THE JOURNAL OF PEDIATRICS • www.jpeds.

com REDISCOVERING
THE PHYSICAL EXAM
It Is Not Just a Skin Tag: Infantile Perianal Pyramidal Protrusion

A
10-month-old girl was referred to Pediatric Gynecol-
ogy after her parents noticed an increasing genital
bulge. Parents reported first noticing what they be-
lieved to be a skin tag in the perineal area at birth. Over the
next few months, the area became more swollen and did not
regress despite recommended sitz baths and barrier creams.
Medical history was unremarkable except for increasing con-
stipation since the introduction of solid food at 6 months of
age. There were no concerns for sexual abuse. Her mother
denied any history of human papilloma virus infection.
Physical examination revealed normal prepubertal genita-
lia. On inspection of the perineum, an elongated, pyramidal
flesh-colored lesion extended from just below the vaginal ves-
tibule to the superior aspect of the anal verge (Figure). No
bleeding or excoriations were noted. Over the next 2 months,
her constipation was treated with dietary modifications and
daily Miralax, and the lesion decreased in size by approxi-
Figure. A 10-month-old girl with perianal protrusion encom-
mately 50%.
passing entire perineum.
Infantile perianal pyramidal protrusion (IPPP), also re-
ferred to as infantile perianal protrusion or infantile perineal
protrusion, was first described in 1996 and then reexamined
and investigated in case reports and observational studies.1-4
It has been described as a benign disease, predominately oc- congenitally weak area of the perineum sensitive to increased
curring in females, and characterized by a pyramidal shaped pressure with Valsalva4,5; (2) acquired, given its association with
protrusion located on the midline raphe of the perineum, diarrhea, fistulas, and anal fissures; this has been speculated
usually anterior to the anus.2,3 to be secondary to mechanical irritation from wiping2,3,5; (3)
Although initially described as skin tags or folds with in- association of IPPP with LSA, attributing the lesion to a re-
creased recognition, subsequent authors have described the wide arrangement of fibrous tissue caused by inflammation related
variety of shapes including pyramidal, papular, peanut shaped, to LSA.1,2
leak-like, tongue-like, and hen’s crest-shaped with colors ranging Many studies noted a spontaneous decrease in size or reso-
from flesh colored to red.1-5 Color may vary given the condi- lution of the lesions over time. If it is suspected to be of the
tion or health of the perineum, considering the recognition acquired form, management of the precipitating condition is
of diaper dermatitis in some infants as well as irritation from recommended, instituting dietary changes and aggressively
constipation, and other skin conditions including lichen treating constipation or diarrhea when present. If there is
sclerosus et atrophicus (LSA). clinical or pathologic evidence of LSA, then treatment of the
The differential diagnosis of perineal lesions includes but underlying condition should be initiated with topical
is not limited to hemorrhoids, hemangiomas, rectal pro- corticosteroids.6 ■
lapse, granulomatous perineal lesions of Crohn’s disease, sexual
abuse, condyloma, molluscum contagiosum, and skin tags. The Alexis Scaparotti, MD
appearance of the lesions can create significant anxiety for Patricia S. Huguelet, MD
parents because of the concern for sexually transmitted in- Department of Obstetrics and Gynecology
fections when the lesions are misclassified as condyloma. Pediatric and Adolescent Gynecology
The pathogenesis of IPPP is unknown, but most experts agree Children’s Hospital Colorado
that the lesions are caused by 1 of 3 mechanisms: (1) embryo- University of Colorado School of Medicine
logic, a congenital weakness of the median raphe or a remnant Aurora, Colorado
of the urogenital septum2,3,5; reports of new lesions develop-
ing after an episode of constipation, supporting a theory of a References available at www.jpeds.com

J Pediatr 2017;181:321
0022-3476/$ - see front matter. © 2016 Elsevier Inc. All rights reserved.
http://dx.doi.org10.1016/j.jpeds.2016.10.038

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References 4. Zavras N, Christianakis E, Tsamoudaki S, Velaoras K. Infantile perianal py-

1. Cruces MJ, De La Torre C, Losada A, Ocampo C, García-Doval I. Infan-
tile pyramidal protrusion as a manifestation of lichen sclerosus et atrophicus.
Arch Dermatol 1998;134:1118-20.
2. Patrizi A, Raone B, Neri I, D’Antuono A. Infantile perianal protrusion: 13
new cases. Pediatr Dermatol 2002;19:15-8.
3. Konta R, Hashimoto I, Takahashi M, Tamai K. Infantile perineal protru-
sion: a statistical, clinical, and histopathologic study. Dermatology
2000;201:316-20.
ramidal protrusion: a report of 8 new cases and a review of the literature.
Case Rep Dermatol 2012;4:202-6.
5. Miyamoto T, Inoue S, Hagari Y, Mihara M. Infantile perianal pyramidal
protrusion with hard stool history. Br J Dermatol 2004;151:229.
6. Kim BJ, Woo SM, Li K, Lee DH, Cho S. Infantile perianal pyramidal pro-
trusion treated by topical steroid application. J Eur Acad Dermatol Venereol
2007;21:263-4.

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