Excellent response to ustekinumab in a 9-year-old girl with severe psoriasis.

We report the case of a 9-year-old girl with severe plaque psoriasis refractory to multiple topical and systemic therapies. Physical examination revealed extensive, erythematous plaques with overlying thick scales that covered more than 80% of her body surface area, which included the face, scalp, trunk, and limbs. Because of the severity of the disease and lack of treatment response to other systemic therapies, she was treated with ustekinumab. Three weeks after ustekinumab was initiated, her psoriatic lesions fully cleared.

Extraocular Sebaceous Carcinoma-A Clinicopathologic Reassessment.

Sebaceous carcinoma is an aggressive adnexal neoplasm with sebaceous differentiation. Few reports have described the histopathologic characteristics of the sebaceous carcinoma occurring extraocularly. Seventy-two cases of extraocular sebaceous carcinoma were identified from the database of a Dermatopathology Laboratory from January 1, 2007 to May 31, 2013. More cases occurred in men (60%), with a mean age at diagnosis of 65.8 years (range 39-99 years). Neoplasms were histopathologically classified as well-differentiated (22%), moderately differentiated (67%), and poorly differentiated (11%). Sixty-seven percent (67%) of cases demonstrated a squamoid growth pattern and thirty-three percent (33%) demonstrated a basaloid growth pattern. A majority of the neoplasms histopathologically classified as well-differentiated (94%) and moderately differentiated (65%) demonstrated a squamoid growth pattern. Ten percent (10%) of cases exhibited cystic histopathologic changes. The histopathological features reported in this study aid in the understanding of extraocular sebaceous carcinoma and its eventual diagnosis and classification.

Histiocytoid Sweet syndrome may indicate leukemia cutis: a novel application of fluorescence in situ hybridization.

BACKGROUND: In patients with malignancy-associated Sweet syndrome, a thorough evaluation for leukemia cutis should be considered. OBJECTIVE: We sought to describe the clinicopathologic characteristics of histiocytoid Sweet syndrome. METHODS: We retrospectively identified patients with histiocytoid Sweet syndrome at our institution from January 1992 through December 2010. We evaluated the underlying cutaneous infiltrate using immunohistochemistry and fluorescence in situ hybridization. RESULTS: We re-evaluated all 22 patients with hematologic malignancy-associated Sweet syndrome. Six patients had a monocytoid infiltrate that was consistent with histiocytoid Sweet syndrome; subsequent evaluation of these patients demonstrated cytogenetic abnormalities on prior bone-marrow biopsy specimens. Fluorescence in situ hybridization analysis was feasible in cutaneous specimens from 5 of the 6 patients and demonstrated the same cytogenetic abnormalities that were identified on prior bone-marrow biopsy specimens in 4 patients. Therefore, these 4 patients may have had a form of leukemia cutis. LIMITATIONS: This was a retrospective study. CONCLUSION: For patients with histiocytoid Sweet syndrome, an underlying hematologic malignancy, and a monocytoid infiltrate on biopsy specimen, fluorescence in situ hybridization of the cutaneous infiltrate may be beneficial to identify cytogenetic abnormalities that may indicate leukemia cutis.

Sweet syndrome: clinical presentation, associations, and response to treatment in 77 patients.

BACKGROUND: Sweet syndrome is a neutrophilic dermatosis with cutaneous tender lesions that can be associated with malignancies, infections, systemic inflammatory disorders, and medications. Although numerous studies have described Sweet syndrome, few studies have systematically investigated Sweet syndrome. OBJECTIVE: We sought to describe characteristics and treatments of patients with Sweet syndrome and evaluate clinical differences depending on the underlying cause. METHODS: A retrospective study was conducted to identify patients with Sweet syndrome evaluated at Mayo Clinic from 1992 to 2010. RESULTS: Of 77 patients with Sweet syndrome (mean age of onset 57 years), 43 (56%) were male. Eighteen patients (23%) reported a preceding infection. A total of 41 (53%) patients were classified as having classic Sweet syndrome, 27 (35%) patients had malignancy-associated Sweet syndrome, and in 9 (12%) patients drug-induced Sweet syndrome was considered. In all, 21 patients had a hematologic malignancy or myeloproliferative/myelodysplastic disorder, whereas 6 patients had solid tumors. The mean hemoglobin level, in both male and female patients (P < .0443 and P < .0035, respectively), was significantly lower in malignancy-associated versus classic and drug-induced Sweet syndrome. Systemic corticosteroids were the most frequently used treatment (70%). LIMITATIONS: This is a retrospective study and represents patients from a single academic center. CONCLUSIONS: Sweet syndrome is a distinctive disorder with certain clinical and histologic characteristics, which usually has a complete response to systemic corticosteroids. It is important to evaluate Sweet syndrome patients who have laboratory evidence of anemia for an underlying malignancy.

Clinical course and factors associated with remission in pemphigus vulgaris patients in Puerto Rico.

OBJECTIVE: To describe and identify those factors associated with remission on pemphigus vulgaris (PV) patients in Puerto Rico. METHODS: This retrospective cohort study evaluated PV patients followed at the University Puerto Rico (UPR) Bullous Diseases Clinic during the 2000-2010 period. Patients included in the study had clinical and pathologic findings consistent with PV and had a disease duration of at least 3 years. Variables including gender, date of birth, time of disease onset, and date of first partial or complete remission were collected from the medical chart for each study participant. The primary outcome was to determine the number of patients who achieved partial or complete remission. Other secondary outcomes were to identify if partial/complete remission were associated to gender, disease duration, and age at onset of disease. RESULTS: Among 35 patients included in this study, 6 (17%) achieved complete remission and 28 (80%) achieved partial remission. A statistically-significant association was found between duration of disease and remission, predicting a 52% probability of remission after ten years of disease duration. Age at onset of disease showed a trend association with remission, although it was not statistically significant. Gender was not associated with remission. CONCLUSION: These findings provide insights into the clinical course of PV and can be of value in the management and care of this patient population.

