Table 2.

Morphological description of skin rash and histopathologic findings in AOSD

Case (DK)DescriptionKey skin biopsy findingsClinicopathologic Correlation
1 (NO) Full-body maculopapular rash, predominantly non-pruritic Superficial perivascular lymphocytic dermatitis Viral exanthem, morbilliform drug eruption 
2 (YES) Extensive blanchable erythematous macules coalescing into patches involving the arms, legs, and torso Upper-epidermal dyskeratosis with sparse superficial perivascular lymphocytic infiltrate PPE of AOSD 
3 (YES) Blanchable, non-pruritic maculopapular rash involving the upper torso, arms, and lateral ankles Upper-epidermal necrotic keratinocytes with superficial mid-dermal perivascular lymphohistiocytic infiltrate; mild dermal mucin PPE of AOSD 
4 (YES) Persistent pruritic linear plaques of the trunk and buttocks Upper-epidermal dyskeratosis with mild perivascular lymphoplasmacytic infiltrate and occasional neutrophils PPE of AOSD 
5 (YES) Linear hyperkeratotic plaques with areas of hyperpigmentation involving the upper and lower back and gluteal region Epidermal dyskeratosis with parakeratosis and spongiosis; mixed perivascular inflammatory infiltrate in the mid-upper dermis PPE of AOSD 
6 (YES) Diffuse erythematous rash involving the abdomen, arms, and legs Sparse epidermal necrotic keratinocytes with focal parakeratosis; prominent superficial and deep perivascular/interstitial eosinophilic infiltrate PPE of AOSD 
7 (YES) Raised, salmon-colored, blanchable maculopapular rash involving the distal extremities Sparse epidermal necrotic keratinocytes with superficial perivascular and interstitial neutrophils PPE of AOSD 
8 (YES) Blotchy erythematous rash involving the back, neck, abdomen, and legs Upper-epidermal dyskeratosis with focal subcorneal pustules PPE of AOSD 
9 (YES) Hyperpigmented macular rash involving the anterior chest, neck, and left inguinal region Upper-epidermal dyskeratosis with parakeratosis and mild spongiosis; superficial perivascular lymphocytic infiltrate with occasional eosinophils PPE of AOSD 
10 (YES) Widespread maculopapular, blanchable coalescing lesions along the arms, legs, and torso Upper-epidermal necrotic/DKs, mild spongiosis, subtle interface vacuolar changes, and mid-dermal perivascular lymphocytic infiltrate with scattered neutrophils PPE of AOSD 
11 (YES) Widespread, symmetric erythematous papules and macules coalescing into patches involving the trunk and extremities Upper-epidermal dyskeratosis with sparse superficial perivascular infiltrate and focal vacuolar interface change PPE of AOSD 
12 (YES) Well-demarcated, flat, and blanchable erythematous lesions on forearms and coalescing to involve most of the body Upper-epidermal necrotic keratinocytes; parakeratosis; mixed superficial and deep perivascular inflammatory infiltrate of plasma cells and eosinophils PPE of AOSD 
13 (NO) Migratory, blanchable, non-raised erythematous rash; occasionally pruritic Nonspecific superficial perivascular and interstitial neutrophilic/lymphocytic infiltrate, mild hyperkeratosis Classic evanescent AOSD rash, urticaria 
14 (NO) Migratory, intermittent blanchable erythematous rash involving the upper body Superficial perivascular and interstitial mixed inflammatory infiltrate (neutrophils and lymphocytes), mild hyperkeratosis, papillary dermal edema Classic evanescent AOSD rash, urticaria 
15 (NO) Pruritic light pink macules and papules with erythematous plaques on trunk/extremities Light superficial perivascular lymphocytic infiltrate with occasional interstitial neutrophils Viral exanthem, morbilliform drug eruption 
16 (YES) Pruritic erythematous urticated plaques on torso and extremities; largely resolved at time of biopsy with scattered excoriated papules and minimal residual erythema Occasional DKs in the mid-to-upper epidermis with otherwise unremarkable epidermis and minimal dermal inflammation PPE of AOSD 

DK, dyskeratotic keratinocytes present in superficial layers (YES) or not (NO).

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