Hidrocystoma
Key Points
Hidrocystomas are benign cystic lesions of sweat glands seen most commonly on the face, especially on the eyelids
Hidrocystomas are categorized into apocrine and eccrine types
Apocrine glands are found only at the eyelid margin, whereas eccrine glands are sparsely present in the dermis of the pretarsal and preseptal eyelid skin
Eccrine and apocrine hidrocystomas are believed to result from ectasias of sweat gland secretory ducts and glands
Apocrine hidrocystomas are intradermal, dome-shaped, translucent cystic nodules with a white layered precipitate
Eccrine lesions are dome-shaped cysts with a clear amber or bluish tint and no precipitate
Treatment is mostly with surgical excision or electrodesiccation
Hidrocystomas are benign cystic lesions of sweat glands seen most commonly on the face, especially on the eyelids, in the medial canthus, and less commonly the lateral canthal angle.1,2,3,4,5,6,7 In a large series of more than 5000 benign and malignant noninflammatory lesions excised from the eyelids, 5.9% were hidrocystomas, and these were equally distributed in the upper and lower eyelids.4 Other studies reported hidrocystomas to account for 8% to 10.5% of benign eyelid lesions.8,9
Based on their histologic characteristics and presumed histogenic derivation, hidrocystomas are categorized into apocrine and eccrine types. Apocrine glands are found only at the eyelid margin in association with cilia, and nowhere else in the eyelid skin. Eccrine glands, however, are not present on either the upper or lower eyelid margins or in the perimarginal skin but are sparsely present in the dermis of the pretarsal and preseptal eyelid skin.10 So eccrine hidrocystomas develop within the eyelid skin away from the eyelid margins and pericilial skin, whereas hidrocystomas on or near the eyelid margins are always apocrine in origin.11,12,13 De Viragh et al12 reported that apocrine or eccrine hidrocystomas are often misdiagnosed because the usual histological criteria are masked, probably secondary to intraluminal pressure in the cysts, which flattens their walls and abolishes decapitation secretions.
Apocrine sweat glands are composed of a coiled secretory portion located in the dermis. A short straight excretory duct inserts into the upper infundibular portion of the hair follicle.14,15,16 The apocrine gland secretes an oily fluid with proteins, lipids, and steroids. Secretions appear on the skin surface mixed with sebum since sebaceous glands open into the same hair follicle.17 Apocrine glands secrete in periodic spurts and release their products by “decapitation,” where membrane-bound cytoplasm from the apical surface of the secretory cells bud off into the lumen of the duct.
Apocrine hidrocystomas were first described by Mehregan in 196418 and are thought to derive from the secretory portion of the apocrine gland. They are usually solitary lesions, but can also be multiple,19 and vary in size from 3 mm to about 15 mm.20 Multiple apocrine hidrocystomas have been described in two rare ectodermal dysplasias. Goltz-Gorlin syndrome (focal dermal hypoplasia syndrome) is a sporadic or X-linked dominant disease most common in females and characterized by atrophic hyperpigmented and hypopigmented macules, linear skin atrophy, microcephaly, microphthalmia, midface hypoplasia, malformation of the ears, apocrine hidrocystoma of the eyelids, syndactyly, and mental retardation.21,22 Schopf-Schulz-Passarge syndrome is an autosomal recessive condition characterized by hypotrichosis, hypodontia, palmar and plantar hyperkeratosis, nail fragility, hypotrichosis, and multiple eyelid apocrine hidrocystomas.23,24
Eccrine glands are composed of an intraepidermal spiral duct, a dermal duct consisting of a straight and coiled portion, and a coiled secretory tubule deep in the dermis.17 The eccrine gland opens directly onto the skin surface through a sweat pore. These glands produce a clear odorless secretion, consisting primarily of water.
Eccrine hidrocystomas were first reported by Robinson in 189325 in workers laboring in humid and warm environments. The cysts tend to enlarge with exercise and in warm environments, due to increased sweat retention.26 Eccrine hidrocystomas are most prevalent in adults between 30 and 70 years and present as small, tense, thin-walled cysts 1 to 6 mm in diameter and can occur as single or multiple lesions.2,20 They are very rare in children, and when present can be associated with ptosis and astigmatism.27,28 Solitary eccrine hidrocystomas occur equally among males and females, but multiple cysts are mainly seen in females.29
Etiology and Pathophysiology
Eccrine and apocrine hidrocystomas are believed to result from ectasias of sweat gland secretory ducts and glands.2,29,31,32,33,34,35 The apocrine sweat glands of Moll, which are present at the lid margin and empty into the infundibular portion of the lash follicles, give rise to cysts along the lash line.10 These cysts derive from the secretory portion of apocrine glands in the dermis and are thought to develop as cystic proliferations of the coil structure of the gland, rather than as a simple retention cyst.36,37,38 Eccrine hidrocystomas, however, are located in the dermis and empty directly onto the skin surface without any communication with the folliculosebaceous apparatus. Eccrine cysts are believed to result from blockage of the excretory duct, with sweat retention and cystic dilation of the eccrine gland.
Apocrine hidrocystomas differ from eccrine cysts by the presence of cyst content containing milky layered apical cytoplasmic decapitation debris. These represent shedding of adluminal snouts that become incorporated into the eosinophilic cyst secretions. Eccrine cysts contain a clear watery secretion with no snouts, and without cytoplasmic contributions.10 Both of these types of cysts are distinguished from simple conjunctival cysts by the absence of goblet cells.