Table 1 Clinical manifestations and histopathological findings of Still’s disease

Wouters et al. [12]

1/66 /F 1/62/M

Polyarthritis, fever, splenomegaly

Leukocytosis, anemia, increased ESRand liver enzymes

Evanescent macular nonpruritic rash on trunk, limbs

-

NSAIDs, Penicillamine

Phillips et al. [21]

1/51/F

Arthralgia, sore throat, lethargy, night sweats

Leukocytosis, anemia, increased ESR, liver enzymes & ferritin

Evanescent erythematous maculopapular pruritic rash on trunk, limbs, face

Perivascular infiltrate in upper dermis and dermal mucin deposition

NSAIDs, Methotrexate

Tay et al. [27]

1/33/F

Fever, arthralgia, malaise, sore throat, headache, lymphadenopathy, hepatomegaly

Leukocytosis, anemia, increased ESR, CRP & immunoglobulins

Evanescent urticarial-like erythematous maculopapular pruritic rash on trunk, limbs

Edema of the upper dermis with perivascular neutrophils and eosinophils

NSAIDs, Prednisolone

Setterfield et al. [30]

1/32/F

Arthralgia, sore throat, fever, anorexia, weight loss

Leukocytosis, anemia, increased ESR, CRP & ferritin

Persistent urticarial annular pruritic lesions on trunk and limbs

Dermal edema, perivascular neutrophilic infiltrate

NSAIDs

Lubbe et al. [33]

1/16/F

Fever, arthritis, sore throat, pericardial effusion, hepatosplenomegaly

Leukocytosis, anemia, increased ESR, CRP & ferritin

Evanescent maculopapular lesions on chest, persistent brownish papules and plaques on face, neck, back

Parakeratosis, acanthosis and scattered intraepithelial keratinocyte necroses

NSAIDs, Prednisone

Salaffi et al. [36]

1/55/F

Fever, arthralgia, fatigue

Leukocytosis, anemia, increased ESR, CRP & ferritin

Urticarial pruritic rash on trunk, limbs and face

Dermal edema with perivascular and interstitial neutrophilic infiltrate

NSAIDs, Prednisone, Methotrexate

Suzuki et al. [18]

1/25/M

Fever, arthralgia, fatigue

Leukocytosis, anemia, increased ESR, CRP, liver enzymes & ferritin

Persistent papules and plaques on trunk, linear pigmentation on chest and back

Mild perivascular infiltration of neutrophils and lymphocytes.

NSAIDs, Prednisolone, Methotrexate

Perez et al. [43]

1/39/M

Polyarthritis, sore throat, fever, chills, lymphadenopathy, hepatosplenomegaly

Leukocytosis, increased ESR, CRP liver enzymes & ferritin

Erythematous papules and plaques on neck, trunk, limbs

Perivascular infiltrate of dermal vessels with eosinophils, histiocytes, lymphocytes and neutrophils within vessels

NSAIDs, Prednisone

Lee et al. [25]

1/46/F

Arthralgia, high fever, headache, nausea, splenomegaly

Anemia, thrombocytopenia, increased ESR, liver enzymes &ferritin

Vesicular and pustular eruption on hands and feet

Fibrin deposit in the vessels, subepidermal bulla and ischemic necrosis of epidermis

NSAIDs, Corticosteroids

Elezoglou et al. [42]

1/47/M

Fever, arthralgia, malaise, sore throat, hepatosplenomegaly glomerulonephritis

Leukocytosis, anemia, increased CRP, liver enzymes & ferritin, cryoglobulinemia

Evanescent maculopapular pruritic rash on head, trunk, buttocks, groin, annular purpuric plaques on ankles and shins

Leuko-cytoclastic vasculitis

Methyl-prednisolone, Methotrexate

Thien Huong et al. [17]

1/23/F

Fever, polyarthralgia, polyadenopathy, myalgia

Leukocytosis, increased ESR & ferritin

Persistent pigmented plaques on trunk

-

Corticosteroids

Tomaru et al. [23]

1/34/F

Fever, polyarthralgia, lymphadenopathy

Leukocytosis, increased CRP, liver enzymes & ferritin

Persistent pruritic erythematous papules with linear arrangement and pigmentary changes on chest and back, evanescent salmon-pink eruption on lower extremities

Mild acanthosis, exocytosis, dyskeratotic cells and liquefaction degeneration in the basal layer, with lichenoid inflammatory reaction

Corticosteroids, Cyclosporine, Methotrexate

Criado et al. [31]

1/52/F

Fever, polyarthralgia, sore throat, mild hepatomegaly, lymphadenopathy

Leukocytosis, increased ESR, CRP, & ferritin, hypergammaglobulinemia

Lenticular urticaria-like rash on face, thorax, abdomen, hands

Interstitial edema in reticular and papillar dermis with neutrophils and leukocytes around vasculitis-free vessels.

