Trichodysplasia spinulosa: a presentation of polyomavirus infection in immunosuppressed patients.

Trichodysplasia spinulosa (TS) is a rare skin condition that occurs mainly in immunosuppressed patients. Although initially postulated to be an adverse effect of immunosuppressants, TS-associated polyomavirus (TSPyV) has since been isolated from TS lesions and is now considered to be the causative agent. Trichodysplasia spinulosa presents with folliculocentric papules with protruding keratin spines, most commonly on the central face. Trichodysplasia spinulosa can be diagnosed clinically, but the diagnosis can be confirmed with histopathological examination. Histological findings include the presence of hyperproliferating inner root sheath cells containing large eosinophilic trichohyaline granules. Polymerase chain reaction (PCR) can also be used to detect and quantify TSPyV viral load. Owing to the paucity of reports in the literature, TS is frequently misdiagnosed and there is no high-quality evidence to guide management. Herein, we present a renal transplant recipient with TS that did not respond to topical imiquimod but improved upon treatment with valganciclovir and reduction of the mycophenolate mofetil dose. Our case highlights the inverse relationship between immune status and disease progression in this condition.

An unusual case of multiple grouped non-familial trichoepitheliomas.

Trichoepitheliomas (TEs) are benign and rare adnexal hamartomas of the pilosebaceous units. Trichoepitheliomas could occur in the setting of an underlying genetic disorder with multiple TEs or as solitary non-hereditary TEs. We report a healthy 32-year-old woman with sporadic multiple clustered and non-segmental TEs without positive family history. There have been two other cases reported in the literature that had non-familial multiple TEs, one was facially disfiguring and the other was in a segmental pattern. Our case has been the only one reported in the English literature which has sporadic, multiple TEs clustered unilaterally and non-segmentally on the trunk.

Ustekinumab to target granulomatous dermatitis in recalcitrant ulcerative necrobiosis lipoidica: case report and proposed mechanism.

We present a 42-year-old woman with no history of diabetes or glucose intolerance who had a 5-year history of ulcerative necrobiosis lipoidica (NL). Despite failure of multiple medications, she experienced clearing of her ulcers after her treatment was changed to ustekinumab. We discuss our patient's disease course and elaborate upon mechanistic reasons for her improvement related to ustekinumab therapy.

100% Complete response rate in patients with cutaneous metastatic melanoma treated with intralesional interleukin (IL)-2, imiquimod, and topical retinoid combination therapy: results of a case series.

BACKGROUND: Patients with cutaneous melanoma metastases have experienced excellent responses to intralesional interleukin (IL)-2. This has led to its recent inclusion into the US National Comprehensive Cancer Network guidelines for management of cutaneous melanoma metastases. Despite this, intralesional IL-2 has not been highlighted in the US literature nor have US physicians adopted it. OBJECTIVE: We sought to evaluate the effectiveness of intralesional IL-2 combined with topical imiquimod and retinoid for treatment of cutaneous metastatic melanoma. METHODS: A retrospective case series of 11 patients with cutaneous metastatic melanoma were treated with intralesional IL-2 combined with topical imiquimod and retinoid. RESULTS: A 100% complete local response rate with long-term follow-up (average of 24 months) was seen in all 11 patients treated with this proposed regimen. Biopsy specimens of treated sites confirmed absence of malignant cells. The most common treatment-related adverse event was rigors. LIMITATIONS: Small number of patients, retrospective review of charts, and lack of a comparison group were limitations. CONCLUSION: Intralesional IL-2 administered concomitantly with topical imiquimod and a retinoid cream is a promising therapeutic option for managing cutaneous melanoma metastases. The regimen was well tolerated and should be considered as a reasonable alternative to surgical excision.

Metastatic melanoma - a review of current and future treatment options.

Despite advances in treatment and surveillance, melanoma continues to claim approximately 9,000 lives in the US annually (SEER 2013). The National Comprehensive Cancer Network currently recommends ipilumumab, vemurafenib, dabrafenib, and high-dose IL-2 as first line agents for Stage IV melanoma. Little data exists to guide management of cutaneous and subcutaneous metastases despite the fact that they are relatively common. Existing options include intralesional Bacillus Calmette-Guérin, isolated limb perfusion/infusion, interferon-α, topical imiquimod, cryotherapy, radiation therapy, interferon therapy, and intratumoral interleukin-2 injections. Newly emerging treatments include the anti-programmed cell death 1 receptor agents (nivolumab and pembrolizumab), anti-programmed death-ligand 1 agents, and oncolytic vaccines (talimogene laherparepevec). Available treatments for select sites include adoptive T cell therapies and dendritic cell vaccines. In addition to reviewing the above agents and their mechanisms of action, this review will also focus on combination therapy as these strategies have shown promising results in clinical trials for metastatic melanoma treatment.

Detailed protocol for administration of intralesional IL-2 for the treatment of Stage IIIc and IV M1a metastatic melanoma based on current NCCN guidelines.

Melanoma claims approximately 9,000 lives in the United States annually. Patients who present with satellite, in-transit, or distant cutaneous metastases have limited treatment options and the prognosis for patients with metastatic disease remains poor. Surgical excision remains the most common treatment modality for cutaneous metastases, but may not address concurrent subclinical in-transit metastases. Other palliative treatment options include Bacillus Calmette-Guérin (BCG) and isolated limb perfusion (ILP). Although intravenous IL-2 has been used for treatment of metastatic melanoma since 1998, intralesional IL-2 has only now been included in the most recent National Comprehensive Cancer Network (NCCN) guidelines after case series and phase I/II clinical trials have shown promising results against Stage IIIc and IV M1a melanoma. Intralesional IL-2 protocols have varied markedly from study to study and there are no consensus guidelines available to help direct treatment. Herein, we present a detailed protocol for the administration of intralesional IL-2 that has been successfully used at two different institutions for treatment of cutaneous melanoma metastases.

Multimodal therapy of idiopathic pyoderma gangrenosum.

A 13-year old girl was admitted to the University of California Davis Medical Center for evaluation and treatment of cutaneous bullae and ulcerations over her lower extremities that were refractory to antibiotic therapy and incision and drainage. Her disease continued to worsen with the appearance of multiple new bullae and the progression of old ones into deep ulcers with undermined borders. Biopsy revealed a neutrophilic dermatosis and diagnostic work-up was negative for infectious or autoimmune etiologies. Given her clinical presentation, biopsy results, and negative work-up, a diagnosis of pyoderma gangrenosum (PG) was made and she was started on immunosuppressive medications. The patient was started on a multidrug regimen of prednisone and cyclosporine but remission was not achieved until the addition of adalimumab. After the inflammatory component of her disease was under control, wound care measures were maximized to promote ulcer healing. Wound care measures included compression and debridement. Upon complete closure of all wounds she was successfully transitioned to mycophenolate mofetil monotherapy for maintenance therapy. This case emphasizes the need for combinational therapy to successfully treat severe cases of PG, which are often refractory to monotherapy with prednisone or cyclosporine. It also highlights the importance of appropriate wound care to achieve complete ulcer healing.