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]
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]
Entire Body System
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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]
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]
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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]
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Physician
Objective: To familiarize physicians with the clinical manifestations, diagnosis, evaluation, and management of a solitary cutaneous mastocytoma. [eurekaselect.com]
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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]
Gastrointestinal
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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]
Skin
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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]
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Flushing
Symptoms may be localized to include pruritus or blistering of the lesion or generalized with flushing and rarely urticaria. [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]
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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]
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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]
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Sweating
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]
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]
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]
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]