Isosexual pseudoprecocious puberty in a 2(1/2)-year-old girl presenting with intense skin pigmentation.

BACKGROUND

Ovarian follicular cyst producing estradiol is a rare cause of isosexual pseudoprecocious puberty. Intense pigmentation of breast papillae, areolae, and labia minora is also rarely reported in the literature.

CASE: We describe a 2(1/2) year old girl presenting with signs of precocious puberty and advanced bone age due to a large follicular cyst. Estradiol and Dehydro-epiandrosterone sulfate (DHEAS) levels were remarkably elevated. Hyperpigmentation was also noted. Salpingoophorectomy resulted in regression of precocity and depigmentation, but DHEAS serum levels remained elevated.

SUMMARY AND

CONCLUSION

High levels of circulating estradiol due to an ovarian follicle can induce precocious puberty and pigmentation of the skin which regresses after surgical removal of the cyst. Elevated DHEAS levels may be the initiating event causing the formation of the large follicular cyst.