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Arerugi = [Allergy] 2022Cutaneous mastocytosis (CM) usually appears in childhood and improves substantially before adolescence. The c-KIT mutation of D816V is present in 36% and 20% of patients... (Review)
Review
Cutaneous mastocytosis (CM) usually appears in childhood and improves substantially before adolescence. The c-KIT mutation of D816V is present in 36% and 20% of patients with childhood-onset CM and diffuse cutaneous mastocytosis (DCM), respectively. In some cases of childhood-onset DCM, the disease can progress to systemic mastocytosis; in others, it resolves spontaneously. Thus, assessing the prognosis is difficult. Herein, we described a case of DCM in an 11-month-old, male patient without a c-KIT mutation. The patient presented with dark brown macules and sporadic erythema topped by bullous lesions. A skin biopsy of the macule on the abdomen revealed accumulation of mast cells which were round to oval-shaped with amphophilic cytoplasm within the upper dermis. The patient had received H1 inhibitor until age 3 years and continued to experience blisters on the trunk. However, no severe symptoms, such as anaphylaxis, occurred. Included in this manuscript is a review of previous reports of childhood-onset DCM in Japan and cases specifically seen at our dermatology clinic.
Topics: Adolescent; Child, Preschool; Humans; Infant; Male; Mast Cells; Mastocytosis, Cutaneous; Prognosis; Proto-Oncogene Proteins c-kit; Skin
PubMed: 35831165
DOI: 10.15036/arerugi.71.397 -
Medical Engineering & Physics Dec 2021Electrochemotherapy (ECT) requires covering the entire tumor and safe margins with a suitable pulsed electric field (PEF). The PEF distribution depends on the biological...
Electrochemotherapy (ECT) requires covering the entire tumor and safe margins with a suitable pulsed electric field (PEF). The PEF distribution depends on the biological and electrical parameters. The biological tissue may have diffractive geometry with non-linear conductivity behavior due to electroporation. That characteristic may provoke ECT-insufficient electric field regions, also known as blind spots. The conductive gels can fill holes and bumps, being a tool to homogenize the electric field. We executed an in vitro vegetal tissue experiment to validate a numerical model under different gels conditions. We used a study case in silico experiment to investigate gel influence on PEF distribution and electrical current. We propose a case-oriented methodology to optimize the gel during the ECT pre-treatment. Results show that the optimized gel completely treats a region of interest while avoiding unnecessary current increase and damage to healthy tissue by over treatment. The optimized gel conductivity may be lower than the previously reported (0.5 to 1 S/m) and may be in the range of the commercially available gels. For a veterinary mastocytoma exophytic nodule ECT case study, using needles electrode, the 0.2 S/m gel is the optimum gel.
Topics: Computer Simulation; Computers; Electric Conductivity; Electrochemotherapy; Electroporation; Gels
PubMed: 34848032
DOI: 10.1016/j.medengphy.2021.10.011 -
Journal of Cutaneous Pathology Feb 2018Cutaneous mastocytoma (CM) is a localized variant of mastocytosis, characterized by an over-accumulation of mast cells in the skin, without extra-cutaneous organ...
Cutaneous mastocytoma (CM) is a localized variant of mastocytosis, characterized by an over-accumulation of mast cells in the skin, without extra-cutaneous organ involvement. It is defined as the presence of up to 3 isolated mast-cell skin lesions and commonly develops in newborns and children. We report the case of a 35-year-old healthy Caucasian woman presenting with a 4-year history of a pruritic brown plaque on her left breast. Hematoxylin-eosin staining showed a dense dermal infiltrate of atypical mast cells extending to the subcutis. The cells presented a marked nuclear pleomorphism with bilobed and multilobed nuclei. Immunohistochemical studies revealed strongly expressed KIT (CD117) and CD25 proteins. Serum tryptase levels and bone marrow biopsy were normal. The diagnosis was a solitary cutaneous pleomorphic mastocytoma. This case can be added to 17 other cases of adult mastocytoma documented in the literature, although, unlike other reported cases, and as far as we are aware, this is the first case of pleomorphic mastocytoma in an adult.
