Indian Journal of Dermatology
QUIZ
Year
: 2015  |  Volume : 60  |  Issue : 4  |  Page : 417--418

Multiple asymptomatic papules on the back of the right side of the chest


Angoori Gnaneshwar Rao 
 Department of Dermatology, SVS Medical College, Mahbubnagar, Telangana, India

Correspondence Address:
Prof. Angoori Gnaneshwar Rao
F12, B8, HIG-II APHB, Baghlingampally, Hyderabad, Telangana
India




How to cite this article:
Rao AG. Multiple asymptomatic papules on the back of the right side of the chest.Indian J Dermatol 2015;60:417-418


How to cite this URL:
Rao AG. Multiple asymptomatic papules on the back of the right side of the chest. Indian J Dermatol [serial online] 2015 [cited 2019 Oct 15 ];60:417-418
Available from: http://www.e-ijd.org/text.asp?2015/60/4/417/160503


Full Text

A 43-year-old male presented with multiple asymptomatic papules on the back of the right side of the chest of 1 year duration. He was asymptomatic a year back then he developed small papules on the right side of the front of the chest initially and later on involved the front and back of the chest. No history was suggestive of leprosy and hyperlipidemias. Family history was negative for similar problem. Examination revealed multiple skin-colored to yellowish papules distributed on the front and back of the chest and shoulder region on the right side [Figure 1]. Also, there were multiple hyperpigmented macules on the right infrascapular region. There was no nerve thickening and no sensory deficit and there were no hypopigmented or anesthetic patches. Systemic examination did not reveal any abnormality. Routine investigations including complete blood picture, blood sugar, complete urine examination, blood urea, serum creatinine, liver function tests and serum lipid profile were normal. Fundus was normal. A slit skin smear for acid fast bacilli was negative. A punch biopsy from the representative lesion subjected to histopathological examination revealed a cyst with an intricately folded wall, lined by two to three layers of flattened squamous epithelium and the absence of the granular layer. Lobules of sebaceous glands were found embedded in cyst lining. The lumen was filled with amorphous eosinophilic material and multiple hair shafts [Figure 2] [Figure 3] [Figure 4].{Figure 1}{Figure 2}{Figure 3}{Figure 4}

Question

What is your diagnosis?

 View AnswerAnswer

Steatocystoma multiplex

 Discussion



Steatocystoma multiplex (SM) is a hamartomatous malformation of the pilosebaceous duct junction, was first described by Jamiesonin in 1873 and subsequently, Pringle coined the term in 1899. [1] It is an autosomal dominantly inherited disorder but sporadic cases have been reported in the literature. [2] The familial SM is associated with mutation in the keratin 17 gene and so far 14 mutations have been described in the literature. [3],[4] It may be associated with other ectodermal dysplasias. The exact origin of the SM is not known. However, various theories have been put forward: Consequence of sebaceous retention cysts of nevoid nature could be hamartomas or could be a variety of dermoid cysts. [5] SM can present as localized, generalized, facial, acral, and suppurative types. [6] The solitary lesions are sporadic and they are known as steatocystoma simplex.

Adolescence or young adulthood is the usual age of onset suggesting a relationship with development of sebaceous glands and hormonal influence. [7] However, sporadic cases with presentation in elderly similar to the index case have also been reported. It presents with multiple skin-colored to yellowish smooth-surfaced papules and nodules predominantly distributed on trunk, front of neck, face and axillae, the areas normally rich in pilosebaceous units. Rarely SM may involve vulva and soles. [8],[9] Usually asymptomatic but pain may be associated in suppurative form. [10] Both sexes are affected equally. However, higher prevalence among males has been reported in the literature. [11] It may be associated with acrokeratosis verruciformis, icthyosis, multiple keratacanthomas, hypertrophic lichen planus, pachynochia congenita, hypotrichosis, hydradenitis suppurativa and rheumatoid arthritis. [12],[13] However, there was no such association in the reported case. Nail changes like hypertrophic nail dystrophy have been reported in SM. However, such changes could not be seen in the index case. Ultrasonography shows well-demarcated, unechoic areas within the dermis and the subcutis. Histopathology of the lesion is confirmatory which shows an intricately folded wall, lined by two to three layers of flattened squamous epithelium and the absence of granular layer. Lobules of sebaceous glands are embedded in cyst lining. The lumen was filled with amorphous material. Immunohistochemistry study shows a calretinin-positive inner epithelial layer of the cyst. [14] However, immunohistochemical study could not be done in the index case. Histopathological differential diagnosis includes eruptive vellus hair cyst, epidermoid cyst, tricholemmal cyst and milium which can be differentiated from one another [Table 1].{Table 1}

