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Table of Contents 
E–CASE REPORT
Year : 2013  |  Volume : 58  |  Issue : 5  |  Page : 409
Linear syringocystadenoma papilliferum: A case report with review of literature


1 Department of Dermatology, STD and Leprosy, Dr. Ram Manohar Lohia Hospital and PGIMER, New Delhi, India
2 Department of Pathology, Dr. Ram Manohar Lohia Hospital and PGIMER, New Delhi, India

Date of Web Publication30-Aug-2013

Correspondence Address:
Amrita Chauhan
Department of Dermatology, STD and Leprosy Dr. RML Hospital and PGIMER, Baba Kharag Singh Marg, New Delhi - 110 001
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.117353

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   Abstract 

We report a rare case of syringocystadenoma papilliferum (SCAP) presenting as multiple papulonodules in a linear pattern over right lower abdomen which has been reported only once before.


Keywords: Adnexal tumour, linear, syringocystadenoma papilliferum


How to cite this article:
Chauhan A, Gupta L, Gautam RK, Bhardwaj M, Gopichandani K. Linear syringocystadenoma papilliferum: A case report with review of literature. Indian J Dermatol 2013;58:409

How to cite this URL:
Chauhan A, Gupta L, Gautam RK, Bhardwaj M, Gopichandani K. Linear syringocystadenoma papilliferum: A case report with review of literature. Indian J Dermatol [serial online] 2013 [cited 2020 Aug 4];58:409. Available from: http://www.e-ijd.org/text.asp?2013/58/5/409/117353

What was known?
Syringocystadenoma papilliferum is an uncommon hamartomatous tumor which is most commonly located on face and scalp.



   Introduction Top


Syringocystadenoma papilliferum (SCAP) is an uncommon benign hamartomatous adnexal tumor. It presumably arises from pleuripotential cells and histology exhibit either apocrine or eccrine differentiation; however, it is still classified under tumors with apocrine gland differentiation. [1] The lesions are usually seen at birth or in childhood. Most tumors are located on face and scalp, often with co-existent Nevus Sebaceus. [2] SCAP frequently presents as multiple warty papules which may be translucent or pigmented.The lesions increase in size with papillomatous expansion at or around the time of puberty. Histopathology is confirmatory and immunohistochemistry further differentiates between apocrine or eccrine origin.


   Case Report Top


A 10-year-old girl presented with multiple asymptomatic papules over right lower abdomen present since the age of 2 months. The lesions were gradually progressive with a rapid enlargement in the last 1 year. There was no history of any neurological, ocular or skeletal abnormality. On examination there were multiple erythematous papules and coalescent plaques (0.5 cm to 5 cm) in a linear pattern over the right lower abdomen [Figure 1]. Most papules were discrete, pink, dome shaped with a few papules showing central umblication and crusting. The papules coalesced to form numerous papillomatous plaques of varying sizes (1 to 5 cm). Yellowish slough and crusting was seen on some of the lesions. The discharge from the lesions foul smelt. No regional lymphadenopathy was present. The routine hematological and biochemical tests, chest radiograph and abdominal ultrasonography were normal.
Figure 1: Linear multiple erythematous papules and plaques with crusting on the right lower abdomen

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The skin biopsy revealed cystic invagination of epidermis with papillary projection lined by two rows of cells. There were columnar cells towards the lumen and cuboidal cells in the outer layer. Decapitation was seen in the luminal columnar cell layer.The fibrovascular core of papillae showed mixed inflammatory infiltrate comprising of plasma cells, lymphocytes and neutrophils. The underlying deep dermis showed apocrine sweat glands [Figure 2]. The histopathologic features were consistent with the diagnosis of syringocystadenoma papilliferum. Immunohistochemistry (IHC) staining using epithelial membrane antigen (EMA), CD56, CK 19, CK 5, P 63 and smooth muscle antigen (SMA) revealed EMA positivity in columnar cells. Focal positivity of CD 56 was seen in columnar cells. CK 19 was positive in both columnar and basal cells. CK 5 and p63 were positive in basal cells.SMA was focally positive in basal cells [Figure 2]. These findings were consistent with the diagnosis of SCAP of apocrine origin. There was no evidence of malignant transformation. The patient was referred to Plastic surgery for surgical excision with skin grafting. However, the patient was lost to follow up.
Figure 2: (H and E, ×40) Cystic invagination of epidermis with papillary projection and ductal invaginations extending into the dermis

