524 SHORT COMMUNICATION
ActaDV ActaDV
Advances in dermatology and venereology Acta Dermato-Venereologica
Two Severe Cases of Adult-onset Still ’ s Disease with Persistent Pruritic Eruptions
Yang LIU 1 # , Tetsuya IKAWA 1 # , Yayoi TADA 1 *, Takamitsu TANAKA 1 , Takamitsu OHNISHI 1 , Shinichi WATANABE 1 , Daisuke TSUKUI 2 , Kurumi ASAKO 2 and Hajime KONO 2 Departments of 1 Dermatology , and 2 Internal Medicine , Teikyo University School of Medicine , 2-11-1 Kaga , Itabashi-ku , Tokyo 173-8606 , Japan . E-mail : ytada-tky @ umin . ac . jp
#
These authors contributed equally to this work . Accepted Jan 23 , 2018 ; Epub ahead of print Jan 24 , 2018
Adult-onset Still ’ s disease ( AOSD ) is a rare systemic inflammatory disease characterized by spiking fevers , increased white blood cell count , arthralgia and skin rash ( 1 ). The typical skin rash of AOSD is salmon pinkcoloured , mildly itchy , and apparent during high fever . Histopathology of biopsy specimens from the eruption show dermal perivascular infiltrates of lymphocytes and neutrophils ( 2 ). Other than the typical rash , different types of skin manifestation of AOSD have been reported , such as dermatographism-like persistent eruption ( 2 ), violaceous to dusky red , scaly lichenoid papules ( 2 ), and intensely pruritic persistent erythema ( 3 ). These atypical eruptions are called persistent pruritic eruptions ( PPE ) ( 4 ). Here , we report 2 patients who were diagnosed as having AOSD with PPE showing peculiar histopathological findings . Prednisolone , 1 mg / kg / day , was not sufficient to resolve their systemic inflammation . The importance of PPE in diagnosing AOSD and predicting the disease outcome is discussed .
CASE REPORTS
Case 1 . A 75-year-old woman presented to the internal medicine department of our hospital with high fever (> 39 ° C ), swollen painful knees , sore throat , and skin rash persisting for one month . Prior to presentation to the internal medicine department , treatment with oral aminobenzyl penicillin , 2,000 mg / day for 10 days , was not effective . Three weeks prior to admission , dark-red plaques with severe pruritus appeared on her chest , which persisted even when the fever subsided . Her body temperature was 37.4 ° C and she had cervical lymphadenopathy on admission . Dark-red to violaceous scaly plaques were seen on her chest ( Fig . S1a 1 ). Laboratory findings revealed neutrophilia ( WBC 6,600 / μl , neutrophil 5,060 / μl ), anaemia ( Hb 6.3 g / dl ), thrombocytopaenia ( 10.0 × 10 4 / μl ), elevation of liver enzymes ( AST 852 U / l , ALT 776 U / l , γGTP 397 U / l ), hyperferritinaemia ( 167,998 ng / ml ), and increased serum soluble interleukin-2 ( IL-2 ) receptor ( 5,995 U / ml ). Anti-nuclear antibody and rheumatoid factor were negative . Histopathology of biopsy specimens of the rash showed focal hyperkeratosis , flattening of rete ridges , and scattered dyskeratotic cells in the epidermis ( Fig . S1b , c 1 ). Perivascular lymphocyte infiltrates and nuclear debris were observed around dermal blood vessels ( Fig . S1d 1 ). The patient was diagnosed as having AOSD according to the diagnostic criteria ( 5 ). She was initially treated with prednisolone , 1 mg / kg / day for 10 days . However , haemophagocytosis , disseminated intravascular coagulation and interstitial pneumonia emerged during her clinical course . The patient was then treated with 7 sessions of plasmapheresis , together with cyclosporine , 2 mg / kg / day , and an interleukin-6 receptor antagonist tocilizumab ( Chugai Pharmaceutical , Tokyo , Japan ), 8 mg / kg / day , and finally her condition was controlled .
1 https :// www . medicaljournals . se / acta / content / abstract / 10.2340 / 00015555-2887
Case 2 . 77-year-old woman presented to the emergency room of our hospital with a history of high fever ( 38 – 39 ° C ) and intensely pruritic skin rash persisting for one week . She had received intravenous cefepime for 5 days at the nursing home , but this treatment failed to resolve her symptoms . On admission , her body temperature was 38.5 ° C and she did not have arthritis . Oedematous erythema with severe pruritus showing linear configurations was seen on her chest , abdomen , back , and inguinal region ( Fig . 1a , b ). The erythema persisted even at the times when she had normal body temperature . Laboratory findings revealed leukocytosis with neutrophilia ( WBC 11,400 / μl [ normal 3,000 – 9,800 ], neutrophil 10,602 / μl [ 40 – 70 %]), anaemia ( Hb 9.5 g / dl [ 10.2 – 15.5 ]), thrombocytopaenia ( 3.7 × 10 4 / μl [ 15 – 38 × 10 4 ]), elevated liver enzymes ( AST 73 U / l [ 1 – 32 ], ALT 26 U / l [ 6 – 35 ], γGTP 36 U / l [ 8 – 33 ]), hyperferritinaemia ( 43,865.7 ng / ml [ 6.4 – 167.1 ]) and increased serum soluble IL-2 receptor ( 3,616 U / ml [ 154 – 472 ]). Anti-nuclear antibody and rheumatoid factor were negative . The erythrocyte sedimentation rate ( ESR ) was increased ( 26 mm / h ). Anti-nuclear antibody , anti-Jo-1 antibody and rheumatoid factor were negative . Histopathological examination of biopsy specimens of the skin rash showed flattening of rete ridges ( Fig . 1c ) and scattered dyskeratotic cells in the epidermis ( Fig . 1d ). Lymphocytes , neutrophils , histiocytes and nuclear debris were seen around superficial dermal blood vessels ( Fig . 1e ). The patient was diagnosed as having AOSD according to the diagnostic criteria ( 5 ). She was initially treated with prednisolone , 1 mg / kg / day for 10 days . However , haemophagocytosis , disseminated intravascular coagulation and liver enzyme elevation persisted . Additional cyclosporine was administered and 7 sessions of plasmapheresis were performed to reduce the dose of prednisolone to 0.3 mg / kg / day . Although AOSD was under control , the patient died 7 months after admission due to concomitant interstitial pneumonia .
DISCUSSION
Both patients fulfilled the criteria of AOSD ( 5 ) and had an extremely elevated serum ferritin level . Autoimmune diseases , malignancy , and infectious diseases were ruled out .
Two kinds of skin symptoms of AOSD have been reported : the typical salmon-pink rash and other atypical ones ( 6 ). The typical rash appears only when the body temperature increases , and usually appears without pruritus . On the other hand , atypical rash emerges during the early phase of AOSD , and its emergence does not depend on body temperature .
Atypical rash was first reported in a patient with AOSD by Lübbe et al . ( 7 ) in 1999 . They described pruritic rashes , which consisted of oedematous erythema or various-coloured scaly papules , sometimes with a linear configuration . The rash could clinically resemble prurigo pigmentosa ( 8 ). Histopathology of the atypical rash was reported to have a characteristic pattern of dyskeratosis doi : 10.2340 / 00015555-2887 Acta Derm Venereol 2018 ; 98 : 524 – 525
This is an open access article under the CC BY-NC license . www . medicaljournals . se / acta Journal Compilation © 2018 Acta Dermato-Venereologica .