Septic, CD-30 Positive Febrile Ulceronecrotic Pityriasis Lichenoides et Varioliformis Acuta
Article first published online: 21 JUL 2005
Volume 22, Issue 4, pages 360–365, July 2005
How to Cite
Herron, M. D., Bohnsack, J. F. and Vanderhooft, S. L. (2005), Septic, CD-30 Positive Febrile Ulceronecrotic Pityriasis Lichenoides et Varioliformis Acuta. Pediatric Dermatology, 22: 360–365. doi: 10.1111/j.1525-1470.2005.22418.x
- Issue published online: 21 JUL 2005
- Article first published online: 21 JUL 2005
Abstract: We report life-threatening febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta in an 8-year-old girl. Hemorrhagic-crusted papules and plaques covered over 90% of the patient's body, leaving her susceptible to Pseudomonas aeruginosa and Staphylococcus epidermidis bacteremia as well as Candida parapsilosis fungemia. Sepsis delayed definitive treatment of the underlying cutaneous disease for 2 weeks. Combined therapy with methotrexate and cyclosporin caused remission of the process. Although immunohistochemistry revealed CD-30 positive cells, suggesting the diagnosis of lymphomatoid papulosis, the histopathology was most compatible with pityriasis lichenoides et varioliformis acuta. A partial loss of CD2 and CD5 in the predominant CD3 T-cell lymphocytes suggested a clonal proliferation. Elevated soluble interleukin-2 receptor levels reflected marked T-cell activation, and the downward trend of the levels during treatment coincided with clinical regression of this inflammatory dermatosis.