urticaria pigmentosa

Summary

Summary: The most common form of cutaneous mastocytosis (MASTOCYTOSIS, CUTANEOUS) that occurs primarily in children. It is characterized by the multiple small reddish-brown pigmented pruritic macules and papules.

Top Publications

  1. Brockow K, Scott L, Worobec A, Kirshenbaum A, Akin C, Huber M, et al. Regression of urticaria pigmentosa in adult patients with systemic mastocytosis: correlation with clinical patterns of disease. Arch Dermatol. 2002;138:785-90 pubmed
    To determine clinical correlates of urticaria pigmentosa (UP) regression in adult patients with systemic mastocytosis (SM).
  2. Azaña J, Torrelo A, Mediero I, Zambrano A. Urticaria pigmentosa: a review of 67 pediatric cases. Pediatr Dermatol. 1994;11:102-6 pubmed
    ..We studied 67 consecutive patients (33 males, 34 females) with urticaria pigmentosa and assessed them fully to determine the presence of systemic involvement...
  3. Mann R, Friedman K, Milgraum S. Urticaria pigmentosa and juvenile xanthogranuloma: case report and brief review of the literature. Pediatr Dermatol. 1996;13:122-6 pubmed
    Juvenile xanthogranuloma and urticaria pigmentosa are well described in the dermatologic literature. We recently observed a 9-month-old boy with the two diseases occurring concomitantly...
  4. Akin C, Valent P, Escribano L. Urticaria pigmentosa and mastocytosis: the role of immunophenotyping in diagnosis and determining response to treatment. Curr Allergy Asthma Rep. 2006;6:282-8 pubmed
    ..Flow cytometric analysis of bone marrow mast cells is therefore a sensitive method of diagnosis of mast cell disease and is expected to find increasing use in determining response to emerging mast cell cytoreductive therapies...
  5. Longley B, Tyrrell L, Lu S, Ma Y, Langley K, Ding T, et al. Somatic c-KIT activating mutation in urticaria pigmentosa and aggressive mastocytosis: establishment of clonality in a human mast cell neoplasm. Nat Genet. 1996;12:312-4 pubmed
    ..discrete mast cell infiltrates are associated increased epidermal melanin, a clinical picture known as urticaria pigmentosa (UP)...
  6. Beghini A, Tibiletti M, Roversi G, Chiaravalli A, Serio G, Capella C, et al. Germline mutation in the juxtamembrane domain of the kit gene in a family with gastrointestinal stromal tumors and urticaria pigmentosa. Cancer. 2001;92:657-62 pubmed
    ..family in which the dominantly inherited trait of hyperpigmented spots was inherited from an individual who developed multiple GISTs with diffuse hyperplasia of the myenteric plexus by his son, who was affected with urticaria pigmentosa.
  7. Bessis D, Guilhou J, Guillot B. Localized urticaria pigmentosa triggered by mesotherapy. Dermatology. 2004;209:343-4 pubmed
  8. Schechter N, Wang Z, Blacher R, Lessin S, Lazarus G, Rubin H. Determination of the primary structures of human skin chymase and cathepsin G from cutaneous mast cells of urticaria pigmentosa lesions. J Immunol. 1994;152:4062-9 pubmed
    ..by protein methods and by analysis of PCR amplification products obtained with cDNA-derived from urticaria pigmentosa (UP) lesions. UP is a disease characterized by skin lesions containing high numbers of mast cells...
  9. Kim K, Roh K, Choi J, Sung K, Moon K, Koh J. Scabies incognito presenting as urticaria pigmentosa in an infant. Pediatr Dermatol. 2002;19:409-11 pubmed
    ..Topical or systemic corticosteroids may modify the clinical presentation of scabies and that situation is referred to as scabies incognito. We describe a 10-month-old infant with scabies incognito mimicking urticaria pigmentosa.

More Information

Publications62

  1. Hollmann T, Brenn T, Hornick J. CD25 expression on cutaneous mast cells from adult patients presenting with urticaria pigmentosa is predictive of systemic mastocytosis. Am J Surg Pathol. 2008;32:139-45 pubmed
    b>Urticaria pigmentosa (UP) is a clinicopathologic term used to describe reddish-brown cutaneous macules and papules, characterized histologically by mast cell infiltration of the papillary and upper reticular dermis and reactive basal ..
