Abstract

A 41-year-old woman with a history of chronic malnutrition and gastroschisis repair with multiple subsequent abdominal surgeries, was admitted to the hospital for vision changes and gastrointestinal bleeding. Laboratory studies demonstrated profound vitamin A deficiency (<5 mcg/mL; reference range = 38-98). Physical examination revealed scattered erythematous, dome-shaped papules with central scale on her bilateral lower extremities. A punch biopsy was performed to rule out a perforating disorder. Histologic sections showed an epidermal invagination, representing a dilated follicular ostia, within which transepidermal elimination of elastic fibers was seen, in addition to mild dermal fibrosis, superficial perivascular lymphocytic inflammation, and loss of sebaceous glands. The clinical presentation and histopathologic findings were consistent with phrynoderma with an associated perforating folliculitis. Aggressive vitamin A repletion was initiated to good effect. Phrynoderma is a rare form of follicular hyperkeratosis, presenting as discrete, follicular, scaly, reddish brown papules on the extensor surfaces of the extremities. While most often attributed to vitamin A deficiency, some authors have also implicated generalized malnutrition, as well as deficiencies of vitamins B, E, and essential fatty acids. Phrynoderma has been reported in patients with malnutrition related to gastrointestinal conditions such as Crohn’s disease, bariatric surgery, bowel resection for primary visceral myopathy, pancreatic insufficiency, and chronic giardiasis. Our patient did not have a history of any of these entities. Further, an associated perforating folliculitis is extremely rare. Our case illustrates that phrynoderma should be considered in patients with a history of extensive bowel surgery for any reason and lends support to perforating folliculitis as a feature of this condition, rather than an incidental concurrent finding.

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