Squamous Cell Carcinoma of the Hand: A Retrospective Study in Immunosuppressed and Immunocompetent Individuals.

BACKGROUND: Cutaneous squamous cell carcinoma (SCC) of the hand presents a treatment challenge because of the anatomical complexity of this location. Immunosuppressed patients are disproportionately affected by cutaneous SCC. Existing data on SCC of the hand are primarily presented in the orthopedic literature, and may thus be affected by referral bias. OBJECTIVE: Characterization of epidemiology and treatment outcomes for hand versus nonhand cutaneous SCC in immunosuppressed versus immunocompetent patients, across all clinical departments. MATERIALS AND METHODS: Single-institution retrospective cohort study of cutaneous SCC evaluated over 3 years and hand SCC over an additional 5 years. RESULTS: A cohort of 522 hand SCC cases (1,746 total SCC) was ascertained among 1,064 patients, of whom 175 were immunosuppressed. Occurrence on the hand was more common for SCC arising in immunosuppressed versus immunocompetent patients (38% vs 24% of cases respectively). Hand SCC cases demonstrated balanced laterality and comparable spectra of differentiation regardless of immunosuppression. No cases of hand SCC metastasis were observed over greater than 2 years' mean follow-up, and digital amputation was only required in approximately 1% of hand SCCs. CONCLUSION: In our cohort, assessment of hand SCC across all clinical departments suggests more favorable prognosis than reflected in the previous literature.

Topical Combination of Fluorouracil and Calcipotriene as a Palliative Therapy for Refractory Extramammary Paget Disease.

IMPORTANCE: Extramammary Paget disease (EMPD), a rare intraepithelial adenocarcinoma, poses a therapeutic challenge with high postoperative recurrence rates and a limited number of effective local treatment options. OBJECTIVE: To describe the use and efficacy of a topical combination of fluorouracil and calcipotriene as a palliative therapy for refractory EMPD. DESIGN, SETTING, AND PARTICIPANTS: This retrospective case series of 3 women with recurrent, refractory EMPD was conducted at Beth Israel Deaconess Medical Center, Boston, Massachusetts and Washington University School of Medicine, St Louis, Missouri. All patients were treated with a 1:1 mixture of fluorouracil, 5%, cream and calcipotriene, 0.005%, cream or ointment. MAIN OUTCOMES AND MEASURES: Clinical and histopathological findings. RESULTS: All 3 women (1 in her 50s, 2 in their 70s) presented with recurrent EMPD (vulvar, perianal, and perioral) after surgery and/or irradiation, and their EMPD was refractory to treatment with imiquimod, 5%, cream. Owing to disease progression and/or intolerable adverse effects from imiquimod, the patients began treatment with a 1:1 mixture of fluorouracil, 5%, cream and calcipotriene, 0.005%, cream. This treatment, which was well tolerated, was followed by clinical improvement in symptoms and appearance of the lesions in all 3 cases and histopathological signs of decreased tumor burden in 2 cases. Patients applied the combination topical therapy to affected areas with differing frequencies, ranging from 1 to 2 days per month to 4 consecutive days every 2 weeks. CONCLUSIONS AND RELEVANCE: Extramammary Paget disease frequently recurs even after aggressive surgical management and can be refractory to many topical and locoregional therapies. Palliative treatment with a combination of fluorouracil and calcipotriene may be a viable option for patients with recurrent, refractory EMPD.

Concurrent metastases of papillary thyroid carcinoma to the scalp and Meckel's cave.

We present the case of a 65-year-old man with severe headaches and unilateral facial weakness, seen in consultation by the dermatology service to rule out primary cutaneous melanoma after brain imaging identified an enlarging mass within the right trigeminal (Meckel's) cave. Examination revealed only a pair of erythematous papules on the scalp, for which biopsy demonstrated metastatic papillary thyroid carcinoma. Further evaluation and subsequent thyroidectomy confirmed the origin of widespread internal disease, followed by definitive excision of scalp lesions and multimodal management of systemic involvement. Whereas presentation of metastasis to the skin is highly variable, a low threshold for biopsy may allow for histological identification of internal disease not otherwise considered in the clinical differential.

Utility of the Hemangioma Severity Scale as a Triage Tool and Predictor of Need for Treatment.

BACKGROUND/OBJECTIVES: Infantile hemangiomas (IHs) are commonly encountered in primary care and most often remain asymptomatic, resolving without sequelae. Certain characteristics are associated with a greater risk of complications, associated anomalies, and disfigurement. The heterogeneous presentation poses a clinical challenge for physicians in determining the need for treatment and subspecialty referral. This study aims to evaluate the utility of the previously published Hemangioma Severity Scale (HSS) to predict the need for treatment. METHODS: This retrospective study included 106 patients with IHs seen in the Indiana University Dermatology Clinic in 2011. Data from electronic medical records and clinical photographs taken at patients' initial visits were used to score the hemangiomas using the HSS. Treatments used over 9 to 14 months of follow-up were recorded. RESULTS: Four HSS score subgroups were identified. Higher HSS scores correlated with the need for treatment; 98% of patients with HSS scores of 10 or greater received local or systemic therapy. Higher HSS scores also correlated with greater frequency of complications and risks of associated structural anomalies and permanent disfigurement. Scores did not correlate with sex, age at initial presentation, history of bleeding or pain, or IH size. CONCLUSIONS: The HSS may be a useful tool for primary care physicians in identifying high-risk IHs that may benefit from therapy. This easy-to-use scale can improve clinical outcomes by identifying which patients need intervention to minimize complications. IHs with total HSS scores of 6 or greater should be referred for subspecialty evaluation.

Myelodysplastic Syndrome Occurring in a Patient with Gorlin Syndrome.

We report a case of myelodysplastic syndrome (MDS) occurring in an African American boy with Gorlin syndrome with a novel PTCH1 mutation. Before developing MDS, the patient had been treated with chemotherapy and radiation for a medulloblastoma. He received a bone marrow transplant for the MDS and eventually died of treatment complications. Secondary hematologic malignancies are a known complication of certain chemotherapeutics, although whether a patient with Gorlin syndrome has a greater propensity for the development of such malignancies is unclear.

Atypical propylthiouracil-induced ANCA-positive vasculitis: report of a case with unusual clinical and histopathologic findings.

The side effects of propylthiouracil, including cytopenia and vasculitis, are well established.  We present an interesting case in which cytopenia and cutaneous vasculopathy occurred concomitantly in a critically ill patient.  The patient was initially treated for suspected infection until dermatologic and rheumatologic workup revealed ANCA-positivity and vasculopathy on histopathology, most consistent with an atypical presentation of ANCA-positive vasculitis.  Upon initiation of immunosuppressive therapy, the patient's condition rapidly improved emphasizing the importance of early recognition of this condition.