Anaphylaxis.

Anaphylaxis is a systemic, life-threatening disorder triggered by mediators released by mast cells and basophils activated via allergic (IgE-mediated) or nonallergic (non-IgE-mediated) mechanisms. It is a rapidly evolving, multisystem process involving the integumentary, pulmonary, gastrointestinal, and cardiovascular systems. Anaphylaxis and angioedema are serious disorders that can lead to fatal airway obstruction and culminate in cardiorespiratory arrest, resulting in hypoxemia and/or shock. Often, these disorders can be appropriately managed in an outpatient setting; however, these conditions can be severe enough to warrant evaluation of the patient in the ED and in some cases, hospitalization, and management in an ICU. Reports suggest that underdiagnosis and undertreatment of anaphylaxis are common. Several new syndromes have been described recently including bird-egg, pork-cat, delayed allergy to mammalian meat and a diverse group of mast cell activation disorders. Conditions such as postural orthostatic tachycardia syndrome, carcinoid syndrome, Munchausen stridor, and factitious anaphylaxis can present similarly and need to be included in the differential diagnosis. Anaphylaxis is a clinical diagnosis, but plasma tryptase and urinary histamine levels are often elevated, allowing diagnostic confirmation; however, diagnostic testing should not delay treatment as results may not be immediately available. The sine qua non of treatment is avoidance of any known triggers and epinephrine, which should never be delayed if this disorder is suspected. Secondary treatments include fluids, bronchodilators, antihistamines, and glucocorticoids. Patients with cardiopulmonary arrest or airway or vascular compromise require mechanical ventilation, vasopressors, and other advanced life support in the ICU.

Myxoid variant of primary cutaneous anaplastic large cell lymphoma: First 2 cases.

Anaplastic large cell lymphoma (ALCL) is a CD30+ T-cell non-Hodgkin lymphoma with 2 main clinical presentations: primary cutaneous ALCL (pcALCL) and systemic ALCL (sALCL). While rare cases of myxoid sALCL have been reported, there are no previous cases of myxoid pcALCL reported. We present 2 unusual cases of pcALCL showing prominent collections of dermal mucin closely intermingling with the anaplastic lymphocytes. Patient 1 was a 30-year-old woman who presented with ulcerated nodules on her neck, abdomen, chest and shoulders. A systemic lymphoma was excluded by physical examination, positron emission tomography and computed tomography (PET-CT) scan, as well as by bone marrow biopsy and flow cytometry studies. The patient was closely followed-up for 10 months without evidence of systemic involvement. The biopsy showed diffuse infiltration of the dermis by a CD2+, CD30+, anaplastic lymphoma kinase (ALK)-negative ALCL. Patient 2 was a 55-year-old woman who presented with a single nodule on her right arm. A systemic lymphoma was excluded by physical examination as well as by a PET-CT scan. The biopsy showed diffuse and dense lymphoid infiltration of the whole biopsy by a CD3+, CD4+, CD30+, ALK-negative ALCL. The atypical lymphocytes were intermingled with large amounts of dermal stromal mucin.

A Retrospective Review of Treatment Results for Patients With Central Centrifugal Cicatrical Alopecia.

INTRODUCTION: Central centrifugal cicatricial alopecia (CCCA) is a form of scarring alopecia primarily affecting women of African descent on the crown of the scalp. Limited data exists regarding evidence-based treatment for CCCA.

OBJECTIVE: To examine photos of subjects with CCCA before and after treatment in order to evaluate results of treatment and compare results of different treatment regimens.

METHODS: Photographs of 15 subjects with CCCA before and after treatment were evaluated by two blinded investigators who assigned disease severity scores to photographs based on a published scale: Central Scalp Alopecia Photographic Scale in African American Women.

RESUTLS: Median change in severity score (post-treatment severity score - pre-treatment severity score) was 0.5 (P = 0.58) for all 15 subjects receiving a series of 7 to 8 intralesional steroid injections along with topical steroids (Class I/II) +/- minoxidil and +/- anti-dandruff shampoo, indicating worsening of disease after treatment. Subjects receiving minoxidil versus those who did not (0.25 vs 0.5; P = 0.38) and subjects receiving anti-dandruff shampoo versus those who did not (0.0 vs 0.5; P = 0.42) demonstrated no statistically significant difference in pre- and post-treatment severity scores. Of 15 subjects, 5/15 (33.3%) had decreased severity scores, 8/15 (53.3%) had increased severity scores, and 2/15 (13.3%) had no change in severity scores.

