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CASE SNIPPET
Year : 2012  |  Volume : 58  |  Issue : 3  |  Page : 210-211

Steatocystoma multiplex - an uncommon lesion with special emphasis on cytological features and cyto-histological correlation


Department of Pathology, Subharti Medical College, NH-58, Delhi Haridwar Bypass, Meerut, Uttar Pradesh, India

Date of Web Publication26-Sep-2012

Correspondence Address:
P Elhence
Department of Pathology, Subharti Medical College, NH-58, Delhi Haridwar Bypass, Meerut, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0022-3859.101405

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How to cite this article:
Elhence P, Bansal R, Sharma S, Jain V. Steatocystoma multiplex - an uncommon lesion with special emphasis on cytological features and cyto-histological correlation. J Postgrad Med 2012;58:210-1

How to cite this URL:
Elhence P, Bansal R, Sharma S, Jain V. Steatocystoma multiplex - an uncommon lesion with special emphasis on cytological features and cyto-histological correlation. J Postgrad Med [serial online] 2012 [cited 2019 Nov 14];58:210-1. Available from: http://www.jpgmonline.com/text.asp?2012/58/3/210/101405


A 40-year-old man presented with multiple, brown to tan-colored, non-tender swellings of two years' duration with a clinical differential diagnosis of diffuse lipomatosis and neurofibromatosis. These swellings were present over the chest, abdomen [Figure 1], nape of the neck and back and ranged in size from 0.5 cm to 2.5 cm. The lesions did not have any punctum nor had any lesion ruptured since its appearance. His nails were normal. No family history of similar lesions was present. Fine needle aspiration cytology (FNAC) was done from three of the cysts. On aspiration, yellowish to dirty brown fluid was aspirated. Smears revealed crystalline structures of varying size and shape [Figure 2], amorphous debris and cells resembling anucleate squames. The crystals were not examined under polarizer. Other epithelial or inflammatory cells could not be identified. A diagnosis of benign cystic lesions was given with recommendation for excision biopsy and further investigation of serum lipid profile and uric acid to rule out hypercholesterolemia and gouty tophi. The lipid profile and serum uric acid were found to be within normal limits subsequently.
Figure 1: Clinical photograph of patient with multiple cystic lesions

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Figure 2: Crystalline structures of varying sizes and shapes, Leishman- Giemsa stain, 400×

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An excision biopsy was performed from one cystic lesion on the trunk. On microscopic examination, sections showed a cyst lined by flattened to stratified squamous epithelium without a granular layer and lobules of sebaceous glands embedded in the cyst wall [Figure 3]. The luminal surface of the cyst wall revealed crenated homogeneous eosinophilic cuticle and the lumen was filled with scanty amorphous keratinous material. A diagnosis consistent with steatocystoma multiplex was given.
Figure 3: Cyst lining with lobules of sebaceous glands and crenated eosinophilic cuticle, Hematoxylin-Eosin stain, 400×

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Steatocystoma multiplex is an inherited condition classically transmitted in an autosomal dominant manner that usually presents in adolescence or early adult life and is characterized by multiple, skin-colored, dome-shaped cystic lesions and nodules over the chest, trunk, proximal extremities, etc. [1] Their size ranges from 3 mm to 3 cm. The inherited form of steatocystoma multiplex is associated with Keratin 17 (K17) gene mutations which are not observed in the sporadic cases. [2] An association with hypertrophic lichen planus and pachyonychia congenita has also been documented. [2] An extensive search of literature revealed only one article by Oertel and Scott in which fine needle aspiration of three cases of steatocystoma multiplex have been discussed. [3]

An average age of 26 years at onset with no gender or racial predilection has been noted. [4],[5] The present case is that of a middle-aged adult with no family history of similar lesions and no evidence of other associated lesions.

It is an uncommon benign disorder of the pilosebaceous unit characterized by numerous asymptomatic sebum-containing dermal cysts though they may cause disfigurement due to inflammation, formation of sinus tracts or scarring as seen in the inflammatory variant, steatocystoma suppurativa. [1] Presentation in the form of a solitary lesion is known as steatocystoma simplex. This patient presented with multiple non-tender cysts which were cosmetically disfiguring.

