CUTANEOUS LUPUS ERYTHEMATOSUS: COMPARISON OF DIRECT IMMUNOFLUORESCENCE FINDINGS WITH HISTOPATHOLOGY

Background. Direct immunofluorescence (DIF) is considered to be a major advance in the diagnosis of connective tissue diseases, particularly lupus erythematosus (LE); however, the reliability of the technique depends on several factors, such as age and site of the lesion, type of immunofluorescence, type of immunoglobulin, etc. False positives and false negatives can occur. Objective and Methods. To determine the diagnostic value of DIF we studied 18 clinically established cases of cutaneous lupus erythematosus (CLE). Lesional biopsies were subjected to routine histopathologic examination and direct immunofluorescence. The results were compared. Results. Direct immunofluorescence was positive in 72.7% and histopathology in 66% cases. Combination of the two techniques (with one or both methods giving characteristic findings) was positive in 83% cases. The most common antibody was IgG, seen in 77.8% cases. A homogeneous pattern of immunofluorescence, with IgG, was seen in 55.5% of the cases. Although histopathology gave positive or suggestive results in all cases, DIF was negative in two cases of early cutaneous LE. Conclusion. Although DIF is an extremely useful diagnostic tool, it should always be used in conjunction with histopathology and the combination of the two methods yields the best results.     

CUTANEOUS LUPUS ERYTHEMATOSUS: DIRECT IMMUNOFLUORESCENCE AND EPIDERMAL BASAL MEMBRANE STUDY

Biopsy specimens of cutaneous discoid lesions of 71 patients with cutaneous lupus erythematosus (CLE) were studied. The material was examined by direct immunofluorescence (DIF) to establish positivity and morphologic patterns of immunoglobulins and complement deposits in the basement membrane zone (BMZ). A correlation between DIF results and thickening of the epidermis basement membrane (BM) stained by periodic acid-Schiff (PAS), obtained from 31 matched biopsy specimens, also is presented. Direct immunofluorescence had positive results in 66.20% of the 71 examinations and 70.97% of the matched examinations, whereas PAS showed BM thickening in 100% of the specimens. This observation stresses the importance of such histopathologic findings in the diagnosis of discoid lesions in CLE. There was no correlation between DIF patterns and PAS-stained BMZ thickening. These findings merit additional study. In conclusion, an accordance between PAS-stained BM thickening and immunoglobulin deposits has occurred in 70.97% of cases. This phenomenon does not depend on the presence of immunocomplexes, for it occurs even in cases in which immunocomplex deposits were not detected.     

CHRONIC DISCOID LUPUS ERYTHEMATOSUS IN THAILAND: DIRECT IMMUNOFLUORESCENCE STUDY

Background. Studies of chronic discoid lupus erythematosus (DLE) lesions by direct immunofluorescence (DIF) were heterogeneous with respect to classes of immunoglobulins and sites where these were deposited. Most of the studies were done in the USA and European countries. Materials and Methods. To obtain representative data from Asiatic countries, we analyzed the direct immunofluorescent abnormalities of 100 DLE lesions in Thai patients who were diagnosed on the basis of clinical and histologic criteria. Results. Granular deposits at the dermoepidermal junction (DEJ) were detected in 90% of cases. The common immunoreactants at the DEJ were IgG (63%) and IgM (47%). The deposits were usually combinations of various classes of immunoglobulins, mostly IgG (53%) and IgM (41%). Deposits of IgG and IgM alone at the DEJ were observed in 12% and 8%, respectively. Deposits at colloid bodies, dermal blood vessel walls, and epidermal nuclei were sometimes also seen. Conclusions. The DIF test of skin biopsy specimens is diagnostically significant in chronic DLE. Our study in Thai patients showed that the most common deposit was a combination of various classes of immunoglobulins, mostly IgG and often IgM as well as C3, and occurred at the DEJ of the involved area.     

