Hidrocystoma

Benjamin Barankin, MD, FRCPC
Alexander K.C. Leung, MD, MBBS, FRCPC, FRCP
Volume 56 - Issue 9 - September 2016

A 47-year-old white woman presented with an asymptomatic cystic lesion in the periorbital area of approximately 4 years’ duration. Over time, the lesion had slowly increased in size. She noted that the size of the lesion was slightly bigger during the summertime. There was no visual disturbance. Nevertheless, she was bothered by it because the lesion was unsightly. She was otherwise healthy with no other problems. The family history was noncontributory.

Physical examination revealed a translucent, smooth-surfaced, fluctuant, cystic lesion on the lateral canthus of her left eye. The lesion measured 4 to 5 mm in diameter. No similar lesions were observed elsewhere on the body. The remainder of the clinical examination revealed no abnormalities.

Based on the appearance of the lesion, a clinical diagnosis of eccrine hidrocystoma was made. The lesion was excised and healed with minimal scarring.

Etiopathogenesis. Hidrocystomas are benign cystic tumors that derive from either the eccrine or apocrine sweat gland.1 The condition was first described by Robinson in 1893.2

Presumably, an eccrine hidrocystoma is due to obstruction of an eccrine sweat gland duct with resultant retention of sweat and the appearance of a dilated cystic structure.1,3 An apocrine hidrocystoma is believed to arise from the proliferation of an apocrine sweat gland.4 A hot and humid environment has been implicated in the pathogenesis of some cases of eccrine hidrocystomas, especially multiple eccrine hidrocystomas.4

Epidemiology. The exact incidence is not known. In a series of 130 sweat gland lesions of the eyelids seen at the Armed Forces Institute of Pathology in Washington, DC, hidrocystomas constituted 22% of all cases.5 Hidrocystomas are most prevalent between 30 and 70 years of age.4 For solitary lesions, the sex ratio is equal. Multiple eccrine hidrocystomas are seen mainly in women.4

Histopathology. Histologically, an eccrine hidrocystoma appears as an unilocular cyst lined by 1 or 2 layers of cuboidal cells.4 Unlike the apocrine counterpart, a myoepithelial layer, secretary cells demonstrating decapitation secretion, and periodic acid–Schiff-positive granules are characteristically absent.3,4,6 The inner luminal layer in the solitary (but not multiple) eccrine hidrocystoma stains positive for S100 protein.4,6 In contrast, an apocrine hidrocystoma appears as an unilocular or multilocular cyst.6 The cyst wall is composed of an inner layer of secretary columnar epithelium and an outer myoepithelial cell layer.4,6 Periodic acid–Schiff-positive granules are observed in the presence of lipofuscin granules.4 Decapitation secretion is characteristic. Papillary projections into the lumen of the cyst can be seen.3

Clinical manifestations. Typically, hidrocystomas present as asymptomatic, dome-shaped papules or nodules.6 The consistency is usually fluctuant and cystic. Eccrine hidrocystomas are usually translucent and may have an amber, brownish, grayish, or bluish hue.6 In contrast, the color of apocrine hidrocystomas ranges from flesh-colored to blue-black; the blue-black color might be the result of Tyndall phenomenon and the presence of lipofuscin pigment.4,6 Eccrine hidrocystomas are usually smaller, ranging from 1 to 6 mm in diameter, while apocrine hidrocystomas usually range from 3 mm to 1.5 cm.4,6 Eccrine hidrocystomas usually do not involve the eyelid margin; rather, they are most frequently located in the malar and the periorbital areas, in particular, the medial or lateral canthus,1,7 as is illustrated in the case presented here. On the other hand, apocrine hidrocystomas are mainly found on the head and neck, in particular along the eyelid margin.4

