William D. James

AAD/ACMS/ASDSA/ASMS 2012 appropriate use criteria for Mohs micrographic surgery: A report of the American Academy of Dermatology, American College of Mohs Surgery, American Society for Dermatologic Surgery Association, and the American Society for Mohs Surgery

The appropriate use criteria process synthesizes evidence-based medicine, clinical practice experience, and expert judgment. The American Academy of Dermatology in collaboration with the American College of Mohs Surgery, the American Society for Dermatologic Surgery Association, and the American Society for Mohs Surgery has developed appropriate use criteria for 270 scenarios for which Mohs micrographic surgery (MMS) is frequently considered based on tumor and patient characteristics. This document reflects the rating of appropriateness of MMS for each of these clinical scenarios by a ratings panel in a process based on the appropriateness method developed by the RAND Corp (Santa Monica, CA)/University of California-Los Angeles (RAND/UCLA). At the conclusion of the rating process, consensus was reached for all 270 (100%) scenarios by the Ratings Panel, with 200 (74.07%) deemed as appropriate, 24 (8.89%) as uncertain, and 46 (17.04%) as inappropriate. For the 69 basal cell carcinoma scenarios, 53 were deemed appropriate, 6 uncertain, and 10 inappropriate. For the 143 squamous cell carcinoma scenarios, 102 were deemed appropriate, 7 uncertain, and 34 inappropriate. For the 12 lentigo maligna and melanoma in situ scenarios, 10 were deemed appropriate, 2 uncertain, and 0 inappropriate. For the 46 rare cutaneous malignancies scenarios, 35 were deemed appropriate, 9 uncertain, and 2 inappropriate. These appropriate use criteria have the potential to impact health care delivery, reimbursement policy, and physician decision making on patient selection for MMS, and aim to optimize the use of MMS for scenarios in which the expected clinical benefit is anticipated to be the greatest. In addition, recognition of those scenarios rated as uncertain facilitates an understanding of areas that would benefit from further research. Each clinical scenario identified in this document is crafted for the average patient and not the exception. Thus, the ultimate decision regarding the appropriateness of MMS should be determined by the expertise and clinical experience of the physician.

Colony-stimulating factors

Recombinant hematopoietic colony-stimulating factors have profound effects on developing and mature granulocytes, macrophages, and lymphocytes. Use of these agents for treatment of disease may result in a variety of adverse cutaneous reactions. The recent discovery of colony-stimulating factor production by keratinocytes and dermal cells suggests that these agents may also be significant in cutaneous homeostasis and in the pathogenesis of cutaneous diseases.

Rothmund-Thomson syndrome: Review of the world literature

Rothmund-Thomson syndrome is a rare, inherited disorder characterized by poikilodermatous skin changes that appear in infancy. The inheritance is autosomal recessive. Patients exhibit variable features including skeletal abnormalities, juvenile cataracts, and a higher than expected incidence of malignancy. This extensive review of the world literature clarifies aspects of the inheritance, incidence of characteristic features, and malignant potential of this disease. Insight into its origin is provided through a review of the clinical and in vitro studies of endocrine function and possible DNA repair abnormalities.

Microbiology of the skin: resident flora, ecology, infection.

Humans exist in an environment replete with microorganisms, but only a few become resident on the skin surface. The skin possesses protective mechanisms to limit colonization, and the survival of organisms on the surface lies in part in the ability of the organisms to resist these mechanisms. Microbial colonization on the skin adds to the skins defense against potentially pathogenic organisms. Although microbes normally live in synergy with their hosts, occasionally colonization can result in clinical infection. Common infections consist of superficial infections of the stratum corneum or appendageal structures that can respond dramatically to therapy but commonly relapse. In rare circumstances, these infections can be quite severe, particularly in immunocompromised patients or in hospitalized patients with indwelling foreign devices. These infections are often resistant to conventional antibiotics and can result in infection with other opportunistic pathogens.

