Journal of the American Academy of Dermatology
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J. Am. Acad. Dermatol. · Dec 2018
ReviewNeutrophilic dermatoses: Pathogenesis, Sweet syndrome, neutrophilic eccrine hidradenitis, and Behçet disease.
Neutrophilic dermatoses are a heterogeneous group of inflammatory skin disorders that present with unique clinical features but are unified by the presence of a sterile, predominantly neutrophilic infiltrate on histopathology. The morphology of cutaneous lesions associated with these disorders is heterogeneous, which renders diagnosis challenging. Moreover, a thorough evaluation is required to exclude diseases that mimic these disorders and to diagnose potential associated infectious, inflammatory, and neoplastic processes. ⋯ Delays in diagnosis and treatment can lead to significant patient morbidity and even mortality. Therapeutic modalities range from systemic corticosteroids to novel biologic agents, and the treatment literature is rapidly expanding. The first article in this continuing medical education series explores the pathogenesis of neutrophilic dermatoses and reviews the epidemiology, clinical and histopathologic features, diagnosis, and management of Sweet syndrome, neutrophilic eccrine hidradenitis, and Behçet disease.
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J. Am. Acad. Dermatol. · Jun 2018
Editorial ReviewDermatology and its unique diagnostic heuristics.
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J. Am. Acad. Dermatol. · Apr 2018
ReviewRe-examining mechanic's hands as a characteristic skin finding in dermatomyositis.
Mechanic's hands is a poorly defined clinical finding that has been reported in a variety of rheumatologic diseases. Morphologic descriptions include hyperkeratosis on the sides of the digits that sometimes extends to the distal tips, diffuse palmar scale, and (more recently observed) linear discrete scaly papules in a similar lateral distribution. ⋯ In this review, most studies have shown that mechanic's hands is commonly associated with dermatomyositis and displays histopathologic findings of interface dermatitis, colloid bodies, and interstitial mucin, which are consistent with a cutaneous connective tissue disease. A more specific definition of this entity would help to determine its usefulness in classifying and clinically identifying patients with dermatomyositis, with implications related to subsequent screening for associated comorbidities in this setting.
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J. Am. Acad. Dermatol. · Mar 2018
ReviewLangerhans cell histiocytosis: A neoplastic disorder driven by Ras-ERK pathway mutations.
Langerhans cell histiocytosis (LCH) is a disorder of myeloid neoplasia of dendritic cells that affects 1 in 200,000 children <15 years of age and even fewer adults. LCH presents with a spectrum of clinical manifestations. High-risk stratification is reserved for infiltration of blood, spleen, liver, and lungs. ⋯ Increased levels of phospho-ERK in lesional tissue, activation of Ras/Raf/MEK/ERK signaling with these mutations in vitro, and the mutual exclusivity of these mutations in a given patient suggest a central role for activation of the Ras/Raf/MEK/ERK oncogenic pathway in LCH. Immunohistochemical assessment of lesional tissue using the VE1 BRAFV600E mutation-specific antibody can serve as a screening tool for BRAFV600E-positive LCH. Case reports suggest that BRAFV600E-positive LCH unresponsive to standard therapy might respond to B-Raf-MEK pathway inhibition, but rigorous randomized clinical trials have yet to be performed.
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J. Am. Acad. Dermatol. · Oct 2017
ReviewPostinflammatory hyperpigmentation: A comprehensive overview: Treatment options and prevention.
Postinflammatory hyperpigmentation (PIH) occurs after various dermatoses, exogenous stimuli, and dermatologic procedures. The clinical course of PIH is chronic and unpredictable, although the probability of resolution of epidermal hyperpigmentation is better than those of dermal hyperpigmentation. PIH can be prevented or alleviated. ⋯ Having a reproducible model for PIH is essential for the development of treatment modalities. The second article in this 2-part continuing medical education series on PIH specifically addresses the evidence that supports medical and procedural treatments of PIH and other forms of acquired hyperpigmentation. It also describes a PIH model and provides an algorithm for clinical practice along with discussion about the prevention of PIH.