Eccrine hidrocystomas can be either solitary (Smith and Chernosky type) or multiple (Robinson type). Multiple eccrine hidrocystomas may occur in association with Graves disease or Parkinson disease.8 An increase in size and number of lesions during hot and humid weather can be seen with eccrine hidrocystomas but not with apocrine hidrocystomas.3,6 Apocrine hidrocystomas are usually solitary.6 Multiple apocrine hidrocystomas are rare. Multiple eccrine and especially apocrine hidrocystomas, are a feature of Schöpf-Schulz-Passarge syndrome characterized by multiple eyelid hidrocystomas, palmoplantar keratoderma, hypodontia, hypotrichosis, nail dystrophy, and Goltz syndrome (also known as focal dermal hypoplasia) characterized by multiple hidrocystomas, dermal hypoplasia, cutis aplasia, papillomas, chorioretinal colobomas, absent/dysplastic teeth, and skeletal anomalies.9,10

Diagnosis. The diagnosis is mainly clinical, based on the typical morphologic features. A biopsy or referral to a dermatologist may be required if the diagnosis is in doubt.

Differential diagnosis. The differential diagnosis includes syringoma, molluscum contagiosum, steatocystoma multiplex, hordeolum, chalazion, cystic or nodular basal cell carcinoma, milium, sebaceous hyperplasia, epidermal inclusion cyst, mucoid cyst, angular dermoid, melanocytic nevus, agminated blue nevus, and melanoma.3,4,6

Complications and prognosis. Hidrocystomas may be considered cosmetically unsightly by some people. When occurring on the eyelid, hidrocystomas may cause blepharoptosis and amblyopia.11 Rarely, hemorrhage within a hidrocystoma may occur.12

Without treatment, hidrocystomas usually grow slowly and persist indefinitely.

Prevention and management. For eccrine hidrocystomas, avoiding hot and humid environments is the most useful preventive measure.

Treatment options include simple needle puncture, surgical excision, electrodessication, carbon dioxide or pulsed dye laser therapy, topical scopolamine, topic atropine, topical/intralesional trichloroacetic acid, and perilesional injections with botulinum toxin A.1,4,6 The choice of the treatment method should depend on the clinician’s comfort level with the various treatment options; the number, location, and size of lesions; and the preference of the patient.

REFERENCES:

  1. Couto Júnior AdS, Batista GM, Calafiori ÍGDF, Radael VC, Mendes WB. Hidrocystoma: surgical management of cystic lesions of the eyelid. An Bras Dermatol. 2010;85(3):368-371.
  2. Robinson AR. Hidrocystoma. J Cutan Genitourin Dis. 1893;11:293-303.
  3. Massone C, Noll P, Hofmann-Wellenhof R. Multiple, bilateral periorbital blue macules. Clin Exp Dermatol. 2014;39(3):423-424.
  4. Sarabi K, Khachemoune A. Hidrocystomas—a brief review. MedGenMed. 2006;8(3):57.
  5. McLean IW, Burnier MN, Zimmermann LE, Jakobiec FA. Tumors of the Eye and Ocular Adnexa. Washington, DC: Armed Forces Institute of Pathology; 1994:101-127. Atlas of Tumor Pathology; series 3, book 12.
  6. Ferenczi K, Elenitsas R. Hidrocystoma (apocrine and eccrine hidrocystoma). Decision Support in Medicine. http://www.decisionsupportinmedicine.com. Accessed September 30, 2015.
  7. Singh AD, McCloskey L, Parsons MA, Slater DN. Eccrine hidrocystoma of the eyelid. Eye. 2005;19(1):77-79.
  8. Lee MR, Ryman W. Multiple eccrine hidrocystomas. Australas J Dermatol. 2004;45(3):178-180.
  9. Castori M, Ruggieri S, Giannetti L, Annessi G, Zambruno G. Schöpf-Schulz-Passarge syndrome: further delineation of the phenotype and genetic considerations. Acta Derm Venereol. 2008;88(6):607-612.
  10. Stevenson DA, Chirpich M, Contreras Y, Hanson H, Dent K. Goltz syndrome and PORCN mosaicism. Int J Dermatol. 2014;53(12):1481-1484.
  11. Hirata A. Eccrine hidrocystoma arising at the tarsal plate during childhood. Case Rep Ophthalmol. 2013;4(2):61-63.
  12. Novitskaya E, Rene C, Dean A. Spontaneous haemorrhage in an eyelid hidrocystoma in a patient treated with clopidogrel. Eye. 2013;27(6):782-783.