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Multiple apocrine hidrocystomas

  • Author(s): Anandasabapathy, Niroshana
  • Soldano, Anthony C
  • et al.
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Multiple apocrine hidrocystomas
Niroshana Anandasabapathy MD, PhD Anthony C Soldano MD
Dermatology Online Journal 14 (5): 12

Department of Dermatology, New York University

Abstract

A 83-year-old woman presented with a 7-year history of translucent papules that were scattered diffusely over her nose, peri-orbital region, and cheeks. These lesions were exacerbated by heat and exercise. Histopathologic examination of a biopsy specimen from the cheek showed a thin-walled cyst lined by a flattened bi-layered epithelium that exhibited decapitation secretion in the upper part of the epidermis. A diagnosis of multiple apocrine hidrocystomas was made based on the clinical and histopathologic findings and the patient is currently considering treatment options. These include electrodesiccation, excision, trichloracetic acid, carbon-dioxide laser, and 1450-nm diode laser.



Clinical synopsis

An 83-year-old woman presented to the Charles C. Harris Skin and Cancer Pavillion in March 2003 for an intermittent eruption on her face that began in 1999. These eruptions occurred in the summer on hot days as well as when exercising. The eruption initially lasted several hours, but at the time of her initial presentation, it had been present constantly for 1 month; she had not received any treatment. Therapy with 2 percent salicyclic acid in isopropyl alcohol twice daily was initiated. Although the patient was advised to return to clinic in 1 month, she was lost to follow up until August 2006. At this time she reported an exacerbation of the eruption, which had been present for the entire summer. She reported no benefit with the 2 percent salicylic acid treatment. She did not seek treatment during the interim period because in the winter months the eruption cleared on its own. A biopsy was taken from a lesion on the left cheek. Medical history includes arthritis and hypertension, for which she takes atenolol. The patient has no known medical allergies.


Physical examination

Numerous, 2-3-mm, translucent papules were present on the nose, nasal bridge, cheeks, and periorbital regions and extended to the hairline laterally.


Figure 1Figure 2

Laboratory data

None


Histopathology

In the upper part of the dermis, there is part of a thin-walled cyst that is lined by a double layer of flattened epithelial cells. The inner layer exhibits decapitation secretion.


Comment

Apocrine hidrocystomas, which are also known as apocrine cystadenomas and apocrine retention cysts, most commonly appear as solitary papules and nodules that are particularly common on the inner canthus of the eyelid during adulthood. They may also occur on the head, neck, trunk, penis, axilla, and anus [1]. The cause is unknown, but they are considered to be benign adenomatous cystic proliferations of the apocrine glands [2]. The closely related eccrine hidrocystomas more commonly occur as multiple lesions and are thought to result from blockage of the sweat duct apparatus. Unlike eccrine hidrocystomas, apocrine hidrocystomas have not been related to differences in temperature [1, 3]. There are no known laboratory abnormalities, and neither sex nor race predilection has been described. Multiple apocrine hidrocytomas are associated with Schopf-Schulz-Passarge syndrome, a rare autosomal recessive syndrome in which ectodermal dysplasia occurs. Schopf-Schulz-Passarge syndrome often features palmoplantar hyperkeratosis, hypodontia, nail dystrophy, and hypotrichosis [4, 5]. An association of multiple apocrine hidrocystomas with a peculiar form of focal dermal hypoplasia also has been reported [6].

Histopathologic examination shows large unilocular or multilocular cystic spaces within the dermis. The cyst wall is lined with epithelial cells that are cuboidal-to-columnar shaped, with their nuclei positioned basally. The cyst cavity shows decapitation secretion, which is characteristic of apocrine secretion. Myoepithelial cells form the peripheral cyst wall layer [7]. The secretory granules stain with periodic acid-Schiff and are diastase-resistant. They may be pigmented providing the brown color of the cystic fluid [1]. Electron microscopy shows secretory cells with numerous, dense, lysosomal-type secretory granules typical of apocrine gland cells and an increased number of annulate lamellae, which are unusual in normal apocrine cells [1]. One study debates the histopathological subclassification of tumors as apocrine hidrocystomas, eccrine hidrocystomas, or apocrine cystadenomas without immunohistostains, which suggests misclassification as apocrine cystadenomas because of cystic wall flattening from increased pressure [8]. Solitary lesions are usually treated with simple excision. Multiple apocrine hidrocystomas are rare, and a variety of therapeutic techniques have been used. Recent reports have included case studies in which trichloracetic acid, carbon-dioxide laser, and 1450-nm diode laser have been employed [9, 10, 11]. Electrosurgery and excision have also been used [13, 14]. Additional therapies to consider are those used for multiple eccrine hidrocystomas, which include botulinum toxin, atropine, pulsed-dye laser, and most recently the 595 nm long-pulsed laser [15, 16, 17, 18].

References

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18. Lee HW, et al. Multiple eccrine hidrocystomas: successful treatment with the 595 nm long-pulsed dye laser. Dermatol Surg 2006;32:296

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