  2. Oranje A, Soekanto W, Sukardi A, Vuzevski V, van der Willigen A, Afiani H. Diffuse cutaneous mastocytosis mimicking staphylococcal scalded-skin syndrome: report of three cases. Pediatr Dermatol. 1991;8:147-51 pubmed
    ..Special care should be taken when these patients are to undergo anesthesia. The risk of complications during and after anesthesia is also present in other forms of mastocytosis...
  3. Comte C, Bessis D, Dereure O, Guillot B. Urticaria pigmentosa localized on radiation field. Eur J Dermatol. 2003;13:408-9 pubmed
    A woman previously treated by radiotherapy for a breast cancer developed urticaria pigmentosa mainly restricted to the irradiation field, without any systemic symptoms...
  4. An D, Wu X, Hu F, Yan B, Stefan H, Zhou D. Association study of lamotrigine-induced cutaneous adverse reactions and HLA-B*1502 in a Han Chinese population. Epilepsy Res. 2010;92:226-30 pubmed publisher
    ..In conclusion, HLA-B*1502 and other HLA alleles are not directly associated with LTG-induced MPE. The possibility that HLA-B*1502 is associated with an increased risk of LTG-induced SJS/TEN could not be excluded...
  5. Depras A, Misery L, Cambazard F. [Mastocytosis with symetric disposition in twin sisters]. Ann Dermatol Venereol. 2002;129:66-7 pubmed
  6. Jordan J, Fritsche Polanz R, Sperr W, Mitterbauer G, Fodinger M, Schernthaner G, et al. A case of 'smouldering' mastocytosis with high mast cell burden, monoclonal myeloid cells, and C-KIT mutation Asp-816-Val. Leuk Res. 2001;25:627-34 pubmed
    ..We report on a 48-year-old female patient with SM with urticaria pigmentosa-like skin lesions and mediator-related symptoms...
  7. Dacosta A, Guy J, Cathebras P, Perrot J, Decousus H, Tardy B, et al. [A rare cause of loss of consciousness: mastocytosis. Apropos of 3 cases]. Arch Mal Coeur Vaiss. 1993;86:1747-52 pubmed
    ..Prevention is by drugs which inhibit the synthesis of histamine, the degranulation of mastocytes and the production of prostaglandin D2...
  8. Mauleón Fernandez C, Saez de Ocariz M, Rodriguez Jurado R, Duran McKinster C, Orozco Covarrubias L, Ruiz Maldonado R. Nodular scabies mimicking urticaria pigmentosa in an infant. Clin Exp Dermatol. 2005;30:595-6 pubmed
  9. Skrabs C. Darier sign: a historical note. Arch Dermatol. 2002;138:1253-4 pubmed
  10. Reich M, Reich A, Rzeszutko M, Iwanczak B. Gastroesophageal reflux disease accompanying urticaria pigmentosa. Acta Dermatovenerol Croat. 2011;19:264-7 pubmed
    ..Here, we present a 17-month-old boy with urticaria pigmentosa who also suffered from GERD...
  11. Walker T, von Komorowski G, Scheurlen W, Dorn Beineke A, Back W, Bayerl C. Neonatal mastocytosis with pachydermic bullous skin without c-Kit 816 mutation. Dermatology. 2006;212:70-2 pubmed
    ..Bullous mastocytosis is an unusual variant of mast cell disease with widespread bullae as the main cutaneous feature induced by mast cell proteases that cause dermoepidermal separation...
  12. Ludolph Hauser D, Schopf P, Rueff F, Przybilla B. [Occult cutaneous mastocytosis]. Hautarzt. 2001;52:390-3 pubmed
    Mastocytosis is diagnosed without difficulty if it presents with easily recognizable lesions of urticaria pigmentosa. Recently, we have identified hardly visible skin lesions of mastocytosis in Hymenoptera venom allergic patients ("..
  13. Guler E, Emir S, Kutluk T, Varan A, Buyukpamukcu M. Urticaria pigmentosa associated with Wilms tumor. Pediatr Dermatol. 2001;18:313-5 pubmed
    b>Urticaria pigmentosa is the most common manifestation of mastocytosis, with the majority of cases undergoing spontaneous resolution, especially in children...
