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E-IJD-CASE REPORT
Year : 2014  |  Volume : 59  |  Issue : 6  |  Page : 634
Asymptomatic solitary cutaneous mastocytoma: A rare presentation


1 Department of Skin and STD, Safdurjung Hospital and Vardhman Mahavir Medical College, New Delhi, India
2 National Institute of Pathology, Safdurjung Hospital, New Delhi, India

Date of Web Publication30-Oct-2014

Correspondence Address:
Poonam Puri
House No. 2, Street No. 6, Shantiniketan, New Delhi - 110 021
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.143588

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   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. The diagnosis of cutaneous mastocytoma was made based upon clinical and histopathological features.


Keywords: Asymptomatic mastocytoma, solitary Darier′s sign


How to cite this article:
Gopal D, Puri P, Singh A, Ramesh V. Asymptomatic solitary cutaneous mastocytoma: A rare presentation. Indian J Dermatol 2014;59:634

How to cite this URL:
Gopal D, Puri P, Singh A, Ramesh V. Asymptomatic solitary cutaneous mastocytoma: A rare presentation. Indian J Dermatol [serial online] 2014 [cited 2022 Jan 21];59:634. Available from: https://www.e-ijd.org/text.asp?2014/59/6/634/143588

What was known?
Majority of cases are symptomatic with positive Darier's sign.



   Introduction Top


Urticaria pigmentosa is the most common presentation of cutaneous mastocytosis both in children and adults followed by solitary mastocytoma. Solitary mastocytoma presents with reddish brown nodular lesion up to 3-4 cm in diameter with peau d'orange appearance. Onset is generally before the age of 6 months. Urtication and blistering are seen if traumatized with blunt object and occasionally attacks of flushing can develop on stroking but solitary mastocytoma can be asymptomatic. It is commonly located on the dorsum of the hand near the wrist.


   Case Report Top


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. 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.
Figure 1: Solitary skin colored plaque present on the extensor aspect of left wrist

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On local examination, a single well-defined skin colored firm mobile plaque of 1 × 2.5 cm is present on the extensor aspect of the left wrist [Figure 1]. Surface of the plaque had peau d'orange appearance. On stroking the lesion, there was neither bulla formation on the plaque nor the appearance of wheals or flushing on other parts of the body. Routine investigations and ultrasonography of abdomen were normal.

Histopathological examination of the biopsy showed elongated rete ridges. Papillary dermis was expanded by a dense infiltrate of closely packed cuboidal cells with abundant cytoplasm and central nucleus [Figure 2]. Intracytoplasmic metachromatic granules were seen on toluidine blue staining [Figure 3]a. Immunohistochemical staining with stem cell factor receptor CD-117/c-kit was strongly positive in the cytoplasm of mast cells [Figure 3]b. A diagnosis of solitary cutaneous mastocytoma was considered.
Figure 2: Photomicrograph showing aggregates of closely packed cuboidal cells in papillary dermis (H and E, ×200)

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Figure 3: (a) Photomicrograph showing intracytoplasmic metachromatic granules (Toluidine blue, ×400), (b) Photomicrograph showing strong cytoplasmic labelling with stem cell factor receptor (CD-117, ×400)

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   Discussion Top


Mastocytosis are group of disorders characterized by abnormal proliferation and accumulation of mast cells, involving the skin only (cutaneous mastocytosis) or the bone marrow and other extracutaneous organs (systemic mastocytosis). In children, skin is the most commonly involved organ. [1]

The pathogenesis of cutaneous mastocytosis is not well understood. A transient dysregulation of stem cell factor, a growth factor necessary for mast cell differentiation and growth, has been implicated as the underlying defect in 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, or plaque on the trunk, extremities, head, or neck. [1],[3],[4] Only 10% of mastocytomas appear beyond 2 years of age. [5] The symptoms associated with solitary mastocytomas are secondary to the release of mast cell mediators (histamine). Symptoms may be localized to include pruritus or blistering of the lesion or generalized with flushing and rarely urticaria. Darier's sign may be elicited in only 50% of patients. [6]

