Delving deeper: Understanding the unique interplay of syringomas and epidermoid cysts of vulva

Dear Sir, A 61-year-old woman reported to the dermatology outpatient clinic with several raised lesions accompanied by pruritus in the vulvar region for the past 2 years. Upon cutaneous examination, several skin-colored to hyperpigmented papules measuring between 0.2 mm × 0.5 mm were observed on the vulva. Few of them were shiny and darker in appearance. Similar lesions were present over the lower orbital area Figure 1a-c. Angiokeratoma, milia, syringoma, and steatocystoma multiplex were retained as differential diagnoses. Hepatitis B, hepatitis C, herpes simplex virus-I and II, and human immunodeficiency virus tests were all negative, as were all standard tests. Speculum examination was not performed. Vulva biopsy sent for histopathological (HP) examination. HP report consisted of both epidermal inclusion cyst (EIC) and syringoma, which showed well-circumscribed cyst having squamous epithelial lining and keratin flakes with areas of round to comma-shaped ductules made up of basaloid cells Figure 2a and b. The patient refused radiofrequency cauterization of the lesions. Topical tretinoin was recommended, but the patient was lost to follow-up.Figure 1: (a and b) Multiple skin coloured papules size ranging from 0.2 mm to 0.5 mm over lower eyelid and few at forehead (black arrow) (c) Multiple tiny skin coloured to hyperpigmented papules size 0.1 mm–0.2 mm over inner side of labia majora and lower side of vulva (black arrow)Figure 2: (a) Microsection from vulval lesions shows stratified squamous keratinized epithelium and underlying superficial dermis shows proliferation of round to comma shaped ductules (black arrow) and pilosebaceous unit and increased fibrosis (H and E, ×10) (b) Microsection shows well circumscribed cyst having squamous epithelial lining and keratin flakes (black star) along with areas showing round to comma shaped ductules (black arrow) made up of basaloid cells (H and E, ×4)Syringomas are eccrine or apocrine tumors that are small, skin-colored papules that range in size from 2 to 4 mm. Although they can appear anywhere on the body, the area around the eyes is where they are most commonly found.1 As in our case, vulvar syringomas can coexist with facial syringomas or develop as the part of an eruptive pattern. Usually occurring in childhood or early adulthood, eruptive syringomas can be associated with trauma, hyperthyroidism, diabetes mellitus, Down syndrome, and antiepileptic medications.2 EICs are yellow-white papulonodules that are usually small, round that range in size from a few millimeters to several centimeters in the vulva. It can affect the face, genitalia, limbs, and trunk. In addition, these cysts may form inside the vagina, primarily over the clitoris. The majority of lesions are solitary, according to the literature. Trauma to the genital area, such as female genital mutilation, could be the cause.3 An epidermal cyst is the sebaceous gland itself, while an inclusion cyst comprises epithelial tissue. When the oil glands are blocked, vulvar sebaceous cysts develop. When it becomes infected, incision, drainage, and excision are necessary.4 However, a rare combination of syringomas and EICs in vulva draw our attention to report this case. Both are benign lesions that are primarily asymptomatic and occur at various places on the body.5 In addition, they have unique HP features that can be used to diagnose them. The eccrine duct’s involvement has been proposed in the formation of both lesions, although their unknown causes. In order to establish the nature of their connection, additional research involving a substantial number of studies is required. 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. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.