  14. Hartmann K, Henz B. Mastocytosis: recent advances in defining the disease. Br J Dermatol. 2001;144:682-95 pubmed
  15. Morrow J, Oates J, Roberts L, Zackert W, Mitchell T, Lazarus G, et al. Increased formation of thromboxane in vivo in humans with mastocytosis. J Invest Dermatol. 1999;113:93-7 pubmed
  16. Dunst K, Huemer G, Zelger B, Zelger B. A new variant of mastocytosis: report of three cases clinicopathologically mimicking histiocytic and vasculitic disorders. Br J Dermatol. 2005;153:642-6 pubmed
    Cutaneous mastocytosis (CM) or urticaria pigmentosa is characterized by abnormal proliferation and accumulation of mast cells...
  17. Bakhshi S, Savasan S, Abella E. Infantile fibrosarcoma associated with urticaria pigmentosa. Pediatr Hematol Oncol. 2002;19:445-7 pubmed
    The authors report the case of an infant with urticaria pigmentosa who developed infantile fibrosarcoma. The tumor was successfully resected but the skin lesions have persisted...
  18. Awad J, Morrow J, Roberts L. Detection of the major urinary metabolite of prostaglandin D2 in the circulation: demonstration of elevated levels in patients with disorders of systemic mast cell activation. J Allergy Clin Immunol. 1994;93:817-24 pubmed
    ..One particular advantage is the evaluation of clinical events, which only in retrospect are suspected to be associated with excessive release of PGD2, yet plasma or serum was obtained proximate to the event...
  19. Boncoraglio G, Brucato A, Carriero M, Maccagnano E, Robbiolo L, Scappatura L, et al. Systemic mastocytosis: a potential neurologic emergency. Neurology. 2005;65:332-3 pubmed
  20. Villeneuve V, Kaufman I, Weeks S, Deschamps A. Anesthetic management of a labouring parturient with urticaria pigmentosa. Can J Anaesth. 2006;53:380-4 pubmed
    To report the anesthetic management of labour pain and Cesarean section in a patient with urticaria pigmentosa at risk for systemic mastocytosis...
  21. Hahn H, Hornick J. Immunoreactivity for CD25 in gastrointestinal mucosal mast cells is specific for systemic mastocytosis. Am J Surg Pathol. 2007;31:1669-76 pubmed
    ..Gastrointestinal (GI) symptoms are common in both SM and cutaneous mastocytosis [urticaria pigmentosa (UP)], and are usually caused by the release of histamine and other inflammatory mediators...
  22. Spada J, Lequio M, Pyke M, Hernandez M, Chouela E. [Urticaria pigmentosa: two different clinical presentations in pediatric patients]. Arch Argent Pediatr. 2011;109:e72-6 pubmed publisher
    b>Urticaria pigmentosa (UP) is the most frequent clinical feature of cutaneous mastocytosis. It usually begins in a bimodal way: a peak of incidence from birth to the age of 3 and the other one between 2(nd) and 6(th) decades of life...
  23. Kalogeromitros D, Gregoriou S, Makris M, Georgala S, Kempuraj D, Theoharides T. Secondary anetoderma associated with mastocytosis. Int Arch Allergy Immunol. 2007;142:86-8 pubmed
    ..The most common manifestation is urticaria pigmentosa (UP), which is characterized by small or large brown-red maculopapules on the skin...
  24. Davidson S, Coates D. Cutaneous mastocytosis extending beyond a radiotherapy site: a form of radiodermatitis or a neoplastic phenomenon?. Australas J Dermatol. 2013;54:e85-7 pubmed publisher
  25. Pitt T, Cisneros N, Kalicinsky C, Becker A. Successful treatment of idiopathic anaphylaxis in an adolescent. J Allergy Clin Immunol. 2010;126:415-6; author reply 416 pubmed publisher
  26. Confino Cohen R, Zilber M, Goldberg A. Multiple life-threatening fainting episodes: fatal systemic mastocytosis. Isr Med Assoc J. 2003;5:752-3 pubmed
  27. Sivapirabu G, Sugo E, Wargon O. Juvenile xanthogranuloma: challenges in complicated cases. Australas J Dermatol. 2011;52:284-7 pubmed publisher
    ..Although JXG has been reported with urticaria pigmentosa, in two of our cases persistent urticaria, in association with JXG is discussed.
  28. Lain E, Hsu S. Photo quiz. Chronic, papular rash that develops a wheal when rubbed. Am Fam Physician. 2004;69:1493-4 pubmed
  29. Farahnak A, Essalat M. A study on cercarial dermatitis in Khuzestan province, south western Iran. BMC Public Health. 2003;3:35 pubmed
    ..This survey was made on people from villages north of Ahwaz city in south west Iran, to estimate cercarial dermatitis in this region...