Topics: Adult; Breast; Female; Humans; Mastocytoma, Skin
PubMed: 29148588
DOI: 10.1111/cup.13080 -
Clinics in Dermatology 2019Cutaneous disease can present with lesions of all colors of the visible spectrum. Lesions of the skin, nail, and mucous membranes with an orange color can be due to a... (Review)
Review
Cutaneous disease can present with lesions of all colors of the visible spectrum. Lesions of the skin, nail, and mucous membranes with an orange color can be due to a variety of etiologies. The conditions may appear as purely orange, yellow-orange, red-orange, tan, or brown with an orange hue. The orange color may also present as a transient phase of a disease process. As with all dermatologic pathology, a key way to distinguish orange-colored lesions is by distribution and morphology. The sclera, palate, lips, gingiva, and nails may also be involved. A literature review using PubMed with keywords, including orange, skin, mucosa, cutaneous, xanthoderma, and granuloma, was conducted to gather all dermatologic conditions that can present with an orange color. The relevant diseases were categorized by etiology and include inflammatory, infectious, neoplastic, and exogenous causes.
Topics: Color; Histiocytosis, Non-Langerhans-Cell; Humans; Leishmaniasis, Cutaneous; Lupus Vulgaris; Mastocytoma; Mastocytoma, Skin; Mouth Mucosa; Pigmentation Disorders; Pityriasis Rubra Pilaris; Sarcoidosis; Sebaceous Gland Neoplasms
PubMed: 31896407
DOI: 10.1016/j.clindermatol.2019.07.014 -
Journal of Clinical Medicine Aug 2022The term mastocytosis refers to a heterogeneous group of disorders characterised by accumulation of clonal mast cells in different organs, most commonly in the skin.... (Review)
Review
The term mastocytosis refers to a heterogeneous group of disorders characterised by accumulation of clonal mast cells in different organs, most commonly in the skin. Little is known about the role of dermoscopy in the diagnostics of mastocytosis. To date, no systematic review on the dermoscopic features of cutaneous mastocytosis has been performed. The aim of this study was to summarise the current knowledge in the field as well as to identify the knowledge gaps to show possible directions for further studies, based on a systematic search of PubMed, Scopus, and Web of Science databases and related references published before 3 January 2022. Dermoscopic features, type of dermoscope, polarisation mode, magnification, and number of cases were analysed. In total, 16 articles were included in this review (3 case series and 13 case reports), analysing 148 patients with different variants of cutaneous mastocytosis; all of the studies analysed had a low level of evidence (V). The main dermoscopic features of urticaria pigmentosa included brown structureless areas, brown lines arranged in a network, and linear vessels distributed in a reticular pattern, with this last finding also being typical of telangiectasia macularis eruptiva perstans. The presence of either circumscribed yellow structureless areas or diffuse yellowish background was a constant pattern of mastocytoma, while nodular, pseudoangiomatous xanthelasmoid, and plaque-type mastocytosis were typified by light-brown structureless areas and/or pigment network, though the first two variants also showed yellow/yellow-orange structureless areas. Finally, pigmented streaks of radial distribution surrounding hair follicles were described to be a pathognomonic dermoscopic feature of pseudoxanthomatous mastocytosis. Although this review shows that the various clinical forms of cutaneous mastocytosis may feature diagnostic dermoscopic clues, it also underlines the need for further investigation as several relevant data are missing, including evaluation of dermoscopic pattern according to anatomical locations or "lesion age", studies on rare mastocytosis variants, evaluation of the prognostic role of dermoscopy in the context of systemic involvement, and comparative analyses with common clinical mimickers.
