Reflections on infantile perianal pyramidal protrusion and Hippocrates

By Warren R. Heymann, MD, FAAD
May 29, 2024
Vol. 6, No. 22

Patient safety and quality improvement have been at the forefront of medical care since the publication of “To Err is Human: Building a Safer Health System” by the Institute of Medicine, which estimated that as many as 98,000 people die in any given year from medical errors occurring in hospitals. Their key recommendation was: “Performance standards and expectations for health care organizations should focus greater attention on patient safety.” (1) The most frequent categories of errors in dermatology included assessment and intervention errors. (2) In broad terms, these are errors of omission due to actions not taken and errors of the commission due to a wrong action. (3) (For example, observing a possible melanoma instead of performing a biopsy is an error of omission while doing a wide excision on a seborrheic keratosis because it could be a melanoma is an error of commission.) The correct aphorism attributed to Hippocrates is “Do good or at least do not harm” (Ώφελέειν ᾔ βλάπτειν) (4) or “In illnesses one should keep two things in mind, to be useful rather than cause no harm.” (ὠφελέειν ή μὴ βλάπτειν) (5) inappropriately translated into Latin as “primum non nocere” (first, do no harm). (5)

Child abuse and neglect remains a significant cause of morbidity and mortality in children. Dermatologists have a critical role in evaluating child abuse and neglect as mandated reporters and experts in cutaneous disease. (6) McCann et al., in their study of perianal findings in prepubertal children selected for non-abuse, state, “The relatively high incidence of perianal soft tissue changes that were found in this study, when compared to the frequency of similar observations in children suspected of having been sexually abused, reemphasizes the caution medical examiners must exercise in rendering an opinion as to the significance of medical findings.” Wedge-shaped smooth areas in the midline, with or without depressions, were found both anterior and posterior to the anus in 21 of 81 (26%) children. Anal skin tags/folds were discovered anterior to the anus in 18 of 164 children (11%). (The number of observed subjects varied due to missing data as a result of changes over time in the number of variables assessed.) Anal skin tags/folds were all found only in girls, located in the midline in the same relative proportion in preschool, school-aged, and preadolescent children. In all but 1 of the 18 children, the tags were located anterior to anal orifice. (7)

In 1996, Kayashima et al. reported 15 infants (14 girls and one boy) with a pyramidal protrusion located only in the midline anterior to the anus. Histological examination revealed acanthosis in the epidermis, marked edema in the upper dermis, and mild dermal infiltrates. The patients had been brought to the hospital because of swelling of the protrusion. However, all protrusions gradually reduced over weeks without any treatment other than petrolatum. In the differential diagnosis, the authors acknowledge that it is necessary to exclude perianal eruption associated with child abuse or skin diseases such as genital warts, granulomatous lesions of inflammatory bowel disease, or rectal prolapse. The authors proposed the term infantile perianal pyramidal protrusion (IPPP) as being more precise than “skin tags/folds” used by McCann et al. (8) (In my read of McCann et al., I would have compared the “wedge-shaped smooth areas” more than the “tags/folds” to the cases presented by Kayashima et al. as being cases of IPPP.)

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Since the initial description of IPPP, many reports have challenged that appellation. Instead of pyramidal, lesions have been called papular, peanut-shaped, leaf-like, tongue-like, and crest shaped. (9) Although lesions usually appear anterior to the anus, they may be present concomitantly anteriorly and posteriorly, prompting Leung to suggest that perineal is a superior term to perianal. (10) Hernandez-Machin et al reported the case of an 11-year-old girl with anal and vulvar lesions of IPPP with a history of lichen sclerosus. (11) Gorcey et al. reported IPPP in 6 adult women, aged 20 to 68 years; 5/6 were unaware of the lesions. The authors surmise that IPPP has persisted since childhood. None had lichen sclerosus. (12)

As previously noted, the histology of IPPP is not specific. Ultrasonography may display a hypoechoic area with increased vascular flow. (13) Dermoscopy reveals a vascular pattern with red globular and dotted vessels that may be arranged linearly. (13, 14)

