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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 1  |  Issue : 1  |  Page : 42-44

Steatocystoma multiplex masquerading as neurofibromas


Department of Pathology, Shrimati Kashibai Navale Medical College and General Hospital, Pune, Maharashtra, India

Date of Web Publication24-Jun-2014

Correspondence Address:
Neha Chandrakumar Bhatt
E-517, The Island, Wakad, Pune - 411 057, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


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  Abstract 

A 26-year-old female patient presented with multiple, smooth, round to oval, well-defined, and skin-colored nodules, without central punctum, over both axillae since past 5 years. Few nodules were observed on chest, right upper arm, and left upper thigh. No other ectodermal abnormalities were observed in the patient. There was no family history of similar lesions. A clinical diagnosis of multiple neurofibromas was made, and largest of the lesion was excised. Histopathological examination showed features of steatocystoma multiplex. Steatocystoma multiplex is an extremely rare cystic disorder of pilosebaceous units. Although previously regarded as an inherited autosomal dominant disorder, sporadic cases have been reported.

Keywords: Pilosebaceous unit, sporadic, steatocystoma multiplex


How to cite this article:
Bhatt NC, Khandeparkar SG, Joshi A, Bhoge A. Steatocystoma multiplex masquerading as neurofibromas. Indian J Dermatopathol Diagn Dermatol 2014;1:42-4

How to cite this URL:
Bhatt NC, Khandeparkar SG, Joshi A, Bhoge A. Steatocystoma multiplex masquerading as neurofibromas. Indian J Dermatopathol Diagn Dermatol [serial online] 2014 [cited 2021 Jun 14];1:42-4. Available from: https://www.ijdpdd.com/text.asp?2014/1/1/42/135198


  Introduction Top


Steatocystoma multiplex is an extremely rare cystic disorder of pilosebaceous units, the exact incidence of which is unknown. It was first described by Jamieson in 1873 and subsequently the term was coined by Pringle in 1899. [1] Although previously regarded as an inherited autosomal dominant disorder, sporadic cases have been reported. When inherited, these cases may be associated with other ectodermal dysplasias. Here, we present a sporadic case of this disorder - a young female presenting with multiple nodules in both the axillae, clinically mimicking neurofibromas. [2]


  Case Report Top


A 26-year-old female patient presented with multiple, smooth, round to oval, well-defined, and skin-colored nodules, without central punctum, over both axillae. Few nodules were observed on chest, right upper arm, and left upper thigh. These nodules were first observed by the patient 5-years back and have been increasing in size gradually since then. The patient had no other symptoms except for mild tenderness. A clinical diagnosis of neurofibroma was made, and the largest lesion was excised. On histopathological examination, a cyst with intricately folded wall, lined by 2-3 layers of flattened squamous epithelium lacking granular layer was seen. Lobules of sebaceous glands were embedded in cyst lining. The lumen was filled with amorphous eosinophilic material and few hair shafts [Figure 1]. Periodic acid -Schiff (PAS) stain accentuated cyst lining [Figure 2]. Past history revealed history of excision of similar swelling measuring 1.5 × 1 × 0.5 cm over right arm around 4-years back, which healed by scar formation. On further probing, patient revealed presence of fever, redness, and tenderness of the swelling and edema of the arm as direct reason for previous surgery. The histopathological examination of this swelling could not reveal the exact nature of the lesion because of dissolution of cyst wall, it being replaced by hemorrhages and acute on chronic inflammatory cells. There was no history of any other ectodermal dysplasias or history of similar lesions in the family.
Figure 1: Intricately folded cyst wall lined by flattened stratified squamous epithelium with absence of granular layer, flattened sebaceous lobules embedded within the cyst lining, and hair shafts within the cyst (arrow) (H and E, ×10). H and E = Hematoxylin and eosin

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Figure 2: Accentuated epithelial lining (PAS, ×10). PAS = Periodic acid– Schiff

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  Discussion Top


Steatocystoma multiplex is an uncommon disorder characterized by presence of multiple variably sized dermal cyst arising from abnormal dilatation of pilosebaceous units. Previously considered to be an inherited autosomal dominant disorder, sporadic cases have been lately reported.

