Morphological description of skin rash and histopathologic findings in AOSD
| Case (DK) | Description | Key skin biopsy findings | Clinicopathologic 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 |
| Case (DK) | Description | Key skin biopsy findings | Clinicopathologic 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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