In their series 13 cases of IPPP, Patrizi et al conclude that IPP (the authors prefer not to use the term “pyramidal” because of varying morphology, so they refer to cases as IPP) may represent different conditions: a) Constitutional IPP, which may sometimes be familial and/or congenital, remains unchanged over the years and may have a papillomatous leaflike shape with a rosy surface; b) Functional IPP is characteristic of patients affected by constipation; and c) IPP associated with LS&A. (15) Dhami et al performed a retrospective review of 27 IPPP patients from the Mayo Clinic. Twenty-four were girls, and 3 were boys from 1 day to 4 years (mean 10.8 months, median 8.6 months). Symptoms were reported in 63% (17/27) of patients. No patient underwent a skin biopsy. The authors opine that conservative management is indicated for constitutional IPPP because lesions often resolve spontaneously within weeks to months. For acquired IPPP, resolution is often seen spontaneously within 3 weeks but may occur or be hastened after treating associated constipation. LSA-associated IPPP are typically treated with topical steroids.

Most significantly, Dhami et al. conclude with a Hippocratic approach: “It is important for treating clinicians to recognize IPPP to prevent unnecessary and potentially harmful investigations and treatment.” (16)

Point to Remember: Dermatologists must recognize infantile perianal pyramidal protrusion (and its clinical variants) to avoid inappropriate diagnostic and therapeutic measures.

Our expert’s viewpoint

Megha M. Tollefson, MD, FAAD
Professor of Pediatrics
Professor of Dermatology
Mayo Clinic

Evaluating skin lesions or eruptions in the genital region of any child can be anxiety-provoking, even for the most seasoned pediatric dermatologist. First and foremost, there is an immense pressure to have the correct diagnosis due to the high stakes nature of recognizing or not recognizing findings that may be concerning for abuse. When a mandatory reporter, such as a physician, identifies a concern or finding that is suspicious for abuse in a child, that person is required to report it under law. While in that situation as a physician you would like to be as sure as possible of the suspected diagnosis, as one of my pediatric mentors always taught us — “in the case of child abuse and neglect reporting, if there are not times that your suspicion ends up being unfounded, then you are not reporting enough.” No one likes to be wrong in that scenario, however I have found that when thoughtfully and without judgement explaining the situation to the parent or guardian, including emphasizing that it is a concern that should be investigated for the safety of their child, then they are often (but not always) more accepting and sometimes encouraging of the report. If you do find yourself in the situation of reporting suspicion of abuse in a child, it is often helpful to work with your local child abuse pediatrics team if one is available.

IPPP is a benign finding. However, the differential diagnosis may include worrisome lesions, such as genital warts or potentially malignant growths. Incorrect diagnosis of conditions that are more worrisome may lead to tests and procedures that may be traumatic, physically and/or psychologically, for both the child and the parent. For this reason, spreading awareness of the clinical scenarios and the clinical findings that are consistent with benign conditions like IPPP that could otherwise mimic worrisome conditions is important.

In differentiating such conditions, the clinical exam is of paramount importance. In an already often squirmy young child, examining the genital and perineal areas can be extremely challenging. When examining the genital and perineal areas of infants, I try to always do the exam on the exam table rather than in the arms of the parent or caregiver but encourage the parent or caregiver to comfort the child during the exam. And, always be wary of unexpected bodily discharges from the diaper area! In the older child, genital and perineal exams can be uncomfortable and anxiety provoking. A rule of thumb is never to force the exam on the child and to always make sure their comfort is the first priority. In a school-aged child, the parent or caregiver is generally always present in the room, we always offer gowns, and we are careful to drape properly. In the older child/adolescent, a chaperone should always be present in the room, along with the use of proper gowning and draping.

Together we can care for and protect our youngest patients!

1. Institute of Medicine (US) Committee on Quality of Health Care in America. To Err is Human: Building a Safer Health System. Kohn LT, Corrigan JM, Donaldson MS, editors. Washington (DC): National Academies Press (US); 2000. PMID: 25077248.