CONCLUSIONS: Although no statistically significant difference was found in pre- versus post-treatment disease severity, this may indicate intralesional steroid injections and topical steroids +/- minoxidil and +/- anti-dandruff shampoo halt disease progression.

J Drugs Dermatol. 2017;16(4):317-320.

The role of spatially-derived access-to-care characteristics in melanoma prevention and control in Los Angeles county.

Among 10,068 incident cases of invasive melanoma, we examined the effects of patient characteristics and access-to-care on the risk of advanced melanoma. Access-to-care was defined in terms of census tract-level sociodemographics, health insurance, cost of dermatological services and appointment wait-times, clinic density and travel distance. Public health insurance and education level were the strongest predictors of advanced melanomas but were modified by race/ethnicity and poverty: Hispanic whites and high-poverty neighborhoods were worse off than non-Hispanic whites and low-poverty neighborhoods. Targeting high-risk, underserved Hispanics and high-poverty neighborhoods (easily identified from existing data) for early melanoma detection may be a cost-efficient strategy to reduce melanoma mortality.

Central Centrifugal Cicatricial Alopecia in Children: A Case Series and Review of the Literature.

BACKGROUND/OBJECTIVES: Central centrifugal cicatricial alopecia (CCCA) is a form of scarring hair loss most commonly seen in middle-aged African and African American women. It is rarely reported in children. The objective of the current study was to document the presence of CCCA in children and to encourage physicians to recognize early signs of CCCA in children of affected adults. METHODS: Six children presented with biopsy-proven CCCA to the Department of Dermatology at Wake Forest Baptist Health and the Nelson R. Mandela School of Medicine from 2012 to 2015. Demographic characteristics, hair styling history, and family history of CCCA were noted. Examination included complete history, skin and scalp examination, photographs of the scalp, and scalp biopsies. RESULTS: Patient ages ranged from 14 to 19 years (mean age at onset 14 years). Five patients reported scalp symptoms such as tender papules, pruritus, and scaling. Four patients reported appreciable hair loss on the vertex of the scalp. One patient had used chemical relaxers and hair dyes in the past. Five patients had a known family history of CCCA. CONCLUSION: Because CCCA is not typically seen or suspected in children, it is likely to be misdiagnosed or underreported. The findings in these cases add weight to the concept that genetic susceptibility rather than hair care practices may play a significant role in causing CCCA.

A potentially common peptide target in secreted heat shock protein-90α for hypoxia-inducible factor-1α-positive tumors.

Deregulated accumulation of hypoxia-inducible factor-1α (HIF-1α) is a hallmark of many solid tumors. Directly targeting HIF-1α for therapeutics is challenging. Our finding that HIF-1α regulates secretion of heat shock protein-90α (Hsp90α) for cell migration raises the exciting possibility that targeting the secreted Hsp90α from HIF-1α-positive tumors has a better clinical outlook. Using the HIF-1α-positive and metastatic breast cancer cells MDA-MB-231, we show that down-regulation of the deregulated HIF-1α blocks Hsp90α secretion and invasion of the cells. Reintroducing an active, but not an inactive, HIF-1α into endogenous HIF-1α-depleted cells rescues both Hsp90α secretion and invasion. Inhibition of Hsp90α secretion, neutralization of secreted Hsp90α action, or removal of the cell surface LRP-1 receptor for secreted Hsp90α reduces the tumor cell invasion in vitro and lung colonization and tumor formation in nude mice. Furthermore, we localized the tumor-promoting effect to a 115-amino acid region in secreted Hsp90α called F-5. Supplementation with F-5 is sufficient to bypass the blockade of HIF-1α depletion and resumes invasion by the tumor cells under serum-free conditions. Because normal cells do not secrete Hsp90α in the absence of stress, drugs that target F-5 should be more effective and less toxic in treatment of HIF-1α-positive tumors in humans.