Characterization of Pathogens Isolated from Cutaneous Abscesses in Patients Evaluated by the Dermatology Service at an Emergency Department.

OBJECTIVE: Emergency department (ED) visits for the treatment of skin abscesses have increased with the emergence of community-associated methicillin-resistant Staphylococcus aureus (CAMRSA). There is limited information about the bacteriology of cutaneous abscesses evaluated in ED in Puerto Rico. The purpose of our study was to characterize the pathogens cultured from abscesses of patients in the ED consulted to the Dermatology Service of University of Puerto Rico School of Medicine. METHODS: Patients with skin abscesses consulted to the Dermatology Service by the ED of P.R. Medical Center from 2012 to 2017 were included. Data retrieved included demographic information, past medical history, prior antibiotic use, distribution of lesions, and treatment provided. Bacteriology results and antimicrobial susceptibility patterns from cultured skin lesions were recorded. RESULTS: Ninety patients diagnosed with skin abscess were evaluated. All patients underwent incision and drainage; this was the sole treatment in two patients. The most frequently administered systemic therapy was oral clindamycin in 32 patients (36%). A total of 66 patients (73%) had S. aureus isolates, most of them (85%) MRSA. Among the isolates with MRSA, 14.3% were resistant to clindamycin. All MRSA strains were susceptible to tetracycline and vancomycin. CONCLUSION: There is a high prevalence of MRSA causing abscesses in the Hispanic population evaluated in an ED in Puerto Rico. Systemic antibiotic use for the treatment of skin abscesses after incision and drainage remains high despite published guidelines arguing against their widespread use. Clindamycin resistance in our patient population appears to be more frequent than previously reported.

Warfarin-induced skin necrosis following heparin-induced thrombocytopenia.

Anticoagulants, such as heparin and warfarin, are commonly used in the treatment and prevention of thromboembolic events. The risk of developing warfarin-induced skin necrosis (WISN) with warfarin is reported to be <1%. However, the risk of WISN may be increased with the initiation of warfarin in the setting of heparin-induced thrombocytopenia and thrombosis syndrome (HITT). WISN can lead to catastrophic tissue necrosis requiring amputations and mass debridement. This report describes a case of WISN following HITT and discusses the appropriate medical management of patients with HITT to avoid secondary WISN.

The prognostic role of the preoperative absolute lymphocyte count and absolute monocyte count in patients with resected advanced melanoma.

OBJECTIVES: Published data have reported that components of the peripheral blood are significant prognostic factors in hematologic and solid malignancies. Thus, we sought to investigate if the preoperative absolute lymphocyte count (ALC) and absolute monocyte count (AMC) affects disease progression and survival after complete surgical resection of advanced malignant melanoma. METHODS: We retrospectively reviewed records of 227 patients with resected advanced malignant melanoma (153 stage III and 74 stage IV) that were treated at the Mayo Clinic from 2000 to 2010. Survival analysis was performed using the Kaplan-Meier method, log-rank tests, and the Cox proportional hazards model for the univariate and multivariate analysis. RESULTS: Surgically resected stage III melanoma patients with a preoperative AMC<0.6×10/L experienced a longer overall survival (OS) versus AMC≥0.6×10/L (median: 63.9 vs. 34.8 mo, respectively, P<0.008). Multivariate analysis showed AMC to be an independent predictor for OS in stage III patients. Stage IV resected melanoma patients with an ALC≥1.9×10/L experienced a superior median relapse-free survival (RFS) compared with patients with an ALC<1.9×10/L (median: 11.4 vs. 5.4 mo, respectively, P<0.006). Multivariate analysis showed ALC to be an independent predictor for RFS in stage IV patients. CONCLUSIONS: These data showed that in surgically resected stage III melanoma, preoperative AMC is an independent prognostic factor for OS. In contrast, a higher preoperative ALC is an independent prognostic for longer RFS in surgically resected stage IV melanoma.

Clinostatic syndrome in Waldenström macroglobulinemia.

We report a case of Waldenström macroglobulinemia revealed by clinostatic syndrome in an 81-year-old woman. A lytic lesion was found in the ilium and acetabulum. There was no evidence of transformation to high-grade lymphoma. Radiation therapy ensured complete resolution of the clinostatic syndrome within 1 month of treatment completion.

Melanoma brain metastases and vemurafenib: need for further investigation.

Brain metastases are a major cause of morbidity and mortality in patients with advanced melanoma. With the development of targeted agents for the treatment of metastatic melanoma, a great deal of interest has focused on whether selective BRAF inhibitors may play a role in the treatment of brain metastases in lieu of or in addition to surgery and/or radiation therapy. However, relatively little is known about the intracranial effectiveness of vemurafenib, the only US Food and Drug Administration-approved selective BRAF V600E inhibitor, because patients with brain metastases have historically been excluded from vemurafenib clinical trials. We describe 3 patients with BRAF V600E mutation metastatic melanoma in whom treatment with vemurafenib resulted in prompt extracranial disease response but progression of metastatic disease in the brain. Further, we discuss possible mechanisms responsible for the suboptimal central nervous system response observed in these patients and alternative therapies for patients with melanoma metastatic to the brain.