Recurrent oral erythema multiforme. Clinical experience with 11 patients.

Recurrent oral erythema multiforme is a distinct, but less-well-recognized variant of the erythema multiforme group of diseases. It is characterized by recurrent blistering of the mouth, periodic recurrences, prolonged duration, and considerable morbidity. The cause is unknown; however, a number of precipitating factors have been suggested.

Acquired reversible autistic syndrome in acute encephalopathic illness in children.

In seeking the neurologic substrate of the autistic syndrome of childhood, previous studies have implicated the medial temporal lobe or the ring of mesolimbic cortex located in the mesial frontal and temporal lobes. During an acute encephalopathic illness, a clinical picture developed in three children that was consistent with infantile autism. This development was reversible.

Brunsting-Perry cicatricial pemphigoid associated with bullous pemphigoid.

Brunsting-Perry cicatricial pemphigoid is a chronic recurrent vesiculobullous eruption that occurs predominantly on the head and neck. Because of the clinical, histologic, and immunologic similarity to cicatricial pemphigoid, it has been suggested that Brunsting-Perry cicatricial pemphigoid represents the cutaneous counterpart of cicatricial pemphigoid and should be included as a member of the pemphigoid spectrum of diseases. The concurrence of Brunsting-Perry cicatricial pemphigoid and bullous pemphigoid, as seen in the patient in this report, supports this viewpoint.

Hodgkin's disease with specific bullous lesions.

A variety of bullous eruptions have been reported in patients with Hodgkin's disease, among them erythema multiforme, herpesvirus infections, bullous impetigo, prurigo-like papules with vesicles, drug eruptions, bullous pemphigoid, dermatitis herpetiformis, and acquired epidermolysis bullosa. We now describe a patient whose bullous eruption was thought initially to be bullous pemphigoid. However, histopathologic examination of a bulla showed a lymphomatous infiltrate beneath an intraepidermal and subepidermal blister.

Cicatricial pemphigoid of Brunsting-Perry. Immunofluorescent studies.

Six patients with a vesiculobullous eruption of the type described by Brunsting and Perry as benign pemphigoid were studied by direct and indirect immunofluorescence. All six showed linear deposits of IgG but not IgM or IgA at the epidermal-dermal junction. One case also showed C3 deposition and one patient had circulating antibasement membrane zone antibodies in a titer of 1280.

Pemphigus vegetans of Hallopeau: immunofluorescent studies.

Immunofluorescent techniques were used in the study of tissue from a patient with pemphigus vegetans of Hallopeau. The findings were typical of pemphigus, confirming the propriety of classifying this entity as a benign variant of pemphigus, rather than a disease of purely microbial etiology. The question is raised whether other types of vegetating pyodermas actually are separate diseases or represent a spectrum of pemphigus with the clinical appearance dependent on the patient's immunity to his disease.

Immunopathology of cicatricial pemphigoid: studies of complement deposition.

Immunopathologic investigations were conducted on the sera and oral mucosal tissue specimens of 23 patients with cicatricial pemphigoid. A linear, continuous basement membrane zone pattern was noted in 83% of oral mucosal biopsy specimens studied. This pattern is indistinguishable from the pattern noted in immunofluorescence studies of bullous pemphigoid, herpes gestationis, and some cases of desquamative gingivitis.

Optic atrophy following prophylactic chemotherapy and cranial radiation for acute lymphocytic leukemia.

Two patients with acute lymphocytic leukemia developed progressive optic nerve and chiasmal lesions eight to nine months after the initiation of identical chemotherapy protocols that included intrathecal medication and prophylactic radiation of only 2,400 rads to the central nervous system. Both patients eventually lost all vision despite additional radiotherapy, and there was no evidence of leukemia involving the central nervous system after acute lymphocytic leukemia was diagnosed. Optic nerve biopsy in one case showed changes consistent with radiation necrosis.

Vesicular pemphigoid.

Seven patients had a chronic eruption of small vesicles that was initially considered to be atypical dermatitis herpetiformis. Histopathologic studies disclosed a subepidermal bulla that was compatible with either bullous pemphigoid or dermatitis herpetiformis. Immunofluorescence studies demonstrated deposition of IgG or C3, Or both, at the basement-membrane zone (BMZ) in six of seven patients.

Proper biopsy technique for immunofluorescence tests on skin.

Successful use of direct immunofluorescence tests on skin depends on selection of the appropriate biopsy site, careful preparation of the specimen, and prompt transportation to a reliable laboratory. Recommendations for selection of biopsy sites in various dermatoses and methods available for handling specimens are discussed.

Pemphigus involving the eyes.

Two persons with pemphigus vulgaris involving the eyes were studied. In one case, in vivo-fixed intercellular deposition of IgG was demonstrated in a specimen from the conjunctiva.

Immunofluorescent studies of ocular cicatricial pemphigoid.

Using direct and indirect immunofluorescent techniques, we studied ten patients with cicatricial pemphigoid (chronic cicatricial conjunctivitis). Serologic and histologic evaluation of 13 conjunctival, 12 mucous membrane, and 11 skin specimens from the ten patients showed that three had immunoglobulin deposition at the basement membrane zone of the conjunctiva. One patient had immunoglobulin deposited in the affected skin and oral mucosa.