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Multiple Asymptomatic Papules on the Back of the Right Side of the Chest Angoori Gnaneshwar Rao

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Multiple Asymptomatic Papules on the Back of the Right Side of the Chest Angoori Gnaneshwar Rao QUIZ Multiple Asymptomatic Papules on the Back of the Right Side of the Chest Angoori Gnaneshwar Rao A 43-year-old male presented with multiple asymptomatic complete blood picture, blood sugar, complete urine examination, papules on the back of the right side of the chest of 1 year blood urea, serum creatinine, liver function tests and serum duration. He was asymptomatic a year back then he developed lipid profile were normal. Fundus was normal. A slit skin smear small papules on the right side of the front of the chest initially for acid fast bacilli was negative. A punch biopsy from the and later on involved the front and back of the chest. No representative lesion subjected to histopathological examination history was suggestive of leprosy and hyperlipidemias. Family revealed a cyst with an intricately folded wall, lined by two to history was negative for similar problem. Examination revealed three layers of flattened squamous epithelium and the absence multiple skin-colored to yellowish papules distributed on the of the granular layer. Lobules of sebaceous glands were found front and back of the chest and shoulder region on the right embedded in cyst lining. The lumen was filled with amorphous side [Figure 1]. Also, there were multiple hyperpigmented eosinophilic material and multiple hair shafts [Figures 2-4]. macules on the right infrascapular region. There was no nerve thickening and no sensory deficit and there were no Question hypopigmented or anesthetic patches. Systemic examination did not reveal any abnormality. Routine investigations including What is your diagnosis? (Original) Multiple skin-colored to yellowish papules on the back of chest Figure 1: Figure 2: (Original) Histopathology of skin showing a cyst with an intricately folded and shoulder region on the right side wall lined by two to three layers of flattened squamous epithelium and the absence of granular layer. (H and E stain, ×100) Figure 3: (Original) Histopathology of skin showing a lobule of sebaceous gland (short thick arrow) embedded in cyst lining with amorphous eosinophilic material (long thick arrow) and cluster of hair (long thin arrow) within the cavity of the cyst. Figure 4: (Original) Histopathology of skin showing crenulated keratin (arrow). (H and E stain, ×400) (H and E stain, ×400) Access this article online From the Department of Dermatology, SVS Medical College, Mahbubnagar, Telangana, India Quick Response Code: Website: www.e‑ijd.org Address for correspondence: Prof. Angoori Gnaneshwar Rao, F12, B8, HIG-II APHB, Baghlingampally, Hyderabad, Telangana, India. DOI: 10.4103/0019‑5154.160503 E-mail: [email protected] 417 Indian Journal of Dermatology 2015; 60(4) IJD® QUIZ Answer absence of granular layer. Lobules of sebaceous glands are embedded in cyst lining. The lumen was filled Steatocystoma multiplex with amorphous material. Immunohistochemistry study Discussion shows a calretinin‑positive inner epithelial layer of the cyst.[14] However, immunohistochemical study could not Steatocystoma multiplex (SM) is a hamartomatous be done in the index case. Histopathological differential malformation of the pilosebaceous duct junction, was diagnosis includes eruptive vellus hair cyst, epidermoid first described by Jamiesonin in 1873 and subsequently, cyst, tricholemmal cyst and milium which can be Pringle coined the term in 1899.[1] It is an autosomal differentiated from one another [Table 1]. dominantly inherited disorder but sporadic cases have been reported in the literature.[2] The familial Various treatment modalities have been tried which SM is associated with mutation in the keratin 17 include isotretinoin, cryosurgery, surgical excision and gene and so far 14 mutations have been described laser treatment. Isotretinoin helps in decreasing the size in the literature.[3,4] It may be associated with other of the lesions but does not eradicate the lesions.[15] Of ectodermal dysplasias. The exact origin of the SM is all the treatments, surgical excision and laser therapy not known. However, various theories have been put are known to produce least scarring.[16,17] forward: Consequence of sebaceous retention cysts Learning points of nevoid nature could be hamartomas or could be a variety of dermoid cysts.[5] SM can present as localized, • SM is a hamartomatous malformation of the generalized, facial, acral, and suppurative types.