ULTRASTRUCTURAL STUTDY OF THE CUTANEOUS ELASTIC FIBRES IN IN LUPUS ERYTHEMATOSUS

Summary.— During the ultrastructural study of the cutaneous lesions of 5 cases of systemic lupus erythematosus (S.L.E.) and 2 cases of discoid lupus erythematosus (D.L.E.), the authors report the changes observed in dermal elastic fibres.
These changes consist in a progressive disappearance of the granular matrix and in the subsequent appearance of the fibrillar component of the elastic fibres. These modifications of which several types are described, end in the formation of fibrillar bodies. The evolution of these changes is compared to the evolution of actinic elastosis.     

CUTANEOUS MANIFESTATIONS OF SYSTEMIC LUPUS ERYTHEMATOSUS IN A TERTIARY REFERRAL CENTER

Background:

Systemic lupus erythematosus (SLE) is an autoimmune disease with multiorgan involvement. The skin is the second most commonly affected organ. SLE with skin lesions can produce considerable morbidity resulting from painful skin lesions, alopecia, disfigurement, etc. Skin lesions in patients with lupus may be specific (LE specific) or may be non specific (LE non specific). Acute cutaneous LE (Lupus specific) has a strong association with systemic disease and non-specific skin lesions always indicate disease activity for which patients present to rheumatologists and internists. Therefore, a thorough understanding of the cutaneous manifestations of SLE is essential for most efficient management.

Aims:

The aims of this study were to evaluate the patterns and prevalence of skin lesions in patients with SLE and to assess the relationship between skin lesions and other systemic involvement.

Materials and Methods:

At the Department of Rheumatology and Clinical Immunology, IPGME&R in Kolkata, 150 patients with SLE fulfilling the clinical and laboratory criteria of the American Rheumatology Association (updated 1982) were examined and followed-up for cutaneous manifestations between January 2002 and January 2007.

Results:

Skin lesions were important clinical features. About 45 patients (30%) presented with skin lesions although all patients had skin lesions during the follow-up period. Skin changes noted were as follows: Lupus specific lesions: malar rash in 120 patients (80%), photosensitive dermatitis in 75 patients (50%), generalized maculopapular rash in 40 patients (26.67%), discoid rash in 30 patients (20%), subacute cutaneous lupus erythematosus (SCLE) in 5 patients (3.34%), lupus profundus in 5 patients (3.34%). The lupus non-specific lesions were non-scarring alopecia in 130 patients (86.67%), oral ulcers in 85 patients (56.67%), vasculitic lesions in 50 patients (33.34%), bullous lesions in 15 patients (10%), Raynaud''s phenomenon in 10 patients (6.67%), pyoderma gangrenosum in 2 patients (1.34%), erythema multiforme in 10 patients (6.67%), and nail fold infarcts in 2 patients (1.34%); however, mucosal discoid lupus, lichenoid discoid lupus, livedo reticularis, sclerodactyly, etc. were not detected. Patients having lupus-specific skin lesions e.g., malar rash were associated with systemic involvement, whereas those having lupus non-specific skin lesions were associated with disease flare.

Conclusions:

Skin lesions in patients with SLE are important disease manifestations and proper understanding is essential for diagnosis and efficient management.     

