Visual Diagnosis: Severe Scaly Pruritic Rash in an 8-year-old Girl With Trisomy 21

Presentation

An 8-year-old girl with trisomy 21 presents with 2 weeks of a rapidly spreading scaly and intensely pruritic rash involving the entire body that is particularly prevalent on her hands and feet. Approximately 6 months ago, the patient’s mother noted white pruritic plaques beginning on the palmar surfaces of both of her daughter’s hands. She was given a diagnosis of eczema by her pediatrician. After failing to improve over a period of 1 month with hydrating creams, she was referred to a dermatologist, who also diagnosed eczema. The patient was given triamcinolone cream, and over 4 months, the rash showed initial improvement. However, it then quickly spread to the chest, axillae, legs, and scalp over 2 weeks, prompting the parents to seek further medical attention.

Upon presentation to our hospital, the patient is febrile and diffusely pruritic. There are irregularly thick white plaques over the palmar surfaces of the hands, with underlying violaceous erythema, hyperkeratosis of the skin, dyskeratosis of the fingernails, and numerous deep skin fissures (Fig 1). She has involvement of intertriginous regions of the axillae and breast skinfolds and inguinal folds as well as scaling of the entire scalp, most notable at the hairline. There is substantial swelling of the lower extremities, with sharply demarcated desquamation, painful hyperkeratotic dermatosis, thick white scales of the medial and anterior aspects of both feet, and active bleeding from excoriated regions (Fig 2 …