A Pediatric Puzzle in the Perianal Area

Presenter: Eric Sandrock, DO, Thomas L Davis, MD, Rick Lin, DO
Dermatology Program:  South Texas Dermatology Residency Program, Bay Area Corpus Christi Medical Center
Program Director: Rick Lin, DO MPH

Submitted on: March 20, 2024

CHIEF COMPLAINT:  “multiple papules on buttocks

CLINICAL HISTORY:

Signs and symptoms: A 6-year-old female presented to our clinic with a four-month history of asymptomatic flesh-colored papules along the intergluteal cleft. The patient’s mother denied any other involved areas. The patient denies any pain, tenderness, or itching of the lesions. The patient has a past history of intellectual delay, right lower extremity hypertrophy, syndactyly of the 2nd through 4th toes on the right foot, venous malformation involving the right lower thigh, knee, and proximal calf, and lichen striatus of the left lower extremity. The papules had been previously treated with over-the-counter cryotherapy without noticeable improvement. The patient follows closely with a vascular malformation clinic in San Antonio for her diagnosis of Klippel Trenaunay Syndrome. Past surgical history includes syndactyly release of the 4th toe. The patient denies constipation, diarrhea, fevers, chills, trouble sitting or standing, unwanted sexual activity, or use of fragrance wipes. No one in the household has similar lesions.

PHYSICAL EXAM:

Physical examination shows a solitary, 1 cm at longest dimension, firm, fixed, and smooth nodule on the distal interphalangeal joint of the left index finger. No erythema or tenderness on palpation noted. 

Images of Chief Complaint:

Additional Physical Exam Findings:

LABORATORY TESTS:

HPV detection by PCR was negative.

DERMATOHISTOPATHOLOGY:

On histopathologic examination, present within the dermis are an increased number of dilated vascular spaces filled with lymph.

DIFFERENTIAL DIAGNOSIS:

1. Molluscum
2. Condyloma
3. Lymphangioma Circumscriptum
4. Nevus Flammeus
5. Spinal Dysraphism
6. Pilonidal Cyst
7. Fibroepithelial Polyp

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