Challenging Pediatric Dermatology Cases: What's the Diagnosis?

Lisa Swanson, MD, a board-certified pediatric dermatologist with Ada West Dermatology in Idaho in Meridian, Idaho, began her presentation, “Peds Derm Challenging Cases” with her “very first patient after moving to Idaho,” she explained. The 6-week-old baby boy had a vascular lesion that had appeared at 1-2 weeks of age and was growing quickly. Swanson’ workup included an ophthalmology exam, MRI/MRA of the head and neck, and an echocardiogram, all normal. Ultimately, the diagnosis was segmented infantile hemangioma, and the child was treated with the beta blocker propranolol. The boy showed improvement after 2 months of propranolol, and at 1 year of age, the lesion had greatly subsided. “For hemangiomas, propranolol is a great treatment,” noted Swanson. She advised 2 mg/kg a day 2 or 3 times a day, always with food. Swanson noted, “it is typically used during the growth period, but can work even beyond the proliferative phase.” Side effects can include sleep disturbance, though Swanson also cited a small study that showed no significant influence on sleep with 24 babies on propranolol and 24 babies who were not on the medication.

For another case, Swanson discussed a 7-week-old patient who presented with a barely-detectable lesion at birth that grew very quickly and started ulcerating 1-2 weeks prior to initial visit. The patient was in pain, cried a lot, and was not able to eat. The diagnosis was, ulcerating hemangioma, and the patient was treated with prednisolone for approximately 2 weeks as well as propranolol. Propranolol was started on 2 mg divided bid, and, when slow to improve, the dosage was increased to 2.5 mg a day, at which point he seemed to improve. Oral nadolol is another treatment for this condition that has proved to be effective (2 mg/day, compounded into suspensions and comes with a constipation warning).

Swanson suggested that health care providers can tend to assume too quickly that presenting lesions are infantile hemangioma. “Every bump on a baby is not necessarily a hemangioma,” explained Swanson. Typical infantile hemangioma is not present (or barely seen) at birth; grows for the first few weeks to months of life; and is soft and squishy. It is most likely not hemangioma if it presents fully developed at birth, firm and does not respond to propranolol.

Swanson also highlighted a case of a 2-year-old girl who started developing rings of blisters 2-3 weeks prior to her visit. The child was not febrile, a little itchy, and had been treated with prednisone with little to no impact. This time the diagnosis was chronic bullous disease of childhood. Treatment for these tense blisters can include dapsone (“this tends to work really well,” said Swanson), though 5 days into the treatment the mother reported that the child’s coloring was “a bit off.” Her tongue was blue, and the girl has a methemoglobin level of 20.9, at which point she was admitted to the PICU for methylene blue injections. The child was not improving, and Swanson consulted with colleagues, some who suggested mycophenolate mofetil, with one colleague suggesting dupilumab. The girl was started on dupilumab and began improving greatly.

Other cases discussed were a 29-year-old male with widespread erythroderma and dry flaky skin that he had been battling since infancy and had managed mostly with petroleum jelly, as topical steroids had been attempted but failed. With a suspicion of CARD-14-associated papulosquamous eruption (which can display symptoms of psoriasis and familial pityriasis rubra pilaris), the man was given ustekinumab, which ultimately resolved the condition.

Swanson’s final case discussion was of a 7-year-old boy with lifelong, severe atopic dermatitis, who also showed symptoms of ADHD and anxiety. Topical steroids had not worked, and the child suffered from constant pruritus and sleep disturbance. After a lengthy battle with the insurance company, Swanson was able to get the child on dupilumab. Swanson’s talk ended on a note of inspiration, urging practitioners to fight insurance companies, when needed, to get their patients the medications required. “Rise up! We are the providers. Insurance companies are not in charge of medical decision making,” she encouraged.

Reference

Swanson, E. Peds derm challenging cases. Presented at: The Society for Dermatology Physician Assistants Annual Summer Meeting. June 16-19, 2022. Austin, Texas.