Childhood Solitary Cutaneous Mastocytoma: Clinical Manifestations, Diagnosis, Evaluation, and Management

Author(s): Alexander K. C. Leung*, Joseph M. Lam, Kin Fon Leong.

Journal Name: Current Pediatric Reviews

Volume 15 , Issue 1 , 2019

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Graphical Abstract:


Abstract:

Background: The diagnosis of solitary cutaneous mastocytoma is mainly clinical, based on lesion morphology, the presence of a positive Darier sign, and the absence of systemic involvement. Knowledge of this condition is important so that an accurate diagnosis can be made.

Objective: To familiarize physicians with the clinical manifestations, diagnosis, evaluation, and management of a solitary cutaneous mastocytoma.

Methods: A PubMed search was completed in Clinical Queries using the key term "solitary cutaneous mastocytoma". The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. Only papers published in English language were included. The information retrieved from the above search was used in the compilation of the present article.

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. The lesion often has a peau d'orange appearance and a leathery or rubbery consistency. A solitary cutaneous mastocytoma may urticate spontaneously or when stroked or rubbed (Darier sign). Organomegaly and lymphadenopathy are characteristically absent. The majority of patients with skin lesions that erupt within the first two years of life have spontaneous resolution of the lesions before puberty. Treatment is mainly symptomatic. Reassurance and avoidance of triggering factors suffice in most cases.

Conclusion: The diagnosis is mainly clinical, based on the morphology of the lesion, the presence of a positive Darier sign, and the absence of systemic involvement. A skin biopsy is usually not necessary unless the diagnosis is in doubt.

Keywords: Indurated, hyperpigmented, macule, papule, plaque, nodule, Darier sign.

[1]
Leung AK, Barankin B. Urticaria pigmentosa. Consult Pediatr 2016; 15: 311-3.
[2]
Hartmann K, Escribano L, Grattan C, et al. Cutaneous manifestations in patients with mastocytosis: Consensus report of the European Competence Network on Mastocytosis; the American Academy of Allergy, Asthma & Immunology; and the European Academy of Allergology and Clinical Immunology. J Allergy Clin Immunol 2016; 137(1): 35-45.
[3]
Ma SP, Hardy TG. Solitary Mastocytoma of the eyelid in an adult patient with prolidase deficiency. Ophthal Plast Reconstr Surg 2017; 33(1): e10-3.
[4]
Pandhi D, Singal A, Aggarwal S. Adult onset, hypopigmented solitary mastocytoma: Report of two cases. Indian J Dermatol Venereol Leprol 2008; 74(1): 41-3.
[5]
Azaña JM, Torrelo A, Matito A. 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. Curr Allergy Asthma Rep 2017; 17(11): 80.
[7]
Auquit-Auckbur I, Lazar C, Deneuve S, et al. Malignant transformation of mastocytoma developed on skin mastocytosis into cutaneous mast cell sarcoma. Am J Surg Pathol 2012; 36(5): 779-82.
[8]
Briley LD, Phillips CM. Cutaneous mastocytosis: A review focusing on the pediatric population. Clin Pediatr (Phila) 2008; 47(8): 757-61.
[9]
Gopal D, Puri P, Singh A, Ramesh V. 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. Allergy Asthma Proc 2010; 31(6): 520-3.
[11]
Tüysüz G, Özdemir N, Apak H, Kutlubay Z, Demirkesen C, Celkan T. Childhood mastocytosis: Results of a single center. Turk Pediatri Ars 2015; 50(2): 108-13.
[12]
Azaña JM, Torrelo A, Matito A. Update on mastocytosis (Part 2): Categories, prognosis, and treatment. Actas Dermosifiliogr 2016; 107(1): 15-22.
[13]
de Giorgi V, Fabroni C, Alfaioli B, et al. Solitary mastocytoma: Tooth eruption as triggering factor. Int J Dermatol 2008; 47(12): 1274-7.
[14]
McKinnon EL, Rand AJ, Proia AD. Solitary mastocytoma in the eyelid of an adult. Am J Ophthalmol Case Rep 2018; 9: 103-5.
[15]
Cohen PR. Solitary mastocytoma presenting in an adult: Report and literature review of adult-onset solitary cutaneous mastocytoma with recommendations for evaluation and treatment. Dermatol Pract Concept 2016; 6(3): 31-8.
[16]
Wiechers T, Rabenhorst A, Schick T, Preussner LM, Förster A, Valent P, et al. Large maculopapular cutaneous lesions are associated with favorable outcome in childhood-onset mastocytosis. J Allergy Clin Immunol 2015; 136(6): 1581-90.
[17]
Castells MC, Akin C. 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. Pediatr Dermatol 2008; 25(3): 381-2.
[19]
Tuxen AJ, Orchard D. Solitary mastocytoma occurring at a site of trauma. Australas J Dermatol 2009; 50(2): 133-5.
[20]
Okun MR. Mast cells, melanocytes, balloon cells. Am J Dermatopathol 1982; 4(5): 478-9.
[21]
Bulat V, Mihić LL, Situm M, Buljan M, Blajić I, Pusić J. Most common clinical presentations of cutaneous mastocytosis. Acta Clin Croat 2009; 48(1): 59-64.
[22]
Conrad H, Gausche-Hill M, Burbulys D. 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. Solitary mastocytoma mimicking granuloma faciale. Int J Dermatol 2014; 53(12): e587-8.
[25]
Exposito-Serrano V, Agut-Busquet E, Leal Canosa L, Herrerías Moreno J, Saez A, Luelmo J. Pleomorphic mastocytoma in an adult. J Cutan Pathol 2018; 45(2): 176-9.
[26]
Gori A, Torneria C, Kelly VM, Zlotoff BJ, Contreras ME. Visual diagnosis: Two infants who have skin lesions that react to minor trauma. Pediatr Rev 2009; 30(7): 280-3.
[27]
Nair B, Sonthalia S, Aggarwal I. Solitary mastocytoma with positive Darier’s sign. Indian Dermatol Online J 2016; 7(2): 141-2.
[28]
Janakiramanan N, Chambers D, Dowling GJ. A rare presentation of solitary mastocytoma in the palm of an infant. J Plast Reconstr Aesthet Surg 2010; 63(2): e197-8.
[29]
Bussmann C, Hagemann T, Hanfland J, Haidl G, Bieber T, Novak N. Flushing and increase of serum tryptase after mechanical irritation of a solitary mastocytoma. Eur J Dermatol 2007; 17(4): 332-4.
[30]
Castells MC, Akin C. Mastocytosis (cutaneous and systemic): evaluation and diagnosis in children. In: Post TW, Ed. Up To DateWaltham, MA. (Accessed on March 30, 2018)
[31]
Castells MC, Akin C. Treatment and prognosis of cutaneous mastocytosis. In: Post TW, Ed. Up To DateWaltham, MA. (Accessed on March 30, 2018)
[32]
Chantorn R, Shwayder T. Death from mast cell leukemia: A young patient with longstanding cutaneous mastocytosis evolving into fatal mast cell leukemia. Pediatr Dermatol 2012; 29(5): 605-9.
[33]
Muraro A, Roberts G, Clark A, et al. The management of anaphylaxis in childhood: Position paper of the European academy of allergology and clinical immunology. Allergy 2007; 62(8): 857-71.


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Article Details

VOLUME: 15
ISSUE: 1
Year: 2019
Page: [42 - 46]
Pages: 5
DOI: 10.2174/1573396315666181120163952

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