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E–CASE REPORT
Year : 2013  |  Volume : 58  |  Issue : 5  |  Page : 405
A case of syringocystadenoma papilliferum on lower leg: A rare location


1 Department of Dermatology and Venereology, Hindu Rao Hospital, Delhi, India
2 Department of Pathology, Hindu Rao Hospital, Delhi, India

Date of Web Publication30-Aug-2013

Correspondence Address:
Vinod K Khurana
Department of Dermatology and Venereology; Hindu Rao Hospital, Delhi - 110007
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.117306

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   Abstract 

An 18 years old male presented with a slowly increasing multiple papulonodular lesions on his left leg since birth. No definite diagnosis was made on clinical ground. But verrucous epidermal naevus with secondary change and appendageal tumor was suspected on clinical examination. Histopathological examination revealed syringocystadenoma papilliferm (SCAP). SCAP is very rare on lower leg. Only one case of SCAP on lower leg has been reported in literature so far, which was superimposed on an organoid nevus. The case is reported for unusual location and unusual presentation.


Keywords: Appendageal tumor, organoid naevus, syringocystadenoma papilliferm


How to cite this article:
Khurana VK, Mehta RK, Chaudhary D, Pant L. A case of syringocystadenoma papilliferum on lower leg: A rare location. Indian J Dermatol 2013;58:405

How to cite this URL:
Khurana VK, Mehta RK, Chaudhary D, Pant L. A case of syringocystadenoma papilliferum on lower leg: A rare location. Indian J Dermatol [serial online] 2013 [cited 2020 Jul 14];58:405. Available from: http://www.e-ijd.org/text.asp?2013/58/5/405/117306

What was known?
Syrigocystadenoma papilliferum is a benign tumor of skin commonly appearing in the head and neck region. Usually it is associated with organoid nevus.



   Introduction Top


Syringocystadenoma papilliferm (SCAP) is an uncommon benign tumor of disputed histogenesis with a predilection for the scalp and forehead. Less common sites of involvement are chest, upper arms, ma le breast, eyelids, scrotum, and thigh. [1] We report a case of SCAP on lower leg in a young adult.


   Case Report Top


An 18 years old male reported to skin outpatient department with complaints of slowly progressive multiple raised nodular lesions on the medial side of his left leg since birth. At birth the lesions were slightly raised and reddish in color. The lesions were asymptomatic and gradually increased in size and thickness. The growth of lesion was relatively faster after the onset of puberty.

On examination, there were 4 skin colored papulonodular lesions measuring from 0.6 × 0.6 cm to 1.2 × 1.2 cm in size on medial side of middle 1/3 of left leg. The lesions were firm and non-tender. Thick crusting noted on 2 of the 4 lesion [Figure 1]. Hairs were sparse on the lesions and surrounding skin. There was some atrophic scarring of skin near the larger nodule because of some trauma sustained during childhood. There was history of occasional serous discharge from the lesion since 8 years of age. There was no other finding noted on local examination. On systemic examination, the patient was clinically normal. The routine laboratory investigation including hemogram, Monteux test and X-ray chest was found to be normal. A provisional diagnosis of appendageal tumor was kept in mind. Excision biopsy of one of the nodule was done for histopathological examination (HPE).
Figure 1: Multiple skin colored papulonodular lesions on the left leg with thick crusts in the centre of upper two lesions

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On HPE the epidermis showed papillomatosis. There were cystic invaginations extending downwards from the epidermis. There were multiple papillomatous projections projecting into cystic invaginations [Figure 2]. The papillary projections and lower part of invaginations were lined by two rows of glandular epithelium [Figure 3]. The outer row consisted of small cuboidal cells and inner row displaying columner cells. Some columner cells were also showing decapitation secretion. The papillary projections and dermis had dense infiltration of plasma cells [Figure 4]. A final diagnosis of SCAP was considered.
Figure 2: Photomicrograph showing papillomatosis of epidermis with cystic invagination and papillary projection (H and E, ×100)

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Figure 3: Photomicrograph displaying papillary projections and cystic invagination lined by two rows of cells (H and E, ×100)

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Figure 4: Photomicrograph depicting numerous plasma cells (H and E, ×400)

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   Discussion Top


SCAP was first described by John Stokes in 1917 under the term nevus syringoadenomatous papilliferum. It's a rare cutaneous adnexal neoplasm with variable clinical appearance and characteristic histology. [2] It appears most commonly in head and neck. In 40% of cases, it arises in sebaceous nevus. It may be present, at birth or appear in early life. The lesions frequently increase in size and become papillomatous or verrucous at puberty. SCAP has been commonly described in head (on scalp, forehead, cheeks, pinna and eyelids) and neck region. It's also rarely reported on several uncommon sites like axilla, lower back, abdomen, scrotum and thigh. SCAP has been reported presenting as a cutaneous horn on scalp over temporal region. [3] As of now, there has been just one case report of SCAP on lower leg from Japan. [4]

