Apocrine Hidrocystoma

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Article initiated by:

Fabliha A. Mukit, M.D.

All contributors:

Hays Torgerson Cape, Muntazim Mukit MD, Obianuju Mercy Anelo MD, Nina Krassilnik MD, Kalyan Dadireddy MD, Paul Phelps, MD, FACS, Jill Wells, MD, Fabliha A. Mukit, M.D., Michael T Yen, MD, Preeti Thyparampil, MD, Cat Nguyen Burkat, MD FACS, Srishti Khullar

Assigned editor:

Sara Lally, MD, Preeti Thyparampil, MD

Review:

Assigned status Up to Date

 by Preeti Thyparampil, MD on April 14, 2026.

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Contributing Editors:	add

Histological examination reveals unilocular cyst in the dermis. (Hematoxylin and eosin, original magnification x4). Pathology interpretation by Obianuju Mercy Anelo MD, Nina Krassilnik MD at UTHSC-Memphis.

2022 ICD-10-CM Diagnosis Code D23. 12: Other benign neoplasm of skin of left eyelid, including canthus.

Description

Apocrine hidrocystomas are uncommon benign cystic adnexal tumors arising from the secretory portion of apocrine sweat glands. These are most frequently found in the head and neck region, particularly around the eyelids and face. They were first described by Mehregan in 1964 as cystic proliferations of apocrine glands. Although their exact etiopathogenesis remains uncertain, apocrine hidrocystomas are generally considered to represent either cystic retention or adenomatous/tumorous proliferation of apocrine secretory epithelium. Clinically, they may be suspected on the basis of a solitary, translucent, dome-shaped cystic lesion; however, definitive diagnosis requires histopathological confirmation, classically demonstrating a cyst lined by apocrine epithelium with decapitation secretion and an outer myoepithelial layer.

Presentation

Apocrine hidrocystomas occur most commonly in the head and neck region, particularly in the periocular area, because apocrine structures are closely associated with hair follicles and specialized apocrine glands such as the glands of Moll are present along the eyelid margin .

Unlike eccrine glands, which open directly onto the skin surface, apocrine glands usually drain into the pilosebaceous unit. Therefore, apocrine hidrocystomas are more frequently encountered in hair-bearing or apocrine-rich sites such as the eyelids, scalp, external ear, axilla, areola, anogenital region, and other head and neck locations.

Apocrine hidrocystomas usually present as solitary, slow-growing, well-circumscribed cystic papules or nodules. They are dome-shaped, translucent to bluish-gray or blue-black, mobile, and asymptomatic, with most lesions measuring approximately 3–15 mm in diameter. Multiple or bilateral lesions are uncommon and should raise the possibility of an associated genodermatosis, particularly Schöpf-Schulz-Passarge syndrome or Goltz-Gorlin syndrome, when supported by other systemic or cutaneous features.

Because their clinical appearance may overlap with several benign cystic, vascular, and adnexal lesions, these lesions should be differentiated from eccrine hidrocystoma, epidermal inclusion cyst, mucoid cyst, cyst of Moll, hemangioma, lymphangioma, blue nevus, and other adnexal tumors. Pigmented or bluish periocular lesions may also mimic malignant entities such as basal cell carcinoma, cutaneous melanoma, or amelanotic melanoma Therefore, although the diagnosis may be suspected clinically, histopathological examination remains essential for confirmation and for excluding malignancy.

Etiology and Pathogenesis

The exact cause of apocrine hidrocystoma remains uncertain. Two main pathogenetic mechanisms have been proposed:

• Cystic retention due to obstruction or impaired drainage of apocrine ducts,
• Benign adenomatous proliferation of apocrine secretory epithelium.

The frequent use of the terms “apocrine hidrocystoma” and “apocrine cystadenoma” reflects this overlap between a retention cyst and a true benign adnexal neoplasm.

Epidemiology

These are rare beningn adnexal tumors and their true incidence is not well established because most published data consist of isolated case reports,small case series, and clinicopathological review. They are most often reported in adults, with no consistent sex predilection, and presentation in childhood or adolescence is uncommon.

Clinical Features

These lesions can present on the upper or lower eyelids, eyebrow, medial or lateral canthi, or around the peri-orbital region. These are usually asymptomatic and are often noticed because of cosmetic concern or gradual enlargement.

Periocular lesions may occasionally cause local irritation, foreign body sensation, mechanical ptosis, visual field obstruction, or difficulty with eyelid closure depending on their size and location.

