SkIndia Quiz 24: Itchy papules over face.

A 36-year-old female presented with gradual onset of multiple itchy raised skin colored lesions over the face since past 10 years. There was no history of antecedent trauma, photosensitivity or any medical or surgical illness or family history of similar illness. There was also no history of oral or topical medication taken for the above complaints. Examination revealed multiple discrete 1–2 mm sized, firm skin colored papules over both cheeks [Figures 1 and 2] with no evidence of similar lesions elsewhere on the body. A skin biopsy was done to confirm the diagnosis that showed mid-dermal cysts containing laminated keratin and multiple vellus hair shafts lined by a flattened squamous epithelium [Figures 3 and 4].

Figure 1

Discrete papules over left cheek

Figure 2

Discrete papules over right cheek

Figure 3

Low Power magnification shows a cyst lined by epithelium containing keratinous material with vellus hair shafts (H and E, ×10)

Figure 4

Multiple vellus hair shafts better appreciated under high power magnification (H and E, ×40)

ANSWER

Facial Variant of Eruptive Vellus Hair Cyst (EVHC).

DISCUSSION

Eruptive vellus hair cyst was first described by Esterly et al., in 1977.[1] It can be familial (autosomal dominant), manifesting at birth or in early infancy, sporadic, appearing in the first or second decade of life,[2] or may be associated with renal failure, anhidrotic ectodermal dysplasia, hidrotic ectodermal dysplasia, pachyonychia congenita, and Lowe syndrome.[3] It has been postulated to be a developmental abnormality that predisposes vellus hair follicles to infundibular occlusion leading to consequent atrophy of the hair bulb.[4] Classically, EVHC manifests with multiple asymptomatic, smooth, soft, rounded, or dome-shaped papules ranging from 1 to 4 mm in diameter, and most commonly seen over anterior chest and extremities. This condition can rarely be associated with pain and itching.[3] There are few reports of generalized[5] and isolated facial[678] variants in the literature.

Diagnosis of EVHC is confirmed by histopathological examination of a cutaneous biopsy specimen, characterized by a mid-dermal cyst lined by a flattened squamous epithelium containing laminated keratin and multiple transversely and obliquely cut vellus hairs. An alternative bedside approach is to make a superficial incision at the top of the lesion for easy expression of the cystic contents followed by microscopic examination of the expressed contents in a potassium hydroxide preparation which shows numerous vellus hairs.[9]

Patients generally seek medical attention for cosmetic reasons. Treatment of EVHC is challenging with modest benefit seen with the topical use of 10% urea, 12% lactic acid, retinoic acid, and 0.1% tazarotene. Other treatment modalities include excision, curettage, needle evacuation, incision and drainage, dermabrasion, and laser therapy, but there are chances of scarring and recurrences.[10] However, spontaneous resolution may occur in approximately 25% of cases.[11]

In summary, We would like to emphasize that the facial variant of EVHC is an uncommon entity which should always be considered in the differential diagnosis of asymptomatic facial papules such as steatocystoma multiplex, milia, infundibular cysts, trichilemmal cysts, acne comedones, molluscum contagiosum, etc., and need to be confirmed on skin biopsy.

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.