Dermatology: Practical and Conceptual 12 Observation | Dermatol Pract Concept 2018;8(1):3 DERMATOLOGY PRACTICAL & CONCEPTUAL www.derm101.com Case Presentation A 14-year-old boy presented with an asymptomatic solitary skin-colored plaque on the frontal area that had been pres- ent for five years. In the beginning, the plaque was small but gradually progressed to a large 4 x 4 cm plaque on the forehead (Figure 1a). On the dermatological examination, two café-au-lait macules on the trunk were found. General physical examination was normal. The patient’s past medi- cal history revealed no significant findings, such as seizure, neurological deficit, or abnormal development, and trauma and manipulation were absent. Family history for similar problem was also negative. Brain CT showed that the lesion was limited to the outside of the skull bones without intracra- nial extension. Routine laboratory tests, comprising of liver function test, urine analysis, and complete blood count were all normal. He was very unhappy with the lesion and had developed depression due to his classmates’ insults. An incisional skin biopsy was performed which showed increased normal collagen bundles in dermis, mild hyper- keratosis, acanthosis, minimal perivascular inflammation in the upper dermis, and dermal thickening (Figure 2a, b). Verhoeff’s elastic stain (VEG) revealed elastorrhexis, that is, fragmentation and thinning of the elastic fibers within the reticular dermis (Figure 2c). Therefore, an isolated cutaneous collagenoma was diagnosed. Due to the large size and poor cosmetic result of the surgery and the great impact of the lesion on quality of life, Satisfactory treatment of a large connective tissue nevus with intralesional steroid injection Nasrin Saki1,2, Azadeh Dorostkar1,2, Alireza Heiran3, Fatemeh Sari Aslani1,4 1 Molecular Dermatology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran 2 Dermatology Department, Shiraz University of Medical Sciences, Shiraz, Iran 3 Student Research Committee, Shiraz University of Medical Sciences, Shiraz, Iran 4 Pathology Department, Shiraz University of Medical Sciences, Shiraz, Iran Key words: collagenoma, isolated collagenoma, connective tissue nevus, steroid Citation: Saki N, Dorostkar A, Heiran A. Sari Aslani F. Satisfactory treatment of a large connective tissue nevus with intralesional steroid injection. Dermatol Pract Concept. 2018;8(1):12-14. DOI: https://doi.org/10.5826/dpc.0801a03 Received: June 26, 2017; Accepted: October 8, 2017; Published: January 31, 2018 Copyright: ©2018 Saki et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Funding: None. Competing interests: The authors have no conflicts of interest to disclose. All authors have contributed significantly to this publication. Corresponding author: Nasrin Saki, MD, Molecular Dermatology Research Center, Dermatology Department, Shiraz University of Medical Sciences, Shiraz, Iran. Postal Code: 7134844119. E-mail: nasrinsa85@yahoo.com Collagenoma is a type of connective tissue nevi, a rare hamartomatous malformation characterized by the predominant proliferation of normal collagen fibers and normal, decreased, or increased elastic fi- bers. Collagenomas present as multiple or solitary, hereditary or sporadic, asymptomatic, skin-colored papules, nodules, and plaques with variable sizes, and are usually located on the trunk, arm, and back. Here, we report on a 14-year-old boy who presented with an isolated giant collagenoma of the frontal area that dramatically responded to intralesional triamcinolone acetonide. ABSTRACT mailto:nasrinsa85@yahoo.com Observation | Dermatol Pract Concept 2018;8(1):3 13 Conclusion Connective tissue nevi are benign, well-defined hamartomas of the der- mis categorized as collagenomas, elas- tomas, and nevi mucinosis based on the prevailing element of extracellular matrix of dermis. Collagenomas are divided into inherited and acquired types. The inherited form consists of familial cutaneous collagenoma and shagreen patch of tuberous sclerosis, which is autosomal dominant. Familial cutaneous collagenoma presents with multiple lesions and is accompanied by we decided to treat it with four monthly sessions of intralesional triamcinolone acetonide injections. Triamcinolone acetonide 10 mg/ml was injected into the bulk of the con- nective tissue nevus in the first session and 20 mg/ml in the other three sessions. The patient was regularly followed and showed remarkable response with good results after the third injection. At follow-up, eight months after the last session, only the medial border of the lesion had relapsed, which was resolved with a single injection of triamcinolone acetonide 10 mg/ml (Figure 1b, c, d). Figure 1. Trend on the improvement of the collagenoma over one year achieved by four intralesional triamcinolone acetonide injections. [Copyright: ©Saki et al.] cardiac disorders such as cardiomyopa- thy and conduction abnormalities [1-5]. Acquired collagenoma includes eruptive collagenoma and isolated