Image 1-A bulge in the right labium majus. Note the increased hair. Image taken from Vargas SO, etal. Am J Surg Pathol. 2005 Aug;29(8):1007-1016

Image 2-T1 weighted MRI postcontrast with soft tissue bilateral enlargement of the labia. Image taken from Vargas SO, etal. Am J Surg Pathol. 2005 Aug;29(8):1007-1016

Image 3-Microscopic sections show broadened fibrous septa surrounding benign fat lobules. Image taken from Vargas SO, etal. Am J Surg Pathol. 2005 Aug;29(8):1007-1016

Issues in Dermatopathology

CALME-No Need to Panic!

Childhood Asymmetric Labium Majus Enlargement-Mimicking a Neoplasm

Vargas SO, Kozakewich HPW, Boyd TK, Ecklund K Fishman SJ, Laufer MR, Perez-Atayde AR.

Am J Surg Pathol 2005; Aug;29(8):1007-1016

Dermatologists are frequently asked to evaluate genital lesions. This article, presented in the surgical pathology literature, documents a new disease entity that will probably be unfamiliar to most dermatologists and pathologists.

The authors present cases from 14 girls ranging in age from 3.9-13.2 years. All of the girls presented with enlargement of the labium majora (Figure 1). Unilateral and bilateral involvement were documented, either clinically or by radiographic findings (Figure 2). The majority of the patients reported no pain but did note a fluctuation in size, ranging from 2-8 cm.

Thirteen of the fourteen patients underwent surgical excision revealing a bland collection of expanded fibrous septa encircling benign fat lobules. A myxoid extracellular matrix was common associated with variable numbers of elastic fibers and fibroblasts.

Clinical follow up for all cases revealed recurrence in 7/14 (50%) cases and the one case that did not undergo surgical excision underwent regression. Immunoperoxidase analysis revealed positivity for estrogen and progesterone receptors. Cytogenetic analysis performed upon 3 of the cases revealed a normal karyotype.

In conclusion, the authors state that CALME is a relatively common and distinctive clinicopathologic entity occurring in the pre- and early pubertal periods. Because these lesions occurred in the milk line, the appearance may coincide with early breast budding. With the presence of hormone receptors, the authors speculate that CALME may represent an asymmetric physiologic enlargement to pre and early pubertal hormonal surges.

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Additional References:

The Doctor's Doctor-Vagina and Vulva