Birt-Hogg-Dubé syndrome: A review of the literature and the differential diagnosis of firm facial papules

Birt-Hogg-Dubé syndrome (BHDS) was originally described in 1977 as the grouping of 3 skin tumors-the fibrofolliculoma, trichodiscoma, and acrochordon-in family members with an autosomal dominant inheritance pattern. In recent years it has become clear that these 3 lesions likely represent only 1 of these tumors, the fibrofolliculoma. More important, evidence now supports a definite susceptibility to malignant renal tumors and pulmonary disease in patients with BHDS. Clinical recognition of this entity is possible in spite of the fact that several syndromes exist that are characterized by the presence of multiple firm facial papules. We will discuss the evolution of BHDS from the original description to the recent discovery of the genetic susceptibility locus, illustrate the clinical differential diagnosis, and highlight the workup needed for newly diagnosed patients and their family members.

Tumor necrosis factor

Tumor necrosis factor is important in systemic and cutaneous defense, homeostasis, and many disease states. The numerous and diverse effects of tumor necrosis factor are best understood when considered as concentration-dependent, with normal homeostasis progressing to defense followed by toxic effects. Understanding tumor necrosis factor is important for the dermatologist as more studies appear in our literature and potential clinical uses of tumor necrosis factor (and possible anti-tumor necrosis factor agents) are realized.

Superficial thrombophlebitis II. Secondary hypercoagulable states

Secondary hypercoagulable states are complex clinical conditions associated with an increased risk of thrombosis in which the exact pathophysiology is poorly understood. Secondary causes of superficial thrombophlebitis include malignancy, pregnancy, use of oral contraceptives, infusion of prothrombin complex concentrates, Behçets disease, Buergers disease, Mondors disease, infectious agents, conditions that promote venous stasis, intravenous catheters and intravenous drug use. Conditions that may stimulate superficial thrombophlebitis include dermatophyte cellulitis at saphenous phlebectomy sites, sarcoidal granulomas, cutaneous polyarteritis nodosa, and hyperalgesic pseudothrombophlebitis in patients who test positive for human immunodeficiency virus. The distinguishing features, clinical evaluation, treatment, and histologic characteristics of the various disorders are reviewed.

Inhibition of human immunodeficiency virus replication in cell culture by endogenously synthesized antisense RNA

Antisense RNA, which has a sequence complementary to mRNA, may provide the basis for antiviral therapies of high selectivity. We have explored the inhibitory effect of six antisense RNAs upon the replication of human immunodeficiency virus (HIV) in cell culture. We chose regions of the HIV genome to test whether sequences required for splicing or for translation initiation were more susceptible to antisense RNA interference. Our results suggest that inhibitory antisense RNAs contain sequences complementary to the AUG initiation codon of the tat gene and have a comparatively low tendency to form intramolecular base pairs which would interfere with intermolecular duplex formation. Inhibition can be substantial (over 70%) but is transient. Transience does not result from mutation of the input virus. Inhibition was not a consequence of the induction of interferon by antisense RNA-mRNA duplex formation. Our results suggest that at least part of the inhibitory effect is at the posttranscriptional level.

Pyroxidine for the Palmar-Plantar Erythrodysesthesia Syndrome

Excerpt To the Editor:Reported cutaneous reactions associated with 5-fluorouracil seem to depend on dose and schedule (1). The palmar-plantar erythrodysesthesia syndrome (PPES) was initially descri...

Cutaneous and ocular manifestations of Down syndrome

A patient with Down syndrome and extensive elastosis perforans serpiginosa is presented. There is an increased incidence of elastosis perforans serpiginosa, alopecia areata, syringomas, and Norwegian scabies in patients with this syndrome. Ocular findings include slanting of the palpebral fissures, Brushfields spots, nystagmus, and epicanthal folds. Serious ocular complications that may impair vision include keratoconus, congenital cataracts, and retinal detachment.