areas with combined features of the two tumors. A polymerase chain reaction performed on lesional tissue was negative for human herpesvirus-8 DNA. CONCLUSION: It is important to note that angiosarcoma may develop in the abdomen in association with chronic lymphedema, as demonstrated by the cases noted in this report. In addition, our case highlights the difficulty in differentiating histologically angiosarcoma from Kaposi's sarcoma in some situations, and demonstrates the value of close clinicopathologic correlation and sequential tissue sampling in evaluating problematic cases.

Management of multifocal primary cutaneous CD30 anaplastic large cell lymphoma.

Primary cutaneous CD30(+) anaplastic large cell lymphoma in adults is rare, but the prognosis is generally excellent. Skin lesions may be localized or, less commonly, multifocal. Although not extensively reported, multifocal primary cutaneous anaplastic large cell lymphoma tends to relapse after systemic chemotherapy and is generally considered more prone to progress to extracutaneous involvement than the localized disease. We report the case of a 21-year-old woman with primary cutaneous CD30(+) anaplastic large cell lymphoma manifesting as widespread papules and nodules. Despite remaining localized to the skin, the disease relapsed after multiple chemotherapy regimens and autologous stem-cell transplantation. Treatment with an experimental anti-CD30 monoclonal antibody was successful. Review of this case and similar cases illustrates that traditional combination chemotherapy may not be best. Newer treatments, including anti-CD30 monoclonal antibodies, show promise. However, further study is needed to develop optimal therapeutic strategies.

Recurrent palmoplantar hidradenitis with exclusive palmar involvement and an association with trauma and exposure to aluminum dust.

Recurrent palmoplantar hidradenitis is a benign, self-limited inflammatory skin condition that has been reported to occur on the soles and palms of otherwise healthy children and young adults. Patients with the disease present with tender, erythematous and edematous plaques and nodules on the palmoplantar skin. We describe a child who had recurrent palmoplantar hidradenitis that occurred after trauma and exposure to aluminum dust and manifested as lesions localized to only the palmar surfaces. This case is presented to add exclusive palmar involvement to the diagnostic spectrum of recurrent palmoplantar hidradenitis and to review the proposed pathogenesis of the disease.

Erythema multiforme associated with phenytoin and cranial radiation therapy: a report of three patients and review of the literature.

BACKGROUND: Intracranial malignancies (primary and metastatic) are often complicated by seizure activity. Phenytoin (Dilantin) is typically employed as prophylactic anticonvulsant in this setting. Uncommonly, erythema multiforme (EM) can develop in such patients at the port site during or soon after cranial radiation and can rapidly progress to EM major. Herein, in addition to a comprehensive literature review of this entity, three additional patients are presented. The acronym 'EMPACT' is suggested (E: erythema; M: multiforme; associated with P: phenytoin; A: and; C: cranial, radiation; T: therapy) to best describe this disorder. METHODS: An extensive review of the English medical literature through the National Library of Medicine (PUBMED) was performed to identify patients who had received or continued to receive radiation therapy while on phenytoin. A total of 24 patients were identified and clinical information of varying detail was available in all cases. Clinical and histological information on three additional patients seen at two institutions (Rochester Methodist Hospital, Rochester, MN, and Fairview-University Medical Center, Minneapolis, MN) by the authors were also compiled. RESULTS: The mean age was 44 years (range: 23-67) and no sexual predisposition was noted. All patients had taken phenytoin for variable time periods (range 16-80 days; mean: 40) and were on the medication when the skin lesions first appeared. These lesions developed within the port site during the radiation treatments (11 cases) or soon after (nine cases) its completion (mean: 16 days; range: 2-35). Subsequent disease evolution to EM major occurred in all cases (Stevens-Johnsons syndrome developed in 73% of patients). No relationship was identified between the extent and the severity of the skin lesions with the phenytoin and radiation dosages and with the histologic type and origin of the intracranial malignancy. None of the patients demonstrated the requisite features of the 'Dilantin hypersensitivity syndrome'. Although, a systemic steroid taper was employed in 10 out of the 14 patients before the development of the skin lesions, the subsequent progression of the skin lesions was not influenced by the use of systemic steroid therapy. Complete recovery occurred in all but two patients typically within 1-8 weeks of discontinuation of phenytoin. CONCLUSIONS: The need for prophylactic anticonvulsant therapy especially utilizing phenytoin in patients undergoing cranial radiation therapy should be assessed on a case by case basis. If anticonvulsants are employed, then they must be administered with caution, and all cutaneous reactions developing subsequently within the radiation site must be promptly evaluated with a high index of suspicion for erythema multiforme.