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14-year-old male with history of ALL presents with perifollicular rash
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A 14-year-old boy with a significant medical history of acute lymphocytic leukemia (ALL) was admitted awaiting blood count recovery after chemotherapy. You were consulted for evaluation of a papule on his gums. The patient has reported poor appetite over the past few months while receiving chemotherapy for treatment of his leukemia. He was fatigued, but was otherwise behaving normally. He denied night sweats, fever, nausea, vomiting, abdominal pain, joint pain or dysuria. However, the patient did report that his gums had been bleeding easily when he brushed his teeth.
Upon exam, there was a 2 mm red to purple friable papule superior to his upper incisors. He also was noted to have red perifollicular hemorrhagic macules surrounding the hair follicles of his legs bilaterally. A significant number of the hairs on his legs were shortened or curled.
Image: Streicher JL
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Case Discussion
Vitamin C (D) (ascorbic acid) is an important nutrient found in fresh fruits and vegetables. The nutrient can easily be destroyed by overcooking, processing of foods and prolonged storage. Scurvy is the clinical presentation of vitamin C deficiency that can be seen within 3 months of deficient intake of the vitamin. Many of the signs seen in vitamin C deficiency are related to its important role in collagen formation. Dermatologic findings in scurvy include corkscrew hairs, perifollicular hemorrhage, and inflamed gingiva with bleeding and erosions. In addition, these patients may develop petechiae and ecchymoses. Systemic symptoms of vitamin C deficiency include fatigue, weakness and arthralgias.
The differential diagnosis includes Henoch-Schönlein purpura, capillaritis, and leukemia cutis. Henoch-Schönlein purpura may be considered given the red, purpuric macules distributed symmetrically over the extensor extremities. Capillaritis also is a diagnostic consideration given the uniform, red nonblanching macules due to red blood cell extravasation into the skin. However, one would not expect to see petechiae surrounding only the hair follicles nor oral mucosal involvement in Henoch-Schönlein purpura or capillaritis. Also, this patient was not experiencing any systemic symptoms usually seen in Henoch-Schönlein purpura, including arthralgias, fever, headache or abdominal pain or a history of an antecedent respiratory infection.
Leukemia cutis should be on the differential in a patient with known leukemia who develops new skin lesions. However, ALL rarely presents with cutaneous lesions as compared with acute myelogenous leukemia where it is seen much more frequently. Leukemia cutis normally presents with firm, red to purple papules that were not present in our patient.
Our patient was found to have low serum vitamin C levels, and supplementation was initiated. The treatment of scurvy in children consists of oral vitamin C supplementation of 100 mg to 200 mg daily. Clinical improvement can be seen within 1 week, but treatment needs to be continued for at least 3 months.
The mucocutaneous findings of vitamin C deficiency (scurvy) typically present with perifollicular hemorrhage, corkscrew hairs, and bleeding gums. Practitioners should be aware of the cutaneous findings of this nutritional deficiency and have a higher suspicion for the disease in patients who report chronically poor oral intake, participate in unusual or strict diets that lack citrus, or in the setting of illnesses that may require increased requirements such as febrile illnesses, diarrhea and iron deficiency.
- References:
- Schaefer SM, Hivnor CM. Nutritional Diseases. In: Bolognia JL, et al, eds. Dermatology. 3rd ed. Vol 1. Philadelphia, Pa.; Elsevier; 2012:737-751.
- Shah D, et al. Vitamin C (Ascorbic Acid). In: Kliegman RM, et al, eds. Nelson Textbook of Pediatrics. 20th ed. Vol. 1. Philadelphia, Pa.; Elsevier; 2016:329-331.
- For more information:
- Jenna L. Streicher, MD, is a pediatric dermatology fellow at The Children’s Hospital of Philadelphia. She can be reached at streicherj@email.chop.edu.
- Marissa J. Perman, MD, is an attending physician at The Children’s Hospital of Philadelphia.
Disclosures: Streicher and Perman report no relevant financial disclosures.