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32-year-old woman presented with a one- year history of asymptomatic cystic lesions around her eyes. She had no other complaints or history of major illnesses and had not taken any medications. To the best of her knowledge, nobody in her family had a similar dermatologic condition.
Physical examination revealed numerous tense translucent cystic nodules, some as large as 8 mm in diameter, on the periorbital areas (Figure A). A skin biopsy specimen was obtained from one of the lesions (Figures B and C).
Your Diagnosis?
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pocrine hydrocystoma is generally regarded as a benign apocrine tumor, which is almost always solitary. It is usually found on the head and neck, but can occasionally be seen elsewhere. It equally affects both sexes and most often appears in middle-aged individuals.1
Apocrine hydrocystoma usually presents as a translucent tumor with a cystic consistency and ranges in color from light gray to dark blue-black. In its usual solitary form, it is not associated with a familial incidence, and in contrast to the eccrine variant, shows no seasonal variation. Multiple apocrine hydrocystomas are only rarely encountered and may be hallmarks of two rare inherited disorders: the Schopf-Schulz-Passarge syndrome and a peculiar form of focal dermal hypoplasia.2
Histologically, apocrine hydrocystoma shows one or several large cystic
spaces located in the dermis into which papillary projections often extend. The
inner surface of the wall and the papillary projections are lined with a row of
secretory cells of variable height, showing decapitation secretion indicative
of apocrine secretion; peripheral to the layer of secretory cells, are
elongated myoepithelial cells.3 Eccrine hydrocystoma differs from
the apocrine variant in the absence of decapitation secretion of PAS-positive
granules and lack of myoepithelial cells.3 Using a monoclonal
antibody to alpha-smooth muscle actin in benign sweat gland tumors,
myoepithelial cells have been identified in most sweat gland tumors that have
an inclination to differentiate toward the secretory coil of the
normal sweat gland. In contrast, in eccrine hydrocystoma and other tumors showing differentiation toward the excretory (ductal) component of the gland, myoepithelial cells are absent.4
The treatment of apocrine hydrocystoma is difficult if they are multiple. There is a report of successful treatment of multiple apocrine hydrocystomas with carbon dioxide (CO2) laser.5
Our patient was a rare case of multiple apocrine hydrocystomas. She was treated surgically by an ophthalmologist and had a cosmetically acceptable outcome.
References
1 Smith JD, Chernosky ME. Apocrine hydrocystoma (cystadenoma). Arch Dermatol. 1974; 109: 700 – 22.
2 Alessi E, Gianotti R, Coggi A. Multiple apocrine hydrocystomas of the eyelids. Br J Dermatol. 1997; 137: 642 – 5.
3 Elder D, Elentisas R, Ragsdale BD. Tumors of the epidermal appendages. In: Elder D, Elentisas R, Jaworsky C, et al, eds. Histopathology of the Skin. Philadelphia , Pa: Lippincott-Raven; 1997: 747 – 803.
4 Eckert F, Betke M, Schmoeckel C, et al. Myoepithelial differentiation in benign sweat gland tumors, Demonstrated by a monoclonal antibody to alpha-smooth muscle actin. J Cutan Pathol. 1992; 19: 294 – 301.
5 Bickley LK, Goldberg DJ, Imaeda S, et al. Treatment of multiple apocrine hydrocystomas with the carbon dioxide (CO2) laser. J Dermatol Surg Oncol. 1989; 15: 599 – 602.
Javad Golchai MD*, Omid Zargari MD*, Mohammad-Reza Sabouri MD**, *Department of Dermatology, **Department of Ophthalmology, Guilan University of Medical Sciences, Rasht, Iran.