Sclerotic Fibroma in a Patient with Birt-Hogg-Dubé Syndrome
Main Article Content
Keywords
Birt-Hogg-Dubé syndrome, BHD, Sclerotic Fibroma, Cowden Syndrome, Multiple Hamartoma Syndrome, Storiform Collagenoma
Abstract
Birt-Hogg-Dubé syndrome (BHD) is a rare genetic disorder characterized by cutaneous lesions and systemic findings such as renal neoplasms, pulmonary cysts, and spontaneous pneumothoraces. Fibrofolliculomas, trichodiscomas, and acrochordons are the most common cutaneous lesions associated with BHD. Some cases with mucocutaneous lesions have also been reported. We report a sclerotic fibroma that developed over a previous thoracotomy scar of a patient with BHD.
References
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2. Stocchero GF. Storiform collagenoma: case report. Einstein (Sao Paulo). Jan-Mar 2015;13(1):103-5. doi:10.1590/s1679-45082015rc2907
3. Lo WL, Wong CK. Solitary sclerotic fibroma. Journal of Cutaneous Pathology. 1990;17(5):269-273. doi:https://doi.org/10.1111/j.1600-0560.1990.tb00101.x
4. Rapini RP, Golitz LE. Sclerotic fibromas of the skin. J Am Acad Dermatol. Feb 1989;20(2 Pt 1):266-71. doi:10.1016/s0190-9622(89)70033-5
5. Metcalf JS, Maize JC, LeBoit PE. Circumscribed storiform collagenoma (sclerosing fibroma). Am J Dermatopathol. Apr 1991;13(2):122-9. doi:10.1097/00000372-199104000-00004
6. Birt AR, Hogg GR, Dubé WJ. Hereditary Multiple Fibrofolliculomas With Trichodiscomas and Acrochordons. Archives of Dermatology. 1977;113(12):1674-1677. doi:10.1001/archderm.1977.01640120042005
7. Gupta N, Seyama K, McCormack FX. Pulmonary manifestations of Birt-Hogg-Dubé syndrome. Fam Cancer. Sep 2013;12(3):387-96. doi:10.1007/s10689-013-9660-9
8. Gunji Y, Akiyoshi T, Sato T, et al. Mutations of the “Birt–Hogg–Dubé” gene in patients with multiple lung cysts and recurrent pneumothorax. Journal of Medical Genetics. 2007;44(9):588-593. doi:10.1136/jmg.2007.049874
9. Pavlovich CP, Walther MM, Eyler RA, et al. Renal tumors in the Birt-Hogg-Dubé syndrome. Am J Surg Pathol. Dec 2002;26(12):1542-52. doi:10.1097/00000478-200212000-00002
10. Patterson JW, Hosler GA, Prenshaw KL. Weedon's skin pathology. Fifth edition ed. Elsevier; 2021.