Solitary Nodular Lesion Over Face

A 24-year-old male visited the dermatology outpatient department with progressively increasing swelling over the left nasolabial fold for three years [Figure 1]. The lesion was asymptomatic but cosmetically unacceptable to the patient. The dermatological examination revealed a single translucent nodule over the left nasolabial fold just adjacent to the angle of the nose. An excision biopsy was done. The histopathological examination revealed centrally filled keratin material with distorted hair follicle surrounded by thick basophilic fibrous stroma [Figure 2]. The dermis showed characteristic multiple thin branching strands of infundibular epithelium surrounded by abundant stroma, along with few cysts lines by single layer of cells [Figure 3].

Figure 1.

A transluscent dome shaped shiny nodule over left nasolabial fold

Figure 2.

Histopathological findings includes centrally filled keratin material with distorted hair follicle surrounded by thick basophilic fibrous stroma. (H and E, 10 X)

Figure 3.

The dermis showed characteristic multiple thin branching strands of infundibular epithelium surrounded by abundant stroma. (H and E, 40 X)

What is the diagnosis?

Answer: Fibrofolliculoma with cystic changes.

Discussion

Fibrofolliculoma is an uncommon benign cutaneous tumour originating from perifollicular mesenchyme. These are slowly evolving asymptomatic connective tissue tumours and appear as small skin-coloured perifollicular dome-shaped papules most commonly on the head and neck region. It usually arises in the form of multiple lesions, which are inherited in an autosomal dominant manner as seen in association with Birt–Hogg–Dube syndrome; comprising of fibrofolliculomas, acrochordons, trichodiscomas, and internal neoplasms. Malignant conversion is rare.[1]

Solitary lesions are rare, non-hereditary, and not associated with other cutaneous abnormalities. There have been only 18 cases of solitary fibrofolliculomas reported till date, most affecting females, site being face, with varying duration and from the Asian continent.[1,2,3,4,5,6] Solitary fibrofolliculomas appear clinically and histologically similar to individual lesions of muliple fibrofolliculomas.

Fibrofolliculoma is a hamartoma, consisting of connective tissue and follicular epithelial components, which are clearly and distinctly seen upon histopathological examination. The central part of the lesion comprises cords of follicular epithelium surrounded peripherally by proliferating bands of connective tissue. IHC expression is in the form of factor XIIIa in the perifollicular cells in a background of CD34-positive spindle cells.[1,5]

Multiple differential diagnosis can be considered clinically, like basal cell carcinoma, trichofolliculoma, epidermoid cyst, angiofibroma, trichoepithelioma, clear cell acanthoma, acrochordon, dermal melanocytic nevus, and dermatofibroma. Histological differentials include trichofolliculoma, perifollicular fibroma, trichoepithelioma, trichilemmoma, trichodiscoma, angiofibroma, and basaloid follicular hamartoma.[2,3,4]

Dermoscopy of multiple fibrofolliculomas associated with Birt–Hogg–Dube syndrome has been reported previously as well-demarcated areas of pallor with central follicular openings. Dermoscopy of solitary fibrofolliculoma has been reported in a single-case report as hypopigmented globules and curvilinear vessels often connecting red dots and globules.[6] These findings are distinct from dermoscopic findings of basal cell carcinoma. This simple tool may thus serve well to differentiate a benign entity from its malignant clinical mimicker, especially so as patients do not want to undergo biopsy for facial lesions.

Surgical excision is considered the optimal therapy as it offers both diagnostic and therapeutic means. There are no standard excision guidelines. Other therapeutic options include shave excision, dermabrasion, electrodessication, CO₂ laser, or erbium-doped YAG laser, which are better suited for multiple or recurrent lesions.[1,2,3]

We report a rare case of solitary large fibrofolliculoma on the cheek adjoining the nasolabial fold depicting cystic changes on histopathology, which has not been described till date in the literature. Owing to the rare occurrence and slow evolution with non-specific clinical features, most cases of solitary fibrofolliculomas are clinically misdiagnosed unless biopsied. Hence, dermatologists should keep this entity in mind as a differential for lesions on the head and neck.

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.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.