[Paraneoplastic pemphigus with tracheobronchial involvement]. / Pemphigus paranéoplasique avec atteinte trachéo-bronchique.

BACKGROUND: Paraneoplastic pemphigus is a bullous skin disease with characteristic polymorphous clinical presentation and precise histological and immunological features. We report a case of paraneoplastic pemphigus associated with chronic lymphoid leukemia involving the tracheobronchial epithelium. CASE REPORT: A patient with chronic lymphoid leukemia developed pluriorificial lesions. There were several conjunctival, buccal and genital erosions associated with erosive plaques on the trunk, Nikolski's sign and bullous lesions suggestive of paraneoplastic pemphigus. Histology examination of a bulla showed intraepidermal blistering and suprabasal acantholysis. Direct immunofluorescence evidenced intercellular IgG and C3 deposits. Search for anti-intercellular substance antibodies was positive with fluorescence on specific paraneoplastic pemphigus substrates. At immunotransfer, the serum recognized several bands corresponding to 250, 230, 210 and 190 kD antigens, confirming the diagnosis of paraneoplastic pemphigus. Several days later, the patient's general condition deteriorated with bronchorrhea. Bronchial endoscopy visualized ulceronecrotic plaques. Tracheal biopsy evidenced acantholytic cells and intraepithelial cleavage. General corticosteroid therapy was initiated and led to improvement of the skin lesions but the patient died rapidly from pneumonia. Autopsy confirmed the presence of epithelial cleavage and acantholysis involving the trachea and bronchi. DISCUSSION: This case illustrates the difficulty of diagnosing paraneoplastic pemphigus in the early stages. The pluriorificial lesions were suggestive of a Stevens-Johnson syndrome. Besides the genital, conjunctival and buccal mucosa, other mucosa can be involved. In our case, despite the absence of an immunological element, histology was highly suggestive of specific tracheobronchial localizations. The presence of such lesions, which should be searched for in all cases with bronchopulmonary manifestations, worsens the prognosis.

Juvenile xanthogranuloma: a congenital giant form leading to a wide atrophic sequela.

Juvenile xanthogranuloma is the most common non-Langerhans cell histiocytosis of infancy and childhood. It is a benign disorder that usually regresses spontaneously, more often without or with limited skin changes. The clinical features are very pleomorphic and recognition of the atypical presentations should facilitate the diagnosis. We report a 9-month-old boy who had an extensive, atrophic lesion on the proximal thigh that followed the course of the congenital giant form. The large size of the lesion does not alter the clinical course or result in extracutaneous involvement, which remains rare. However, uncommon clinical presentations raise the problem of differential diagnosis, requiring biopsy to eliminate other tumors with a less favorable prognosis.