Serdar GÖKREM, Gürcan ASLAN, Ali Cemal YILMAZ, Nesrin Tan BASER, Ünzile Balci AKBUGA

Rare clinical variants of pilomatricoma

Pilomatrikoma (Malherbe ’nin kalsifi ye epitelyoması saç matriks hücrelerinden gelişen benign adneksal bir tümördür. Pilomatrikoma genellikle solid kitleler olarak izlenir. Sıklıkla yaşamın 1. ve 6. dekadında pik yapar. Kadın/erkek görülme oranı 3/2 ’dir. Lezyonların %50 ’den fazlası baş boyun bölgesinde, %25-30 ’luk kısmı üst ekstremitede lokalizedir. Pilomatrikoma nadir değildir. Bunun yanında ailesel multiple pilomatrikoma ve perfore pilomatrikomanın ise nadir görüldüğü bildirilmektedir. Kural olarak pilomatrikomanın herediter olmadığı söylensede literatürde bildirilmiş sınırlı sayıda ailesel pilomatrikoma vakası vardır. Burada 3 yıllık bir peryodda kliniğimize başvuran ve histopatolojik olarak pilomatrikoma tanısı alan 10 vaka incelendi. Literatürde nadir rastlandığı bildirilen ailesel multiple pilomatrikoma ve pilomatrikomanın nadir bir klinik varyantı olan perfore pilomatrikoma vakaları sunuldu. Bu vakalar literatür eşliğinde gözden geçirildi. Serimizdeki hasta sayısının az olmasına rağmen ailesel multiple pilomatrikoma ve perfore pilomatrikoma olgularımızın literatüre katkısı olacağını düşünüyoruz.

Pilomatrikomanın nadir klinik varyantları

Pilomatricoma (calcifi ying epithelioma of Malherbe ) is a benign adnexal tumor of hair matrix cells. Pilomatricomas are usually observed as solitary lesions. Pilomatricoma mostly makes peaks in the fi rst and sixth decades. Female to male ratio is 3/2. More than 50% of the lesions are found on head and neck region, and 25-30% of the lesions are found on the upper extremities. The rest occurs in the trunk and rarely in the lower extremities. Pilomatricoma is not rare entity. On the contrary, multiple and perforating forms of pilomatricoma are rare entities. Although pilomatricoma is not hereditary as a rule, there are limited familial pilomatricoma cases which are published in the literature. The patients, who come to our clinic during three years and histologically diagnosed as having the pilomatricoma were analysed. Rare clinic variants of pilomatricoma that familial multiple pilomatricoma, perforating pilomatricoma are reported. In spite of the small number of the patients in our series, we believe that familial multiple pilomatricoma and perforating pilomatricoma cases will supply the literature.

PDF

___

1. Elder D, Elentistas R, Ragsdale BD. Tumors of epidermal appendages. In: Elder D, Elantistas R, Jaworsky C, Johnson B Jr, editors. Lever’s histopatology of the skin, 8th ed. Philadelphia: Lippincott-Raven.; 1997. p.747.
2. Fernandes R, Holmes, Mullenix C. Giant pilomatricoma ( epithelioma of Melherbe): Report of a case and review of literature. J Oral Maxillofac Surg 2003;61:634-6.
3. Fetil E, Özkan S, Ilknur T, et.al. Multiple pilomatricoma with perforation. Int J Dermatol 2002;41:892-3.
4. Forbis R, Helwig EB. Pilomatrixoma (calcifying epithelioma). Arch. Dermatol 1961; 83: 606-8.
5. Margogi A, Wick MR, Dehner LP. Pilomatrical neoplasm in childern and young adults. Am J Dermatopathol 1992; 87:14.
6. Moehlenbeck, F. W. Pilomatrixoma (calcifying epithelioma): A statistical study. Arch. Dermatol 1973; 108: 532-4.
7. Migirov L, Fridman Y, Talmi YP. Pilomatrixoma of the retroauriculerarea and arm. J Pediat Surg 2002;8: 1.
8. Sarı A, Yavuzer R, Isık I, et al. Atypical presentation of pilomatricoma: A case report . Dermatol Surg 2002;28:603-5.
9. Aslan G, Erdogan B, Akoz T, et.al. Multiple occurrence of pilomatrixoma. Plast Reconstr Surg 1996; 98: 510-3.
10. Onishi T, Nakamura Y, Watanebe S. Perforating pilomatricoma in a process of total elimination. J Am Acad Dermatol 2003;49:146-7.
11. Alli N, Gungor E, Artuz F. Perforating pilomatricoma. J Am Acad Dermatol 1996;35: 116-8.
12. Grabczynska SA, Budny P, Calonjet E, et al. Case 3. Clin Exp Dermatol 2002;27 :343-4.
13. Hills RJ, Ive FA. Familyal pilomatrixomas. Br J Dermatol 1992;127:194-5.
14. Graells J, Servitje O, Badell A. Multiple familial pilomatricomas associated with myotonic dystrophy. Int J Dermatol 1996; 35: 732-3.
15. Haskimoto K, Nelson RG, Lewer WF. Calcifiying epithelioma of Malherbe: histochemical and electronmicroscopic studies. J Invest Dermatol 1996;46:391-408.
16. Kligman AM. The human hair cycle. J Invest Dermatol 1959;33:307-16.
17. Farrier S, Morgan M. bcl-2 expression in pilomatricoma. Am J Dermatopathol 1997; 19: 254-7.
18. Headington JT. Tumors of hair folliculer diferantiation. In: Farmer ER, Hood AF, editors. Pathology of the skin. Norwalk, Conn. & San Meteo, Calif.: Appleton & Lange.; 1990. p.596-614.
19. Chan EF, Gat U, McNiff JM, et.al. A common human skin tumour is caused by activating mutations in betacatenin. Nat Genet 1999; 21: 410-3.
20. Park SW, Suh KS, Wang HY. ß catenin expression in the transitional cell zone of pilomatrixoma. Br J Dermatol 2001;145:624-9.
21. Pirouzmanes A, Reinisch J, Gonzolez- Gomez I, et al Pilomatrixoma: A review of 346 cases. Plast Reconstr Surg 2003;112:1784-89.
22. Duflo S, Nicollas R, Roman S. Pilomatrixoma of the head and neck in children: a study of 38 cases and a review of the literature. Arch Otolaryngol Head Neck Surg 1998 Nov; 124: 1239-42.
23. Yoshimoto S, Ichinose M, Udagawa A, et al. Are Multiple pilomatricomas rare? Plast Reconstr Surg 2002;109:816-17.
24. Julian CG, Bowers PW. A clinical review of 209 pilomatricomas. J Am Acad Dermatol 1998 Aug; 39: 191-5.
25. Hernandez-Perez E, Cestoni-Parducci RF. Pilomatricoma (calcifying epithelioma): a study of 100 cases in El Salvador. Int J Dermatol 1981 Sep; 20: 491-4.