Various treatment modalities have been tried which include isotretinoin, cryosurgery, surgical excision and laser treatment. Isotretinoin helps in decreasing the size of the lesions but does not eradicate the lesions. [15] Of all the treatments, surgical excision and laser therapy are known to produce least scarring. [16],[17]

Learning points

SM is a hamartomatous malformation of the pilosebaceous duct junction inherited as autosomal dominant disorder, due to mutation in the keratin 17 geneAdulthood is the usual age of onsetIt presents with multiple skin-colored to yellowish smooth-surfaced papules and nodules predominantly distributed on trunk, front of neck, face and axillaeHistopathology of the lesion is confirmatory, which shows an intricately folded wall lined by two to three layers of flattened squamous epithelium and the absence of the granular layer with lobules of sebaceous glands embedded in cyst liningTreatment modalities include isotretinoin, cryosurgery, surgical excision and laser treatment.

First three correct entries are:

Sarina Jain, FaridkotKeshavmurthy Adya, SBMP Medical CollegeAtishay Bukharia, RIMS, Ranchi

"Others who have sent correct answers are mentioned below:"

Anup Kumar Tiwary, Monali Pattnaik, Akhilesh Shukla, Neha virmani, Vinay K, Surajit Gorai, Gillian Ruth Britto, Nidhi Kamra, G. V. Seethalakshmi, Vishal Gupta, Riti Bhatia, Mrinal Gupta, Poonam Puri, Vishalakshi S. Pandit, Sandeep Kaur, Urmi Khanna, Meenaz Khoja, Dr. Anuradha, Smita Rani Samal, Dipti Das, Shailee Patel , Geeti Khullar, Shiti Bose, Priyanka Jain, Mahima Agrawal, R.Kusuma, Rinu Ruth George, Gautham krishna reddy, Shouvik Ghosh, Taniya Sharma, Ashish Amrani, Sanchika Gupta, S. Murugan, C. Vijay Krishna, Anuj Tenani, Ganesh Avhad, P. M. Kheni, C.Aruna, Minu Jose Chiramel, Ankita Srivastava, Surekha Avvaru, S. Chidambara Murthy

References

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4Covello SP, Smith FJ, Sillevis Smitt JH, Paller AS, Munro CS, Jonkman MF, et al. Keratin 17 mutations cause either steatocystoma multiplex or pachyonychia congenita type 2. Br J Dermatol 1998;139:475-80.
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14Riedel C, Brinkmeier T, Kutzne H, Plewig G, Frosch PJ. Late onset of a facial variant of steatocystoma multiplex - calretinin as a specific marker of the follicular companion cell layer. J Dtsch Dermatol Ges 2008;6:480-2.
15Friedman SJ. Treatment of steatocystoma multiplex and pseudofolliculitis barbae with isotretinoin. Cutis 1987;39:506-7.
16Choudhary S, Koley S, Salodkar A. A modified surgical technique for steatocystoma multiplex. J Cutan Aesthet Surg 2010;3:25-8.
17Mumcuoðlu CT, Gurel MS, Kiremitci U, Erdemir AV, Karakoca Y, Huten O. Er: Yag laser therapy for steatocystoma multiplex. Indian J Dermatol 2010;55:300-1.