drug rash with eosinophilia and systemic symptoms ( dress ) syndrome or drug - induced hypersensitivity syndrome ( dihs ) is a rare , life - threatening adverse drug reaction , mostly to anticonvulsants and sulfonamides that can present with clinical , paraclinical and histopathological findings similar to lymphomas . cases of antibiotic , antihypertensive , and antiepileptic drugs such as phenytoin , carbamazepine , lamotrigine - induced dress , and pseudolymphoma have in fact been reported . we report here the first case of lamotrigine - induced dress with histologic features of a cutaneous cd30 + lymphoma . a 30-year - old woman presented to the hospital with a morbilliform and pruritic generalized rash [ figure 1 ] , along with high - grade fever , fatigue , and malaise for 4 days . physical examination and blood tests revealed enlarged cervical and inguinal nodes , leukocytosis ( up to 66,800 white blood cell / mm ) , and elevated levels of hepatic transaminases , lactate dehydrogenase , -2 microglobulin , and erythrocyte sedimentation rate . the whole body computed tomography showed multiple neck , chest , and abdominal lymphadenopathy with splenomegaly suggestive of lymphoma [ figure 2a ] . a skin biopsy specimen revealed epidermotropism of atypical lymphoid cells , with cd30 positive staining as well as a dense perivascular and perifollicular lymphoid infiltrate [ figure 2b ] . an excisional biopsy of a 1.0 cm cervical node showed diffuse polymorphic lymphoid hyperplasia with effacement of the normal architecture with strong and diffuse staining for cd30 on immunohistochemistry , positive anti - leukocyte common antigen , and high proliferation index ( ki67 80% ) . however , osteomedullary biopsy showed no evidence of lymphoproliferative disorder , was absent for immunomarkers and high eosinophils proliferation rate . t - cell receptor rearrangement studies on the skin , lymph node , and bone marrow biopsies , failed to detect monoclonality . a detailed review of her past medical history revealed that she was started on lamotrigine 50 mg / day for somatization disorder 40 days prior to the onset of symptoms . the diagnosis of dihs / dress was made ; lamotrigine was discontinued and treatment with oral corticosteroids ( prednisolone , 60 mg / day ) was started with rapid tapering . the patient made a full recovery with no organ deficit on follow - up visits at 1 , 2 , 4 , and 6 months . ( a ) striking facial edema and erythema ( b ) morbilliform generalized desquamative rash ( a ) pelvic computed tomography scan showing inguinal lymphadenopathy ( blue arrows ) ( b ) skin biopsy showing lymphocyte epidermotropism and dense perivascular infiltrate ( h and e , 100 ) to our knowledge , this is the first case of a confirmed lamotrigine - induced dress with histologic features of a cd30 + cutaneous and systemic lymphoma . because of the widespread lymphadenopathy , hepatosplenomegaly , leukocytosis , and the positive cd30 staining on both skin and lymph node biopsies , this case could have been easily mistaken for systemic lymphoma . dress / dihs is , in fact , a severe adverse reaction associated with multisystemic involvement that can present clinical and histopathologic features similar to lymphomas , with epidermotropism and cutaneous cd30 + lymphocytes . the latter is usually seen in lymphoproliferative disorders such as lymphomatoid papulosis , hodgkin 's disease , and primary cutaneous anaplastic large cell lymphoma but have been described in drug reactions . even monoclonal t - cell receptor have been seen with drug reactions , rendering the diagnosis of dress versus lymphoma more difficult . although both dress and pseudolymphoma are drug - induced have been reported with lamotrigine separately and can present with histopathologic features of lymphoma , studies have shown that they can be differentiated on the basis of the clinical presentation . compared to dress , patients with pseudolymphoma have less acute and less systemic involvement but more severe lymph node distortion and atypical lymphocytes . this is not surprising since the mechanisms that have been implicated in the development of both entities include genetic predisposition and association with human leukocyte antigen , virus reactivation , and accumulation of reactive drug metabolites . this case highlights the atypical presentation of a lamotrigine - induced dress / dihs in the presence of a cutaneous and a lymph node cd30 + lymphocytic infiltrate mimicking systemic lymphoma . pathologists and clinicians must be aware of this  lymphomatous  presentation of drug reactions . the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s ) has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal . the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed . lamotrigine can induce drug rash with eosinophilia and systemic symptoms with features of cd30 + lymphomasany atypical presentation of systemic or skin lymphomas such as hyperacute presentation or absent t - cell receptor monoclonality should raise the suspicion of a drug reaction even in the presence of large anaplastic cd30 + cells.subjects have given their informed consent and that the study protocol has been approved by the institute 's committee on human research . lamotrigine can induce drug rash with eosinophilia and systemic symptoms with features of cd30 + lymphomas any atypical presentation of systemic or skin lymphomas such as hyperacute presentation or absent t - cell receptor monoclonality should raise the suspicion of a drug reaction even in the presence of large anaplastic cd30 + cells . subjects have given their informed consent and that the study protocol has been approved by the institute 's committee on human research . the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s ) has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal . the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed . lamotrigine can induce drug rash with eosinophilia and systemic symptoms with features of cd30 + lymphomasany atypical presentation of systemic or skin lymphomas such as hyperacute presentation or absent t - cell receptor monoclonality should raise the suspicion of a drug reaction even in the presence of large anaplastic cd30 + cells.subjects have given their informed consent and that the study protocol has been approved by the institute 's committee on human research . lamotrigine can induce drug rash with eosinophilia and systemic symptoms with features of cd30 + lymphomas any atypical presentation of systemic or skin lymphomas such as hyperacute presentation or absent t - cell receptor monoclonality should raise the suspicion of a drug reaction even in the presence of large anaplastic cd30 + cells . subjects have given their informed consent and that the study protocol has been approved by the institute 's committee on human research . the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s ) has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal . the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed . lamotrigine can induce drug rash with eosinophilia and systemic symptoms with features of cd30 + lymphomasany atypical presentation of systemic or skin lymphomas such as hyperacute presentation or absent t - cell receptor monoclonality should raise the suspicion of a drug reaction even in the presence of large anaplastic cd30 + cells.subjects have given their informed consent and that the study protocol has been approved by the institute 's committee on human research . lamotrigine can induce drug rash with eosinophilia and systemic symptoms with features of cd30 + lymphomas any atypical presentation of systemic or skin lymphomas such as hyperacute presentation or absent t - cell receptor monoclonality should raise the suspicion of a drug reaction even in the presence of large anaplastic cd30 + cells . subjects have given their informed consent and that the study protocol has been approved by the institute 's committee on human research .
drug rash with eosinophilia and systemic symptoms ( dress ) syndrome or drug - induced hypersensitivity syndrome ( dihs ) is a severe adverse drug reaction . 
 it can present with clinical , paraclinical , and histological findings mimicking skin and/or systemic lymphomas . 
 we report the first case of a lamotrigine - induced dress with histologic features of a cutaneous cd30 + lymphoma . 
 the patient responded well to a tapering course of oral steroids . 
 this case highlights the atypical presentation of a lamotrigine - induced dress / dihs in the presence of a cutaneous and a lymph node cd30 + lymphocytic infiltrate mimicking systemic lymphoma . 
 pathologists and clinicians must be aware of this  lymphomatous  presentation of drug reactions .