Journal of the American Academy of Dermatology; Elmets CA, Leonardi CL et. al.
Feb 17th, 2019 - Psoriasis is a chronic, inflammatory, multisystem disease that affects up to 3.2% of the US population. This guideline addresses important clinical questions that arise in psoriasis management and care, providing recommendations on the basis of available evidence.
Pediatric Dermatology; Nanda A, Al-Abboh H et. al.
Feb 12th, 2019 - Neutrophilic panniculitis (NP) with myelodysplasia has been described in adults but not in children. We report a case of NP associated with myelodysplasia in a child with MYSM1 deficiency, a newly described syndrome with primary immunodeficiency (PI), bone marrow failure, and developmental aberrations.
Journal of the American Academy of Dermatology; Abisror N, Mekinian A et. al.
Feb 9th, 2019 - Episodic Angioedema with eosinophilia (EAE, Gleich's syndrome) is a rare disorder consisting of recurrent episodes of angioedema, hypereosinophilia and frequent elevated serum Immunoglobin M. We conducted a retrospective multicenter nationwide study regarding the clinical spectrum and therapeutic management of patients with EAE in France. Thirty patients were included with a median age at diagn...
Journal of the American Academy of Dermatology; Geller S, Gomez CJ et. al.
Feb 4th, 2019 - Follicular mucinosis (FM), which is defined by mucin accumulation within follicular epithelium, may occur in mycosis fungoides (MF). FM without MF is occasionally reported in systemic hematologic malignancies and may be diagnostically challenging. To describe clinicopathologic characteristics of FM in patients with hematologic malignancies other than MF. Clinical data and histopathology feature...
Journal of the European Academy of Dermatology and Venereology : JEADV; Baek YS, Seo JY et. al.
Jan 17th, 2019 - Li-Fraumeni syndrome (LFS) is a rare, autosomal dominant inherited cancer predisposition syndrome associated with germline mutation of the TP53 tumor suppressor gene [1, 2]. It is traditionally characterized by early-onset of multiple primary tumors: sarcoma, breast cancer, brain tumor, leukemia, and adrenocortical carcinoma.  Classically, LFS is clinically diagnosed when a patient present w...
Clinical and Experimental Dermatology; Wu X, Huang J et. al.
Jan 4th, 2019 - Congenital self-healing reticulohistiocytosis (CSHR) is a rare disorder characterized by benign skin lesions with a tendency to self-heal. Multiple skin lesions are usually present in CSHR. It is very difficult to distinguish between CSHR and an invasive Langerhans cell histiocytosis. We present a case of a 5-month-old infant girl who had hypopigmented skin lesions distributed over her neck, th...
Pediatric Dermatology; Song H, Dzuali F et. al.
Dec 14th, 2018 - We report a child with a past medical history notable for congenital deafness, palmoplantar keratoderma (PPK), and hypothalamic glioma who initiated a MEK inhibitor trametinib for cancer-directed therapy at 11 years of age and was incidentally noted to have marked improvement in his PPK. Trametinib withdrawal led to worsening in the patient's PPK. We speculate that the patient's PPK improved be...
The Journal of Dermatology; Ishikawa M, Endo Y et. al.
Dec 11th, 2018 - Histiocytoses, including Langerhans cell histiocytosis (LCH), juvenile or adult xanthogranuloma (AXG) and Rosai-Dorfman disease (RDD), are rare disorders characterized by the proliferation of cells derived from monocyte/macrophage lineages. A few cases of LCH coexisting with xanthogranuloma or RDD have been reported. The etiology of these diseases remains unclear. However, oncogenic BRAFV 600E ...
Hematological Oncology; Merlo G, Cozzani E et. al.
Nov 29th, 2018 - In recent years, dermatologic manifestations in oncohematologic patients have become more common. The aim of our study was to determine the incidence and heterogeneity of skin manifestations in patients followed at our Hematology Department. This observational monocentrical study was conducted on 60 patients. We divided the observed conditions in exanthematous, purpuric, vesicular-bullous, papu...
Clinics in Dermatology; Gupta MA, Pur DR et. al.
Nov 17th, 2018 - Several antiepileptic drugs (AEDs) are approved by the US Food and Drug Administration for the treatment of bipolar disorder (valproic acid, divalproex, lamotrigine, carbamazepine) and some cutaneous neuropathic pain syndromes (carbamazepine, gabapentin, pregabalin). The AEDs may be effective in the management of (1) chronic pruritus, including pruritus due systemic disease, including uremia, n...
