Indian Journal of Pathology and Microbiology

: 2018  |  Volume : 61  |  Issue : 2  |  Page : 287--289

Ectopic hidradenoma papilliferum of eyelid: A rare entity with diagnostic challenge

Prakriti Shukla, Anil K Malaviya 
 Department of Pathology, Era's Lucknow Medical College and Hospital, Lucknow, Uttar Pradesh, India

Correspondence Address:
Prakriti Shukla
Department of Pathology, Era's Lucknow Medical College and Hospital, Lucknow - 226 003, Uttar Pradesh


Hidradenoma papilliferum is a rare cutaneous adnexal tumour of middle aged females arising mainly in the apocrine sweat glands of perianal and genital region. Ectopic forms of this entity have been described with majority of them occurring on the head and neck region. We report a case of 16 years young male who presented with two small, well circumscribed, greyish-brown, firm, nodules on his left upper and lower eyelid. A clinical impression of basal cell carcinoma was assumed but on histopathological examination it was diagnosed as hidradenoma papilliferum of eyelid. Immunohistochemistry was performed to support the diagnosis due to its unusual clinical presentation. To the best of our knowledge, only six cases in the upper eyelid and one case in the lower eyelid have been reported in the English literature. This is the first case report of hidradenoma papilliferum involving both upper and lower left eyelid in an adolescent boy.

How to cite this article:
Shukla P, Malaviya AK. Ectopic hidradenoma papilliferum of eyelid: A rare entity with diagnostic challenge.Indian J Pathol Microbiol 2018;61:287-289

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Shukla P, Malaviya AK. Ectopic hidradenoma papilliferum of eyelid: A rare entity with diagnostic challenge. Indian J Pathol Microbiol [serial online] 2018 [cited 2021 Apr 15 ];61:287-289
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Full Text


Hidradenoma papilliferum (HP) also known as papillary hidradenoma is a rare, benign adnexal tumor that has its origin in the apocrine sweat glands of genital and perianal region of middle-aged females.[1],[2] In rare instances, it can be seen at other sites such as head, neck, and extremities.[3],[4],[5] It presents as a slow-growing, solitary, cystic, dermal nodule that may sometimes bleed or ulcerate.[1] Clinically, it mimics other cutaneous neoplasms requiring histopathological examination. Immunohistochemistry can help support the diagnosis in tumors arising at ectopic sites. The clinical presentation, morphological features, and management of the lesion remain the same in both anogenital and ectopic cases.

 Case Report

A 16-year-old young male of average built and height presented with a 9-month history of two painless nodules on the lateral one-third aspect of the left eye involving both upper and lower eyelids. These lesions had slowly grown in the past and were not associated with ulceration or bleeding. His personal, medical, and family history was unremarkable. On examination, two small, nontender, well-circumscribed, grayish-brown, firm nodules of size approximately 1 cm each were present on the lateral aspect of the left eyelid [Figure 1]. Surgical excision was performed and clinical diagnosis of basal cell carcinoma had been presupposed. The excised nodules were sent for histopathological examination.{Figure 1}

Microscopic examination showed a well-circumscribed, cystic neoplasm in the dermis with no connection to the epidermis. The tumor was composed of elongated tubules and large papillary folds [Figure 2]a. The papillae were made of central fibrous connective tissue lined by two layers of epithelial cells, a basal layer of myoepithelial cells and a luminal layer of columnar cells [Figure 2]b. The cystic cavity and the lumina of the tubular structures contained active decapitation secretions. The core of papillary structures showed mild lymphoplasmacytic cell infiltrate. The above microscopic features were consistent with the diagnosis of HP. Immunohistochemistry revealed strong gross cystic disease fluid protein 15 (GCDFP-15) [Figure 2]c and epithelial membrane antigen (EMA) positivity in the tumor cells [Figure 2]d.{Figure 2}


HP is a benign, sweat gland adenoma with apocrine differentiation.[1] Apocrine sweat glands are formed in utero but remain inactive until stimulated by hormonal changes at puberty. As they empty their secretions into the isthmus part of hair follicle, they tend to arise within their vicinity. Therefore, apocrine glands are greatly concentrated in the anogenital region and axilla with most cases of HP reported in these locations. The ectopic forms have been described at several sites including head and neck region, chest, and extremities.[3],[4],[5] This is attributed to the presence of heterotrophic and specialized version of apocrine glands in these areas. To the best of our knowledge, only seven cases of HP have been reported in eyelids.

It has been assumed that estrogen stimulation plays a significant role in the development of this tumor in the females as all cases have been reported after the onset of puberty [6] In men, the role of estrogen in ectopic HP needs further evaluation. Often, it has been mistaken for malignant tumor due to its complex adenomatous pattern, although malignant transformation is rare. The overall prognosis of this neoplasm is good regardless of location. Surgical excision is the treatment of choice with no reported incidence of recurrence.

Histopathologically, a well-demarcated dermal tumor exhibiting both papillary and glandular architecture is seen with no connection to the overlying epidermis. The luminal cells are typically cuboidal to columnar, with pale eosinophilic cytoplasm and are surrounded by a thin layer of myoepithelial cells. The lumen of these glands shows active decapitation secretion. This tumor typically expresses EMA, carcinoembryonic antigen, GCDFP, and human milk fat globule membrane antigen immunomarkers indicating its differentiation toward apocrine sweat glands.[7]

The peculiar feature in our study is that the patient was a young male who presented with a 9-month short history of two nodules in the eyelid involving both upper and lower lids of the left eye. This is the first case reporting two nodules instead of a solitary lesion presenting on both upper and lower eyelids. Histologically, the tumor was of mixed differentiation with focal areas showing infiltration by plasma cells and lymphocytes. This finding suggests its differentiation toward syringocystadenoma papilliferum, a closely related tumor. However, syringocystadenoma papilliferum shows cystic invaginations extending downward from the epidermis. The other histopathological differential diagnoses include tubular apocrine adenoma, clear cell (apocrine) adenoma, and intraductal carcinoma.[4]


Papillary hidradenoma is an uncommon benign adnexal tumor usually affecting the anogenital region of women. Appearance of this neoplasm on unusual sites makes its identification troublesome as it lacks distinctive clinical characteristics. Therefore, understanding this entity is essential for clinical practitioners. Diagnosis is based on histopathological examination, and wide surgical excision is the key to definitive cure.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.


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