Vulvar Primary Cutaneous CD8+ Aggressive Epidermotropic Cytotoxic T-Cell Lymphoma.

Cutaneous T-cell lymphomas may present with a clinical course that is incongruent with the associated histologic findings. Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma classically presents as an abrupt eruption of disseminated ulcerated annular plaques with aggressive behavior and a poor prognosis. Herein we describe a vulvar primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma with a locally aggressive clinical course that was strikingly responsive to radiation therapy. As aggressive therapy involving systemic chemotherapy is indicated for primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma, appropriate clinico-pathologic correlation is crucial for preventing potentially excessive or insufficient therapeutic intervention. Our case also highlights the pivotal role of both radiation therapy and infection control in the management of aggressive cutaneous vulvar lymphomas.

Successful Mastectomy and Chemotherapy in a Patient with Breast Cancer and Active Generalized Pyoderma Gangrenosum.

INTRODUCTION: Pyoderma gangrenosum (PG) is a rare, ulcerating neutrophilic dermatosis often associated with inflammatory bowel disease, rheumatoid arthritis, and myeloproliferative disorders. The classic description of PG includes irregularly shaped ulcers with undermined edges with a gun-metal gray or violaceous hue. The etiology remains unclear but appears to be related to genetically predisposed dysregulation of the innate immune system. Diagnosis of PG can be difficult as it can present with symptoms similar to cutaneous infections including erythema, edema, ulceration, fever and leukocytosis. Surgical procedures are generally contraindicated in patients with PG due to the risk of pathergy, excessive cutaneous injury, or ulceration in response to trauma. CASE REPORT: The authors report the development of PG with the initiation of chemotherapy in a 46-year-old woman with breast cancer. The patient had a complicated clinical course after multiple surgical debridements due to an initial misdiagnosis of necrotizing fasciitis. The patient's rapid onset of post-procedural ulceration was consistent with the pathergy of PG. The diagnosis of PG was confirmed by skin biopsy, which revealed a diffuse neutrophilic infiltrate, and with the patient's negative cultures and response to steroids. The patient was treated with perioperative prednisone and intravenous immunoglobulin prior to a mastectomy for her breast cancer. The surgery was not complicated by pathergy. CONCLUSIONS: This unique case highlights the challenging aspects in the medical and perioperative management of active PG in a patient with breast cancer.

Perioperative management of pyoderma gangrenosum.

Pyoderma gangrenosum (PG) classically presents with an acute inflammatory stage, characterized by rapid evolution of painful ulcerations. The pathergy associated with PG lesions complicates disease management. Although PG is commonly treated with immunosuppression, some patients have refractory noninflammatory ulcers. In this subpopulation, there are case reports of successful surgical treatment. However, there is no consensus on optimal perioperative treatment for patients with PG undergoing surgery of any kind, PG related or otherwise. Therefore, we conducted a comprehensive literature review describing perioperative management practices and risk factors that may predict response to surgical intervention. We identified 126 cases of surgical intervention in patients with active PG; among these, only 16.7% experienced postoperative disease progression. No perioperative treatments or clinical risk factors were identified as statistically significant predictors of disease recurrence. Although limited by case series design and publication bias, this study is a valuable means of hypothesis generation for this rare condition.

Hidradenitis suppurativa associated with sorafenib initiation.

Sorafenib is a multi-kinase inhibitor approved for the treatment of renal cell and hepatocellular carcinoma. Adverse cutaneous reactions are a very common side effect of the medication. We report the development of hidradenitis suppurativa (HS) in a patient after initiation of treatment with sorafenib. HS is marked by recurrent deep painful nodules, fluctuant abscesses, and draining sinus tracts most frequently occurring in the groin and axilla. To our knowledge, sorafenib-induced HS in the axillary and inguinal skin folds has not been previously reported.

A Rare Variant of Penile Squamous Cell Carcinoma in a Man with Paraplegia.

Verrucous carcinoma (VC) is a rare variant of squamous cell carcinoma (SCC). It is described as a low grade, slow growing, locally infiltrative neoplasm that accounts for 3%-8% of penile SCCs. Here we report a case of destructive VC of the glans penis in a paraplegic man resulting in a hypospadias from the tip of the glans to the corona. Histology demonstrated exophytic squamous epithelial proliferation with characteristic round, pushing borders. In situ hybridization was positive for both low-risk and high-risk strains of human papillomavirus.

Atrophic dermatofibroma in an elderly male - a rarely described variant of a common lesion.

Dermatofibroma frequently presents as a red-brown nodule on the extremities of the middle aged. Atrophic dermatofibroma is a rare variant that has been most commonly described as an atrophic depressed, erythematous lesion in females. The correct diagnosis of atrophic dermatofibroma is often hindered by its infrequent presentation. It has a female preponderance with an occurrence ratio of 10:1. We describe a case of an atrophic dermatofibroma on the back of an elderly man. Skin biopsy demonstrated a spindle cell proliferation in a storiform pattern, loss of elastic fibers, and substantial atrophy of both the underlying dermis and subcutaneous tissue. An aggregation of elastic fibers was found in the periphery of the tumor. These histologic features supported the diagnosis of atrophic dermatofibroma. The dermal and adipocyte atrophy was likely responsible for the retracted appearance of the lesion.

A Vascular-appearing Spindle Cell Xanthogranuloma in a Child.

Spindle cell xanthogranuloma is a rare variant of juvenile xanthogranuloma that most commonly presents in adults as papulonodules. We describe a vascular-appearing case of spindle cell xanthogranuloma on the nose of a 10-year-old boy. The lesion was a dark red, well-demarcated, dome-shaped papule. Histopathology revealed spindle-shaped histiocytes in a storiform pattern that stained positive for cluster of differentiation 68 (CD68) and the nuclear antigen Ki-67. No vascular features were found. To our knowledge, this is the first reported spindle cell xanthogranuloma to mimic an angiomatous lesion.