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
- 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]
- Swelling
The lesion used to itch and swell on scratching, bathing and toweling of the area. The child was otherwise healthy and no other systemic manifestations were noted. [ncbi.nlm.nih.gov]
Respiratoric
- Aspiration
Her bone marrow aspirate and biopsy were negative for clusters of mast cells, c-KIT mutation at codon 816, and mast cell expression of CD2 and/or CD25 by flow cytometry or immunohistochemistry. [ncbi.nlm.nih.gov]
Gastrointestinal
- 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]
- Abdominal Pain
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]
Skin
- Darier's Sign
By the end of third month, complete subsidence of the lesion was noticed with residual hyperpigmentation, negative Darier's sign, and no signs of atrophy. [ncbi.nlm.nih.gov]
- Pruritus
Symptoms may be localized to include pruritus or blistering of the lesion or generalized with flushing and rarely urticaria. [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]
- Skin 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]
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]