Mast cell degranulation within solitary mastocytomas may be triggered by a variety of factors, including physical stimuli (heat, cold, friction, and pressure), emotional factors, certain medications (non-steroidal anti-inflammatory drugs, opioids, dextromethorphan, vancomycin, and general anesthetics), and radiocontrast media. [5],[7] The diagnosis of a solitary mastocytoma is suspected by the presence of a characteristic skin lesion and confirmed by presence of mast cell clusters in biopsy and/or toluidine blue, Giemsa staining and specific mast cell marker c-kit/CD-117 staining. [8] Laboratory evaluation is rarely needed unless lesions fail to regress overtime or systemic symptoms are present. The initial laboratory evaluation includes a complete blood count with differential, comprehensive metabolic profile and a serum tryptase level in symptomatic cases and ultrasonography of the abdomen for any systemic involvement. A bone marrow biopsy is not necessary in infants and children unless extracutaneous organ involvement is suspected. 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. However, no intervention is required if the lesion is asymptomatic. The mainstay of therapy involves avoidance of potential triggers and oral antihistamines (H1-blockers). Other therapeutic options include psoralens UV-A, short-term application of a topical steroid under an occlusive dressing, application of calcineurin inhibitors, or targeted laser therapy. [9] Surgical excision can be curative if unresponsive to other therapies. In addition, families should be educated on the natural history of solitary mastocytomas.

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, and only 10-15% of children have symptoms that persist into adulthood. [2],[11]

 
   References Top

1.Akoglu G, Erkin G, Cakir B, Boztepe G, Sahin S, Karaduman A, et al. Cutaneous mastocytosis: Demographic aspects and clinical features of 55 patients. J Eur Acad Dermatol Venereol 2006;20:969-73.  Back to cited text no. 1
    
2.Heide R, Tank B, Oranje AP. Mastocytosis in childhood. Pediatr Dermatol 2002;19:375-81.  Back to cited text no. 2
    
3.Hannaford R, Rogers M. Presentation of cutaneous mastocytosis in 173 children. Australas J Dermatol 2001;42:15-21.  Back to cited text no. 3
    
4.Wolff K, Komar M, Petzelbauer P. Clinical and histopathological aspects of cutaneous mastocytosis. Leuk Res 2001;25:519-28.  Back to cited text no. 4
    
5.Briley LD, Phillips CM. Cutaneous mastocytosis: A review focusing on the pediatric population. Clin Pediatr (Phila) 2008;47:757-61.  Back to cited text no. 5
    
6.Thappa DM, Jeevankumar B. Solitary mastocytoma. Indian Pediatr 2005;42:390.  Back to cited text no. 6
    
7.Bains SN, Hsieh FH. Current approaches to the diagnosis and treatment of systemic mastocytosis. Ann Allergy Asthma Immunol 2010;104:1-10.  Back to cited text no. 7
    
8.Valent P, Akin C, Escribano L, Födinger M, Hartmann K, Brockow K, et al. Standards and standardization in mastocytosis: Consensus statements on diagnostics, treatment recommendations and response criteria. Eur J Clin Invest 2007;37:435-53.  Back to cited text no. 8
    
9.Heide R, Beishuizen A, De Groot H, Den Hollander JC, Van Doormaal JJ, De Monchy JG, et al. Mastocytosis in children: A protocol for management. Pediatr Dermatol 2008;25:493-500.  Back to cited text no. 9
    
10.Ben-Amitai D, Metzker A, Cohen HA. Pediatric cutaneous mastocytosis: A review of 180 patients. Isr Med Assoc J 2005;7:320-2.  Back to cited text no. 10
    
11.Kettelhut BV, Metcalfe DD. Pediatric mastocytosis. J Invest Dermatol 1991;96:15-18S.  Back to cited text no. 11
    

What is new? The asymptomatic presentation of solitary mastocytoma is rare and therefore reported.


    Figures

  [Figure 1], [Figure 2], [Figure 3]

This article has been cited by
1 Solitary mastocytoma in children: a retrospective analysis of the clinical course and timing of regression
E. I. Kasikhina, M. N. Ostretsova, G. P. Tereshchenko
Meditsinskiy sovet = Medical Council. 2021; (8): 72
[Pubmed] | [DOI]



 

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