kikuchi - fujimoto disease ( kfd ) or histiocytic necrotizing lymphadenitis is a rare , benign , and self - limited disorder characterized by regional cervical lymphadenopathy , mild fever , and night sweats . recognition of kfd is crucial especially because it can be mistaken for lymphoma , tuberculosis or even , for carcinoma . treatment is symptomatic and includes analgesics - antipyretics , non - steroidal anti - inflammatory drugs and , rarely , corticosteroids . kfd can occur isolated , or associated with severe and autoimmune diseases including mixed connective tissue diseases and systemic lupus erythematosus ( sle ) . here we report a case of necrotizing lymphadenitis preceding the occurrence of subacute cutaneous erythematosus lupus ( scle ) in a 42-year - old woman . a 42-year - old moroccan female was admitted to the hospital with fever lasting one month , night sweats and left cervical lymphadenopathy . laboratory exams showed leukocytes 3300/mm , erythrocytes 4.370.000/mm , hemoglobin 12.7 g / dl , thrombocytes 156.000/mm , ldh 843u / l ( normal values : 230460 ) . serologic investigations excluded viral , bacterial and parasitic infections , including syphilis and cytomegalovirus , epstein - barr virus , hiv , rickettsia coronii , brucella spp , salmonella typhi , salmonella paratyphi , toxoplasma , leishmania donovani infections . chest computed tomography ( ct ) showed multiple , small - sized bilateral hilar and mediastinal lymphadenopathies . abdominal ct revealed additional small interaortocaval , para - aortic mesentheric and iliac lymphadenopathies . due to the presence of multiple lymphadenopathies , histology showed a mild reactive t lymphocytosis , consisting of a regular number of interstitial lymphocytes which showed mainly a cd3 + , cd 20-phenotype . the lymph node structure was completely effaced by diffuse necrotic areas with abundant karyorrhectic debris ( figure 1 ) ; the search for dna of mycobacterium tuberculosis was negative . a mediastinal lymph node biopsy and a liver biopsy showed normal histological findings . at this time after two months new laboratory investigations showed a leukocyte count of 3830/mm , erythrocytes 3360000/mm , hgb 9,2 g / dl , thrombocytes 353.000/mm , ast 44 u / i , alt 45 u / i , ldh 138 u / l . since the patient developed recurrent papulo - squamous lesions on her scalp , ears , face , and trunk , she was referred to the dermatology department . skin examination showed papular and scaly plaques on the face , the scalp , the v - area of the neck , upper chest , back and shoulder in a photodistributed pattern . skin lesions were biopsied with a presumptive diagnosis of lupus . at histology , the epidermis was thinned and displayed focal parakeratosis and vacuolar degeneration of basal keratinocytes , with occasional cytoid bodies . the dermis showed conspicuous interstitial mucin deposits , dense perivascular and perifollicular lymphocytic infiltrates with interface dermatitis ( figure 2 ) and superficial nuclear debris with karyorrhexis . direct immunofluorescence failed to demonstrate immunoglobulin or complement deposits along the dermoepidermal junction or around the vessels . immunologic serum investigations showed absence of antinuclear antibodies , antibodies ( anti)-la / ss - b , anti - ds dna , anti - phospholipids , anti - panca , anti - jo1 , anti - scl-70 , anti - rnp . antibodies anti - ro / ss - a ( e.i.a ) were 59 ( normal value : 010 ) , ena ( e.i.a ) 18.7 ( normal value : 03 ) . the patient s complement levels were low with c3 71 mg / dl ( normal values : 88201 mg / dl ) , c4 5 mg / dl ( normal values : 1647 mg / dl ) . on the basis of clinical , laboratory and histology findings , a diagnosis of scle was made . skin lesions improved rapidly and healed without scarring . the dosage of corticosteroids was gradually tapered without relapse of skin lesions . laboratory investigations are all within normal value , except for persistent complement deficiency in c3 and c4 . common presentation signs are acute symmetrical painful swelling of predominantly cervical lymph nodes , fever and systemic signs . the nature of the clinical and pathophysiological relationships between kfd and sle remains a matter of debate , in particular whether kfd can be considered an atypical manifestation of sle . both conditions share the predilection for young females and similar clinical features , including lymphadenopathy , mainly in the cervical lymph nodes , fever , arthralgia , and leukopenia . histopathologic findings of kfd are crucial in characterization of this condition . diagnosis is generally made upon on pathological examination of the excised lymph nodes . typical morphological features include apoptotic necrosis in paracortical areas with abundant karyorrhectic debris , infiltration of histiocytes , cd123 + plasmacytoid dendritic cells , cd8 + t cells , and the absence of neutrophils . lupus lymphadenitis ( ll ) is characterized by prominent necrosis , strikingly similar to that described in patients with kfd . some histological findings , such as the presence of hematoxylin bodies , prominent plasma cells and neutrophils support the diagnosis of ll . the first one is a sle with cutaneous and lymph - node involvement , mainly if histopathology of skin lesions show interface dermatitis . in this case kfd may in fact be a histopathologic characteristic of sle supporting the hypothesis that kfd is a rare manifestation of sle . extra - nodal involvement in kfd is uncommon ; skin manifestations are observed in 530% of patients . various skin lesions , such as urticaria - like erythematous papules , nodules , plaques , and indurate lesions , have been reported . in addition , also lupus - typical skin manifestations , as malar rash and photosensitivity , have been observed . in such cases , histopathology of skin lesions usually show lymphohistiocytic infiltrates accompanied by non - neutrophilic karyorrhetic debris , similar to those observed in the involved lymph nodes . in our patient the lymph node biopsy showed extensive necrosis suggesting the possibility of ll , however aggregates of nuclear debris or so - called hematoxylin bodies were not found . she presented leukopenia and photosensitivity , but she did not fulfill acr ( american college of rheumatology ) criteria for the diagnosis of sle . however , since we have a 38-month follow - up , a clinical evolution of our patient s scle toward sle in the future can not be definitely excluded . kfd may be the initial diagnosis in patients who go on to develop sle later on . a lag time of 10 months to 3 years has been observed between the diagnosis of kfd and the subsequent onset of sle . it is suitable to test for autoimmune disease repeatedly even if anas are initially negative .
kikuchi - fujimoto disease ( kfd ) is an uncommon clinicopathological entity characterized by fever and lymphadenopathy , predominantly involving cervical lymph nodes , accompanied by chills and leukopenia . 
 the diagnosis relies primarily on the presence of typical morphological features in the swelling lymph nodes . 
 kfd can occur as a benign and self - limiting lymphadenopathy , but it can sporadically precede , postdate or coincide with the diagnosis of systemic lupus erythematosus ( sle ) . 
 the authors report a case of subacute cutaneous lupus erythematosus ( scle ) in a 42-year - old female preceded by prolonged fever , anemia , leukopenia , and cervical necrotizing lymphadenopathy . 
 about two months later , the patient developed facial and scalp plaques suggestive of lupus skin disease . 
 histologic and immunologic investigations lead to the diagnosis of scle . 
 it is not clear whether kfd associated with lupus skin disease are true kfd or a histopathologic feature of sle .