Syringocystadenoma papilliferum in an unusual location

Abstract

Syringocystadenoma papilliferum is a rare benign hamartomatous adnexal tumor of the apocrine or eccrine sweat glands. Most patients present a solitary lesion in the head and neck region. Presentation outside the head and neck region is even more uncommon. We present a case of Syringocystadenoma papilliferum with papulonodular lesion located on the vulva of an infant girl. This case illustrates the atypical location of this rare disease and adds to the differential diagnosis of lesions on the vulva.

Contiguous verrucous proliferations in syringocystadenoma papilliferum: A retrospective analysis with additional evaluation via mutation‐specific BRAFV600E immunohistochemistry

“…On review of the literature, there have been only a handful of isolated cases with explicit descriptions of SCAP arising in contiguity with verruca . However, after more careful review, we were able to identify multiple other published cases of SCAP which lacked explicit mention of this association, but nevertheless showed these changes in the published images . Out of suspicion that this may be an underappreciated feature, we sought to explore this possibility further through retrospective review of all sporadic cases of SCAP at our institution.…”

A red nodule of the scalp

“…A possible derivation from eccrine or undifferentiated pluripotential cells has also been reported [ 1 – 4 ]. SCAP affects males and females equally [ 2 ], developing at birth (50% of cases) or in childhood/adolescence [ 3 , 5 ]. At puberty a dimensional growth may be displayed [ 2 , 3 ].…”

“…SCAP affects males and females equally [ 2 ], developing at birth (50% of cases) or in childhood/adolescence [ 3 , 5 ]. At puberty a dimensional growth may be displayed [ 2 , 3 ]. SCAP usually arises on the scalp, face and neck, and less commonly on other regions, such as the trunk, eyelids and genitalia [ 2 – 4 ].…”

“…Clinically it appears as a hairless, smooth, or verrucous single papule or plaque, but multiple elements may occur, especially on SN [ 2 – 4 ]. SCAP may also be associated with different types of lesions, both benign (viral warts, SN, apocrine poroma, hidradenoma papilliferum) and malignant (basal cell carcinoma, sebaceous carcinoma) [ 2 , 3 ]. SCAP is most frequently related to SN (1/3 of cases) [ 2 , 4 , 6 ].…”

“…Despite the uncommon malignant transformation into basal cell carcinoma (10%), squamous cell carcinoma, or syrigocystadenocarcinoma, surgical excision is considered the treatment of choice in order to arrive at a final histological diagnosis [ 3 , 4 ]. After histopathologic confirmation, carbon dioxide laser treatment may be considered an option [ 2 , 3 ].…”