collagenoma. Eruptive collagenoma is described as discrete, firm, skin-colored, and elevated papules, nodules, or plaques distrib- uted on the trunk and extremities sym- metrically [6]. Isolated collagenoma exhibits numerable lesions localized on the palms, soles, and rarely other parts of the body [7,8]. The pathogenesis of collagenoma is ambiguous; however, sporadic collagenoma might be related to trauma, since it appears in areas sub- jected to friction. In addition, collageno- mas might be associated with pseudohy- poparathyroidism, hypogonadism, and Down syndrome [9-11]. Apparently, underproduction of col- lagenase and the consequent diminished collagen degradation is the key event underlying collagenoma pathogenesis [12]. Additionally, the required time for fibroblasts mitosis, abundant within collagenoma, is reduced and this con- tributes to the increase in the collagen bundles [13]. Histologically, in all types of collagenoma, coarse and dense colla- gen fibers accumulate in the dermis and the content of elastic fibers is diminished [14]. To the best of our knowledge, there is no conclusive effective therapy for cuta- neous collagenoma except for surgical removal of the lesions [15]. Three stud- ies investigating the therapeutic effect of triamcinolone acetonide on familial cutaneous collagenoma [16], acquired linear nodular collagenoma [17], and isolated corymbose collagenoma [18] reported the improvement of the lesions in varying degrees. The therapeutic effects of triamcinolone acetonide might be the result of the underproduction of transforming growth factor β1 (TGF- β1) in the fibroblasts and overproduc- tion of basic fibroblast growth factor (bFGF), which in turn inhibits fibroblast mitosis and collagen synthesis [19]. In the present case we tried intralesional triamcinolone acetonide and observed 14 Observation | Dermatol Pract Concept 2018;8(1):3 10. Kakinoma Y, Endo H, Tsukahara T, Futoeda T, Saito Y, Shinkai H. Collagenoma with pseudohypoparathyroidism. Br J Dermatol. 2000;143(5):1122-1124. 11. Sacks HN, Crawley IS, Ward JA, Fine RM. Familial cardio- myopathy, hypogonadism, and collagenoma. Ann Intern Med. 1980;93(6):813-817. 12. Nico MM, Valente NY, Machado KA. Isolated plantar col- lagenoma. Acta Derm Venereol. 2003;83(2):144. 13. Uitto J, Bauer EA, Santa Cruz DJ, Holtmann B, Eisen AZ. De- creased collagenase production by regional fibroblasts cultured from skin of a patient with connective tissue nevi of the collagen type. J Invest Dermatol. 1982;78:136–140. 14. Romiti R, Romiti N. Papulolinear collagenoma. J Am Acad Der- matol. 2004;50:797-798. 15. Al-Breiki SH, Bukhari IA. Childhood solitary collagenoma. Saudi Med J. 2006;27(3):395-396. 16. Dawn ME, Deng AC, Petrali J, Wessely C, Jaffe D, Gaspari AA. Familial cutaneous collagenoma. Skinmed. 2008;7:43–45. 17. Sardana K, Bansal S, Garg VK, Khurana N. Linear nodular col- lagenoma – successful treatment with intralesional triamcinolone acetonide. Pediatr Dermatol. 2009; 26:626–628. 18. Yadav S, Khullar G, Saikia UN, Dogra S. Isolated corymbose collagenoma responding to intralesional triamcinolone acetonide and hyaluronidase injections. Dermatol Ther. 2013;26(5):419- 423. 19. Robles DT, Berg D. Abnormal wound healing: keloids. Clin Der- matol. 2007; 25(1):26-32. a dramatic response. Hence, intralesional triamcinolone ace- tonide may be a promising therapy for non-operable forms of collagenoma. References 1. Botella-Estrada R, Alegre V, Sanmartin O, Ros C, Aliaga A. Isolated plantar cerebrifirm collagenoma. Arch Dermatol. 1991;127(10):1589-1590. 2. Jones MS, Helm KF. A solitary warty plaque. Isolated cerebriform collagenoma. Arch Dermatol. 1997; 133(7):911-912. 3. Kumar S, Singh SK, Banasal A, Banasal M. Isolated collagenoma on the scalp: a rare presentation. Int J Tricology. 2013;5(2):88. 4. Amato L, Mei S, Gallerani I, Moretti S, Cipollini EM, Palleschi GM, et al. Familial cutaneous collagenoma: report of an affected family. Int J Dermatol. 2005;44(4):315-317. 5. Boente Mdel C, Primc NB, Asial RA, Winik BC. Familial cutane- ous collagenoma: a clinicopathologic study of two new cases. Pediatr Dermatol. 2004;21(1):33-38. 6. Ju Q, Song N, Sun J. Eruptive cutaneous collagenoma in a Chinese patient. J Dermatol. 2011;38(4):399-401. 7. Yahya H, Rafindadi AH. Eruptive collagenoma in Nigerian girl. Int J Dermatol. 2006;45(11):1344-1346. 8. Madke B, Doshi B, Nayak C, Prasannan R. Isolated pedunculated collagenoma (collagen nevi) of the scalp. Indian J Dermatol. 2013;58(5):411. 9. Togawa Y, Nohira G, Shinkai H, Utani A. Collagenoma in down syndrome. Br J Dermatol. 2003;148(3):596-597. Figure 2. Microscopic findings: (a) Increased normal collagen bundles in the dermis, mild hyperkeratosis, acanthosis, minimal perivascular inflammation in upper dermis, and dermal thickening (H&E x40). (b) Partial replacement of the subcutaneous fat by broad collagen bundles (H&E x200). (c) Elastorrhexis within reticular dermis (Verhoeff’s elastin stain x200). [Copyright: ©Saki et al.]