SkIndia Quiz 29: Multiple facial papules.

A 40-year-old woman presented with gradual onset of multiple raised skin-colored lesions over face since two months. There was no history of any associated complaints such as burning, itching, or pain. There was no history of antecedent trauma, seasonal variation, systemic ailments, or a family history of similar complaints. There was also no history of any oral or topical medication.

Examination revealed multiple discrete 1–2 mm sized, firm skin-colored papules over periorbital area, dorsum of nose, and upper lip [Figure 1] with no evidence of similar lesions elsewhere on the body. On puncture of a lesion with a sterile needle, small amount of serous clear fluid was exuded. A skin biopsy was done to confirm the diagnosis, which showed a unilocular cyst lined by double layer of cells in the dermis [Figure 2a–c].

Figure 1

Multiple dome-shaped papules over periorbital area, dorsum of nose, and upper lip

Figure 2

(a) Haematoxylin and Eosin staining shows a unilocular cyst in the upper dermis lined by double lining of cells. (b) Low power magnification (10x) shows a unilocular cyst containing eosinophilic fluid. (c) High power magnification (40x) shows a portion of cyst lined by double lining of cells

ANSWER

Eccrine hidrocystoma.

DISCUSSION

Hidrocystomas[1] are uncommon benign cystic tumors of the sweat glands, which can be classified into two types, namely, eccrine and apocrine on the basis of their presumed histogenetic division. Eccrine hidrocystomas originate from the eccrine sweat ducts and usually result from dilatation of cystic excretory eccrine glands due to retention of sweat or blockage of the sweat duct.[23] They usually present as cystic dome-shaped papules with an amber, brown, or bluish tint ranging from 1 to 6 mm in diameter over the periorbital and malar areas of middle-aged or elderly women. Skin lesions usually worsen in hot and humid weather.

Eccrine hidrocystomas are classified into two major groups: The most prevalent Smith and Chernosky or solitary type, and the Robinson or multiple type. Difficulty may be encountered in differentiating it from apocrine hidrocystoma, which usually presents as dome-shaped, solitary, clear, cystic nodule with a smooth surface and blue-black tinge, with a diameter of 3–15 mm. These lesions are found mostly on the head and neck and along the eyelid margin near the inner canthus but unlike eccrine lesions, they are less likely to occur at periorbital regions with no evidence of seasonal variations.

Under the microscope, eccrine hidrocystomas appear as unilocular cysts composed of one or two layers of cuboidal cells located within the mid-dermal to superficial layers of the skin with no evidence of decapitation as seen in the apocrine counterpart. Histochemically, they stain positive for S-100 protein (solitary type), whereas apocrine hidrocystomas do not.

The inherited disorders most commonly associated with the presence of multiple eccrine or apocrine hidrocystomas are Goltz–Gorlin syndrome and Schopf–Schulz–Passarge syndrome. Graves’ disease has also been associated with multiple eccrine hidrocystomas.[4]

Cystic lesions such as syringomas, epidermal inclusion cysts, mucoid cysts, cystic pigmented type of basal cell carcinoma, hemangioma, and lymphangioma need to be considered in the differential diagnosis, necessitating the need for skin biopsy to exclude these diagnoses.

No definitive treatment has been described for eccrine hidrocystomas. However, simple needle puncture is the commonest approach to the treatment of solitary hidrocystomas (eccrine and apocrine, although not producing lasting improvement in Robinson type of hidrocystomas). Other methods that have shown success include topical atropine,[56] glycopyrrolate[7] and scopolamine, botulinum toxin A,[8] electrocautery, carbon dioxide laser vaporization, and 585-nm flash lamp pumped pulse dye laser.[9] Finally, avoiding hot temperatures or humid conditions will prevent worsening of symptoms in eccrine hidrocystomas.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.