Pediatric Dermatology Consult

What's the diagnosis?

Publish date: August 18, 2023

A 4-month male was referred to the pediatric dermatology clinic for a rash on the scalp, torso, and the diaper area since he was 2 months of age. He has been treated with nystatin, clotrimazole, and zinc oxide paste with partial improvement. After 2 months of partial improvement the rash worsened again, and he was referred to pediatric dermatology. The mother also reported asymptomatic left upper lateral eyebrow swelling noted a few weeks prior.

Dr. Catalina Matiz and Dr. Laurie Tyrell

Picture 1

He has been growing well with no fevers or other symptoms. He was born via spontaneous vaginal delivery without complications. The mother had prenatal care. On physical exam he had waxy scaly papules and plaques on the scalp. Some telangiectasias were noted on the temporal area (Picture 1.)
Dr. Catalina Matiz and Dr. Laurie Tyrell

Picture 2

On the torso and diaper area, he had multiple scaly pink papules. On the groin he had eroded pink scaly plaques (Picture 2).
On his back he had a 3-mm yellow papule (Picture 3).

Dr. Catalina Matiz and Dr. Laurie Tyrell

Picture 3

On the left upper lateral eyebrow there was some swelling noted without a defined mass, and there was no tenderness or increased vascularity noted on the area. He did not have any enlarged liver or spleen or associated lymphadenopathy. He was treated with fluocinolone oil and topical mupirocin for 2 weeks. He came back a week later for lack of improvement of the skin rash and worsening swelling on the lateral eyebrow.

The most likely diagnosis is:

Atopic dermatitis with bacterial superinfection

Cutaneous candidiasis

Seborrheic dermatitis

Langerhans cell histiocytosis

Allergic contact dermatitis

Multidisciplinary care

The diagnosis of LCH in infants necessitates a combination of clinical, radiological, and histopathologic findings. In infants, cutaneous involvement is a frequent initial presentation, with characteristic lesions that are often misdiagnosed as other dermatologic conditions. Timely recognition of these lesions and appropriate skin biopsies for histological examination are essential steps in achieving an accurate diagnosis.

Radiological imaging, including x-rays, CT, and MRI, plays a crucial role in assessing the extent of involvement.

The management of LCH in infants requires a well-coordinated multidisciplinary approach involving pediatric oncologists, dermatologists, radiologists, orthopedic surgeons, and other relevant specialists. Treatment strategies vary depending on the extent of disease involvement and the presence of risk factors. In localized cases, observation with close monitoring may be considered, as some cases of LCH in infants may undergo spontaneous regression. However, cases with severe symptoms, extensive organ involvement, or high-risk features may require systemic therapies.

Dr. Catalina Matiz, a pediatric dermatologist at Southern California Permanente Medical Group, San Diego

Dr. Catalina Matiz

Chemotherapy agents, including vinblastine and prednisone have been utilized in the treatment of infantile LCH with varying success. The selection of treatment regimens should be tailored to each individual case, considering disease severity, potential toxicities, and long-term effects. In cases of bone lesions causing significant deformities or functional impairment, surgical intervention may be necessary. Skin only disease can be treated with topical corticosteroids.

Prognosis

Survival rates in patients with single-organ involvement without risk-organ involvement is close to 100% and with risk-organ involvement of 98% at 5 years.

Long-term follow-up is essential for infants diagnosed with LCH, as recurrence and late effects can occur even after successful treatment. Continued monitoring allows for the timely detection of relapses or the development of secondary complications.

Infants thought to have common skin conditions like eczema, seborrheic dermatitis, or diaper dermatitis not responding to treatment should be referred to pediatric dermatology for evaluation to rule out the possibility of LCH.

Dr. Matiz is a pediatric dermatologist at Southern California Permanente Medical Group, San Diego.

References

Krooks J et al. J Am Acad Dermatol. 2018 Jun;78(6):1035-44.

Krooks J et al. J Am Acad Dermatol. 2018 Jun;78(6):1047-56.

Leung AKC et al. World J Pediatr. 2019 Dec;15(6):536-45.

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