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Urticaria pigmentosa

Gabriel Marrero Alemán, Constantin El Habr, Diana Islas Norris, Társila Montenegro Dámaso, Leopoldo Borrego, Omar P Sangueza
Cutaneous mastocytosis is defined by the presence of mast cells within the skin in the absence of other criteria for the diagnosis of systemic mastocytosis. Mast cells are characterized by an abundant granular cytoplasm and a round to oval or spindle-shaped nuclei. The presence of mast cells with bilobed and multilobed nuclei in cutaneous mastocytosis is a rare phenomenon and has been rarely reported in the literature. To our knowledge, there are only 4 reported cases of cutaneous mastocytosis with atypical mast cells...
April 2017: American Journal of Dermatopathology
Adam Heinze, Travis J Kuemmet, Yvonne E Chiu, Sheila S Galbraith
BACKGROUND/OBJECTIVES: Urticaria pigmentosa (UP) is the most common form of mastocytosis in children and is associated with systemic signs, symptoms, and triggers. To our knowledge, the effect of UP on children's quality of life has not been studied. The objective of the current study was to characterize the natural history, triggers, and complications of pediatric UP, identify prognostic indicators, and determine its effect on quality of life. METHODS: Between 2002 and 2007, children with three or more mastocytomas diagnosed by a pediatric dermatologist were recruited during visits at the Children's Hospital of Wisconsin Dermatology Clinic (Milwaukee, WI)...
March 2017: Pediatric Dermatology
Karl B Alstadhaug, Randi Fykse Halstensen, Francis Odeh
BACKGROUND: Progressive multifocal leukoencephalopathy (PML) is a rare opportunistic brain infection caused by the human polyomavirus JC (JCPyV). A particular problem with the drug cladribine seems to be prolonged suppression of the CD4+ T-cells, a well-known risk factor for PML. CASE DESCRIPTION: A 67-year-old male presented with a 3-weeks history of unsteady gait, dysarthria and a dysfunctional right arm. Seven years earlier, he had been diagnosed with urticaria pigmentosa, and 2 years later aggressive systemic mastocytosis...
March 2017: Journal of Clinical Virology: the Official Publication of the Pan American Society for Clinical Virology
Shannon Tew, Brad M Taicher
Service dogs are beneficial in providing assistance to people with multiple types of disabilities and medical disorders including visual impairment, physical disabilities, seizure disorders, diabetes, and mental illness. Some service animals have been trained as a screening tool for cancer. We review a case involving a 6-year-old female with a history of mast cell mediator release and immediate hypersensitivity due to the urticaria pigmentosa variant of cutaneous mastocytosis who underwent a cystourethroscopy...
2016: Case Reports in Pediatrics
H Zaouri, H Amarouch, N Elmakrini, N Tazi, N Ismaili, L Benzekri, K Senouci, B Hassam
Mastocytosis is a group of diseases related to abnormal accumulation and proliferation of mast cells in one or more organs. They may be associated with an acquired point mutation and the activation of the receptor tyrosine-kinase c-KIT of CFS (mast cell growth factor). The clinical manifestations are varied and secondary to the release of mast cell mediators and/or infiltration of various organs. There are two main types of mastocytosis: pure cutaneous mastocytosis and systemic mastocytosis when more than two organs are involved in mast cell infiltration (bone marrow, gastrointestinal tract, bone, liver and spleen, lymph nodes)...