NSAIDs, Methotrexate, Thalidomide

Yang et al. [35]

1/47/F

Fever, polyarthralgia, sore throat, myalgia, pleural effusion, splenomegaly

Leukocytosis, elevated liver enzymes & ferritin

Persistent violaceous scaly maculopapular rash with linear lesions on forehead, neck, elbows, knees

Necrotic keratinocytes in the upper epidermis and perivascular infiltrate of lymphocytes and neutrophils

Methyl-prednisolone, Azathioprine

Wolgamot et al. [39]

1/55/F

Fever, polyarthralgia

Leukocytosis, increased ESR, CRP

& ferritin

Pruritic maculopapular rash, plaques on face, extremities, trunk

Pattern with dyskeratotic keratinocytes in upper epidermis and stratum corneum

Prednisone, Methotrexate, Etanercept, Anakinra

Yanai et al. [41]

1/43/M

Fever, arthralgia, myalgia, myositis

Increased fibrinogen CRP, creatine kinase & ferritin

Salmon-pink rash on upper arm

Perivascular lymphocytes infiltration and fragmentation of blood cells compatible with leukocytoclastic vasculitis

NSAIDs, corticosteroids

Fortna et al. [40]

3/15-54/F

Fever, polyarthralgia, myalgia, sore throat

Leukocytosis, increased ESR, CRP, liver enzymes & ferritin

Pruritic erythematous blanchable papules and plaques on back, neck, abdomen, limbs

Hyperkeratosis with patchy parakeratosis, areas of dyskeratosis to upper layers of epidermis, and mild acanthosis.

Methyl-prednisolone, Methotrexate, Anakinra

Criado et al. [26]

2/27-34/F

1/26/M

Fever, arthritis, sore throat, lymphadenopathy, pleural effusion, splenomegaly

Leukocytosis, anemia, increased ESR, CRP & liver enzymes, hyperferritinemia

Urticarial pruritic rash, linear lesions (dermographism) on trunk, limbs, face

Perivascular and interstitial inflammatory cell infiltrate of lymphocytes and neutrophils with leukocytoclasia.

Prednisone, Chloroquine, Methotrexate,

Nagai et al. [34]

18/16-60/F

Fever, arthralgia, sore throat, splenomegaly, lymphadenopathy

Leukocytosis, increased CRP, liver enzymes & ferritin

Evanescent salmon-pink maculopapular eruption, persistent papules and plaques with linear erythema similar to prurigo pigmentosa, edema of eyelids mimicking dermatomyositis

Parakeratosis and necrotic keratinocytes in epidermis, inflammatory infiltrates of lymphocytes in the papillary and mid-dermis

Corticosteroids, Methotrexate, Mizoribine Cyclosporin, Cyclophosphamide

Lee et al. [14]

30/17-67/F

6/17-67/M

Fever, arthralgia, sore throat, splenomegaly, lymphadenopathy

Leukocytosis, elevated liver enzymes & ferritin

Evanescent rash, persistent pruritic urticarial, violaceous papules and plaques, dermatographism-like, prurigo pigmentosa-like and dermatomyositis-like eruption on trunk, neck, face, limbs

Normal epidermis with perivascular infiltrate of neutrophils, nectrotic keratinocytes in upper epidermis.

NSAIDs, Corticosteroids, Methotrexate, Azathioprine

Yoshifuku et al. [16]

1/27/F

Fever, polyarthralgia, sore throat, hepatosplenomegaly lymphadenopathy

Leukocytosis, increased CRP, elevated liver enzymes & ferritin

Pruritic pigmented erythematous plaques and dark-reddish papules on abdomen and back

Mild hyperkeratosis, and presence of dyskeratotic keratinocytes in upper epidermis

Corticosteroids, Cyclosporin,

Said et al. [37]

1/23/M

Fever, sore throat, myopericarditis, arthralgia, hepatosplenomegaly

Leukocytosis, increased ESR &CRP, elevated ferritin, raised cardiac enzymes

Urticated and erythematous plaques and papules on the dorsum of right hand and fingers

Superficial and deep perivascular infiltrates of lymphocytes and neutrophils

Corticosteroids,

NSAIDs, Azathioprine

Cho et al. [24]

1/38/F

Fever, polyarthralgia, sore throat hepatosplenomegaly lymphadenopathy

Leukocytosis, elevated liver enzymes & ferritin

Prurigo pigmentosa-like persistent papules and plaques on anterior chest, abdomen, back

Parakeratosis, and perivascular infiltrations of lymphocytes, eosinophils and neutrophils in upper dermis

Methyl-prednisolone, Methotrexate, Hydroxychloroquine

Sarkar et al. [19]

1/36/M

Fever, arthritis, hepatosplenomegaly hemophagocytic lymphohistiocytosis

Leukocytosis, anemia, increased ESR, CRP, liver enzymes & ferritin, hypoalbuminemia

Pigmented patches and plaques on chest, dermal and mucosal hyper-pigmentation

Multiple necrotic keratinocytes in aggregates in upper epidermis

Corticosteroids

Cossi et al. [38]

1/35/M

Fever, cough, dyspnea myopericarditis

Increased CRP, liver enzymes & ferritin hypergammaglobulinemia

Pruritic erythematous-edematous plaques on trunk, upper limbs

Epidermal spongiosis, dermal infiltrate of perivascular lymphocytes and histiocytes, intra- vascular CD15⁺neutrophils

Methyl-prednisolone, Immunoglobulin IV Methotrexate