Septic, CD-30 positive febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta.
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.
Herron
Mark D
MD
Department of Dermatology, University of Utah School of Medicine, Salt Lake City, Utah, USA.
Bohnsack
John F
JF
Vanderhooft
Sheryll L
SL
D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D016470:Bacteremia / Q000188:drug therapy / Q000382:microbiology; D002175:Candida / Q000302:isolation & purification; D002648:Child; D004359:Drug Therapy, Combination; D005260:Female; D005334:Fever / Q000188:drug therapy / Q000209:etiology*; D016469:Fungemia / Q000188:drug therapy / Q000382:microbiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D017730:Ki-1 Antigen / Q000032:analysis*; D008214:Lymphocytes / Q000276:immunology / Q000378:metabolism; D017514:Pityriasis Lichenoides / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D011550:Pseudomonas aeruginosa / Q000302:isolation & purification; D015375:Receptors, Interleukin-2 / Q000097:blood; D012128:Respiratory Distress Syndrome / Q000628:therapy; D018805:Sepsis / Q000188:drug therapy / Q000382:microbiology*; D012883:Skin Ulcer / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D013212:Staphylococcus epidermidis / Q000302:isolation & purification
D000900:Anti-Bacterial Agents; D007166:Immunosuppressive Agents; D017730:Ki-1 Antigen; D015375:Receptors, Interleukin-2