November 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Enzo Errichetti, Giuseppe Stinco
Over the last few years, dermoscopy has been shown to be a useful tool in assisting the noninvasive diagnosis of various general dermatological disorders. In this article, we sought to provide an up-to-date practical overview on the use of dermoscopy in general dermatology by analysing the dermoscopic differential diagnosis of relatively common dermatological disorders grouped according to their clinical presentation, i.e. dermatoses presenting with erythematous-desquamative patches/plaques (plaque psoriasis, eczematous dermatitis, pityriasis rosea, mycosis fungoides and subacute cutaneous lupus erythematosus), papulosquamous/papulokeratotic dermatoses (lichen planus, pityriasis rosea, papulosquamous sarcoidosis, guttate psoriasis, pityriasis lichenoides chronica, classical pityriasis rubra pilaris, porokeratosis, lymphomatoid papulosis, papulosquamous chronic GVHD, parakeratosis variegata, Grover disease, Darier disease and BRAF-inhibitor-induced acantholytic dyskeratosis), facial inflammatory skin diseases (rosacea, seborrheic dermatitis, discoid lupus erythematosus, sarcoidosis, cutaneous leishmaniasis, lupus vulgaris, granuloma faciale and demodicidosis), acquired keratodermas (chronic hand eczema, palmar psoriasis, keratoderma due to mycosis fungoides, keratoderma resulting from pityriasis rubra pilaris, tinea manuum, palmar lichen planus and aquagenic palmar keratoderma), sclero-atrophic dermatoses (necrobiosis lipoidica, morphea and cutaneous lichen sclerosus), hypopigmented macular diseases (extragenital guttate lichen sclerosus, achromic pityriasis versicolor, guttate vitiligo, idiopathic guttate hypomelanosis, progressive macular hypomelanosis and postinflammatory hypopigmentations), hyperpigmented maculopapular diseases (pityriasis versicolor, lichen planus pigmentosus, Gougerot-Carteaud syndrome, Dowling-Degos disease, erythema ab igne, macular amyloidosis, lichen amyloidosus, friction melanosis, terra firma-forme dermatosis, urticaria pigmentosa and telangiectasia macularis eruptiva perstans), itchy papulonodular dermatoses (hypertrophic lichen planus, prurigo nodularis, nodular scabies and acquired perforating dermatosis), erythrodermas (due to psoriasis, atopic dermatitis, mycosis fungoides, pityriasis rubra pilaris and scabies), noninfectious balanitis (Zoon's plasma cell balanitis, psoriatic balanitis, seborrheic dermatitis and non-specific balanitis) and erythroplasia of Queyrat, inflammatory cicatricial alopecias (scalp discoid lupus erythematosus, lichen planopilaris, frontal fibrosing alopecia and folliculitis decalvans), nonscarring alopecias (alopecia areata, trichotillomania, androgenetic alopecia and telogen effluvium) and scaling disorders of the scalp (tinea capitis, scalp psoriasis, seborrheic dermatitis and pityriasis amiantacea)...
December 2016: Dermatology and Therapy
Catherina Moll-Manzur, Esteban Araos-Baeriswyl, Camila Downey, María T Dossi
Urticaria pigmentosa, also known as maculopapular mastocytosis, is the most common type of paediatric mastocytosis. It presents with yellow to brown macules or papules, usually located on trunk and extremities. Regarding its diagnostic and therapeutic implications, the objective of this article is to serve as an update for the paediatrician on the most relevant aspects of this pathology.
August 1, 2016: Archivos Argentinos de Pediatría
Loren Wissner Greene, Kamyar Asadipooya, Patricia Freitas Corradi, Cem Akin
Systemic Mastocytosis (SM) is characterized by accumulation of clonal, neoplastic proliferations of abnormal mast cells (MC) in one or more organ system other than skin. Presence of these multifocal clusters of abnormal mast cells is an essential feature of SM. Frequently associated with D816V (KIT) mutation, the presence of this mutation and elevated serum tryptase are minor criteria for diagnosis. SM manifestations depend on the degree of mast cell proliferation, activation and degranulation. SM has a variable prognosis and presentation, from indolent to "smoldering" to life-threatening disease...
September 2016: Reviews in Endocrine & Metabolic Disorders
M Gehlen, A D Lazarescu, C Hinz, N Schmidt, M Pfeifer, M Werner, H F Weidemann, M Schwarz-Eywill, A Maier
Systemic mastocytosis is defined as a clonal increase of mast cells. We report on four patients with severe osteoporosis and histologically confirmed systemic mastocytosis. In spite of antiresorptive therapy the patients developed further vertebral fractures and suffered from ostealgia. Systemic mastocytosis is an important differential diagnosis in patients with therapy refractive and unexplained osteoporosis. Skin involvement (urticaria pigmentosa) is a rare occurrence and in most cases an isolated involvement of bone marrow is present...
