Livedo, dementia, thrombocytopenia, and endotheliitis without antiphospholipid antibodies: seronegative antiphospholipid-like syndrome.

[sneddon syndrome]

We report a 51 -year -old woman who presented with dementia , livedo racemosa , polyarthralgia , mild renal insufficiency , proteinuria , and thrombocytopenia . Cutaneous and renal biopsy specimens both showed an identical specific occlusive arteriolopathy consistent with Sneddon syndrome and antiphospholipid syndrome . However , no antiphospholipid antibodies were detected and we , therefore , diagnosed seronegative antiphospholipid-like syndrome . We discuss the nosology of this entity and its association with non-antiphospholipid antibody -related Sneddon syndrome . The common denominator of Sneddon syndrome and antiphospholipid syndrome with or without antiphospholipid antibodies seems to be the endothelial damage and occlusive arteriolopathy . Skin biopsy is useful to confirm the diagnosis of seronegative antiphospholipid-like syndrome .

Diseases
Validation

Diseases presenting "skin biopsy" symptom

adrenomyeloneuropathy

cadasil

cutaneous mastocytosis

dentinogenesis imperfecta

dracunculiasis

epidermolysis bullosa simplex

erdheim-chester disease

erythropoietic protoporphyria

fabry disease

harlequin ichthyosis

inclusion body myositis

junctional epidermolysis bullosa

kindler syndrome

lamellar ichthyosis

malignant atrophic papulosis

monosomy 21

oligodontia

primary effusion lymphoma

primary hyperoxaluria type 1

pyruvate dehydrogenase deficiency

sneddon syndrome

waldenström macroglobulinemia

x-linked adrenoleukodystrophy

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