Biomedical subjects

A Campo-Voegeli

Publications and source records attributed to A Campo-Voegeli.

4 recordsLinked to original sources

PubMed2002

Neonatal pemphigus vulgaris with extensive mucocutaneous lesions from a mother with oral pemphigus vulgaris.

The clinical phenotype of pemphigus is well explained by the combination of desmoglein (Dsg) 1 and Dsg3 distribution pattern and antiDsg autoantibody profile (Dsg compensation theory). It has been reported that neonatal skin has a similar Dsg distribution pattern to adult mucosal epithelia. We describe a newborn girl with mucocutaneous pemphigus vulgaris (PV) from a mother with mucosal dominant PV. The mother had had painful oral erosions for at least 7 months. Histopathological examination and direct and indirect immunofluorescence studies confirmed the diagnosis of PV and neonatal PV in the mother and daughter, respectively. The mother had a high titre of anti-Dsg3 IgG and a low titre of antiDsg1 IgG, while the neonate had only a high titre of anti-Dsg3 IgG, but no detectable antiDsg1 IgG. AntiDsg3 IgG, which caused the oral dominant phenotype in the mother, induced extensive oral as well as cutaneous lesions in the neonate. Our case provides clinical evidence for the Dsg compensation theory in neonatal PV.

Adult↗

PubMed1998

Systemic xanthohistiocytoma: a variant of xanthoma disseminatum?

We present a 66-year-old man who had maculopapular pigmented lesions on the skin of the head, neck and trunk suggesting generalized eruptive histiocytoma (GEH). These lesions had a yellowish centre in a target-like pattern that has not been previously described. The patient suffered from diplopia and had a severe sensorimotor polyneuropathy causing progressive paresis of the limbs. The explorations performed disclosed the presence of specific xanthomatous infiltrates in the skin, lungs, respiratory tract, peripheral nerves and meninges, suggesting xanthoma disseminatum (XD) or juvenile xanthogranuloma. Multiple osteolytic lesions of large bones were also found. The infiltrate was CD68, MAC 387 and factor XIIIa positive and S-100 and CD1 negative. Some cells contained worm-like bodies visible by electron microscopy. Our patient presented clinical and immunohistochemical findings suggestive of GEH, juvenile xanthogranuloma or XD, supporting the idea of a wide spectrum of non-Langerhans cell histiocytosis. These specific target-like xanthomatous lesions seem to be unique for this new variant of XD.

Aged↗

PubMed1998

Acrocyanosis induced by interferon alpha(2a).

A 56-year-old woman with mycosis fungoides developed acrocyanosis each time treatment with interferon alpha(2a) was started. Acrocyanosis disappeared after discontinuing therapy. Immunological study performed while acrocyanosis was present showed elevated antinuclear antibody (ANA) titers and circulating immune-complex levels. Long-term interferon treatment has been related to autoimmune side effects. Raynaud's phenomenon has been observed in patients undergoing interferon treatment associated with elevated ANA titers, to cryoglobulinemia or to arterial occlusion. Acrocyanosis has never before been described as side effect in patients undergoing this treatment. We believe that our patient's acrocyanosis must be considered a side effect of interferon.

Antibodies, Antinuclear↗

PubMed1998

Subacute cutaneous lupus erythematosus associated with cinnarizine and thiethylperazine therapy.

We report the case of a woman who developed subacute cutaneous lupus erythematosus (SCLE) after exposure to the sun while taking cinnarizine and thiethylperazine. The patient recalled that 10 years previously, a similar eruption had appeared after sunbathing, while she was taking only cinnarizine. SCLE development in this patient was probably drug related and there is some evidence that cinnarizine played an important pathogenic role.

Adult↗