September 2016: Zeitschrift Für Rheumatologie
Johannes Junginger, Florian Geburek, Muhammad Akram Khan, Gundi Müller, Achim D Gruber, Marion Hewicker-Trautwein
BACKGROUND: Cutaneous mastocytosis is a rare benign disease occurring in domestic animals and humans. In previous reports, dermal findings in foals were accompanied by systemic mast cell infiltrations, whereas lesions in human cutaneous mastocytosis, including urticaria pigmentosa and solitary mastocytoma, are usually restricted to the skin. OBJECTIVES: To describe a new variant of equine cutaneous maculopapular mastocytosis lacking systemic involvement. ANIMALS: A 2...
April 24, 2016: Veterinary Dermatology
Bjorn van Anrooij, Johanna C Kluin-Nelemans, Mary Safy, Bertine M J Flokstra-de Blok, Joanne N G Oude Elberink
BACKGROUND: Presently, no validated data exists on symptom severity and disease-specific quality of life (QoL) for mastocytosis patients. Simultaneously, clinical trials and drug application processes increasingly mandate reporting patients' perspectives on symptoms and QoL. We report on the development and validation of the mastocytosis quality of life questionnaire (MQLQ) and the mastocytosis symptom assessment form (MSAF). METHODS: Both outcome measures were developed in a standardised stepwise method, starting with the identification of items in focus groups (n= 12), item reduction and subsequent cross-sectional validation in a 63% female cohort of 164 adult indolent systemic mastocytosis patients...
April 19, 2016: Allergy
Yuki Kato, Emi Kashiwagi, Koichi Masuno, Kae Fujisawa, Noriko Tsuchiya, Shuuichi Matsushima, Mikinori Torii, Nobuo Takasu
Cutaneous mastocytosis, which resembles a subset of urticaria pigmentosa in humans, is rare in dogs. We herein report unrepresentative neoplastic proliferation of mast cells in ventral skin removed routinely from a nine-month-old female laboratory beagle dog at necropsy. A histological examination revealed diffuse extensive cellular infiltration from the superficial to deep dermis in most parts of the skin around the fourth and fifth mammary papilla without nodule formation. Tumor cells were fairly monomorphic, well-differentiated mast cells with round nuclei of small distinct nucleoli and moderate to abundant, slightly eosinophilic and granular cytoplasm...
January 2016: Journal of Toxicologic Pathology
Amy S Aloysi, Gabriella M Ahle, Emma Geduldig, Charles H Kellner, Ethan O Bryson
Urticaria pigmentosa is a rare disorder characterized by an abnormal systemic proliferation of mast cells. In this condition, various triggers can induce either cutaneous histamine release, resulting in rash, or generalized histamine release, resulting in symptomatic hypotension, syncope, or in its severest form, an anaphylactoid reaction resistant to most resuscitative measures. Many anesthetic agents and adjuncts are known potential triggers, and patients who require surgery or procedures under anesthesia must be managed carefully...
September 2016: Journal of ECT
María Soledad Zegpi-Trueba, Paula Hasbún-Acuña, Daniela Berroeta-Mauriziano
INTRODUCTION: Mastocytosis represents a group of diseases characterised by an excesive accumulation of mastocytes in one or multiple tissues. It can affect only the skin, or have a systemic involvement. It has a low prevalence, and the prognosis is benign in children. OBJECTIVE: To report a case of urticaria pigmentosa as a subtype of cutaneous mastocytosis, and present a literature review focused on clinical findings, diagnosis and initial basic management. CLINICAL CASE: A child of six months of age presenting with multiple blemishes and light brown papules located on the trunk, arms and legs...
May 2016: Revista Chilena de Pediatría
Karin Hartmann, Luis Escribano, Clive Grattan, Knut Brockow, Melody C Carter, Ivan Alvarez-Twose, Almudena Matito, Sigurd Broesby-Olsen, Frank Siebenhaar, Magdalena Lange, Marek Niedoszytko, Mariana Castells, Joanna N G Oude Elberink, Patrizia Bonadonna, Roberta Zanotti, Jason L Hornick, Antonio Torrelo, Jürgen Grabbe, Anja Rabenhorst, Boguslaw Nedoszytko, Joseph H Butterfield, Jason Gotlib, Andreas Reiter, Deepti Radia, Olivier Hermine, Karl Sotlar, Tracy I George, Thomas K Kristensen, Hanneke C Kluin-Nelemans, Selim Yavuz, Hans Hägglund, Wolfgang R Sperr, Lawrence B Schwartz, Massimo Triggiani, Marcus Maurer, Gunnar Nilsson, Hans-Peter Horny, Michel Arock, Alberto Orfao, Dean D Metcalfe, Cem Akin, Peter Valent
Cutaneous lesions in patients with mastocytosis are highly heterogeneous and encompass localized and disseminated forms. Although a classification and criteria for cutaneous mastocytosis (CM) have been proposed, there remains a need to better define subforms of cutaneous manifestations in patients with mastocytosis. To address this unmet need, an international task force involving experts from different organizations (including the European Competence Network on Mastocytosis; the American Academy of Allergy, Asthma & Immunology; and the European Academy of Allergology and Clinical Immunology) met several times between 2010 and 2014 to discuss the classification and criteria for diagnosis of cutaneous manifestations in patients with mastocytosis...
