A 9-month-old baby girl presented with solitary 1 × 0.5 cm, well-defined, yellowish, waxy, nonscaly plaque on dorsum of right ring finger [Figure 1]. Parents stated that the lesion started at the age of 2 months as a clear vesicle which healed spontaneously within a week. It was followed by appearance of a gradually progressive skin colored plaque at the same site. There was history of recurrent vesiculation within the plaque whenever the child rubbed the lesion. She was apparently in good general health. Systemic examination was unremarkable. Biopsy from the lesion revealed dense upper and middermal infiltrate of uniformly looking cells with rounded central nuclei and abundant amphophilic cytoplasm [Figures 2 and 3].
Figure 1.
Solitary, well defined, yellowish, waxy, non scaly plaque on finger
Figure 2.
Histology showing dense upper and mid dermal infiltrate of uniformly looking cells with rounded central nuclei and abundant amphophilic cytoplasm (H and E ×40)
Figure 3.
A higher magnification showing abundant uniformly looking cells (H and E ×100)
Question
What is your diagnosis?
Answer
Solitary mastocytoma.
Discussion
Mastocytosis encompasses a spectrum of disorders characterized by overproliferation and accumulation of mast cells with altered immunophenotype commonly within skin but may affect other organs of body, especially bone marrow and gastrointestinal tract.[1] The clinical expressions of cutaneous mastocytosis (CM) include solitary mastocytoma, maculopapular CM (urticaria pigmentosa) and diffuse CM.[2] Telangiectasia macularis eruptiva perstans is an entity that presumably does not exist, so it has not been recognized by the World Health Organization.[3] Urticaria pigmentosa is the commonest pattern of CM.[1] In a series of Indian children with CM.[4] Solitary mastocytoma was the second commonest clinical pattern observed after urticaria pigmentosa. All forms may display vesicular or bullous variant.[5,6]
Mastocytomas are usually solitary and present as red, pink, or yellowish nodule or plaque in infancy or early childhood, measuring up to 3-4 cm in diameter. They tend to blister if rubbed and occasional attacks of flushing can be induced.[1] Though commonly involve the extremities and trunk, solitary mastocytoma has also been described on lower eyelid as rare location.[7] Extracutaneous involvement is rare.[8] Although the course of mastocytomas is generally benign and the lesions usually involute over several years, there have been reports of solitary mastocytomas eventuating in generalized urticaria pigmentosa.[5]
Footnotes
Source of support: Nil
Conflict of Interest: Nil.
References
- 1.Grattan CE, Kojba BA. Urticaria and mastocytosis. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's Textbook of Dermatology. 8th ed. Oxford: Blackwell publishing; 2010. pp. 22.30–6. [Google Scholar]
- 2.Gysel VD, Schaik RH, Oranje AP. Mastocytosis. In: Irvine AD, Hoeger PH, Yan AC, editors. Harper's Textbook of Pediatric Dermatology. 3rd ed. Oxford: Wiley-Blackwell; 2011. pp. 1–15.75. [Google Scholar]
- 3.Soter NA. Mastocytosis and the skin. Hematol Oncol Clin North Am. 2000;14:537–55. doi: 10.1016/s0889-8588(05)70295-1. [DOI] [PubMed] [Google Scholar]
- 4.Kanwar AJ, Sandhu K. Cutaneous mastocytosis in children: An Indian experience. Pediatr Dermatol. 2005;22:85–9. doi: 10.1111/j.1525-1470.2005.22124.x. [DOI] [PubMed] [Google Scholar]
- 5.Paller AS, Mancini AJ, editors. Hurwitz Clinical Pediatric Dermatology. 3rd ed. Philadelphia: Elsevier Inc; 2006. Cutaneous tumors and tumor syndromes; pp. 9.225–9. [Google Scholar]
- 6.Nayak S, Acharjya B, Devi B, Behera SK. Bullous mastocytosis. Indian J Dermatol. 2007;52:201–3. [Google Scholar]
- 7.Naysan J, Kodsi SR, Kristal L. Solitary mastocytoma of the eyelid. J AAPOS. 2013;17:443–4. doi: 10.1016/j.jaapos.2013.04.002. [DOI] [PubMed] [Google Scholar]
- 8.Abdullah L, Abbas O. Dermacase: Can you identify this condition? Solitary mastocytoma. Can Fam Physician. 2012;58:766. 770. [PMC free article] [PubMed] [Google Scholar]