The usual age of presentation is adolescence or young adulthood, suggesting a relation with development of sebaceous glands and hormonal influence on growth of the lesion. [1],[3] Our patient is 26-years old, and earliest noticeable lesions were present when she was 21-years old, which is the usual age for presentation of this lesion. Both the sexes are equally affected.

Multiple yellow- to skin-colored, well-defined, smooth-surfaced, and firm nodules ranging in size from 0.3 mm to 3 cm are seen over anterior face, trunk, arms, and axillae where normally pilosebaceous units are numerous. Individual case reports involving the foot and vulva have been reported. [4],[5] Our case presented with lesions over both axillae, right upper arm, chest, and left upper thigh. Many associated findings have been reported in patients with steatocystoma multiplex such as ichthyosis, koilonychia, acrokeratosis verruciformis of Hopf, hyperkeratotic lichen planus, hidradenitis suppurativa, hypotrichosis, multiple keratoacanthomas, and rheumatoid arthritis and pachyonychia congenital. [6],[7]

In our case, there were no other associated ectodermal dysplasias or autoimmune disorders and any positive family history. Usually the lesions are asymptomatic, however, when complicated by inflammation, rupture, or hemorrhage may present with local redness, pain, tenderness, and fever. When inflammatory involvement is extensive, the condition is referred to as steatocystoma multiplex suppurativa. [1] Our patient had history of excision of such inflamed lesion. Inflammatory healing or conservative surgical methods of removal usually results in disfiguring scars with associated psychosocial stigma, which these young patients have to face.

Many treatment options have been tried like antibiotics (in Steatocystoma multiplex suppurativa), isotretinoin, aspiration of lesions, surgical excision, cryosurgery, and laser treatment. Out of these, incisional excision and laser therapy have been shown to produce least scarring. [8],[9] One study mentions yttrium aluminum garnet (YAG) laser treatment showing good results with minimal scarring and no recurrences. [10]

Our experience with present case highlights the importance of appropriate diagnosis of this lesion and its differentiation from other clinical mimickers like neurofibromas and lipomatosis for optimal treatment of the patient.

 
  References Top

1.Fekete GL, Fekete JE. Steatocystoma multiplex generalisata partially suppurativa: Case report. Acta Dermatovenerol Croat 2010;18:114-9.  Back to cited text no. 1
    
2.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.  Back to cited text no. 2
[PUBMED]  Medknow Journal  
3.Kaur T, Kanwar AJ. Steatocystoma multiplex in four successive generations. J Dermatol 2003;30:559-61.  Back to cited text no. 3
    
4.Jain M, Puri V, Katiyar Y, Sehgal S. Acral steatocystoma multiplex. Indian Dermatol Online J 2013;4:156-7.  Back to cited text no. 4
[PUBMED]  Medknow Journal  
5.Rongioletti F, Cattarini G, Romanelli P. Late onset vulvar steatocystoma multiplex. Clin Exp Dermatol 2002;27:445-7.  Back to cited text no. 5
    
6.Hansen KK, Troy JL, Fairley JA. Multiple papules of the scalp and forehead. Steatocystoma multiplex (facial papular variant). Arch Dermatol 1995;131:835,8.  Back to cited text no. 6
    
7.Kumar JC, Siddappa K, Ghosh S. Steatocystoma multiplex. Indian J Dermatol Venereol Leprol 1996;62:369-70.  Back to cited text no. 7
[PUBMED]  Medknow Journal  
8.Choudhary S, Koley S, Salodkar A. A modified surgical technique for steatocystoma multiplex. J Cutan Aesthet Surg 2010;3:25-8.  Back to cited text no. 8
[PUBMED]  Medknow Journal  
9.Schmook T, Burg G, Hafner J. Surgical pearl: Mini-incisions for the extraction of steatocystoma multiplex. J Am Acad Dermatol 2001;44:1041-2.  Back to cited text no. 9
    
10.Mumcuoðlu 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.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2]



 

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