January 2016: Journal of Allergy and Clinical Immunology
Minh Van Hoang, Phuoc Van Dang, Duc Van Bui, Haider Mejbel, Divya Thomas Mani, Bruce Robert Smoller, Thuy Linh Phung
Cutis laxa is characterized by dramatic wrinkling of skin that is lacking in elasticity due to inherent defects in dermal elastic fibers. Cutis laxa can be caused by genetic and metabolic disorders. It can also be acquired, possibly resulting from inflammatory processes with destruction of elastic fibers. This report describes a 26-year old woman who developed acquired cutis laxa and cutaneous mastocytosis leading to premature aging. She represents a unique co-occurrence of these two separate disease entities...
July 2015: Dermatology Online Journal
Kinga Cristina Slavescu, Roxana Chiorean, Sorina Danescu, Madalina Bota, Liliana Rogojan, Adrian Baican
Cutaneous mastocytosis is a disease characterized by the infiltration and proliferation of mast cells in the skin. In children, the most common form of presentation is urticaria pigmentosa, while the diffuse cutaneous bullous mastocytosis is one of the rarest subtypes seen. The aim of this paper is to present a case of diffuse bullous mastocytosis with detection of IgM deposits at dermo-epidermal junction using direct immunofluorescence (DIF) microscopy. The diagnosis of diffuse bullous mastocytosis is a challenge, and DIF microscopy is necessary in order to exclude an autoimmune bullous disorder...
March 2016: Journal of Cutaneous Pathology
T Gülen, H Hägglund, B Dahlén, G Nilsson
Mastocytosis is a complex disorder characterized by the accumulation of abnormal mast cells (MC) in the skin, bone marrow and/or other visceral organs. The clinical manifestations result from MC-derived mediators and, less frequently, from destructive infiltration of MCs. Patients suffer from a variety of symptoms including pruritus, flushing and life-threatening anaphylaxis. Whilst mastocytosis is likely to be suspected in a patient with typical skin lesions [i.e. urticaria pigmentosa (UP)], the absence of cutaneous signs does not rule out the diagnosis of this disease...
March 2016: Journal of Internal Medicine
Gülen Tüysüz, Nihal Özdemir, Hilmi Apak, Zekayi Kutlubay, Cuyan Demirkesen, Tiraje Celkan
AIM: We aimed to retrospectively evaluate histopathological, demographic and clinical findings of children with mastocytosis diagnosed with mastocytosis in our clinic. MATERIAL AND METHODS: The files of 21 patients diagnosed with mastocytosis between 2000 and 2014 in our clinic were retrospectively analyzed. RESULTS: All patients had cutaneous mastocytosis, 19 patients had urticaria pigmentosa and 2 patients had mastocytoma. The male-female ratio was: 1/1...
June 2015: Türk Pediatri Arşivi
Melody C Carter, Sarah T Clayton, Hirsh D Komarow, Erica H Brittain, Linda M Scott, Daly Cantave, Donna M Gaskins, Irina Maric, Dean D Metcalfe
BACKGROUND: The management of children with pediatric mastocytosis poses a challenge. This is because there is limited information as to the application of clinical and laboratory findings and bone marrow histopathology as they relate to medical intervention and communication. OBJECTIVE: We sought to examine clinical aspects of pediatric mastocytosis in relationship to serum tryptase levels and bone marrow pathology to provide practical guidance for management. METHODS: Between 1986 and 2012, 105 children were evaluated at the National Institutes of Health...
December 2015: Journal of Allergy and Clinical Immunology
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