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   Discussion Top


Syringocystadenoma papilliferum is a benign hamartomatous adnexal tumor. It was earlier known by the names of Adenoma cystoma intracanaliculare [3] and Nevus syringoadenomatous papilleferus. [4] It is an uncommon tumor but surprisingly, Saha et al. in 2011, reported 17.39% SCAP among a small series of 30 cases of adnexal tumors studied by them. [5] It is postulated that SCAP arises from the pleuripotent cells with the potential to exhibit either apocrine or eccrine lineage of which apocrine differentiation is more common. The tumor may arise within a Nevus sebaceus (30%). There are three recognized clinical forms; plaque, solitary nodular, and linear. The linear variant is rare and only 14 cases of linear SCAP have been reported. [6],[7],[8],[9],[10] So far only 1 report of linear SCAP over abdomen is available. [11]

Most of the SCAP's are sporadic cases and are diagnosed on histopathology. The clinical presentation is often non-specific and misleading. The majority of SCAP present as solitary lesion over the head and neck region often in association with a nevus sebaceus. The uncommon sites include trunk, arms, breast, eyelids, axilla, scrotum, lower limb, inguinal and perineal regions. [12] The localization of SCAP over abdomen has been documented only twice before. The first histopathology confirmed case from Japan in 2006 had solitary red-coloured gradually enlarging eroded tumor with apocrine poroma on right side of abdomen. [13] The age of onset was 61 years. The second case of SCAP on abdomen reported had presented at 40 years of age. [11] Our case had clinical similarity to the second case with lesions in a linear distribution over right side of abdomen at an age of 13 years; though she had the lesions since infancy. Abdomen is a rare site for the development of SCAP because ectopic apocrine glands on the abdomen are usually poorly developed and extremely sparse. [11] Further, the development of multiple SCAP lesions is an uncommon event and along the Blaschko's lines is exceptional. [11] The linear variant is usually present at birth (70-85%) or may develop before puberty (15-30%). The lesions consist of multiple linear papulo-vesicles with dome-shaped surfaces at birth which may ulcerate. They tend to increase in size and number at puberty and may become verrucous and papillomatous. [11] Nearly all the cases of linear SCAP involve neck, though they may rarely involve chest, arm, thigh and abdomen. Surprisingly, no case has been reported with nevus sebaceous. Other adnexal tumors which show linear arrangements include nevus comedonicus, trichodiscoma, trichoepithelioma, basaloid follicular hamartoma, cylindroma, eccrine nevus, syringoma, eccrine poroma, eccrine spiradenoma, and basal cell carcinoma.

The clinical features of SCAP vary widely but the histopathology is invariably uniform and confirmatory. IHC is important in defining the origin from either apocrine or eccrine cell lineage. There are only a few reports available on immunohistochemical findings in SCAP and in its malignant form. SCAP has been reported to be positive for CEA, CK AE1 and EMA but variable for SMA. Various studies have reported divergent immunohistochemical findings. This can be explained by the use of different staining procedures and different antibody clones. However, p63 has proved to be the best antibody for visualizing basal cells. [14]

SCAP has long been considered to be a hamartoma arising from pleuripotent cells. Mutation in Patched gene (PTCH) or P16 tumor suppressor gene has been demonstrated in some cases. [2] It is believed to recapitulate the formation of folliculo-apocrine unit in embryonic life.Hence, recapitulation of various fetal cytokeratins can be demonstrated. SMA positivity is normally the feature of myoepithelial cells but SCAP do not contain any myoepithelial cell but demonstrate such positivity in focal basal cells thereby indicating the immaturity of the tumor. Cytokeratin expression in the luminal layer of the tumor in the secretory and ductal portion of sweat glands has admixture of 2 types of cells. To conclude, the tumor epithelium consist of several cell types demonstrating various development stages. The basal tumor cells demonstrate a tendency to differentiate towards myoepithelial lineage and luminal cells towards ductal or secretory epithelium. [1]

Rarely, malignant transformation to basal cell carcinoma, squamous cell carcinoma, or sweat gland carcinoma has been documented in plaque and solitary nodular types. Further, SCAP is known to be associated with malignant tumors such as, basal cell carcinoma (10%), sebaceus carcinoma, verrucous carcinoma and ductal carcinoma. Only one case of malignant transformation of SCAP to syringocystadenocarcinoma papilliferum (SCACP) has been documented amongst 12 cases of SCACP described in the literature, [15] however, no case of linear type has turned malignant probably because of their origin.

