Cutaneous Manifestations of Familial Transthyretin Amyloid Polyneuropathy.

Familial amyloid polyneuropathy (FAP) is a rare inherited autosomal dominant form of systemic amyloidosis, which classically presents with severe motor, sensory, and autonomic dysfunction. Cutaneous involvement does not become clinically apparent until late stage symptomatic disease and is rarely reported in modern literature. Here, the authors review the clinical and histologic cutaneous findings of FAP previously described in the literature and report on 3 patients with unique genetic mutations (Thr60Ala and Gly6Ser; Trp41Leu; Glu89Gln) for which cutaneous involvement has not previously been described. Histologically, our patients showed variable amyloid deposition in the subcutaneous adipose tissue, papillary dermis, and dermal blood vessel walls. A review of the literature suggests cutaneous transthyretin deposition is an underrecognized feature of FAP that occurs early on in disease, even before neural involvement and related symptoms as seen in one of our patients. As such, a cutaneous punch biopsy can serve as quick, easy, and relatively noninvasive diagnostic tool in suspected cases.

The Use of Anti-Keratin 903 Antibodies to Visualize Colloid Bodies and Diagnose Lichen Planopilaris.

Cytokeratins are a major component of colloid bodies that are essentially diagnostic of lichen planopilaris (LPP). Here, the authors assess the ability of the cytokeratin 903 antibody (CK-903) to stain colloid bodies and differentiate LPP from other histologically similar appearing primary cicatricial alopecias. A retrospective review of all specimens submitted to the dermatopathology department over a 2-year window identified 18 cases of LPP and 20 cases of histologically similar appearing entities (discoid lupus erythematosus or central centrifugal cicatricial alopecia) through a combination of H&E, elastic van gieson, and periodic acid-schiff stains. All 38 samples were then prospectively stained with CK-903. Colloid bodies were identifiable in 3 of the 18 LPP cases based on H&E alone but were seen in 9 of 18 cases when CK-903 was used. There were no cases where colloid bodies were seen on H&E but not subsequently identified with CK-903. Additionally, there was no CK-903 staining in any of the 20 cases of similar appearing entities except 1 case of discoid lupus erythematosus, which is known to occasionally show colloid bodies. The authors conclude that CK-903 is a useful adjunctive tool that will allow for a quicker, less costly, and more accurate diagnosis of LPP given its ability identify colloid bodies even in the setting of significant inflammation and fibrosis and its advantages over direct immunofluorescence of low cost, short preparation time, and lack of need for a specialized fluorescent microscope.

Porokeratotic Adnexal Ostial Nevus-Report of a Case With Unusual Clinical and Histologic Features.

An 11-year-old Tanzanian girl presented with diffuse verrucous lesions of varying morphology, scarring alopecia, and keloid scars over the face with a predilection for the ears. Physical examination revealed dark keratoderma and patches of hypopigmentation near the midline of the dorsal trunk (Figure 1a). Her forearms were densely covered by verrucous lesions with the exception of a clear linear patch on the dorsal aspect of the left forearm (Figure 1b). The perioral area was notable for white spires projecting from verrucous papules (Figure 1c) while the oral mucosa and teeth appeared normal on visual examination. The rest of her body, including the palms and soles, was covered by patchy, scaly lesions of varying severity.

Evaluating Dermatology Residency Program Websites.

BACKGROUND: Internet resources play an important role in how medical students access information related to residency programs.Evaluating program websites is necessary in order to provide accurate information for applicants and provide information regarding areas of website improvement for programs. To date, dermatology residency websites (D  WS) have not been evaluated.This paper evaluates dermatology residency websites based on availability of predefined measures. METHODS: Using the FREIDA (Fellowship and Residency Electronic Interactive Database) Online database, authors searched forall accredited dermatology program websites. Eligible programs were identified through the FREIDA Online database and had a functioning website. Two authors independently extracted data with consensus or third researcher resolution of differences. This data was accessed and archived from July 15th to July 17th, 2015.Primary outcomes measured were presence of content on education, resident and faculty information, program environment, applicant recruitment, schedule, salary, and website quality evaluated using an online tool (WooRank.com). RESULTS: Out of 117 accredited dermatology residencies, 115 had functioning webpages. Of these, 76.5% (75) had direct links found on the FRIEDA Online database. Most programs contained information on education, faculty, program environment, and applicant recruitment. However, website quality and marketing effectiveness were highly variable; most programs were deemed to need improvements in the functioning of their webpages. Also, additional information on current residents and about potential away rotations were lacking from most websites with only 52.2% (60) and 41.7% (48) of programs providing this content, respectively. CONCLUSIONS: A majority of dermatology residency websites contained adequate information on many of the factors we evaluated. However, many were lacking in areas that matter to applicants. We hope this report will encourage dermatology residencyprograms to improve their websites and provide adequate content to attract the top residents for their respective programs.

Histopathology of Measles: Report of 2 Cases With New Findings.

