Cystic fibrosis dermatitis arthritis syndrome: A series of four cases.

Dermatologic manifestations of cystic fibrosis (CF) include nutrient deficiency dermatoses, vasculitis, transient reactive papulotranslucent acrokeratodema, digital clubbing, and increased rates of atopy and drug reactions. Few cases of a characteristic eruption in patients with episodic arthritis of CF have been described with prior reports primarily occurring outside of the dermatology literature. We report four cases consistent with this presentation to add to the literature and propose a new and unifying name to recognize this entity as cystic fibrosis dermatitis arthritis syndrome (CF-DAS). Clinical suspicion should remain high in young female patients with cystic fibrosis presenting with episodic joint pain and rash, independent of pulmonary exacerbations.

Cutaneous manifestations of cystic fibrosis.

Cystic fibrosis (CF) is caused by a mutation in the Cystic fibrosis transmembrane conductance regulator (CFTR) gene, and features recurrent sinus and pulmonary infections, steatorrhea, and malnutrition. CF is associated with diverse cutaneous manifestations, including transient reactive papulotranslucent acrokeratoderma of the palms, nutrient deficiency dermatoses, and vasculitis. Rarely these are presenting symptoms of CF, prior to pulmonary or gastrointestinal sequelae. Cutaneous drug eruptions are also highly common in patients with CF (PwCF) given frequent antibiotic exposure. Finally, CFTR modulating therapy, which has revolutionized CF management, is associated with cutaneous side effects ranging from acute urticaria to toxic epidermal necrolysis. Recognition of dermatologic clinical manifestations of CF is important to appropriately care for PwCF. Dermatologists may play a significant role in the diagnosis and management of CF and associated skin complications.

Cutaneous manifestations of sickle cell disease: an updated review.

Sickle cell disease is a common and highly morbid genetic condition that is characterized by multi-system involvement, including numerous cutaneous manifestations. Persistent and recurrent leg ulceration has long been considered the dermatological hallmark of those with sickle cell disease, but there have been recent descriptions of associated infection with rare pathogens and episodes of liverdoid vasculopathy. Growing considerations for this population in the literature include cutaneous reactions to common treatments such as hydroxyurea and clinical management of the intersection between sickle cell disease and conditions like plaque psoriasis. Recent studies have also demonstrated an increased resistance to skin carcinogenesis for those with sickle cell disease through unclear mechanisms. However, though the body of knowledge regarding cutaneous manifestations and considerations of sickle cell disease is slowly expanding, it does not match the considerable disease and symptom burden faced by these patients. More research is needed to better delineate our understanding of these cutaneous manifestations of sickle cell disease to improve outcomes and further management.

Review and reappraisal of assessment parameters of second intention healing after Mohs micrographic surgery.

Mohs micrographic surgery (MMS) is used to remove cutaneous tumors in cosmetically sensitive anatomic areas. Surgeons can provide several reconstructive options to patients following tumor removal, including primary closure, skin grafts, local, regional or free flaps, and secondary intention healing (SIH). Notably, with the advancement of surgical and reconstructive techniques, the use of second intention healing has declined and may be underutilized. This review aims to critically evaluate the literature regarding indications, anatomical considerations, advantages, and complications of second intention wound healing following Mohs surgery. We also offer reappraisal of SIH following MMS to promote evidence-based postoperative care.

Surgical Site Infection After Dermatologic Procedures: Critical Reassessment of Risk Factors and Reappraisal of Rates and Causes.

While rates are low, surgical site infections are the most common complication of dermatologic surgery. Surgical site infections have important consequences including impairment of wound healing, suboptimal cosmetic outcome, hospitalization, increased healthcare costs, and rarely, systemic infection. It is imperative to understand the risk factors and existing preventative measures to minimize the development of infection. This article reviews the available literature regarding surgical site infections following dermatologic procedures, to evaluate the standard of diagnosis and role of wound culture, risk factors, mimicking conditions, and significance of antibiotic prophylaxis. We offer a critical reassessment of the current literature on risk factors and reappraisal of infection rates to promote evidence-based patient care. We conclude that the strongest evidence suggests that diabetes mellitus is likely associated with increased surgical site infections. Immunosuppression is often clinically considered a risk factor; however, the evidence is mixed. In general the addition of antibiotics does not confer benefit except in high-risk sites. Conclusively, Mohs micrographic surgery has been proven safe in office and inpatient settings. We agree that sterile glove use for simple procedures is likely not a significant factor in the development of surgical site infections; however, we hypothesize that the overall sterile technique and setting may play a role in longer and/or more complex procedures.

Beyond Mohs surgery and excisions: A focused review of treatment options for subtypes of basal cell carcinoma.

Basal cell carcinoma (BCC) is the most common nonmelanoma skin cancer. It originates from undifferentiated cells in the basal cell layer of the epidermis or from the outer root sheath of the hair follicle. The most important factor in development of BCC is ultraviolet radiation. Surgery is considered the gold standard of treatment for BCC. However, nonsurgical options are available for individuals who are unsuitable for surgery. The purpose of this review is to summarize the efficacy and indications of alternative, nonsurgical treatments that can be used in the management of BCC. An extensive literature review was performed for the nonsurgical options for the treatment of BCC. Resources searched included PubMed and Google Scholars, limited to the years 1995 to 2020. Key words searched included BCC, destructive methods, photodynamic therapy (PDT), radiotherapy, topical medication, laser, hedgehog pathway inhibitors (HPIs). The most relevant results such as systematic reviews, randomized controlled trials, or comparative studies were selected to provide a summary for the most common nonsurgical methods used for treating BCC. Effective nonsurgical treatments for BCC include destructive methods (eg, curettage alone, cryosurgery, or electrodessication), PDT, topical medications, radiotherapy, laser, and HPIs. Nonsurgical therapeutic alternatives are safe and effective for the treatment of BCC. Factors such as tumor location, size, and histopathological subtype should be taken into consideration when selecting optimal treatment. In addition to clinical factors, cosmetic results and patient preference should be considered.

Anti-PD1 immune checkpoint inhibitor-induced bullous pemphigoid in metastatic melanoma and non-small cell lung cancer.

Anti-programmed cell death 1 (PD1) targeted immune checkpoint inhibitors such as nivolumab and pembrolizumab are increasingly used to treat advanced malignancies such as melanoma, non-small cell lung cancer, urothelial cancer, and renal cell carcinoma. A rare but increasingly reported adverse effect of anti-PD1 therapy is bullous pemphigoid (BP), an autoimmune blistering disease directed against BP antigen 1 and BP antigen 2 in the basement membrane of the epidermis. We present 3 cases of BP secondary to anti-PD1 immunotherapy in patients with melanoma and non-small cell lung cancer to highlight the diagnosis and treatment of this condition and emphasize the importance of the dermatologist in the care of patients with immunotherapy-related skin disease.