SCAP is a benign hamartomatous tumor of skin. Histogenesis of this tumor is still controversial. [5] The evidence of decapitation secretion in the luminal cells of SCAP suggests an apocrine origin. But it is paradoxical to observe the fact that 90% of SCAP are found on location devoid of apocrine glands. [6] Pinkus stated that presence of more or less malformed pilosebaceous complex is obligatory and furnish the hamartomatous foundation on which the tumor develops. [7] Mammino and Vidmar after reviewing 145 cases concluded that histogenesis of SCAP is a curious mix of eccrine and apocrine elements on the basis of microscopic, immunohistochemical and ultrastructural studies. [8]

Studies by Yamamoto using anticytokeratin antibodies and several other markers in SCAP support the classical concept that SCAP is a hamartomatous tumor that arises from pluripotent cells. In another study by Kurokawa et al from Japan based on cytokeratin expression (CK), squamous epithelium in SCAP seems to differentiate towards infrainfundibulum. [9] SCAP shows focal positivity to CK-15 a relatively specific marker of hair follicle stem cells in the bulge. [10] Moss et al., showed mutation in PTCA or P-16 tumor suppressor gene in SCAP. [10] Basal cell carcinoma, rarely secondary adenocarcinoma (syringocystadenocarcinoma papilliferum) and apocrine ductal carcinoma may develop in SCAP. [10]

SCAP is usually a benign tumor and surgical excision is curative. To conclude, SCAP is an uncommon tumor and location on leg is a rarity. We must consider it in the differential diagnosis of any longstanding nodular lesion with crusted surface that may ooze serosanguinous fluid because this is a reflection of the fact that the papillae of a SCAP are in continuity with the epidermal surface permitting extrusion of secretory products. [11]

 
   References Top

1.Tumors of cutaneous appendages page 780. In: Weedon's Skin Pathology 3 rd ed. Weedon D. editor, Churchill Livingstone Elsevier; UK, 2010.  Back to cited text no. 1
    
2.Rammeh-Rommani S, Fezaa B, Chelbi E, Kammoun M, Ben Zilani S, Zermani R. Syringocystadenoma papilliferum: Report of 8 cases. Pathologica 2006;98:178-80.  Back to cited text no. 2
    
3.Wen SY. Syringocystadenoma presenting as a cutaneous horn: British association of dermatologist. Br J Dermatol 2000; 142:1242-4.  Back to cited text no. 3
[PUBMED]    
4.Yoshii N, Kanekura T, Setoyama M, Kanzaki T. Syringocystadenoma papilliferum: Report of first case on the lower leg. J Dermatol 2004;31:939-42.  Back to cited text no. 4
[PUBMED]    
5.Yamamoto O, Doi Y, Hamada T, Hisaoka M, Sasaguri Y. An immunological and ultrastructural study of syringocystadenoma. Br J Dermatol 2002;31:936-45.  Back to cited text no. 5
    
6.Malhotra P, Singh A, Ramesh V. Syringocystadenoma papilliferumon the thigh: An unusual location. Indian J Dermatol Venereol Leprol 2009;75:170-2.  Back to cited text no. 6
[PUBMED]  Medknow Journal  
7.Pinkus H. Life history of naevus syringocystadenoma papilliferum. Arch Dermatol Syph 1954;69:305-22.  Back to cited text no. 7
    
8.Mammino JJ, Vidmar DA. Syringocystadenoma papilliferum. Int J Dermatol 1991;30:763-6.  Back to cited text no. 8
[PUBMED]    
9.Kurokawa I, Mizutani S, Nishijima S, Kusumoto K, Tsubura A. Keratinizing squamous epithelium associated with syringocystadenoma papilliferum differentiate towards infrainfundibulum: A case report with immunohistochemical study of cytokeratins. J Int Med Res 2005;33:590-3.  Back to cited text no. 9
    
10.Moss C, Sahidullah H. Naevi and other developmental defects page 18.15. In: Burn T, Breathnach S, Cox N, Griffiths C, editors. Rook's Text Book of Dermatology 8 th ed. Blackwell Publishing; UK, 2010.  Back to cited text no. 10
    
11.McCalmont Timothy H. Adnexal Neoplasms page 1706-7 in Dermatology. 2 nd ed. In: Bolognia JL, Jorizzo JL, Rapini RP, editors. Mosby Elsevier; Spain. 2008.  Back to cited text no. 11
    

What is new?
Syrigocystadenoma papilliferum can rarely develop on leg. It is not always associated with organoid nevus. We must suspect the diagnosis irrespective of its location and consider histopathological examination for confirmation.


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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