[6] The pilosebaceous duct junction inherited as autosomal solitary lesions are sporadic and they are known as dominant disorder, due to mutation in the keratin 17 steatocystoma simplex. gene • Adulthood is the usual age of onset Adolescence or young adulthood is the usual age of • It presents with multiple skin‑colored to yellowish onset suggesting a relationship with development of smooth‑surfaced papules and nodules predominantly [7] sebaceous glands and hormonal influence. However, distributed on trunk, front of neck, face and axillae sporadic cases with presentation in elderly similar to • Histopathology of the lesion is confirmatory, which the index case have also been reported. It presents shows an intricately folded wall lined by two to with multiple skin‑colored to yellowish smooth‑surfaced three layers of flattened squamous epithelium and papules and nodules predominantly distributed on the absence of the granular layer with lobules of trunk, front of neck, face and axillae, the areas normally sebaceous glands embedded in cyst lining rich in pilosebaceous units. Rarely SM may involve • Treatment modalities include isotretinoin, cryosurgery, [8,9] vulva and soles. Usually asymptomatic but pain may surgical excision and laser treatment. be associated in suppurative form.[10] Both sexes are affected equally. However, higher prevalence among References [11] males has been reported in the literature. It may be 1. Ahn SK, Hwang SM, Lee SH, Lee WS. Steatocystoma multiplex associated with acrokeratosis verruciformis, icthyosis, localized only in the face. Int J Dermatol 1997;36:372‑3. multiple keratacanthomas, hypertrophic lichen planus, 2. Punia RP, Samra SG, Mohan H. Steatocystoma multiplex: pachynochia congenita, hypotrichosis, hydradenitis A report of two sporadic cases. Indian J Pathol Microbiol suppurativa and rheumatoid arthritis.[12,13] However, 2007;50:824‑6. there was no such association in the reported case. 3. Chu HD. Steatocystoma multiplex. Dermatol Online J Nail changes like hypertrophic nail dystrophy have 2003;9:18. been reported in SM. However, such changes could 4. Covello SP, Smith FJ, Sillevis Smitt JH, Paller AS, Munro CS, not be seen in the index case. Ultrasonography shows Jonkman MF, et al. Keratin 17 mutations cause either steatocystoma multiplex or pachyonychia congenita type 2. Br well‑demarcated, unechoic areas within the dermis and J Dermatol 1998;139:475‑80. the subcutis. Histopathology of the lesion is confirmatory 5. Lima AM, Rocha SP, Batista CM, Reis CM, Leal II, Azevedo LE. which shows an intricately folded wall, lined by two to What is your diagnosis. case for diagnosis. An Bras Dermatol three layers of flattened squamous epithelium and the 2011;86:165‑6. Table 1: (Original) histopathological differential diagnosis of steatocystoma multiplex Steatocystoma Eruptive % Epidermoid cyst Tricholemmal cyst Milium multiplex vellus hair cyst Granular layer Absent Present Present Absent Present Vellus hair in the cyst Present Present Absent Absent Absent Sebaceous glands in the cyst wall Present Absent Absent Absent Absent Keratin in the cyst Absent Absent Laminated keratin Homogenous keratin Lamella ted keratin Indian Journal of Dermatology 2015; 60(4) 418 IJD® QUIZ 6. D’Souza P, Joshi A, Gujral S, Ramam M. Linear steatocystoma 14. Riedel C, Brinkmeier T, Kutzne H, Plewig G, Frosch PJ. Late multiplex. Acta Derm Venereol 1997;77:161. onset of a facial variant of steatocystoma multiplex ‑ calretinin 7. Kaur T, Kanwar AJ. Steatocystoma multiplex in four successive as a specific marker of the follicular companion cell layer. generations. J Dermatol 2003;30:559‑61. J Dtsch Dermatol Ges 2008;6:480‑2. 8. Jain M, Puri V, Katiyar Y, Sehgal S. Acral steatocystoma 15. Friedman SJ. Treatment of steatocystoma multiplex and pseudofolliculitis barbae with isotretinoin. Cutis multiplex. Indian Dermatol Online J 2013;4:156‑7. 1987;39:506‑7. 9. Rongioletti F, Cattarini G, Romanelli P. Late onset vulvar 16. Choudhary S, Koley S, Salodkar A. A modified surgical steatocystoma multiplex. Clin Exp Dermatol 2002;27:445‑7. technique for steatocystoma multiplex. J Cutan Aesthet Surg 10. Adams B, Shwayder T. Steatocystoma multiplex suppurativum. 2010;3:25‑8. Int J Dermatol 2008;47:1155‑6. 17. Mumcuoğlu CT, Gurel MS, Kiremitci U, Erdemir AV, Karakoca Y, 11. Ohtake N, Kubota Y, Takayama O, Shimada S, Tamaki K. Huten O. Er: Yag laser therapy for steatocystoma multiplex. Relationship between steatocystoma multiplex and eruptive Indian J Dermatol 2010;55:300‑1. vellus hair cysts. J Am Acad Dermatol 1992;26:876‑8. 12. Hansen KK, Troy JL, Fairley JA. Multiple papules of the How to cite this article: Rao AG. Multiple Asymptomatic Papules on scalp and forehead. Steatocystoma multiplex (facial papular the Back of the Right Side of the Chest. India J Dermatol 2015:60:417-9 variant). Arch Dermatol 1995;131:835‑8. 13. Kumar JC, Siddappa K, Ghosh S. Steatocystoma multiplex. Received: December, 2014. Accepted: February, 2015. Indian J Dermatol Venereol Leprol 1996;62:369‑70. Source of support: Nil, Conflict of Interest: Nil. 419 Indian Journal of Dermatology 2015; 60(4).
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