Thomas K. Burnham

Systemic lupus erythematosus and coexisting bullous pemphigoid: Immunofluorescent investigations

An 18-year-old black woman with systemic lupus erythematosus (SLE) who subsequently developed a bullous eruption is presented. Direct immunofluorescent studies of a bulla and peribullous skin demonstrated a tubular band at the dermoepidermal junction diagnostic for bullous pemphigoid (BP). However, an atrophic plaque clinically and histologically characteristic for lupus erythematosus (LE) also demonstrated a tubular band instead of one of the LE bands. Indirect immunofluorescent studies employing normal human skin revealed peripheral, homogeneous, and particulate antinuclear antibody patterns with anti-IgG but were negative for circulating anti-basal zone antibodies. Therefore BP was dominant cutaneously, whereas SLE prevailed serologically. This case illustrates the diagnostic problems of a bullous eruption in an SLE patient and points out some unusual immunofluorescent findings.

Lichen planus and coexisting lupus erythematosus versus lichen planus-like lupus erythematosus

Abstract A middle-aged black woman presented initially with painful cutaneous plaques that were located at various sites and that were diagnosed histologically as lichen planus. Standard light microscopic examination showed histopathologic variants of lichen planus. Direct immunofluorescence of a skin lesion had negative results for any of the lupus erythematosus bands but did reveal hyaline bodies in the deep cellular layer of the epidermis and the superficial layer of the dermis. These findings were compatible with either lichen planus or lupus erythematosus. However, both the clinical course of the eruption and the antinuclear antibody tests showed that the immunofluorescent antinuclear antibody pattern of large, speckle-like threads were consistent with lupus erythematosus. Furthermore, the large, speckle-like, thready antinuclear antibody pattern, which has been shown to be a marker for a benign subset of lupus erythematosus, is not seen in lichen planus. Lichen planus-like lupus erythematosus was therefore the more likely diagnosis.

Antinuclear antibodies as indicators for the procainamide-induced systemic lupus erythematosus—like syndrome and its clinical presentations

Fifty patients on a regimen of procainamide were studied in regard to the association between antinuclear antibodies (ANA) and the development of drug-induced systemic lupus erythematosus (SLE)-like syndrome. Four groups were identified: Group 1 was ANA-positive, with clinical manifestations (serologic and clinical findings); Group 2 was ANA-positive, without clinical manifestations (serologic findings only); Group 3 was ANA negative (no patients with clinical manifestations); and Group 4 had SLE persisting after discontinuance of procainamide. The leukocyte-specific ANA (LSANA) patterns were predominant, with peripheral LSANA confined to Groups 1 and 4. Furthermore, the titer of the homogeneous LSANA, to which peripheral LSANA converts on dilution, was clinically significant. A homogeneous LSANA titer of 160 or greater was seen essentially only in patients with clinical manifestations of the SLE-like syndrome. Serial ANA determinations are therefore necessary to monitor patients receiving procainamide.

Multiple Cutaneous and Conjunctival Keratoacanthomata

A case of multiple keratoacanthomata affecting both the skin and conjunctiva is reported. Although the patient was carefully studied on several occasions, the correct diagnosis was long delayed for the following reasons: (1) a history of pulmonary tuberculosis, (2) the disparity between the microscopic findings in solitary keratoacanthoma and those in some cases of multiple keratoacanthomata, (3) the unusual and heretofore unreported coexistence of cutaneous and conjunctival lesions.

Serologic profiles as immunologic markers for different clinical presentations of lupus erythematosus

The clinical and laboratory features of 55 patients with lupus erythematosus (LE), grouped on the basis of six nuclear immunofluorescent pattern results commonly encountered in this disease were examined. Serologic profiles of antinuclear antibodies (ANA), anti-DNA and anti-ENA results can serve as immunologic markers in LE for a benign subset and two other groups with a different incidence of certain clinical characteristics. The large speckle-like thready pattern without antibodies to DNA or ENA is an immunologic marker for a benign LE subset, with generalized skin lesions with or without joint involvement only. Significant levels of the anti-DNA antibodies with the shrunken peripheral, peripheral, or leukocyte-specific ANA with a particulate pattern are markers for severe systemic involvement. The thready pattern with antibodies to ENA (Sm antigen) and leukocyte-specific ANA without a particulate pattern, with or without antibodies to DNA or ENA, indicate less severe systemic disease.

Large speckle-like thready and thready antinuclear antibody patterns as markers for different clinical presentations in lupus erythematosus

Fifty-one patients with lupus erythematosus were studied retrospectively. They were chosen on the basis of their antinuclear antibody (ANA) immunofluorescent pattern. Only those with the thready or the large speckle-like thready patterns were studied. Autoantibody profiles consisting of ANA, anti-single-stranded deoxyribonucleic acid (ssDNA) antibody, and anti-extractable nuclear antigen (ENA) antibody determinations were obtained. The patients with the thready ANA pattern and anti-ENA (Sm) antibodies had a significantly higher incidence of pulmonary, joint, and renal involvement than the anti-ENA negative patients with the large speckle-like thready pattern. There was also a significantly higher incidence of Raynauds phenomenon in patients with the thready pattern than in those with the large speckle-like thready pattern. Photosensitivity was seen significantly more frequently in the patients with the large speckle-like thready pattern than in those with the thready pattern.