Table 1.
Cutaneous manifestation | Clinical presentation | Histopathology |
---|---|---|
Chilblain-like or pernio-like | Dusky, erythematous to edematous papules, nodules, plaques or, less frequently, bullae formation on acral surfaces |
Superficial and deep perivascular lymphocytic infiltrate of predominantly CD3+/CD4+ T cells Negative immunofluorescence |
Maculopapular or morbilliform | Erythematous macules with areas of normal-appearing skin on the trunk and extremities with pruritus | Spongiotic dermatitis with eosinophils in earlier lesions, or mixed perivascular lymphocytic infiltrate with histiocytes in later lesions |
Urticarial | Migratory, pruritic, edematous, variably-sized wheals within 24 h without bruising or hyperpigmentation ± angioedema | Perivascular lymphocytic infiltrate, scattered eosinophils, and upper dermal edema with no virally induced cytopathic changes |
Vesicular or varicella-like | Small, scattered monomorphic vesicles on the trunk with mild or absent pruritus, pain, or a burning sensation | Vacuolar degeneration of basal layer, apoptotic keratinocytes, mild inflammatory infiltrate, and multinucleated keratinocytes |
Livedoid |
Livedo reticularis: symmetric, lace-like dusky patches forming pale-centered rings along blood vessels Livedo racemosa: irregular and asymmetrical dusky patches along blood vessels |
Epidermal necrosis, superficial and deep dermis thrombosis vasculopathy in small and medium vessels, sweat gland necrosis, mild perivascular lymphocytic infiltrate and complement deposition in the vessel walls |
Erythema multiforme-like | Target (3 rings) or targetoid (2 rings) confluent macules, papules, and plaques of varying sizes with hemorrhages and central crusts | Superficial and deep perivascular perieccrine CD3+/CD4+/CD8+ lymphocytic infiltrate with perieccrine involvement and scattered vasculopathic changes |
Multisystem inflammatory syndrome | Polymorphous maculopapular eruptions on the trunk and flexural areas, acral erythema, extremity swelling and desquamation, mucositis, and fissured lips |
In children: Varies from biopsies with leukocytoclastic vasculitis to erythema multiforme-like histopathology In adults: Varies from biopsies with superficial perivascular lymphocytic infiltrate with rare neutrophils and extravasated erythrocytes to superficial and deep perivascular and periadnexal lymphocytic infiltrate histopathology |