Cutaneous angiosarcoma presenting as bilateral periorbital edema.

A 66-year-old man presented with chronic bilateral periorbital edema with associated yellowish hue, scattered violaceous smooth macules and contracture of the forehead. He had undergone dental surgery 3 months prior to symptom onset. Laboratory workup for common causes of eyelid edema was unremarkable and MRI of the orbits was unrevealing. The patient did not respond to oral corticosteroids or antibiotics. Punch biopsies were obtained which revealed atypical lymphatic endothelial cells consistent with a diagnosis of cutaneous angiosarcoma.The patient was deemed not to be a surgical candidate and underwent 3 cycles of immunotherapy with limited response. He declined further treatment and transitioned to hospice care. Although cutaneous angiosarcoma uncommonly involves the periorbital region, it should be considered in the differential diagnosis of eyelid edema as early recognition and treatment are critical to prevent rapid intradermal spread and metastases.

Association of antinuclear antibody status with clinical features and malignancy risk in adult-onset dermatomyositis.

BACKGROUND: The clinical significance of antinuclear antibody (ANA) status in adults with dermatomyositis (DM) has yet to be fully defined. OBJECTIVE: We compared the incidence of amyopathic disease, risk of malignancy, and clinical findings in ANA+ and ANA- patients with adult-onset DM. METHODS: This was a retrospective cohort study of patients with ANA+ or ANA- adult-onset DM determined by enzyme-linked immunosorbent assay. RESULTS: Of 231 patients, 140 (61%) were ANA+ and 91 (39%) were ANA-. Compared with the ANA- patients, the ANA+ patients had a lower frequency of dysphagia (15% vs 26% [P = .033]) and heliotrope rash (38% vs 53% [P = .026]). In all, 54 patients (23%) developed malignancy within 3 years of diagnosis of their DM; 11% of the ANA+ patients developed malignancy versus 43% of the ANA- patients (P < .001). There was a strong association between ANA positivity and lower likelihood of malignancy in multivariable analysis (odds ratio, 0.16; P < .001). Conversely, ANA positivity was not associated with amyopathic disease (odds ratio, 0.94; P = .87). LIMITATIONS: The retrospective nature of the study was a limitation. CONCLUSION: In patients with adult-onset DM, ANA negativity is associated with increased likelihood of development of malignancy within 3 years of diagnosis of their DM. Particularly close follow-up and frequent malignancy screening may be warranted in ANA- individuals with DM.

Persistent dermatomal eruption on a leg.

Unilateral linear capillaritis (ULC) is a rare variant of pigmented purpuric dermatoses (PPD) that is characterized by a linear or pseudo-dermatomal eruption on a single extremity. Although clinically distinct from the other PPD, it shares histopathologic features with this group. Herein, we present a man in his 50s who presented with asymptomatic macules and scaly papules on the left lower extremity in a linear distribution. The eruption persisted despite treatment with topical triamcinolone 0.1% and oral rutocide.

Prognostic impact of fibrosarcomatous transformation in dermatofibrosarcoma protuberans: a cohort study.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade cutaneous malignancy that sometimes transforms into a high-grade fibrosarcomatous variant (DFSP-FS). Limited data compare clinical features and biological behavior of these 2 entities. OBJECTIVE: We sought to compare clinical features and biological behavior of DFSP and DFSP-FS. METHODS: This was a retrospective cohort study of ambulatory patients with DFSP or DFSP-FS treated between January 1955 and March 2012 in the dermatology department of a tertiary care academic medical center. RESULTS: Of 188 patients, 171 (91%) had DFSP and 17 (9%) had DFSP-FS. Recurrence-free survival differed significantly between the groups over time (P = .002). The 1-year and 5-year recurrence-free survival was 94% and 86%, respectively, for DFSP, vs 86% and 42%, respectively, for DFSP-FS. Metastatic disease occurred in no patients with DFSP and in 18% (3 of 17) with DFSP-FS (P < .001). There were no statistically significant differences in age at diagnosis, sex, race, symptomatology, maximum tumor size, muscle/bone invasion, or duration of tumor before diagnosis. LIMITATIONS: The retrospective nature of study was a limitation. CONCLUSIONS: DFSP-FS exhibits more aggressive behavior than DFSP, with lower recurrence-free survival and greater metastatic potential. Their similar clinical presentation mandates histopathological differentiation for prognosis.

Leprosy as a Diagnostic Challenge in the United States.

A 63-year-old woman from Central Florida presented to an outside clinic with a 2-year history of a progressive, asymptomatic cutaneous eruption and arthralgias. Her past medical history was significant for reported seronegative rheumatoid arthritis, for which adalimumab, methotrexate, and low-dose prednisone therapy were initiated 5 years prior. The skin eruption occurred shortly after a 4-week hospitalization during which these medications were withheld. At her initial outside evaluation, a biopsy was performed and interpreted as subacute cutaneous lupus erythematosus (SCLE). She was treated with hydroxychloroquine without improvement. A repeat biopsy was reported as consistent with interstitial granulomatous dermatitis (IGD). There was no improvement with potent topical corticosteroids.

Evaluating the Efficacy, Safety, and Tolerability of the Combination of Tazarotene, Azelaic Acid, Tacrolimus, and Zinc Oxide for the Treatment of Melasma: A Pilot Study.

Background: Melasma is a common hyperpigmentation disorder of the skin. Combination therapy of topical retinoids, corticosteroids, and hydroquinone has been effective in treating melasma, but long-term use is limited by corticosteroid atrophy and exogenous ochronosis. The aim of this pilot study (NCT02730819) was to determine the efficacy, safety, and tolerability of a novel composition (2013-MCN-333) comprising tazarotene 0.075%, azelaic acid 20%, tacrolimus 0.1%, and (microfine) zinc oxide 10% for the treatment of melasma. Methods: Sixteen patients with moderate-to-severe melasma were treated daily with sunscreen and 2013-MCN-333 for 20 weeks. Primary outcome measure was change in Melasma Area and Severity Index (MASI) score. Results: Twenty-five percent of patients met the primary endpoint of a MASI score of less than eight points at Week 20. MASI score also decreased significantly from baseline (median: 18.9 points) through Week 4 (median: 17.3 points; p=0.006), Week 12 (median: 16.0 points; p=0.001), and Week 20 (median: 13.3 points; p=0.001). Treatment-related adverse events were mild, most of which decreased or resolved over the course of the study. Limitations: The small sample size and nonblinded nature of treatment intervention are potential limitations. Conclusion: Our results suggest daily 2013-MCN-333 could potentially be an effective, safe, and tolerable treatment for moderate-to-severe melasma.