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E Cozzani

Publications and source records attributed to E Cozzani.

50 records · Page 3Linked to original sources

Linear IgA bullous dermatosis with autoantibodies to a 290 kd antigen of anchoring fibrils.

We describe a patient with a papulovesicular eruption associated with scarring and severe mucosal lesions that led to blindness. Direct immunofluorescence showed linear IgA deposits at the dermoepidermal junction. Indirect immunofluorescence microscopy showed that the patient's serum reacted with the dermal side of salt-split skin. Direct immunoelectron microscopy showed the IgA deposits to be associated with anchoring fibrils, whereas with Western blot analysis the patient's serum reacted with a 290 kd dermal antigen. On the basis of these findings, we suggest that our case may represent a form of IgA-mediated epidermolysis bullosa acquisita.

Adult↗

Linear IgA bullous dermatosis of childhood with autoantibodies to a 230 kDa epidermal antigen.

Linear IgA bullous dermatosis (LABD) is an autoimmune, subepidermal disease defined on the basis of direct immunofluorescence findings. However, more recent techniques used to study bullous dermatoses suggest that LABD may be heterogeneous. A patient with LABD of childhood (chronic benign disease of childhood, CBDC) was studied by indirect immunofluorescence on salt-split skin and by Western blot in an attempt to characterize the involved autoantigen. This young girl's periorificial (mouth, genitalia), erythematovesicular lesions were diagnosed initially as herpes simplex. Histologic examination revealed eosinophilic spongiosis, suggesting the diagnosis of an autoimmune blistering disease. Direct immunofluorescence showed an exclusive linear IgA deposit at the dermoepidermal junction. Indirect immunofluorescence revealed circulating IgA autoantibodies that reacted with the epidermal side of salt-split skin; these reacted by Western blot with a 230 kDa epidermal antigen, as in bullous pemphigoid. This case, fulfilling the diagnostic clinical and direct immunofluorescence criteria for LABD/CBDC, seems to represent IgA bullous pemphigoid. It further underscores the nosologic heterogeneity of LABD, which probably includes, apart from bullous pemphigoid, epidermolysis bullosa acquisita and cicatricial pemphigoid.

Autoantibodies↗

Immunohistopathological study of autoimmune pemphigus. Lack of strictly specific histological and indirect immunofluorescence criteria for paraneoplastic pemphigus.

Paraneoplastic pemphigus (PNP) is a form of pemphigus considered to bear characteristic immunohistopathological features (vacuolar-interface dermatitis, keratinocyte dyskeratosis and reactivity of patients' sera with transitional epithelia). The present study was undertaken in order to investigate whether these criteria are specific enough so as to allow the diagnosis of PNP in the absence of clinical data. A retrospective study of 66 biopsies of pemphigus revealed that one third of them comprised histological signs of PNP; one of the corresponding sera reacted with mouse bladder epithelium. However, no evidence of a neoplastic disease was present in any of the patients, and Western blotting further excluded the diagnosis of PNP. These results suggest that some of the features considered characteristic of PNP are not strictly specific for this variety of pemphigus; hence this diagnosis cannot be reliably established by histology or immunofluorescence but requires biochemical studies.

Acantholysis↗

[Pemphigoid in children].

INTRODUCTION: Bullous pemphigoid is rare during the childhood. The authors report the 51st case. CASE REPORT: Jeremie, a seven-month-old baby, had atypical bullous lesions. Histological examination, direct and indirect immunofluorescence were in favor of bullous pemphigoid. COMMENTS: Clinical and biological features of bullous pemphigoid are similar in the adult and in the infant. First treatment is represented by corticosteroids. Evolution is generally very good.

Facial Dermatoses↗

[The desmosome: structure, function and acquired pathology].

The essential function of the skin is that of a barriere against the outside. The epidermis is the tissue which assures the solidity and the resistance of the skin against forces of traction. The desmosomes, allowing the adhesion of epidermal cells, the keratinocytes, between them, are the structures of resistance of the epidermis. This adhesion function is possible thanks to the interactions between the desmosomial molecules and cytosqueletal filaments. Currently fifteen desmosomal proteins implicated in this process have been characterized. The role of desmosome in integrity of epidermis is demonstrated by the existence of the auto immune dermatosis, the pemphigus, in which the inflammatory reaction against desmosomal proteins results in the loss of the cohesion of keratinocytes.

Cytoskeletal Proteins↗

Ultrastructural study of chronic lesions of erythema elevatum diutinum: "extracellular cholesterosis" is a misnomer.

Erythema elevatum diutinum (EED) is a rare disease of unknown origin that belongs to the spectrum of leukocytoclastic vasculitis. Chronic lesions of EED contain lipid deposits, for which the term extracellular cholesterosis has been coined. We studied a typical case of EED with long-standing lesions. Findings of electron microscopic examination revealed a heavy, exclusively intracellular lipid deposition that consisted of lipid droplets, myelin figures, and rare cholesterol clefts within histiocytes but also within epidermal keratinocytes, mast cells, pericytes, and lymphocytes. These findings are in keeping with the results of previous ultrastructural studies and suggest that the term extracellular cholesterosis is a misnomer; intracellular lipidosis would more accurately describe the lipid deposition.

Chronic Disease↗

Development of pemphigus foliaceus in a patient with psoriasis: a simple coincidence?

Several cases of psoriasis associated with bullous diseases have been reported in the literature. Bullous pemphigoid is the most frequent association observed, while pemphigus-related disorders are less frequently described. The authors report a case of a patient with long-standing psoriasis who developed pemphigus foliaceous and discuss the possible relationship between the diseases.

Adult↗