Autoimmune keratolysis in a patient with leukocytoclastic vasculitis: unusual erythema elevatum diutinum with granulomatous pattern.
OBJECTIVE
Leukocytoclastic vasculitis (LCCV) is an immune complex-mediated, small vessel disease that is clinically characterized by the presence of palpable purpuric lesions, most often in association with rheumatic diseases. Ocular manifestations of LCCV are rare.


METHODS
We describe a patient with an unusual granulomatous pattern of erythema elevatum diutinum (EED) associated with autoimmune keratolysis.


RESULTS
We studied a 64-year-old man with decreased visual acuity and nodular lesions in both hands. Ocular examination revealed bilateral superior corneal melting with perforation in the left eye and conjunctival thickening in both eyes, in association with a severe inflammatory reaction. Histopathologic examination of the conjunctiva revealed granulomatous vasculitis with neutrophilic infiltrate, giant cells, and fibroblastic proliferation. A punch biopsy taken from his skin showed similar characteristics that suggested EED; however, there were no giant cells.


CONCLUSIONS
To our knowledge, autoimmune keratolysis secondary to cutaneous LCCV (EED) has not been described previously, and there has been no description of granulomatous reaction (in the conjunctiva) in EED.
Casanova
F H
FH
Department of Ophthalmology, Federal University of São Paulo, Paulista School of Medicine, Brazil. casanova@oftalmo.epm.br
Meirelles
R L
RL
Tojar
M
M
Martins
M C
MC
Rigueiro
M P
MP
de Freitas
D
D
D001327:Autoimmune Diseases / Q000209:etiology* / Q000473:pathology; D003228:Conjunctiva / Q000473:pathology; D003316:Corneal Diseases / Q000209:etiology*; D004890:Erythema / Q000150:complications* / Q000473:pathology; D006099:Granuloma / Q000150:complications* / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D012422:Rupture, Spontaneous; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000150:complications* / Q000473:pathology