On examination:

• The lesion is typically a well-defined, cystic, fluctuant or firm papule ornodule with a smooth surface.
• The overlying skin is usually intact, and the lesion is generally non-tender and mobile.
• Color may vary from skin-colored or translucent to yellowish, bluish-gray, blue-black, or purple, depending on the depth of the cyst, its contents, and the Tyndall effect from intracystic fluid.
• Most lesions are solitary. Multiple or bilateral lesions are uncommon and should prompt evaluation for associated cutaneous or systemic findings, especially when they occur with ectodermal abnormalities or features suggestive of an inherited syndrome.
• Unlike eccrine hidrocystomas, which may enlarge with heat, humidity, or sweating, apocrine hidrocystomas are usually less temperature-dependent, although clinical overlap can occur.
• In the eyelid and periocular region, careful examination should document the exact site, size, mobility, cystic nature, pigmentation, relationship to the lid margin, lash loss, ulceration, bleeding, and recurrence after previous treatment.
• Features such as rapid growth, irregular pigmentation, ulceration, madarosis, induration, fixation, or spontaneous bleeding should raise concern for an alternative diagnosis, including basal cell carcinoma, melanoma, or another adnexal tumor, and should prompt biopsy or complete excision with histopathological evaluation.

In order to rule out malignancy, it is recommended that excised lesions be submitted for histopathological examination.

Unilocular cyst is lined by a thin layer of squamous epithelium consisting of two cell layers. (hematoxylin and eosin, original magnification x10). Pathology interpretation by Obianuju Mercy Anelo MD, Nina Krassilnik MD at UTHSC-Memphis.

Differential Diagnoses

The main clinical mimics of apocrine hidrocystoma and the features that help distinguish them are summarized in Table 1.

Table 1. The key differential diagnoses of apocrine hidrocystoma and their distinguishing clinical features.

Differential diagnosis	Usual clinical appearance	Distinguishing features from apocrine hidrocystoma
Eccrine hidrocystoma	Translucent cystic papule; may be solitary or multiple.	Often worsens with heat, humidity, or sweating. Histology lacks apocrine decapitation secretion.
Cyst of Moll	Small translucent cyst at the eyelid margin.	Very close mimic; arises from glands of Moll. Some authors consider it within the apocrine cyst spectrum. Histology confirms apocrine lining.
Epidermal inclusion cyst	Smooth, firm, dome-shaped subcutaneous nodule; may show a punctum.	Usually more firm and keratin-filled; central punctum may be present. Histology shows keratinizing squamous epithelium with laminated keratin.
Dermoid cyst	Firm, painless, deep-seated mass, commonly near lateral brow/orbital rim.	Usually congenital or long-standing; deeper and less translucent. Imaging may be required if orbital extension is suspected.
Chalazion	Firm eyelid nodule within the tarsus; may follow inflammation.	Related to meibomian gland obstruction; may have tenderness, inflammation, or recurrence. Not typically translucent or blue-black.
Milia	Tiny white-to-yellow superficial keratin cysts.	Smaller, often multiple, superficial, pearly-white lesions; not bluish or cystic-translucent.
Syringoma	Multiple small skin-colored/yellowish papules, often on lower eyelids.	Usually multiple and symmetric; solid adnexal lesion rather than a cystic/translucent lesion.
Hemangioma / venous malformation	Red-blue compressible vascular lesion.	Blanching, compressibility, change with posture or Valsalva, and vascular pattern on imaging/dermoscopy favor a vascular lesion.
Lymphangioma / lymphatic malformation	Translucent or bluish cystic lesion; may be multiloculated.	May enlarge after upper respiratory infection or hemorrhage; “frog-spawn” appearance may be seen clinically or on imaging.
Blue nevus	Blue-black, well-defined papule or macule.	Usually solid rather than cystic; dermoscopy and histology help distinguish it from pigmented hidrocystoma.
Sebaceous hyperplasia / sebaceous adenoma	Yellowish papule, sometimes with central dell.	Yellow lobulated appearance and sebaceous differentiation; not typically translucent cystic. Atypical lesions should be distinguished from sebaceous carcinoma.
Basal cell carcinoma	Pearly papule/nodule with telangiectasia; may ulcerate.	Red flags include ulceration, rolled borders, telangiectasia, lash loss, induration, bleeding, or progressive growth. Requires biopsy.
Cutaneous melanoma / amelanotic melanoma	Pigmented or non-pigmented enlarging lesion with irregularity.	Irregular pigmentation, asymmetry, rapid growth, bleeding, ulceration, or atypical vascularity should prompt urgent biopsy.
Other adnexal tumors	Variable papules or nodules of eyelid/periocular skin.	Often solid, recurrent, enlarging, or atypical. Histopathology is needed for definitive classification.

Histological Appearance

Microscopically, apocrine hidrocystomas can appear as a unilocular or multilocular cystic lesion composed of two layers.  

