Faculty Bibliography

Calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome is a form of a rare, clinical subtype of systemic sclerosis, known as limited systemic sclerosis. Limited systemic sclerosis, including CREST syndrome, manifests as fibrotic skin changes restricted to the hands and face, with vascular, musculoskeletal, and visceral involvement. We present a case of a 75-year-old woman with a longstanding history of CREST syndrome complicated by a digital ulceration and persistent pain associated with recalcitrant Raynaud phenomenon. After failing a number of first-line pharmacologic therapies such as diltiazem, sildenafil, and topical nitropaste, the patient was started on a trial of botulinum toxin for the left second digit, with 10 unit injections into both webspaces for a total of 20 units. Following injection, the patient reported no further baseline pain in the affected finger and an over fifty-percent improvement in discomfort with manipulation of the digit at a follow-up time of one week. The ulceration started healing within the following three weeks. This result was maintained at a follow-up time of six weeks.

BACKGROUND:Multiple placebo controlled trials have assessed locally applied topical nitrate preparations in treating Raynaud's phenomenon (RP). OBJECTIVES/OBJECTIVE:The objective of this meta-analysis was to assess the effects of local topical nitrates in primary and secondary RP with respect to a combined endpoint integrating parameters of digital blood flow and clinical severity. METHODS:A systematic review was performed using MEDLINE, Embase and the Cochrane library. Only trials comparing locally applied topical nitrates to placebo comparators were included. Studies were appraised for bias by two independent reviewers. RESULTS:Seven placebo controlled trials including 346 patients were used in meta-analysis. Four trials used nitroglycerin ointments, two used MQX-503 and one used compounded nitrite. Meta-analysis results support a moderate to large treatment effect in Raynaud's phenomenon, standardized mean difference (SMD)=0.70, (0.35 to 1.05, p<0.0001). Subgroup analyses showed a large treatment effect in secondary RP, SMD=0.95 (0.25 to 1.65, p=0.008) and moderate effect in primary RP, SMD=0.45 (0.05 to 0.85, p=0.03). LIMITATIONS/CONCLUSIONS:Limitations include the inclusion of multiple topical nitrate preparations and integration of different outcomes assessments. CONCLUSION/CONCLUSIONS:Local topical nitrates have significant efficacy in the treatment of both primary and secondary Raynaud's phenomenon.

Lichen myxedematosus is condition characterized by localized areas of dermal deposition of mucin, presenting with firm papules localized to few areas of the body. The condition needs to be excluded from scleromyxedema, which, in addition to the firm papular eruption, has areas of induration and is usually associated with a monoclonal gammopathyand systemic symptoms. We present a 62-year-old woman with a several-year history of asymptomatic, firm papules over the face and arms with no evidence of thyroid disease or a monoclonal gammopathy,which is consistent with a diagnosis of localized lichen myxedematosus, the discrete papular variant. The patient is being treated with a topical calcineurininhibitor.

Anetoderma is a rare benign elastolytic disorder that is characterized by focal loss of elastin fibers on histopathology and is often recalcitrant to treatment. We present a case of a patient with a 20-year history of pruritic and painful hyperpigmented atrophic papules clustered on the neck, axillae, inframammary folds, and right medial thigh. Although the histopathologyof her axillary lesions was consistent with anetoderma, her clinical presentation is unusual given the extent of involvement, reported pain and pruritus, and sharp demarcation of the distribution. The diagnosticuncertainty of this case led to added difficulty in management of a disease that is already notoriously difficult to treat and may significantly impact patient's quality of life.

New all-oral direct acting antivirals (DAA) have changed the hepatitis C virus (HCV) treatment landscape. Given that dermatologists frequently encounter HCV-infected patients, knowledge of the current treatment options and their utility in treating HCV-associated dermatologic disorders is important. In addition to highlighting the new treatment options, we review four classically HCV-associated dermatologic disorders - mixed cryoglobulinemia (MC), lichen planus (LP), porphyria cutanea tarda (PCT), and necrolytic acral erythema (NAE) - and examine the role for all-oral direct acting antiviral (DAA) regimens in their treatment. A literature search of English-language publications was conducted of the PubMed and EMBASE databases using search terms including 'hepatitis C,' 'direct acting antivirals,' 'cutaneous,' 'mixed cryoglobulinemia,' 'necrolytic acral erythema,' 'lichen planus,' 'porphyria cutanea tarda,' 'rash,' as well as specific drug names, related terms, and abbreviations. Currently, limited data exists on the use of DAAs in HCV patients with cutaneous side effects, although treatment of the underlying HCV is now recommended for nearly all patients, with the new drugs offering much-improved dosage schedules and side effect profiles. The most data exists for MC, in which several studies suggest that DAAs and achievement of sustained virologic response (SVR) improves cutaneous symptoms. Studies of both older and newer regimens are limited by their small size, retrospective nature, lack of appropriate controls, and wide variability in study protocols. Given the strong association, screening for HCV should be considered in patients with MC, LP, PCT, and NAE