PubMed: 36012900
DOI: 10.3390/jcm11164649 -
Veterinary and Comparative Oncology Mar 2019In humans, advanced mast cell (MC) neoplasms are rare malignancies with a poor prognosis. Only a few preclinical models are available, and current treatment options are... (Review)
Review
In humans, advanced mast cell (MC) neoplasms are rare malignancies with a poor prognosis. Only a few preclinical models are available, and current treatment options are limited. In dogs, MC neoplasms are the most frequent malignant skin tumours. Unlike low-grade MC neoplasms, high-grade MC disorders usually have a poor prognosis with short survival. In both species, neoplastic MCs display activating KIT mutations, which are considered to contribute to disease evolution. Therefore, tyrosine kinase inhibitors against KIT have been developed. Unfortunately, clinical responses are unpredictable and often transient, which remains a clinical challenge in both species. Therefore, current efforts focus on the development of new improved treatment strategies. The field of comparative oncology may assist in these efforts and accelerate human and canine research regarding diagnosis, prognostication, and novel therapies. In this article, we review the current status of comparative oncology approaches and perspectives in the field of MC neoplasms.
Topics: Animals; Antineoplastic Agents; Dog Diseases; Dogs; Humans; Mastocytoma; Species Specificity
PubMed: 30136349
DOI: 10.1111/vco.12440 -
Indian Journal of Dermatology,... 2014
Topics: Flushing; Humans; Infant; Infant, Newborn; Male; Mast Cells; Mastocytosis, Cutaneous
PubMed: 25382519
DOI: 10.4103/0378-6323.144189 -
The Medical Journal of Australia Mar 2021
Topics: Darier Disease; Diagnosis, Differential; Humans; Infant; Male; Mastocytoma; Skin; Skin Neoplasms
PubMed: 33715187
DOI: 10.5694/mja2.50947 -
European Journal of Medicinal Chemistry Feb 2018The main transporter for biotin is sodium dependent multivitamin transporter (SMVT), which is overexpressed in various aggressive cancer cell lines such as ovarian (OV... (Review)
Review
The main transporter for biotin is sodium dependent multivitamin transporter (SMVT), which is overexpressed in various aggressive cancer cell lines such as ovarian (OV 2008, ID8), leukemia (L1210FR), mastocytoma (P815), colon (Colo-26), breast (4T1, JC, MMT06056), renal (RENCA, RD0995), and lung (M109) cancer cell lines. Furthermore, its overexpression was found higher to that of folate receptor. Therefore, biotin demand in the rapidly growing tumors is higher than normal tissues. Several biotin conjugated organic molecules has been reported here for selective delivery of the drug in cancer cell. Biotin conjugated molecules are showing higher fold of cytotoxicity in biotin positive cancer cell lines than the normal cell. Nanoparticles and polymer surface modified drugs and biotin mediated cancer theranostic strategy was highlighted in this review. The cytotoxicity and selectivity of the drug in cancer cells has enhanced after biotin conjugation.
Topics: Antineoplastic Agents; Biotin; Cell Proliferation; Dose-Response Relationship, Drug; Humans; Molecular Structure; Neoplasms; Organic Chemicals; Proteins; Structure-Activity Relationship
PubMed: 29324341
DOI: 10.1016/j.ejmech.2018.01.001 -
Actas Dermo-sifiliograficas 2016Mastocytosis is a term used to describe a heterogeneous group of disorders characterized by clonal proliferation of mast cells in various organs. The organ most often... (Review)
Review
Mastocytosis is a term used to describe a heterogeneous group of disorders characterized by clonal proliferation of mast cells in various organs. The organ most often affected is the skin. Mastocytosis is a relatively rare disorder that affects both sexes equally. It can occur at any age, although it tends to appear in the first decade of life, or later, between the second and fifth decades. Our understanding of the pathophysiology of mastocytosis has improved greatly in recent years, with the discovery that somatic c-kit mutations and aberrant immunophenotypic features have an important role. The clinical manifestations of mastocytosis are diverse, and skin lesions are the key to diagnosis in most patients.
Topics: Female; Humans; Male; Mast Cells; Mastocytosis; Proto-Oncogene Proteins c-kit; Skin
PubMed: 26546030
DOI: 10.1016/j.ad.2015.09.007