 
   References Top

1.Yamamoto O, Doi Y, Hamada T, Hisaoka M, Sasaguri Y. An immunohistochemical and ultrastructural study of syringocystadenoma papilliferum. Br J Dermatol 2002;147:936-45.  Back to cited text no. 1
[PUBMED]    
2.E. calonje. Tumours of the skin appendages. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's textbook of Dermatology. 8 th ed. UK: Wiley Blackwell; 2010. p. 19-20.  Back to cited text no. 2
    
3.Helwig EB, Hackney VC. Syringadenoma papilliferum; lesions with and without naevus sebaceous and basal cell carcinoma. AMA Arch Derm 1955;71:361-72.  Back to cited text no. 3
[PUBMED]    
4.Pinkus H. Life history of naevus syringadenomatosus papilliferus. AMA Arch Derm Syphilol 1954;69:305-22.  Back to cited text no. 4
[PUBMED]    
5.Saha A, Das NK, Gharami RC, Chowdhury SN, Datta PK. A clinico-histopathological study of appendageal skin tumors, affecting head and neck region in patients attending the dermatology opd of a tertiary care centre in eastern India. Indian J Dermatol 2011;56:33-6.  Back to cited text no. 5
[PUBMED]  Medknow Journal  
6.Gönül M, Soylu S, Gül U, Kaya I, Albayrak L, Unal T. Linear syringocystadenoma papilliferum of the arm: A rare localization of an uncommon tumour. Acta Derm Venereol 2008;88:528-9.  Back to cited text no. 6
    
7.Narang T, De D, Dogra S, Saikia UN, Handa S. Linear papules and nodules on the neck. Syringocystadenoma papilliferum (SP). Arch Dermatol 2008;144:1509-14.  Back to cited text no. 7
    
8.Yaghoobi R, Zadeh SH, Zadeh AH. Giant linear syringocystadenoma papilliferum on scalp. Indian J Dermatol Venereol Leprol 2009;75:318-9.  Back to cited text no. 8
[PUBMED]  Medknow Journal  
9.Pahwa P, Kaushal S, Gupta S, Khaitan BK, Sharma VK, Sethuraman G. Linear syringocystadenoma papilliferum: An unusual location. Pediatr Dermatol 2011;28:61-2.  Back to cited text no. 9
[PUBMED]    
10.Kar M, Kar JK, Maiti S. Giant linear syringocystadenoma papilliferum of the back. Indian J Dermatol Venereol Leprol 2012;78:123.  Back to cited text no. 10
[PUBMED]  Medknow Journal  
11.Yap FB, Lee BR, Baba R. Syringocystadenoma papilliferum in an unusual location beyond the head and neck region: A case report and review of literature. Dermatol Online J 2010;16:4.  Back to cited text no. 11
    
12.Malhotra P, Singh A, Ramesh V. Syringocystadenoma papilliferum on the thigh: An unusual location. Indian J Dermatol Venereol Leprol 2009;75:170-2.  Back to cited text no. 12
[PUBMED]  Medknow Journal  
13.Suzuki T, Ikeda H, Hamasaki Y, Hatamochi A, Yamazaki S. Syringocystadenoma papilliferum associated with apocrine poroma. J Dermatol 2006;33:249-51.  Back to cited text no. 13
[PUBMED]    
14.Lindboe CF, Brekke H, Schønhardt I, Houge U. Syringocystadenocarcinoma papilliferum in situ: Case report with immunohistochemical observations. Sch Res Exch 2009;2009:4.  Back to cited text no. 14
    
15.Hoekzema R, Leenarts MF, Nijhuis EW. Syringocystadenocarcinoma papilliferum in a linear nevus verrucosus. J Cutan Pathol 2011;38:246-50.  Back to cited text no. 15
[PUBMED]    

What is new?
Our case is unique due to development of SCAP over abdomen (3rd such case in literature) and its linear distribution (which has been reported only once before).


    Figures

  [Figure 1], [Figure 2]



 

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    Abstract
   Introduction
   Case Report
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    References
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