Epidermal cell necrosis with direct epidermal infiltration of Epstein–Barr virus (EBV)-encoded small nuclear RNA-positive T lymphocytes in a patient with EBV-associated haemophagocytic syndrome

Abstract

strongly indicates that lipo-PGE 1 treatment of livedoid vasculitis with cryoglobulinaemia is effective and safe.There are two known mechanisms by which cryoglobulins can result in disease. The first is by precipitation within the vascular lumen, typically cold induced, with hyaline plug formation and minimal early-phase inflammation. Typical clinical lesions would be minimally inflammatory cutaneous infarction with or without associated livedo reticularis, or noninflammatory retiform purpura. The second mechanism is that of immune complex vasculitis. As nearly all type II (monoclonal component, usually IgM; polyclonal component, usually IgG) and III (one or more polyclonal components) cryoglobulins are immune complexes, they should all be capable of inducing an immune complex vasculitis, although many do not. Histological examination of an early purpuric lesion in our patient clearly showed cryoglobulin thrombus formation within the dermal vessels. The scarcity of polymorphonuclear leucocytes and leucocytoclasia among the histopathological findings argue against a primary vasculitic process. On the other hand, histology of a recurrent painful nodule revealed leucocytoclastic vasculitis. This is considered to be a secondary event rather than the primary event involved in the pathogenesis. These features favour a thrombo-occlusive vasculopathy rather than a vasculitis per se. Cryoglobulins have been implicated in intravascular thrombotic processes with secondary leucocytoclastic vasculitis that can result in skin infarction and necrosis.In conclusion, we believe that lipo-PGE 1 treatment combined with low-dose oral corticosteroid effectively eliminated cryoglobulins and improved leg purpura and skin ulcers in our patient with livedoid vasculitis. Further studies will be needed to clarify the clinical application of lipo-PGE 1 , but we expect that lipo-PGE 1 treatment will be effective among selected patients with cryoglobulinaemic vasculitis. References1 Acland KM, Darvay A, Wakelin SH, Russell-Jones R. Livedoid vasculitis: a manifestation of the antiphospholipid syndrome? Br J Dermatol 1999; 140:131-5. 2 Boyvat A, Kundakci N, Babikir MO, Gurgey E. Livedoid vasculopathy associated with heterozygous protein C deficiency. Br J Dermatol 2000; 143:840-2. 3 Dispenzieri A, Gorevic PD. Cryoglobulinemia. Hematol Oncol Clin North Am 1999; 13:1315-49. 4 Dammacco F, Sansonno D, Piccoli C et al. The cryoglobulins: an overview. Eur J Clin Invest 2001; 31:628-38. 5 Browning CE, Callen JP. Warfarin therapy for livedoid vasculopathy associated with cryofibrinogenemia and hyperhomocysteinemia. Arch Dermatol 2006; 142:75-8. 6 Ahn JH, Kim MR, Kim HC. Therapeutic effect of lipoprostaglandin E 1 on sudden hearing loss. Am J Otolaryngol 2005; 26:245-8. 7 Koga T, Az-ma T, Yuge O. Prostaglandin E 1 at clinically relevant concentrations inhibits aggregation of platelets under synergic interaction with endothelial cells. Acta Anaesthesiol Scand 2002; 46:987-93. 8 Gruss JD. Effects of adjuvant PGE 1 therapy following profundaplasty in pa...

Toxic epidermal necrolysis and hemophagocytic lymphohistiocytosis: a case report and literature review

“…Our patient's favorable outcome is consistent with other pediatric case reports . One patient relapsed , but was effectively managed, demonstrating the need for close surveillance.…”

“…A two-hit hypothesis has support in three other cases [12,13,16], whereby nonspecific viral upper respiratory tract symptoms temporally overlapped with medications commonly implicated with TEN. Two other TEN cases [11,17] reported no mucosal involvement and isolated EBV from the skin lesions, suggesting a single viral entity. Our patient's favorable outcome is consistent with other pediatric case reports [11,12,17,18].…”

“…Two other TEN cases [11,17] reported no mucosal involvement and isolated EBV from the skin lesions, suggesting a single viral entity. Our patient's favorable outcome is consistent with other pediatric case reports [11,12,17,18]. One patient relapsed [19], but was effectively managed, demonstrating the need for close surveillance.…”

“…Membranous desquamation prior to HLH diagnosis has been documented in case reports [11][12][13]. This may be a spurious observation, or herald a pivotable aspect of disease progression.…”

TEN‐like eruption in setting of EBV positive T‐cell lymphoproliferative disease with HLH, in a child

“…There has been no previous report of a blistering eruption in the setting of HLH complicating EBV+ve T‐cell LPD of childhood. There is one report of a generalised eruption with TEN or Steven–Johnson syndrome (SJS)‐like histology in a case reported by Kawachi and colleagues Like our patient, there was prominent epidermal infiltration by EBV‐infected CD8 + T‐lymphocytes. The patient presented with fever, myalgia and generalised oedematous confluent areas of macular erythema followed by a generalised desquamative dermatitis.…”