The authors report 2 cases of measles demonstrating novel skin pathology that may be useful in establishing early diagnosis. Syncytial epithelial giant cells, which are characteristic of measles, were found to be present in the dermis, indicating that these cells are not specific to the lymphoid tissue and epithelia of which they are classically attributed to. The cells were not prominent, and required step sectioning to observe. These results were confirmed by electron microscopy, which showed virus capsid particles within the endoplasmic reticulum, secretory vesicles, and cytoplasm of multinucleated cells. One of the cases also demonstrated an unusual mixed infiltrate of eosinophils and fibrin thrombi, which has not been previously described. Both patients in this report recovered with supportive therapy.

Therapies to improve the cosmetic symptoms of rosacea.

Rosacea is a commonly encountered chronic inflammatory skin disease with a predilection for highly visible areas of the skin such as the face. The cosmetic symptoms of rosacea can be substantial and may greatly reduce a patient's quality of life. Although there is no definitive cure for rosacea, effective treatment of symptoms can mitigate the deleterious effects of this condition and improve quality of life. In this article, we review both existing and emerging cosmetic treatments for rosacea, including topical medications, systemic pharmacologic therapies, light-based modalities, and procedural interventions, and assess their ability to improve the cosmetic symptoms of rosacea.

Acne scarring: a review of cosmetic therapies.

Acne vulgaris is one of the most commonly encountered skin conditions and frequently is seen in both adolescent and adult populations. Scarring is a common result of acne and may take the form of atrophic or hypertrophic scars. Acne scarring often occurs in highly visible areas such as the face, thus resulting not only in an un-desirable cosmetic appearance but also potential impairment of mental health, social functioning, and overall well-being. There is a wide variety of medical and surgical therapies available for treatment of acne scarring. In this article, we review some of the most commonly used cosmetic therapies for acne scarring, including dermabrasion, laser resurfacing, radiofrequency (RF), subcision, skin needling, punch techniques, chemical peels, soft-tissue augmentation, intralesional therapy, cryotherapy, and silicone dressings, with a focus on cosmetic outcomes.

Long-term safety of left renal vein division and ligation to expedite complex abdominal aortic surgery.

BACKGROUND: Left renal vein division and ligation (LRVDAL) is performed to facilitate complex abdominal aortic surgery. Surgeons restore continuity of the vein due to concern that ligation could cause renal compromise or hematuria. However, we report the short and long-term safety of left renal vein division and ligation. METHOD: Between 1992 and 2007, we divided the left renal vein in 56 patients (40 males, 16 females) ages 57 to 84 (average 74-years-old) who were treated for aortic occlusive disease (9) or abdominal aortic aneurysm (47). Patients requiring concomitant renal artery reconstruction were excluded from this review. Suprarenal cross-clamp was used in 51 patients with temporary vessel-loop control of the renal arteries. Creatinine (Cr) and glomerular filtration rates (eGFR) were measured pre-, post-, and long-term after surgery. Outpatient records of all patients that had survived more than 12 months were also reviewed in order to evaluate the late effects on renal function or symptoms possibly related to LRVDAL. RESULTS: Median procedure duration was 157 (61-375) minutes. Median cross-clamp time was 16 (10-45) minutes. Median intensive care unit (ICU) and hospital length of stays were 2 (1-11) days and 7 (4-58) days, respectively. There were no deaths. There were no complications directly related to renal vein ligation. Hematuria, seen in 2 patients, was a result of traumatic insertion of a Foley catheter. Median pre-op and discharge Cr levels were 1.1 mg/dL (0.7-2.4 mg/dL) and 1.1 mg/dL (0.6-2.1 mg/dL), respectively (P < .5). Median change in Cr was 0.0 mg/dL and only increased in 14 patients (maximum increase 0.9 mg/dL). Median pre-op and discharge eGFR was 61 mL/minute (28-137 mL/minute/1.73 m2) and 67 mL/minute (32-138 mL/minute/1.73 m2), respectively (P < .5). Cr and eGFR in the 2 patients with a Cr of >2.0 mg/dL remained unchanged post-op. Only 2 patients with a Cr of <2.0 mg/dL had a post-op Cr >2.0 mg/dL and both returned to normal by day 3 post-op. Thirty-six patients have been followed for more than a year (median 34.5 months, maximum 144 months) and Cr has remained stable in all but 2 patients. These 2 patients, both with a pre-op Cr of 1.5 mg/dL, subsequently developed Cr levels of 2.1 mg/dL and 2.4 mg/dL but maintained baseline Cr levels for 25 and 34 months, respectively, before demonstrating these elevated levels which have proven to be unrelated to renal vein ligation. Hematuria and flank pain have never been recorded after discharge. CONCLUSION: Restoration of left renal vein continuity after LRVDAL may be unnecessary since renal compromise and hematuria was not encountered in this long-term analysis.

A Practical Approach to Chemical Peels: A Review of Fundamentals and Step-by-step Algorithmic Protocol for Treatment.

