A Case of Antiepiligrin Cicatricial Pemphigoid with Nephrotic Syndrome

Abstract

We report a 71-year-old woman with antiepiligrin cicatricial pemphigoid associated with nephrotic syndrome. She presented with a six-month history of pruritic blisters over her trunk and legs. She also had episodes of recurrent painful oral erosions. A skin biopsy showed a subepidermal bulla, and a direct immunofluorescence (DIF) study revealed linear deposition of IgG and C3 at the basement membrane zone (BMZ). Indirect immunofluorescence (IIF) staining of 1 M NaCl-split skin demonstrated circulating IgG autoantibodies reactive with the dermal side. Immunoprecipitation studies of the patient's serum disclosed IgG autoantibodies directed against a set of polypeptides that corresponded to laminin 5 (beta 3 gamma 2). Based upon the long-standing edema of her legs and her hypoproteinemia, she was diagnosed with nephrotic syndrome. To our knowledge, the association of antiepiligrin cicatricial pemphigoid with nephrotic syndrome has not been reported previously.

Metastatic ovarian carcinoma-associated subepidermal blistering disease with autoantibodies to both the p200 dermal antigen and the γ2 subunit of laminin 5 showing unusual clinical features

“…Most patients with ALMMP have autoantibodies to the α3 subunit. Some patients have antibodies to the β3 subunit, and others show a reactivity to the α3 and γ2 subunits or to the β3 and γ2 subunits 10 . However, patients with autoantibodies to the γ2 subunit alone are relatively rare 11,12 .…”

“…Some patients have antibodies to the b3 subunit, and others show a reactivity to the a3 and c2 subunits or to the b3 and c2 subunits. 10 However, patients with autoantibodies to the c2 subunit alone are relatively rare. 11,12 There have been a few reports concerning mucous membrane pemphigoid (MMP) with more than two autoantibodies to distinct molecules, including a combination of BP180 and laminin 5, 13 a combination of b4 integrin, BP180 and BP230, 14 and a combination of BP180, BP230 and laminin 5.…”

“…Immunohistochemically, the c2 subunit of laminin 5 can be detected in the skin and the basement membranes of various epithelial tissues, including the bronchus, lung, small intestine, stomach and kidney. 10 There are several case reports of ALMMP showing an association with malignant tumours of different tissues, i.e. large cell carcinoma of the bronchus, 17 adenocarcinoma of the lung, 18 gastric carcinoma [19][20][21][22] and metastatic endometrial carcinoma.…”

Cicatricial pemphigoid with widespread bullous eruption

“…This patient presented with widespread cutaneous blisters indistinguishable from those of BP. Although CP with widespread bullous eruptions has been reported, 3–7 typical patients with CP only develop a small number of skin lesions, if any. Thus, disseminated cutaneous blisters are an uncommon feature of CP and seem to be suggestive of BP.…”

“…Skin lesions appear in 25–35% of patients, and the number and the area of the cutaneous lesions are usually small 2 . To date, only six cases of CP with mucosal scarring and widespread bullous eruption have been reported 3–7 . We report a case of CP presenting as widespread cutaneous bullous lesions with immunological features similar to those of bullous pemphigoid (BP).…”