Edit concept Question Editor Create issue ticket

Mastocytoma


Presentation

  • Case Report A 50-day-old female child presented with a skin colored raised lesion on the dorsal aspect of the left wrist [ Figure 1 ] since the age of 10 days, which gradually increased in size to attain the present size.[ncbi.nlm.nih.gov]
  • There are a number of cases in which more than one mastocytoma was present. Demis 2 states that four lesions may be present. I describe, herein, a patient with nine mastocytomas.[jamanetwork.com]
  • Asymptomatic solitary cutaneous mastocytoma: A rare presentation. Indian J Dermatol 2014; 59(6): 634. [10] Krishnan KR, Ownby DR. A solitary mastocytoma presenting with urticaria and angioedema in a 14-year-old boy.[eurekaselect.com]
  • They usually present within 2 years of age, mostly within first 3 months 1.[ncbi.nlm.nih.gov]
  • A case of an adult with severe prolidase deficiency who developed cutaneous mastocytoma of the eyelid was presented. To the authors’ knowledge, adult-onset solitary mastocytoma of the eyelid has never been reported previously.[journals.lww.com]
Asymptomatic
  • Abstract A 50-day-old female child presented with asymptomatic skin colored raised lesion on the dorsal aspect of the left wrist since the age of 10 days.[ncbi.nlm.nih.gov]
  • Since the mastocytoma was otherwise asymptomatic and she had no mastocytosis-associated general symptoms, she elected to monitor the residual lesion.[ncbi.nlm.nih.gov]
  • Asymptomatic solitary cutaneous mastocytoma: A rare presentation. Indian J Dermatol 2014; 59(6): 634. [10] Krishnan KR, Ownby DR. A solitary mastocytoma presenting with urticaria and angioedema in a 14-year-old boy.[eurekaselect.com]
Weight Loss
  • She has no prior history of itching, flushing, fevers, fatigue, night sweats, weight loss or skin rashes. She had experienced diarrhea whenever she ate wheat, several years earlier, but this subsided once she eliminated gluten from her diet.[ncbi.nlm.nih.gov]
Physician
  • Objective: To familiarize physicians with the clinical manifestations, diagnosis, evaluation, and management of a solitary cutaneous mastocytoma.[eurekaselect.com]
Recurrent Infection
  • Prolidase deficiency is an inherited connective tissue disorder that has systemic sequelae, such as intractable skin ulceration, poor wound healing, recurrent infections, and intellectual impairment.[journals.lww.com]
Vomiting
  • There was no history of appearance of bullae over the lesion, wheals on other parts of the body, flushing of body, pruritus, vomiting, and diarrhea on scratching. General physical and systemic examination was normal.[ncbi.nlm.nih.gov]
  • One of the women had experienced only one episode of gastrointestinal distress characterized by severe nausea and vomiting, diarrhea and abdominal pain [ 11 ].[ncbi.nlm.nih.gov]
Abdominal Cramps
  • Her bowel movements are normal and she has no abdominal cramping or reflux. Cutaneous examination of her right abdomen showed an 8 x 4 mm brown patch with irregular borders ( Figure 1 ).[ncbi.nlm.nih.gov]
Nausea
  • One of the women had experienced only one episode of gastrointestinal distress characterized by severe nausea and vomiting, diarrhea and abdominal pain [ 11 ].[ncbi.nlm.nih.gov]
Darier's Sign
  • If there is a Dariers sign and a strong clinical suspicion this presentation is specific enough that I do not think biopsy is mandatory.[ncbi.nlm.nih.gov]
  • Keywords: Asymptomatic mastocytoma, solitary Darier's sign Introduction What was known? Majority of cases are symptomatic with positive Darier's sign.[ncbi.nlm.nih.gov]
  • Post-rubbing urtication (signifying a positive Darier’s sign) was noted in 79% (11/14) of the mastocytomas. None of the patients had systemic mastocytosis. However, five patients (31%of 16 individuals) had generalized symptoms.[ncbi.nlm.nih.gov]
Flushing
  • Symptoms may be localized to include pruritus or blistering of the lesion or generalized with flushing and rarely urticaria.[ncbi.nlm.nih.gov]
  • Systemic symptoms were noted in 5 patients: flushing (3 women) and pruritus (3 women); gastrointestinal symptoms and headaches, flushing and/or anaphylaxis were each noted in one woman.[ncbi.nlm.nih.gov]
  • A 6-month old with total body flushing and a macular-papular lesion. Pediatr Emerg Care 2007; 23(5): 321-3. [23] Horny HP, Sotlar K, Valent P. Mastocytosis: State of the art. Pathobiology 2007; 74(2): 121-32. [24] Bourji L, Kurban M, Abbas O.[eurekaselect.com]
Papule
  • Results: Typically, a solitary cutaneous mastocytoma presents as an indurated, erythematous, yellow- brown or reddish-brown macule, papule, plaque or nodule, usually measuring up to 5 cm in diameter.[eurekaselect.com]
  • […] cutaneous mastocytosis.[ 2 ] On an average, 10-15% of all patients with cutaneous mastocytosis have a solitary mastocytoma.[ 3 ] The majority of mastocytomas present during infancy, typically by 3 months of age, as a single indurated, red -brown macule, papule[ncbi.nlm.nih.gov]
  • Conclusions A mastocytoma presents as a reddish-brown papule that consists of an accumulation of mast cells in the dermis.[ncbi.nlm.nih.gov]
Skin Atrophy
  • These immunomodulators offer advantages over corticosteroids in terms of a more selective action, no associated systemic side-effects, and the absence of associated skin atrophy, depigmentation and telangiectasia.[ncbi.nlm.nih.gov]
Ulcer
  • Prolidase deficiency is an inherited connective tissue disorder that has systemic sequelae, such as intractable skin ulceration, poor wound healing, recurrent infections, and intellectual impairment.[journals.lww.com]

