Hidrocystomas - A Brief Review

Khashayar Sarabi, BS, MSIV; Amor Khachemoune, MD, CWS

September 06, 2006

Associated Syndromes

The inherited disorders that are most commonly associated with the presence of multiple eccrine/apocrine hidrocystomas are Goltz-Gorlin syndrome and Schopf-Schulz-Passarge syndrome.

Goltz-Gorlin (also known as Jessner-Cole syndrome, or focal dermal hypoplasia) tends to occur sporadically, with few familial cases having X-linked dominant transmission; it occurs mostly in females. Its cardinal features are microcephaly; midfacial hypoplasia; malformed ears; microphthalmia; periocular multiple hidrocystomas; papillomas of the lip, tongue, anus, and axilla; skeleton abnormalities; and mental retardation.^[7]

Schopf-Schulz-Passarge is an autosomal recessive syndrome characterized by multiple eyelid apocrine hidrocystoma, palmoplantar hyperkeratosis, hypodontia, and hypotrichosis. It is further characterized by hypotrichosis, cysts of the eyelids, and multiple periocular apocrine hidrocystomas.^[8]

Graves' disease has also been associated with multiple eccrine hidrocystomas,^[9] possibly due to hyperhidrosis, which is seen in hyperthyroid patients. This is further supported by the disappearance of lesions after treatment of hyperthyroidism.^[9]

References

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Table. Differentiation Between Eccrine and Apocrine Hidrocystomas
	Eccrine Hidrocystomas	Apocrine Hidrocystomas
No. of lesions	Solitary or multiple	Primarily solitary (but occasionally manifesting as multiple lesions)
Color	Ranges from flesh-colored to light-blue tint	Ranges from dark-blue tint to black
Size	1 to 6 mm in diameter	3 to 15 mm in diameter
Age group affected	30-75 years	30-75 years
Gender distribution	More prevalent among females than among males	Females and males are affected in equal numbers
Epithelium types	1-2 layers of cuboidal epithelium	Single/double cuboidal-columnar epithelium
Stains	S-100 positive (solitary type), PAS negative	S-100 negative, PAS positive
Special cellular features	No decapitation of cells, no secretory cells	Decapitation of secretory cells, papillary projections seen under microscope
Body location	Malar, periorbital, chest, axilla, neck	Face, ears, head, chest, shoulders
Possible treatments	Puncture and drainage, electrodessication, carbon dioxide laser, anticholinergic topical creams; placement in cold temperature	Puncture and drainage, electrodessication, carbon dioxide laser, anticholinergic topical creams
Special features	Worse in hot, humid weather	No change with temperature

PAS = periodic acid-Schiff

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Hidrocystomas - A Brief Review

Associated Syndromes

Tables

References

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