Clinical and Experimental Dermatology; Marcoval J, Talavera-Belmonte A et. al.
Nov 15th, 2018 - Sebaceous neoplasms (SN) may appear sporadically in the general population but may also be part of the Muir-Torre variant of Lynch syndrome (MT-LS). There are few studies in southern Europe on the incidence of MT-LS in the population of patients with SN. To retrospectively review patients with SN and to analyse their clinical features and the incidence of MT-LS. Patients with SN diagnosed betwe...
Nov 7th, 2018 - Pregnancy does not necessarily increase a woman’s risk for melanoma, nor is it clear that becoming pregnant affects melanoma’s disease course, according to current evidence. This guidance is among several updates added to newly released guidelines for managing patients with primary cutaneous melanoma.
Clinical and Experimental Dermatology; Velasco R, Cordero P et. al.
Oct 26th, 2018 - Painful papule on the right arm of a woman.|2018|Velasco R,Cordero P,Andrade V,Martín JM,Smith V,|diagnosis,pathology,diagnosis,pathology,diagnosis,pathology,diagnosis,pathology,diagnosis,pathology,
Pediatric Dermatology; Ridaura-Sanz C, Durán-McKinster C et. al.
Oct 19th, 2018 - Silvery hair syndrome is a rare, autosomal-recessive entity characterized by silvery gray hair, eyebrows, and eyelashes and may be associated or not with immunologic or neurologic alterations. Two main types have been recognized: Chediak-Higashi syndrome and Griscelli syndrome. Hair shaft examination under light microscopy has been a useful tool to differentiate Chediak-Higashi syndrome from Gr...
The Journal of Dermatology; Fukayama M, Miyagaki T et. al.
Oct 15th, 2018 - Anetoderma is a rare cutaneous disorder characterized by focal loss of dermal elastic tissue, resulting in macular atrophy or herniated saclike skin. Some families with hereditary anetoderma have been described, but there have been no reports on Japanese familial anetoderma so far. We herein report two Japanese sibling cases of primary anetoderma. A healthy 13-year-old Japanese girl and a healt...
Dermatology Online Journal; Abbas A, Braswell M et. al.
Oct 14th, 2018 - A young man presented with blue nodules on the trunk, face, and extremities that gradually increased in number and size. His mother had similar lesions. Initially, blue rubber bleb nevus syndrome was suspected, but histological findings confirmed the diagnosis of hereditary glomuvenous malformations. Making the correct diagnosis spares the patient unnecessary evaluation for the arteriovenous ma...
American Journal of Clinical Dermatology; Wang CX, Anadkat MJ et. al.
Oct 9th, 2018 - Hematopoietic stem cell transplants (HSCTs) are used to treat a variety of conditions, including hematologic malignancies, bone marrow failure syndromes, and immunodeficiencies. Over 60,000 HSCTs are performed annually worldwide, and the numbers continue to increase. Indeed, as new conditioning regimens develop, more and more individuals, including those of older age, will be eligible for trans...
Journal of the European Academy of Dermatology and Venereology : JEADV; Pereira MP, Zeidler C et. al.
Oct 5th, 2018 - Chronic prurigo (CPG) is a distinct disease characterized by chronic pruritus, history and/or signs of prolonged scratching and multiple pruriginous lesions. It may present with various clinical manifestations, including papules, nodules, plaques or umbilicated lesions. Some patients with chronic pruritus show pruriginous linear and scaring scratch lesions (LSSL) and it is unclear whether these...
Journal of the European Academy of Dermatology and Venereology : JEADV; Chen W, Song Z et. al.
Oct 4th, 2018 - First reported from Taiwan mistakenly as acral acanthosis nigricans in 1991, pigmented carpotarsal hyperkeratosis or hyperkeratosis nigricans carpi et tarsi displays a peculiar distribution of velvety brown-grey hyperpigmented plaques symmetrically on the flexural side of the wrists and ankles and on the dorsal sides of the hands and feet. A marked epidermal hyperkeratosis with typically mild a...
Journal of the American Academy of Dermatology; Kurosawa M, Uehara R et. al.
Sep 30th, 2018 - Autosomal recessive congenital ichthyosis (ARCI) and ichthyosis syndrome (IS) are rare genetic skin disorders. To estimate the number of patients with ARCI and IS in Japan and clarify the clinicoepidemiologic features of these diseases. We performed a nationwide survey of patients treated for ARCI or IS during January 2005-December 2009. We developed diagnostic criteria and conducted a primary ...