• The first layer is the inner cyst wall that is composed of secretory columnar epithelium.  The secretory columnar epithelium can consist of a single layer or a double layer of cuboidal-columnar epithelium.
• The inner layer of the cysts lies in front of the myoepithelial cells which comprise the outer or peripheral layer.  
• The characteristic diagnostic feature is apocrine “decapitation secretion,” in which the apical portion of the epithelial cell protrudes into the cyst lumen and is shed as part of the secretory process. A peripheral myoepithelial cell layer may be seen outside the epithelial lining. These findings support apocrine differentiation and help distinguish apocrine hidrocystoma from eccrine hidrocystoma, which typically lacks true decapitation secretion.
• The cyst lumen may contain clear, eosinophilic, or pigmented fluid.
• When lipofuscin granules are present, PAS-positive granules can also be seen histologically.
• Apocrine hidrocystomas also exhibit papillary projections which appear in the lumen of the cyst as an outgrowth from the wall. Papillary projections consist of vascular connective tissue covered by secretory epithelium.
• Immunohistochemistry is not routinely required in typical cases but may be useful when the morphology is unusual or when distinction from other adnexal tumors is difficult.
• Markers supporting apocrine differentiation may include epithelial membrane antigen, gross cystic disease fluid protein-15, androgen receptor, cytokeratin 7, and myoepithelial markers such as p63, smooth muscle actin, or calponin highlighting the outer myoepithelial layer.

Inlet with higher magnification showing double layer epithelial lining (hematoxylin and eosin, original magnification x20). Pathology interpretation by Obianuju Mercy Anelo MD, Nina Krassilnik MD at UTHSC-Memphis

Treatment

Management depends on lesion size, symptoms, cosmetic concern, and diagnostic certainty. Small, typical-appearing, asymptomatic lesions may be observed if there are no suspicious features. However, because apocrine hidrocystoma can clinically mimic other benign and malignant periocular lesions, excision is often preferred when the diagnosis is uncertain.

Complete surgical excision with narrow margins is the most commonly recommended treatment for solitary apocrine hidrocystoma. Excision provides both definitive treatment and histopathological confirmation. Care should be taken to remove the cyst wall completely, as incomplete removal or simple drainage may increase the likelihood of recurrence.

Alternate treatments include needle puncture, cyst puncture followed by hypertonic glucose sclerotherapy and include cyst puncture with trichloroacetic acid injection and botulinum toxin A are currently being explored. Needle puncture has been found to have higher rate of recurrence compared to complete cystic excision including the walls of the cyst.  Cyst puncture followed by hypertonic glucose sclerotherapy or followed by trichloroacetic acid injection and botulinum toxin A have been found to be an effective therapeutic modality.

Prognosis

The prognosis of apocrine hidrocystoma is excellent. These lesions are benign and have no metastatic potential. Complete surgical excision is usually curative, and recurrence is uncommon when the cyst wall is removed completely.

Most patients do not develop functional complications after treatment, especially when lesions are small and superficial. In periocular lesions, outcome depends on size, location, and proximity to the eyelid margin, lacrimal drainage system, or orbit. Large, recurrent, or orbital lesions may require more careful surgical planning to preserve eyelid function and cosmesis.

Malignant transformation of a typical apocrine hidrocystoma has not been clearly established in routine clinical practice and appears to be exceedingly rare. Nevertheless, histopathological evaluation remains important because clinically similar lesions may represent malignant tumors rather than true apocrine hidrocystoma.

Complications

• Complications of apocrine hidrocystoma are uncommon because the lesion is benign. Large periocular lesions may rarely cause cosmetic concern, irritation, mechanical ptosis, incomplete lid closure, or visual field obstruction.
• Recurrence may occur after incomplete excision, cyst rupture, or simple drainage without removal of the cyst wall.
• Procedure-related complications are uncommon but may include bleeding, infection, scarring, eyelid edema, pigmentary change, or injury to adjacent eyelid or lacrimal structures.
• The main clinical concern is misdiagnosis, as apocrine hidrocystoma may mimic basal cell carcinoma, melanoma, blue nevus, epidermal inclusion cyst, chalazion, or other adnexal tumors. Atypical, recurrent, ulcerated, bleeding, indurated, irregularly pigmented, or lash-loss-associated lesions should undergo histopathological evaluation.

Clinical Pearls

• Apocrine hidrocystoma is a benign cystic adnexal lesion arising from apocrine glands.
• Periocular lesions are commonly related to the glands of Moll.
• Typical lesions are solitary, slow-growing, dome-shaped, translucent to bluish cystic nodules.
• Important mimics include eccrine hidrocystoma, epidermal inclusion cyst, blue nevus, basal cell carcinoma, and melanoma.
• Histopathology confirms the diagnosis by showing apocrine epithelial lining with decapitation secretion.
• Complete excision is usually curative, and recurrence is uncommon when the cyst wall is fully removed.