Treatment

  • Prucha H, Schnopp C, Akdis C, et al. : Pimecrolimus, a topical calcineurin inhibitor used in the treatment of atopic eczema.[ncbi.nlm.nih.gov]
  • Treatment is mainly symptomatic. Reassurance and avoidance of triggering factors suffice in most cases.[eurekaselect.com]
  • Recommendations for the evaluation and treatment of individuals with adult-onset solitary mastocytoma are proposed.[ncbi.nlm.nih.gov]
  • Standards and standardization in mastocytosis: Consensus statements on diagnostics, treatment recommendations and response criteria. Eur J Clin Invest. 2007; 37 :435–53. [ PubMed ] [ Google Scholar ] 9.[ncbi.nlm.nih.gov]

Prognosis

  • Treatment and prognosis of cutaneous mastocytosis. In: Post TW, Ed. Up To DateWaltham, MA. (Accessed on March 30, 2018) [32] Chantorn R, Shwayder T.[eurekaselect.com]
  • Fortunately, the vast majority of children with solitary mastocytomas have a good prognosis with reduction or complete resolution of symptoms by puberty.[ 10 ] Due to spontaneous involution, solitary mastocytomas rarely remain symptomatic in older children[ncbi.nlm.nih.gov]

Epidemiology

  • Mastocytosis (cutaneous and systemic): Epidemiology, pathogenesis, and clinical manifestations. In: Post TW, Ed. Up To DateWaltham, MA. (Accessed on March 30, 2018) [18] Koh MJ, Chong WS. Red plaque after hepatitis B vaccination.[eurekaselect.com]
  • Albeit less common, solitary mastocytomas have been described in 16 adults: 8 men and 6 women; 2 individuals did not have any epidemiologic features reported.[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Pathophysiology

  • Update on mastocytosis (Part 1): pathophysiology, clinical features, and diagnosis. Actas Dermosifiliogr 2016; 107(1): 5-14. [6] Klaiber N, Kumar S, Irani AM. Mastocytosis in Children.[eurekaselect.com]

Prevention

  • Pathogenetically and from the mechanism of action of tacrolimus, which prevents mast cell degranulation, an improvement of mastocytoma can be expected.[ncbi.nlm.nih.gov]
  • The goal of management of a symptomatic solitary mastocytoma is to prevent the release of mast cell mediators and alleviate symptoms associated with mediator release, particularly pruritus.[ncbi.nlm.nih.gov]

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!