ULTRASTRUCTURE OF CUTANEOUS LESIONS IN LUPUS ERYTHEMATOSUS: A COMPARISON BETWEEN THE CUTANEOUS AND SYSTEMIC TYPES

The author investigated the basal lamina of cutaneous lesions in lupus erythematosus (LE) at the dermal-epidermal junction ultrastructurally, and discussed the differences between cutaneous LE (CLE) and systemic LE (SLE) and also among the types of cutaneous eruptions. The findings included micro-depression of the cytoplasm of basal cells, widening of the zona lucida, dissociation between anchoring filaments and the basal lamina, separation of the lamina from epidermal cells, focal defects and bifurcations of the basal lamina, and newly formed fine basal laminae at the original sites adjacent to the basal cells. The basal lamina deeply invaded the dermis with branching or multiplication which looked like loops or labyrinths and was thickened. These changes were all considered to be antecedents to the multiplication of the basal lamina. The basal lamina was multiple and sometimes more than five-layered. The laminae of the latter are thought to be specific to LE. The distances from the outer leaflet of the plasma membranes of basal cells to the deepest points of the multilayered basal laminae in the dermis were measured. They were regarded as an index of the degree of the multiplication of the lamina. Their mean value was significantly greater in CLE than in SLE. This fact indicated that the multiplication of the basal lamina in CLE was higher than in SLE. There were no significant differences among the types of cutaneous eruptions. The basal lamina is known to be formed beneath half-desmosomes without participation of the dermis. Thus multiplication is thought to be a secondary phenomenon following repeated damage to the epidermal cells.     

CUTANEOUS MANIFESTATIONS OF SYSTEMIC LUPUS ERYTHEMATOSUS: STUDY FROM LAHORE, PAKISTAN

Background. Systemic lupus erythematosus (SLE) is an autoimmune disorder in which cutaneous lesions occur in 85% of patients. This study from Lahore, Pakistan, was conducted to determine the pattern and incidence of such lesions in SLE. Methods. Forty patients with SLE fulfilling the clinical and laboratory criteria of the American Rheumatism Association (ARA) (1982) were examined between October 1992 and September 1993 for the presence of cutaneous manifestations. Results. Skin changes noted were: noncicatricial diffuse alopecia (82.5%), malar rash (60%), mucosal lesions (60%), discoid eruption (57.5%), photosensitivity (60%), nail involvement (55%), vascular lesions (50%), pruritus (45%), and pigmentary changes (37.5%). Peripheral gangrene, chronic ulcers, Raynaud's phenomenon, urticaria, chilblains, thrombophlebitis, palmar erythema, and erythema multiforme were rare. Antinuclear antibody reaction was positive in 80% and anti-dsDNA antibodies in 70%. Conclusion. A different clinical pattern was noted in our patients than reported previously.     

INCIDENCE OF LUPUS ERYTHEMATOSUS IN NIGERIA

A retrospective review of twelve Nigerian patients treated at the University Teaching Hospital, Lagos, Nigeria for Lupus erythematosus within a ten-year period is presented. Both the masquerading clinical spectrum and prognosis of this disease are similar to those encountered in cold countries of the world. This paper highlights hypothetical explanations for the rarity of this disease in Nigeria, while it draws attention to tropical clinical simulators of the disease.     

DISCOID LUPUS ERYTHEMATOSUS LESIONS DEVELOPED ON LUPUS ERYTHEMATOSUS PROFUNDUS NODULES

We describe this case of LEP for its unusual way of presentation. It first appeared with a LEP pattern, followed by a typical DEE of the skin, overlying the nodules only. Moreover, our histologic findings showed the typical pattern of lymphocytic lobular panniculitis, with hyaline necrosis of fat, the lymphoid nodule, and even the lymphocytic nuclear “dust.” The epidermal changes, with the liquefaction degeneration of the basal cell layer, a moderate follicular hyperkeratosis, and a perivascular and periappendeal lymphocytic infiltrate, were also observed in the abdominal lesion that developed last without clinically evident DLE.     

LUPUS ERYTHEMATOSUS PROFUNDUS

SUMMARY. —The clinical and histological features of 6 cases of lupus erythematosus (L.E.) profundus are described. The disease was chronic with crops of subcutaneous nodules, usually on the head and arms, that healed with deep scarring. Signs of classical discoid L.E. were usually few and delayed. The histology showed a characteristic lower dermal and subcutaneous necrobiosis with some vasculitis and a striking lack of cellular response. The dying collagen showed overlapping types of change; in some areas it stained faintly and was swollen whilst still retaining some fibrillary structure; in others it was replaced by an eosinophilic material that was either in homogenous puddles or broken into amorphous clumps. The differentiation of these changes from other forms of panniculitis is discussed and it is concluded that the changes are usually sufficiently distinctive to make a diagnosis possible in what may clinically be a perplexing disease.