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Periocular keratotic nodule

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Periocular keratotic nodule
S Shanmugam1, R Gahlaut2, S Edwards2, W Hussain1
Dermatology Online Journal 19 (3): 11

1. Leeds Centre for Dermatology
2. Department of Histopathology
Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom


Figure 1
Figure 1. A 6 x 6 mm keratotic nodule on the right lower cutaneous eyelid

A 51-year-old gentleman presented with a 6-week history of a nodular lesion on the right lower cutaneous eyelid. It was painful and itchy. He was otherwise well with no relevant past medical history.


On examination a 6 x 6 mm dome shaped keratotic nodule was seen (Figure 1). Clinically, this was suspected to be a squamous cell carcinoma and he therefore underwent urgent excision with flap reconstruction.


Figure 2aFigure 2b
Figure 2a. A well-defined dermal lesion, appearing as an invagination from the epidermis with the base composed of papillary projections (H&E Low power)

Figure 2b. H&E x2.5 magnification

Histopathology revealed a dermal lesion, appearing as an invagination from the epidermis with the base composed of papillary projections (figure 2A and 2B). The papillae were composed of a central fibrovascular core lined by double-layered epithelium. The outer zone consisted of small cells with oval hyperchromatic nuclei and scant cytoplasm and the inner zone consisted of multilayered tall columnar cells with abundant eosinophilic cytoplasm and large vesicular nuclei. Decapitation secretions were also identified in one of the ducts. There was no evidence of neoplasia.


Syringocystadenoma papilliferum


Syringocystadenoma papilliferum is a benign adnexal tumor predominantly of apocrine differentiation, which commonly occurs in the scalp and the forehead [1]. Occasionally, eccrine differentiation has also been reported. In majority of cases, syringocystadenoma papilliferum arises within a pre-existent sebaceous naevus of Jadassohn [2, 3].

It can present as a hairless plaque in the scalp or linear group of nodules present at birth or develop in infancy. They can become more nodular, verrucous or crusted at puberty. Solitary nodules commonly develop at puberty and are predominantly found in the trunk, axillae, groin and upper limbs.

Histologically, Syringocystadenoma papilliferum is characterized by endophytic invaginations of the epithelium into the dermis lined by double-layered outer cuboidal and luminal high columnar epithelium. Tumor cells show a positive staining reaction with carcinoembryonic antigen and gross cystic disease fluid protein-15.

Malignant transformation to basal cell carcinoma can occur in longstanding SCAP lesions. Syringocystadenocarcinoma papilliferum is a rare form of adenocarcinoma of the skin, which is considered as the malignant counterpart of SCAP. This usually develops on the scalp in a long standing SCAP. Thus surgical excision is the treatment of choice.

Syringocystadenoma papilliferum is unusual on the lower eyelid and to our knowledge, there are only around 17 cases published in the literature predominantly in ophthalmology and plastic surgery journals.

Barbarino et al published a case review in 2009 in which they looked at 14 cases with SCAP of the eyelid. The lesions were frequently misdiagnosed clinically as basal cell carcinoma or cyst. None of the lesions showed malignant transformation. One lesion recurred after incomplete excision [5].

Syringocystadenocarcinoma papilliferum presenting as an ulcerated, painless right lower eyelid lesion in an 86-year-old man has also been reported.


1. Mammino JJ, Vidmar DA. Syringocystadenoma papilliferum. Int J Dermatol 1991;30:763-6. [PubMed]

2. Helwig EB, Hackney VC. Syringoadenoma papilliferus. Lesion with and without naevus sebaceous and basal cell carcinoma. Arch Dermatol 1955;71:361-372. [PubMed]

3. Jordan JA, Brown OE, Biavati MJ, Manning SC. Congenital syringocystadenoma papilliferum of the ear and neck treated with the CO2 laser. Int J Pediatr Otorhinolaryngol 1996;38:81-7. [PubMed]

4. Askar S, Kilinc N, Avtekin S. Syringocystadenoma papilliferum mimicking Basal cell carcinoma on the lower eyelid: a case report. Acta Chir Plast. 2002;44:117-9. [PubMed]

5. Barbarino S, McCormick SA, Lauer SA et al. Syringocystadenoma papilliferum of the eyelid. Ophthal Plast Reconstr Surg. 2009;25:185-8. [PubMed]

6. Hoguet AS, Dolphin K, McCormick SA et al. Syringocystadenocarcinoma papilliferum of the eyelid. Ophthal Plast Reconstr Surg. 2012;28:e27-9. [PubMed]

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