  30. Möhrenschlager M, Engst R, Müller Weihrich S, Spiessl W, Rüdisser K, Weigl L, et al. Association of urticaria pigmentosa with café-au-lait spots, neurofibromas and neurofibroma-like neoplasms: a mere coincidence?. Dermatology. 2003;206:297-302 pubmed
    b>Urticaria pigmentosa (UP) is characterized by dense aggregates of mast cells in the dermis. There is consistent evidence from the literature that mast cells may play a pathogenetic role in the development of neurofibromas and other tumors.
  31. Ben Amitai D, Metzker A, Cohen H. Pediatric cutaneous mastocytosis: a review of 180 patients. Isr Med Assoc J. 2005;7:320-2 pubmed
    ..Mastocytosis is a heterogeneous group of diseases characterized by the abnormal infiltration of mast cells in the skin and, sometimes, other organs. Some patients may experience symptoms related to mast cell mediator release...
  32. Gambichler T, Terras S, Kreuter A. Treatment regimens, protocols, dosage, and indications for UVA1 phototherapy: facts and controversies. Clin Dermatol. 2013;31:438-454 pubmed publisher
    ..for UVA1 include subacute prurigo, lichen sclerosus, dyshidrotic dermatitis, cutaneous T cell lymphoma, urticaria pigmentosa, and pityriasis rosea; nevertheless, there are some unknowns, uncertainties, and controversies concerning ..
  33. Stern R, Manders S, Buttress S, Heymann W. Urticaria pigmentosa presenting with massive peripheral eosinophilia. Pediatr Dermatol. 1997;14:284-6 pubmed
    ..The patient's course improved with antihistamines and the removal of mast cell degranulating agents. Clinicians need to be aware that mastocytosis should be considered in the differential diagnosis of eosinophilia...
  34. Kim C, Kim H, Jung M, Lee J, Park J, Lee D, et al. Cutaneous mastocytosis associated with congenital alopecia. Am J Dermatopathol. 2012;34:529-32 pubmed publisher
    ..This case showed an atypical clinical presentation of congenital alopecia areata, but histopathological results confirmed the diagnosis of cutaneous mastocytosis...
  35. Renke J, Lange M. [Mastocytosis in childhood]. Przegl Lek. 2006;63:551-3 pubmed
    ..It concerns 5,4 caser per 1000 of cured children. The disease has no original treatment. There is a chance that thanks to to popularisation of knowledge of this disease will be more frequently diagnosed and known better...
  36. Wallenfang K, Stadler R. [Association between UVA1 and PUVA bath therapy and development of malignant melanoma]. Hautarzt. 2001;52:705-7 pubmed
    After 18 months of intensive UVA1- and bath PUVA therapy for urticaria pigmentosa, a female patient developed a malignant melanoma on the right thigh...
  37. Has C, Misery L, David L, Cambazard F. Recurring staphylococcal scalded skin syndrome-like bullous mastocytosis: the utility of cytodiagnosis and the rapid regression with steroids. Pediatr Dermatol. 2002;19:220-3 pubmed
    ..This case report emphasizes the utility of cytodiagnosis in extensive blistering diseases in infancy and the possibility of obtaining rapid healing by using steroids...
  38. Avela K, Alen R, Huttunen M, Pärssinen O. Megalocornea-urticaria pigmentosa syndrome--a new syndrome?. Eur J Med Genet. 2009;52:430-2 pubmed publisher
    We describe a Finnish boy with megalocornea, urticaria pigmentosa, mild delay in speech and motor development, and slightly dysmorphic facial features. The karyotype and the array-CGH analysis did not reveal any abnormalities...
  39. Pérez Elizondo A, Zepeda Ortega B, del Pino Rojas G. [Urticaria pigmentosa: a current approach]. Rev Alerg Mex. 2009;56:124-35 pubmed
    The term urticaria pigmentosa (UP) denotes a heterogeneous group of disorders characterized by abnormal growth and accumulation of mast cells (MC) in the skin...
  40. Tsujita J, Doi K, Nakahara M, Nakahara T, Kaku Y, Nishio K, et al. Overexpression of p16(INK4a) in Mastocytosis (Urticarial Pigmentosa). Fukuoka Igaku Zasshi. 2016;107:12-7 pubmed
    ..0 ± 14.1%) of tryptase-positive tumor cells was immunoreactive to p16(INK4a) in all of 4 mastocytosis. The p16(INK4a) overexpression may induce the senescence of neoplastic mast cells to undergo spontaneous regression of mastocytosis. ..