Background: Chemoexfoliation, also known as chemical peeling, is a method of targeted cutaneous ablation using specific caustic agents that allow for rapid, predictable, and uniform thickness of chemoablation to a desired cutaneous depth, ultimately resulting in an improved appearance of skin. Objective: In this review, we provide an up-to-date analysis of all currently available chemical peels for dermatologic use, as well as a step-by-step instructional protocol for an algorithmic approach to treatment. Methods: A comprehensive search of the Cochrane Library, MEDLINE, and PUBMED databases was performed to identify relevant literature investigating chemical peeling agents. In addition, a search of all commercially available, prescription-based peeling agents was performed to identify all products currently available in the United States market. Results and Conclusion: Chemical peels are the third most commonly performed noninvasive cosmetic procedure in the United States, with over 1,300,000 procedures performed in 2016 alone. There has been a paradigm shift in recent years, with lasers largely supplanting deep peels. Despite this shift, superficial peels have proliferated in both popularity and product diversity. When used for the appropriate indication and with proper technique, nearly all peeling agents have demonstrated excellent clinical efficacy and remain an indispensable cost-effective tool in the dermatologist's aesthetic toolbox.

Basal Cell Carcinoma: A Comprehensive Review of Existing and Emerging Nonsurgical Therapies.

Basal cell carcinoma is the most commonly occurring cancer in the world and overall incidence is still on the rise. While typically a slow-growing tumor for which metastases is rare, basal cell carcinoma can be locally destructive and disfiguring. Given the vast prevalence of this disease, there is a significant overall burden on patient well-being and quality of life. The current mainstay of basal cell carcinoma treatment involves surgical modalities, such as electrodessication and curettage, excision, cryosurgery, and Mohs micrographic surgery. Such methods are typically reserved for localized basal cell carcinoma and offer high five-year cure rates, but come with the risk of functional impairment, disfigurement, and scarring. Here, the authors review the evidence and indications for nonsurgical treatment modalities in cases where surgery is impractical, contraindicated, or simply not desired by the patient.

Actinic keratosis as a marker of field cancerization in excision specimens of cutaneous malignancies.

Field cancerization is the process in which a singular cell accumulates genetic mutations following carcinogen exposure and then divides to create a "field" of monoclonal premalignant cells. In this study, microscopically identified actinic keratoses (AKs) were used as markers of field cancerization in all excision specimens of squamous cell carcinomas (SCCs), basal cell carcinomas (BCCs), and malignant melanomas (MMs) received by our institution's dermatopathology department over a 3- to 6-month period. Our findings provide additional evidence for the theory of field cancerization, its association with cutaneous malignancies, and the need to assess the extent of field damage when determining treatment strategies.

Enlarged facial pores: an update on treatments.

Enlarged facial pores remain a common dermatologic and cosmetic concern from acne and rosacea, among other conditions, that is difficult to treat due to the multifactorial nature of their pathogenesis and negative impact on patients' quality of life. Enlarged facial pores are primarily treated through addressing associative factors, such as increased sebum production and cutaneous aging. We review the current treatment modalities for enlarged or dense facial pores, including topical retinoids, chemical peels, oral antiandrogens, and lasers and devices, with a focus on newer therapies.

New Oral Therapies for Psoriasis: A Comprehensive Review.

Conventional oral therapies for psoriasis, including methotrexate, cyclosporine, and acitretin, have generally unfavorable safety profiles and are not ideal for long-standing use. Thus, new oral therapies are necessary for patients with more moderate disease, patients who prefer oral treatments to injectable biologies, and patients who failed conventional therapies. The authors review here the clinical and safety evidence of phosphodiesterase 4 inhibitor, apremilast, janus kinase inhibitors, including tofacitinib, and fumarie acid esters as additional options in oral psoriasis therapy.

An update on neurotoxin products and administration methods.

Since onabotulinumtoxinA for nonsurgical aesthetic enhancement of glabellar lines was initially reported, the popularity of botulinum neurotoxin (BoNT) products among both clinicians and consumers has rapidly grown, and we have seen several additional BoNT formulations enter the market. As the demand for minimally invasive cosmetic procedures continues to increase, we will see the introduction of additional formulations of BoNT products as well as new delivery devices and administration techniques. In this article, we provide a brief update on current and upcoming BoNT products and also review the literature on novel administration methods based on recently published studies.

Therapies for actinic keratosis with a focus on cosmetic outcomes.

Actinic keratosis (AK) is a commonly encountered premalignant epidermal lesion that has a predilection to manifest on highly visible areas such as the face, head, and hands. Lesions may be cosmetically unappealing and have been reported to reduce patients' quality of life (QOL), but appropriate treatment can resolve these issues. In this article, we review the efficacy of the most commonly utilized treatments for AKs including topical medications, procedural modalities, and light-based therapies, and we discuss the relevant cosmetic considerations and outcomes.