Bullous Systemic Lupus Erythematosus and Cicatricial Pemphigoid

Abstract

Bullous systemic lupus erythematosus is a rare distinctive subepidermal bullous disease seen in patients with systemic lupus erythematosus (SLE). It has characteristical clinic, pathologic, and immunologic findings including antibodies to type VII collagen, laminin 332, laminin 331, and bullous pemphigoid antigen 230. Clinical presentation combined with histopathology, immunological testing, and concomitant diagnosis of SLE according to the criteria of American College of Rheumatology, are required to distinguish bullous SLE from these bullous diseases. In patients with bullous SLE, SLE disease progression and complications may be worse. Cicatricial pemphigoid is a chronic subepidermal blistering disease which is characterized by erosive lesions of mucous membranes and skin. Pathogenesis of cicatricial pemphigoid is characterized by linear deposition of Immunoglobulin G, A, or complement 3 along the epithelial basement membrane zone. The main target antigens are bullous pemphigoid antigens 180-230, laminin 331-332, type VII collagen, and β-4 integrin subunit. Cicatricial pemphigoid may lead to serious complications such as blindness and airway obstruction. Herein, clinical, histological, immunopathological features, the diagnosis and treatment of bullous SLE and cicatricial pemphigoid diseases are mentioned to raise awareness among the dermatologists about this important but rare heterogeneous bullous disease.

A case of bullous systemic lupus erythematosus: Diagnostic challenges and clinical implications

“…Immunopathological studies have revealed DIF to show linear IgG deposits at the BMZ in BSLE, with occasional granular IgA, IgM, and C3 deposits. 14,15 Salt-split skin indirect immunofluorescence (IIF) has also been used to demonstrate IgG deposition at the dermal side of cleavage. In addition, the presence of autoantibodies against Type VII collagen can also be detected in patients with this condition.…”

Localized Blistering Eruption of the Face and Neck – A Case Study and Differential Considerations

“…In EBA circulating antibodies react with type VII collagen of anchoring fibrils with a dermal pattern on salt-split skin IF (binding to the floor of the blister), whereas in MMP they are mainly, but not exclusively, directed to the carboxyterminal end of BP180 antigen, and in 50–80% of cases produce linear deposits on either epidermal or the dermal side of splitted skin. 3 , 25 , 26 BPMMP is even more heterogeneous in terms of immunological findings and circulating antibodies, if detectable, recognize different BMZ antigens. 11 , 23 , 26 The main identified target antigen was BP180 (or specifically NC16a domain of BP180) localized in the upper part of lamina lucida and characteristic of bullous pemphigoid, laminin 332 (laminin 5, formerly epiligrin) localized in the upper part of lamina densa and characteristic of anti-epiligrin cicatricial pemphigoid, and carboxyterminal end of BP180 at the lamina lucida-lamina densa border characteristic of MMP.…”

“…11 , 26 Differential diagnosis is additionally complicated by the fact that in some patients with BPMMP, antibodies to type VII collagen, localized in the lower lamina densa and sub-lamina densa have been described which are typical for EBA. 3 , 6 , 10 , 21 , 23 , 28 Consequently, some authors have suggested that Brunsting-Perry CP may represent a clinical, localized variant of EBA 3 , 28–32 . In the study by Joly et al the patient had the typical clinical features of Brunsting-Perry MMP, however DIF studies on salt-split peribullous skin showed IgG and C3 deposits located exclusively on the floor and not on the roof of the blister in a pattern usually found in patients with EBA.…”

“… 1 In classic MMP, oral mucosa and conjunctiva are typically affected and may cause significant dysfunction, including blindness. 2 , 3 Skin involvement is observed in 25–35% of MMP patients with well-tense blisters localized mainly on the head, neck, and upper torso that heal, leaving atrophic scars and milia. 1 , 4 , 5 They result from subepidermal split without acantholysis accompanied by mixed-cell infiltrates of lymphocytes, plasmacytes, histiocytes, neutrophils, and eosinophils.…”

Esophageal Stricture Recalcitrant to Repeated Dilation in Complex Rheumatological Disease

“… 3 Rupture of bullae can lead to scar and stricture formation. 9 , 11 Esophagitis dissecans can also be seen in bullous pemphigoid. 9 Diagnosis is made by biopsy in both diseases and can show superficial inflammatory infiltrate containing lymphocytes, histiocytes, and eosinophils and shows linear deposits of IgG and/or C3.…”