  41. van Doormaal J, van der Veer E, van Voorst Vader P, Kluin P, Mulder A, van der Heide S, et al. Tryptase and histamine metabolites as diagnostic indicators of indolent systemic mastocytosis without skin lesions. Allergy. 2012;67:683-90 pubmed publisher
    ..systemic mastocytosis (ISM) in adults with clinical suspicion of ISM without accompanying skin lesions [urticaria pigmentosa (UP)] are lacking...
  42. Longley B, Metcalfe D. A proposed classification of mastocytosis incorporating molecular genetics. Hematol Oncol Clin North Am. 2000;14:697-701, viii pubmed
    ..This system provides a theoretic framework for mast cell researchers and helps practicing physicians in estimating prognosis and determining therapeutic options for individual patients...
  43. Rodriguez Acosta A, Reyes Lugo M. Severe human urticaria produced by ant (Odontomachus bauri, Emery 1892) (Hymenoptera: Formicidae) venom. Int J Dermatol. 2002;41:801-3 pubmed
    ..Ant sting reactions are becoming an increasing problem in tropical countries. It is important for physicians to be aware of their possible cutaneous and systemic manifestations...
  44. Peretz E, Grunwald M, Hallel Halevy D, Halevy S. Brownish reticulate maculopapular eruption on the flexures. Arch Dermatol. 2003;139:381-6 pubmed
  45. Syed N, Moiz B. Systemic mastocytosis: a rare entity. J Pak Med Assoc. 2006;56:338-40 pubmed
    ..Because of the benign course of the disease, patient was offered only anti-histamine drugs that were quite successful in alleviating her symptoms...
  46. Gobello T, Mazzanti C, Sordi D, Annessi G, Abeni D, Chinni L, et al. Medium- versus high-dose ultraviolet A1 therapy for urticaria pigmentosa: a pilot study. J Am Acad Dermatol. 2003;49:679-84 pubmed
    There is currently no definitive cure for urticaria pigmentosa (UP). Psoralen plus ultraviolet A therapy is efficacious in alleviating symptoms and reducing cutaneous lesions.
  47. Viscasillas X, Puigdemont G, Salvador C. A slowly enlarging, unilateral, erythematous macular lesion. Arch Dermatol. 2006;142:641-6 pubmed
  48. Krilis S, Austen K, Macpherson J, Nicodemus C, Gurish M, Stevens R. Continuous release of secretory granule proteoglycans from a cell strain derived from the bone marrow of a patient with diffuse cutaneous mastocytosis. Blood. 1992;79:144-51 pubmed
  49. Chamlin S, Cowper S, Longley B, Williams M. Generalized bullae in an infant. Arch Dermatol. 2002;138:831-6 pubmed
  50. Marja Leena L, Tarja R. Quest for the diagnosis. Case 1: An infant with skin rash and episodes with heavy crying. Acta Paediatr. 2007;96:1698-1700 pubmed
  51. Granerus G, Lonnqvist B, Nystrand J, Roupe G. Serum tryptase measured with B12 and G5 antibody-based immunoassays in mastocytosis patients and its relation to histamine turnover. Br J Dermatol. 1998;139:858-61 pubmed
    ..Twelve patients had uncomplicated cutaneous mastocytosis (urticaria pigmentosa) and 13 had urticaria pigmentosa with systemic symptoms...
  52. Vasiadi M, Mondolfi A, Alysandratos K, Therianou A, Katsarou Katsari A, Petrakopoulou T, et al. Neurotensin serum levels and skin gene expression are increased in atopic dermatitis. Br J Dermatol. 2013;169:695-9 pubmed publisher
    ..We also showed that NT stimulates human mastcell release of vascular endothelial growth factor, and that CRH is increased in the serum of patients with atopic dermatitis (AD), an inflammatory skin condition involving mast cells...
  53. Brockow K, Akin C, Huber M, Metcalfe D. IL-6 levels predict disease variant and extent of organ involvement in patients with mastocytosis. Clin Immunol. 2005;115:216-23 pubmed
    ..There was an inverse correlation to hemoglobin. sIL-6R levels were not elevated. These observations demonstrate that IL-6 is a useful surrogate marker of severity of hematologic disease and suggest that IL-6 contributes to pathology...