Classically, the common sites involved are regions with greater density of pilosebaceous glands, usually the trunk, axilla, proximal extremities and chest but lesions may occur on the scrotum, thighs and back. [6] Acral steatocystoma multiplex has been described recently. [1],[7]

On FNAC, these cysts yield a syrupy fluid, the lipid contents of which get removed on histopathology processing. Oertel and Scott aspirated thick, whitish to yellowish, oily material from three patients' smears which revealed predominantly acellular, granular debris, rare anucleated squamous cells and rare cholesterol crystals. [3] In our case, crystalline structures were seen on the smears which were not subsequently demonstrated on histopathology sections. It was contemplated that as the crystals were seen only on Leishman-Giemsa stain on FNAC and not on smears fixed in either Hematoxylin-Eosin or Papanicolaou stain, the various concentrations of alcohols used in histopathology processing probably resulted in dissolution of these crystals.

On histopathology, these cysts are located in the mid-dermis and characterized by folded and crenated cyst wall lined by stratified squamous epithelium with wavy corrugated eosinophilic cuticle towards the luminal surface. [1] No granular layer is usually seen. The differential diagnosis of steatocystoma includes eruptive vellus hair cysts, trichilemmal cysts and infundibular cysts. Shet, Rege and Naik analyzed FNAC smears from 12 pilar cysts and 40 epidermal cysts with respect to background, cellularity, and amount of keratin, and correlated the relevant clinical data with histopathological features. They concluded that diagnosis on FNAC is possible and can be suspected on cytology as aspirates from epidermal cysts showed a clean background, singly dispersed anucleate and nucleate squames and relatively less keratinous material as compared to pilar cysts. [8] Hybrid lesions comprising histologic features of both steatocystoma and vellus hair cysts have been described. [9] Similarly, eruptive vellus hair cysts (EHVC) can be confirmed as vellus hairs may be demonstrated. Kaya et al., confirmed EHVC with a 10% KOH wet mount of cyst contents expressed via a small incision. [10] Hence, while examining a patient with multiple cystic lesions, steatocystoma multiplex should be considered in the differential diagnosis and the diagnosis can be suspected on cytology.

 
 :: References Top

1.Weedon D. Weedon′s Skin Pathology. 3 rd ed. Churchill Livingstone Elsevier; 2010. p. 448.  Back to cited text no. 1
    
2.Davey MA, Elston DM, Burkhart CN, Morrel DS, Farmer ER, Vinson RP, et al. Steatocystoma Multiplex. [Internet]. Available from: http://www.emedicine.medscape.com/article/1059725-overview [Last cited on 2009 April 14].  Back to cited text no. 2
    
3.Oertel YC, Scott DM. Fine needle aspiration of three cases of steatocystoma multiplex. Ann Diagn Pathol 1998;2:318-20.  Back to cited text no. 3
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4.Cho S, Chang SE, Choi JH, Sung KJ, Moon KC, Koh JK. Clinical and histologic features of 64 cases of steatocystoma multiplex. J Dermatol 2002;29:152-6.  Back to cited text no. 4
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5.Siadat AH, Adibi N, NilfroushZadeh MA, Rajabi P, Darougheh A, Baghbaderani AZ, et al. Dermatology case diagnosis. J Res Med Sci 2007;12:222-5.  Back to cited text no. 5
    
6.Naik NS. Steatocystoma multiplex. Dermatol Online J 2000;6:10.  Back to cited text no. 6
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7.Rollins T, Levin RM, Heymann WR. Acral steatocystoma multiplex. J Am Acad Dermatol 2000;43:396-9.  Back to cited text no. 7
[PUBMED]    
8.Shet T, Rege J, Naik L. Cytodiagnosis of simple and proliferating trichilemmal cysts. Acta Cytol 2001;45:582-8.  Back to cited text no. 8
[PUBMED]    
9.Ahn SK, Chung J, Lee WS, Lee SH, Choi EH. Hybrid cysts showing alternate combination of eruptive vellus hair cyst, steatocystoma multiplex, and epidermoid cyst, and an association among the three conditions. Am J Dermatopathol 1996;18:645-9.  Back to cited text no. 9
[PUBMED]    
10.Kaya TI, Tataroglu C, Tursen U, Ikizoglu G. Eruptive vellus hair cysts: An effective extraction technique for treatment and diagnosis. J Eur Acad Dermatol Venereol 2006;20:264-8.  Back to cited text no. 10
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