eMedicine Specialties > Dermatology > Benign Neoplasms
Eruptive Vellus Hair Cysts
Updated: Nov 20, 2008
Introduction
Background
Eruptive vellus hair cysts (EVHCs) were first described in 1977 by Esterly and colleagues who reported 2 children with symmetrically distributed follicular papules on the chest and flexor extremities.1 Histologically, these papules had the unique characteristic of dermal cysts containing small vellus hairs.
Cases of EVHC may either be acquired or inherited in an autosomal dominant fashion. Generally, they are isolated findings. However, they have been associated with other skin disorders (eg, anhidrotic ectodermal dysplasia, hidrotic ectodermal dysplasia, pachyonychia congenita).
Pathophysiology
Eruptive vellus hair cysts (EVHCs) are thought to arise as a developmental abnormality of vellus hair follicles. A predisposition exists for follicular occlusion at the level of the infundibulum, which results in cystic dilatation of the proximal hair follicle and secondary atrophy of the hair bulb.
Frequency
International
The frequency is unknown. More than 50 cases have been reported in the literature; however, eruptive vellus hair cysts (EVHCs) may occur more frequently than is suggested by reports, possibly because the diagnosis is only definitively confirmed with biopsy.
Mortality/Morbidity
Eruptive vellus hair cysts (EVHCs) are asymptomatic; however, they may be cosmetically bothersome. Morbidity may be associated with treatment of the disorder.
Race
Eruptive vellus hair cysts (EVHCs) have no racial predilection.
Sex
Males and females are affected equally.
Age
In familial cases, eruptive vellus hair cysts (EVHCs) are first noted at birth or during infancy. In sporadic cases, cysts usually develop in the late-first or second decade of life.
Clinical
History
- Eruptive vellus hair cysts (EVHCs) typically develop as an acneiform eruption on the chest and extremities.2 EVHCs have also been reported on the eyelids and periorbital skin.3
- Often, no antecedent trauma or triggering factor is reported.
- EVHCs may be congenital or acquired. Several reports have noted familial cases in which the condition is thought to be inherited as an autosomal dominant trait.4,5
- EVHCs may be present at birth or arise in childhood.
- In sporadic cases, lesions typically arise in the first and second decades with the onset of puberty.
Physical
- Eruptive vellus hair cysts (EVHCs) appear clinically as discrete follicular papules.
- Papules may appear erythematous, yellow, bluish-gray, or even slightly brown.
- The chest and flexor or extensor surfaces of the extremities are the most common sites of involvement.
- Generalized EVHCs and facial variants are reported.
- A single case report describes unilateral EVHCs of the face.6
- Patients may have just a few cysts, or, often, they may have several hundred.
- More than 400 cysts were seen on a patient with generalized EVHC.
- Cysts are usually dome-shaped with a smooth surface, measuring 1-4 mm in diameter.
- Cysts sometimes demonstrate central puncta, hyperkeratotic crusts, or umbilication.
- Mechanical irritation of the cysts may produce transepidermal elimination of retained vellus hairs, similar to that seen in perforating folliculitis.
Causes
Eruptive vellus hair cysts (EVHCs) occur as a developmental abnormality of vellus hair follicles. A gradual disruption occurs between the proximal and distal parts of the follicle, usually at the level of the infundibulum. This results in the retention of hairs, cystic dilatation of the proximal follicle, and subsequent atrophy of the hair bulb.
EVHCs have also been considered a type of follicular hamartoma. The cyst wall may consist of keratinization patterns from different levels of the follicular infundibulum. The keratinization is generally that of the epidermis or the outer root sheath, but foci of trichilemmal keratinization have also been reported.
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References
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Further Reading
Keywords
follicular papules, vellus cyst, eruptive cyst, EVHC, vellus hair follicle abnormality, acneiform eruption, pachyonychia congenita, hidrotic ectodermal dysplasia, steatocystoma multiplex, epidermoid cysts, hybrid cysts
Overview: Eruptive Vellus Hair Cysts