Causes of erythroderma | Key histopathologic features |
Idiopathic erythroderma | Nonspecific features including hyperkeratosis, parakeratosis, acanthosis, and inflammatory infiltrate |
Inflammatory dermatoses | |
Psoriasis | Epidermal acanthosis, parakeratosis, Munro microabscesses, dilated tortuous papillary blood vessels |
Atopic dermatitis | Acanthosis, hyperkeratosis, spongiosis |
Dermatitis (other than atopic) | Acanthosis, hyperkeratosis, spongiosis |
Pityriasis rubra pilaris | Acanthosis, hyperkeratosis, alternating ortho- and parakeratosis (horizontal and vertical) |
Lichen planus | Lymphohistiocytic infiltrate with band-like distribution upper dermis; obscures the dermoepidermal junction |
Chronic actinic dermatitis | Acanthosis, hyperkeratosis, spongiosis, perivascular chronic inflammatory cell infiltrate, occasional atypical cerebriform lymphoid cells |
Papuloerythroderma of Ofuji | |
Sarcoidosis | Noncaseating epithelial cell-like granulomas in the deep dermis |
Drug reactions | Histology varies according to the reaction pattern
|
Malignancies: hematologic | |
Cutaneous T cell lymphoma, Sézary syndrome | Findings may be subtle and variable. Sézary cells (highly convoluted cerebriform lymphocytes) along the basal layer, lichenoid infiltrate, epidermotropism, Pautrier microabscesses. Absence of spongiosis. |
Other lymphomas (Hodgkin's, non-Hodgkin's) | Atypical lymphocytes in dermis and epidermis |
Leukemias | Leukemic cells in dermis and epidermis |
Malignancies: solid organ | Nonspecific, variable findings |
Renal cell carcinoma | |
Hepatocellular carcinoma | |
Lung | |
Colon | |
Immunobullous disease | |
Pemphigus foliaceus, vulgaris | Acantholysis |
Bullous pemphigoid | Subepidermal separation |
Paraneoplastic pemphigus | Acantholysis, lichenoid infiltrate, necrotic keratinocytes |
Connective tissue disease | |
Dermatomyositis | Interface dermatitis |
Subacute lupus erythematosus | Interface dermatitis |
Infections | |
Scabies | Scabies mite at junction of epidermis and stratum corneum; spongiosis, dermal lymphocytic infiltrate |
Dermatophytosis | Hyphae |
Congenital cutaneous candidiasis | Hyphae and yeast-like forms |
Staphylococcus scalded skin syndrome | Vesiculation at level of granular cell layer |
Beta-hemolytic streptococcal erythroderma syndrome | |
Blood disorders | |
Hypereosinophilic syndrome | Dermal eosinophilic infiltrates |
Mastocytosis | Dermal mast cell infiltrates |
Graft-versus-host disease (GVHD) | Interface dermatitis |
Erythroderma occurring in the first year of life (congenital/neonatal/infant): additional diagnoses to be considered | |
Inherited ichthyoses | |
Epidermolytic hyperkeratosis (bullous congenital ichthyosiform erythroderma) | Hyperkeratosis, acanthosis, superficial vacuolation, clumps of irregular eosinophilic granules in the granular layer |
Nonbullous congenital ichthyosiform erythroderma | Acanthosis, hyperkeratosis, focal parakeratosis |
Netherton syndrome | Epidermal psoriasiform hyperplasia |
Rare ichthyoses | Hyperkeratosis |
Primary immunodeficiencies | Nonspecific features including hyperkeratosis, parakeratosis, acanthosis, and inflammatory infiltrate |
Severe combined immunodeficiency (SCID) | |
Severe combined immunodeficiency - Omenn syndrome (a form of SCID) | |
X-linked ectodermal dysplasia with immunodeficiency | |
Ectodermal dysplasias | |
Ankyloblepharon-ectodermal defects-cleft lip/palate syndrome | Nonspecific features including hyperkeratosis, parakeratosis, acanthosis, and inflammatory infiltrate |
Miscellaneous | |
Menkes disease |
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