Table 3.

Clinical differential diagnosis for singly dispersed necrotic keratinocytes in the superficial epidermis and cornified layers

ConditionKey non-cutaneous clinical featuresTypical cutaneous findingsOther histological findings
Systemic inflammatory disease 
AOSD (1, 4, 13, 15, 16, 17) High fever, myalgias, hyperferritinemia, and pharyngitis Evanescent salmon-coloured eruption
Linear urticaria
Persistent pruritic papular eruption 
Vacuolar interface dermatitis; mild dermal infiltrate of eosinophils/neutrophils 
aGVHD (33, 34) History of transplant, gastrointestinal symptoms Erythematous macules and papules with varying body surface area, rarely bullae or erythroderma Confluent or diffuse keratinocyte necrosis; variable superficial inflammatory infiltrates without neutrophils; vacuolar interface dermatitis with hair follicle involvement 
Bacterial-toxin-associated–mediated erythemas 
TSS (35) Recent surgery, high fever, myalgias, pharyngitis, and shock Erythroderma or flexure-predominant macular eruption
Desquamation of palms and soles 
Variable dermal perivascular inflammatory infiltrate; epidermal spongiosis without interface dermatitis 
Photodermatologic disorders 
Acute inflammatory erythema (sunburn) (36) Exposure history Erythema of sun-exposed skin with burning sensation and possible edema or blistering Minimal dermal inflammation without eosinophils or vacuolar interface dermatitis 
Phototoxicity (37) Exposure to phototoxic agent and UV radiation Erythema of sun-exposed skin with burning sensation and possible edema or blistering Variable spongiosis and/or interface dermatitis; variable dermal inflammatory infiltrate 
Other inflammatory skin diseases 
Early acute phase of prurigo pigmentosa (38) Young adult female, systemically well except for ketosis Pruritic erythematous papules and vesicles symmetrically distributed on the back, neck, and/or chest evolving into a reticulated configuration of hyperpigmented macules Spongiotic dermatitis with intraepidermal and superficial perivascular lymphocytes; possible confluent keratinocyte necrosis and/or intraepidermal neutrophilic microabscesses; abundant bacteria within hair follicles 
Irritant contact dermatitis (39) History of exposure Painful and/or pruritic erythema, edema and/or bullae on exposed sites Spongiotic dermatitis with intraepidermal and superficial perivascular lymphocytes and neutrophils 
Genodermatoses 
Verrucous phase of incontinentia pigmenti (40) Family history, pediatric onset, dental, hair (“woolly”), nail, central nervous system, eye, and breast abnormalities Erythematous, vesicular or verrucous linear plaques following lines of Blaschko Eosinophilic spongiosis in earlier lesions; verrucous epidermal hyperplasia in later lesions; dermal lymphocytes, eosinophils, and/or melanophages, without neutrophils 

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