2. Marsch A, Khodosh R, Porter M, Raad J, Samimi S, Schultz B, Chaney Strowd L, Vera L, Wong E, Smith GP. Implementing Patient Safety and Quality Improvement in Dermatology Part 1: Patient Safety Science. J Am Acad Dermatol. 2022 Feb 7:S0190-9622(22)00207-9. doi: 10.1016/j.jaad.2022.01.049. Epub ahead of print. PMID: 35143912.

3. Rodziewicz TL, Houseman B, Hipskind JE. Medical Error Reduction and Prevention. 2023 May 2. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan–. PMID: 29763131.

4. Yapijakis C. Hippocrates of Kos, the father of clinical medicine, and Asclepiades of Bithynia, the father of molecular medicine. Review. In Vivo. 2009 Jul-Aug;23(4):507-14. PMID: 19567383.

5. Retsas S. First do no harm: The impossible oath. BMJ 2019;366:l4734.

6. Bentivegna K, Grant-Kels JM, Livingston N. Cutaneous manifestations of child abuse and neglect: Part I. J Am Acad Dermatol. 2022 Sep;87(3):503-516. doi: 10.1016/j.jaad.2021.11.067. Epub 2022 Mar 23. PMID: 35339586.

7. McCann J, Voris J, Simon M, Wells R. Perianal findings in prepubertal children selected for nonabuse: a descriptive study. Child Abuse Negl. 1989;13(2):179-93. doi: 10.1016/0145-2134(89)90005-7. PMID: 2743179.

8. Kayashima K, Kitoh M, Ono T. Infantile perianal pyramidal protrusion. Arch Dermatol. 1996 Dec;132(12):1481-4. PMID: 8961878.

9. Adnan M, Sankaran D, Mydam J, Malviya P, Khan I. A Case of Infantile Perianal Pyramidal Protrusion Masquerading As Imperforate Anus at Birth. Cureus. 2021 Oct 5;13(10):e18491. doi: 10.7759/cureus.18491. PMID: 34754652; PMCID: PMC8569653.

10. Leung AK. Concomitant anterior and posterior infantile perianal protrusions. J Natl Med Assoc. 2010 Feb;102(2):135-6. doi: 10.1016/s0027-9684(15)30514-9. PMID: 20191927.

11. Hernandez-Machin B, Pablo Almeida P, Lujan D, Montenegro T, Borrego L. Infantile pyramidal protrusion localized at the vulva as a manifestation of lichen sclerosus et atrophicus. J Am Acad Dermatol 2007; 56 (2) S49–S50.

12. Gorcey L, Spratt EG, Marmon S, Pomeranz MK. Infantile perianal pyramidal protrusions identified in adult women. J Eur Acad Dermatol Venereol. 2015 Mar;29(3):611-2. doi: 10.1111/jdv.12410. Epub 2014 Feb 21. PMID: 24673710.

13. Lamberti A, Filippou G, Adinolfi A, Fimiani M, Rubegni P. Infantile perianal pyramidal protrusion: a case report with dermoscopy and ultrasound findings. Dermatol Pract Concept. 2015 Apr 30;5(2):125-8. doi: 10.5826/dpc.0502a25. PMID: 26114069; PMCID: PMC4462916.

14. Di Bartolomeo L, Borgia F, Pedaci FA, Li Pomi F, Vaccaro M, Filippeschi C, Guarneri F, Oranges T. Dermoscopy features of infantile perianal pyramidal protrusion. Pediatr Dermatol. 2023 Feb 8. doi: 10.1111/pde.15270. Epub ahead of print. PMID: 36754623.

15. Patrizi A, Raone B, Neri I, D'Antuono A. Infantile perianal protrusion: 13 new cases. Pediatr Dermatol. 2002 Jan-Feb;19(1):15-8. doi: 10.1046/j.1525-1470.2002.00010.x. PMID: 11860563.

16. Dhami RK, Isaq NA, Tollefson MM. Infantile perianal pyramidal protrusion: A retrospective review of 27 patients. Pediatr Dermatol. 2023 May-Jun;40(3):468-471. doi: 10.1111/pde.15307. Epub 2023 Mar 26. PMID: 36967585.

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