Capurro NJ, Carignano AM, Ottino A

Language: Spanish
References: 19
Page: 351-354
PDF size: 340.98 Kb.

ABSTRACT

Palmar or plantar Cerebriform Fibrous Proliferation (Connective Tissue Nevi, Cutaneous Subcutaneous Hyperplasia) is characterized by conspicuous thickening of the skin exhibiting a clinical particular pattern. Microscopically there is dense fibrosis involving both the dermis and subcutis (almost exclusively of type I collagen), and epidermis with hyperkeratosis, acanthosis, and papillomatosis. It is pathognomonic, although not mandatory for the diagnosis of Proteus Syndrome (PS), hamartomatous disorder of unknown etiology with multiple manifestations. A fifteen years old male, attended to consultation showing cerebriform cutaneous lesion, measuring 17,3 x 10,0 cm, in external and plantar surfaces of the right foot. Two biopsies of 1,5 and 1,2 cm. were initially received, and then surgical specimen measuring 18,0 X 10,5 cm., with cerebriform surface. The material was routinely processed and stained with Hematoxilin - Eosin (H.E.) and Orcein. Histologically it showed extensive fibrosis, involving both papillary and reticular dermis, with densely packed conspicuous collagen bundles, and occasional fat cells. Overlying epidermis with acanthosis and hyperparakeratosis. Macro microscopic findings correspond to Cerebriform Fibrous Proliferation. Its importance is based on the close association with PS. The Pathologist should recommend exhaustive evaluation of the patient in order to detect malformations in limbs and raquis, vascular alterations, hepatic, urogenital, and pulmonary and digestive involvement.

REFERENCES

1. Twede JV, Turner JT, Bieseccker LG, Darling TN. Evolution of skin lesions in Proteus syndrome. J Am Acad Dermatol 2005;52:834-8.

2. Velásquez-Fragua R, Castroviejo P. Dos casos de síndrome de Proteus. An Pediatr 2003;58:496-501.

3. Kenneth-Lyons J. Atlas de malformaciones congénitas. 4a ed. México: McGraw-Hil Interamericana, 1990;pp:508-9.

4. Kenneth Lyons J. Smith. Patrones reconocibles de malformaciones humanas. 6ª ed. Madrid: Elsevier, 2007;pp:600-1.

5. Enzinger FM, Weiss´s SW. Soft tissue tumors. 5th ed. St. Louis: Mosby, 2008;pp:294-5.

6. Winik BC, Boente MC, Asial RA. Cerebriform plantar hyperplasia: ultrastructural study of two cases. Eur J Dermatol 2000;10:551-4.

7. Sarma N, Malakar S, Lahiri K. Case report: unilateral Proteus syndrome. Ind J Dermatol. Venereol Leprol 2005;71:122-4.

8. Cohen MM .Proteus syndrome: an update. Am J Med Genet C Semin Med Genet 2005;137:38-52.

9. Valle LE, Coma MV, Rueda ML, Yarza M, Taslik C. Síndrome de hemihiperplasia - lipomatosis múltiple: a propósito de un caso, diferenciación con el síndrome. Arch Argent Dermatol 2003;53:21-26.

10. Yavuzer R, Uluoglu O, Sari A, Boyacioglu M, et al. Cerebriform fibrous proliferation vs. Proteus syndrome. Ann Plast Surg 2001;47:669-72.

11. Samlaska CP, Levin SW, James WD, Benson PM, et al. Proteus syndrome. Arch Dermatol 1989;125:1109-14.

12. Debi B, Kayak S, Da RP, Acharjya B. Proteus syndrome. Indian J Dermatol Venereol Leprol 2005;71:357-9.

13. Nguyen D, Turner JT, Olsen C, Biesecker LG, Darling TN. Cutaneous manifestations of Proteus syndrome: correlations with general clinical severity. Arch Dermatol 2004;140:947-53.

14. Moise LL. Clínicas pediátricas de Norteamérica. Volumen 4. México: Mc Graw-Hill Interamericana, 2001;pp:850.

15. Burke JP, Bowell R, O’Doherty N. Proteus syndrome: ocular complications. J Pediatr Ophthalmol Strabismus 1988;25:99-102.

16. Newman B, Urbach AH, Orenstein D. Proteus syndrome: emphasis on the pulmonary manifestations. Pediatr Radiol 1994;24:189-93.

17. Farajzadeh S, Zahedi MJ, Moghaddam SD. A new gastrointestinal finding in Proteus syndrome: report of a case of multiple colonic hemangiomas. Int J Dermatol 2006;45:35-138.

18. Karabulut A, Karabulut AA, Sunay M, Emir L, Erol D. Proteus syndrome with multiple genitourinary abnormalities. Urol Int 2006;76:91-93.

19. Bouzas EA, Krasnewich D, Koutroumanidis M, Papadimitriou A, et al. Ophthalmologic examination in the diagnosis of Proteus syndrome. Ophthalmology 1993;100:334-8.