Septic, CD‐30 Positive Febrile Ulceronecrotic Pityriasis Lichenoides et Varioliformis Acuta

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.

Pityriasis lichenoides chronica: stratification by molecular and phenotypic profile

“…Second, lymphoid atypia including cells with a cerebriform appearance, is frequent. Lymphoid atypia was demonstrated with regularity in this series and has been described in other articles [9,11,12,19]. The extent of lymphoid atypia could be correlated with the molecular findings.…”

Febrile Ulceronecrotic Mucha-Habermann Disease: Treatment with Infliximab and Intravenous Immunoglobulins and Review of the Literature

“…Weinberg et al [47] in a retrospective and prospective analysis of PLEVA tissue samples found that 8 of 14 (57%) specimensdemonstrated monoclonal T cell receptor gene rearrangements.These findings lead to the conclusion that PLEVA is a benign clonal T cell disorder. Herron et al [31] reported the presence of CD30+ T lymphocytes and elevation of soluble IL-2 receptor level in a report of -febrile ulceronecrotic PLEVA. Elevated sIL-2R represents systemic T cell activation, seen in cutaneous T cell lymphomas.…”

“…Immunosuppressive monotherapy with methotrexate [6,8,11,13,15,19,25,28,29,36,40] or cyclosporine [31,35] or a combination of one of these with a high dose of glucocorticoids [6,8,11,13,25,28,29,36,41,43] has been used in -several cases. Antibiotics like erythro-mycin, acyclovir, ultraviolet photother-apy (PUVA) [11,19], IVIG [5,28,36,41,] photochemotherapy [41], 4,4-diaminodiphenyl sulfone [7,8,10,23,] and debridement with skin grafting [23] have also been used to treat FUMHD.…”

“…A febrile ulceronecrotic severe variant of PLEVA, called febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is more destructive and associated to large coalescent skin necrosis with a rapid progression [1]. 48 cases of PLEVA fulminans have been reported to date in the literature with 9 lethal forms (20%) (table 1) [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44]. Oral, genital and conjunctival mucosa can be affected [2,6,9,12,13].…”

Review of systemic methotrexate therapy in pediatric dermatoses

“…Combination of MTX with systemic steroids or cyclosporine and antibiotics has induced remission even in life-threatening cases. [39][40][41][42] …”