Pemphigus is an autoimmune blistering disease of the skin, mucous membranes, or both. There are two main categories of pemphigus: pemphigus foliaceus (PF) and pemphigus vulgaris (PV). PV is further subdivided into mucosal dominant and mucocutaneous types, according to the extent of cutaneous lesions. These classes of pemphigus have distinct histopathologic and serologic findings, with most cases falling into these subtypes. We report 4 cases that clinically showed blisters and erosions in the skin only, without mucosal involvement. Histologic examination of cutaneous lesions demonstrated suprabasilar acantholysis, a typical finding for PV. These patients had predominant anti-desmoglein 1 (Dsg1) IgG autoantibodies as well as anti-Dsg3 IgG autoantibodies, as determined by enzyme-linked immunosorbent assay. The desmoglein compensation theory posits that this rare phenotype can be produced by pathogenically weak anti-Dsg3 IgG in the presence of potent anti-Dsg1 IgG autoantibodies. Thus, cutaneous type PV without apparent mucosal involvement is observed as a rare clinical and histologic expression of pemphigus. This expression can be a transient phenotype that may develop from, or evolve into, other subtypes of pemphigus.

Cutaneous type pemphigus vulgaris: a rare clinical phenotype of pemphigus.

Background: Cases of paraneoplastic pemphigus (PNP) have been reported associated with various lymphoproliferative malignancies and benign Castleman tumors, with the most severe course and fatal outcome seen in patients with bronchiolitis obliterans. Objective: The aim was to establish immunologic associations by coexistence of Castleman tumor, myasthenia gravis, and bronchiolitis obliterans and to evaluate the treatment modalities. Methods: Clinical studies included computed tomography of the mediastinum, computed tomography and magnetic resonance imaging of the abdominal cavity, and quantitative electromyography. Direct and indirect immunofluorescence on various substrates, immunoblot analysis, immunoprecipitation, and specific enzyme-linked immunosorbent assay using recombinant desmogleins (Dsg) were performed as immunologic assays. Results: Direct and indirect immunofluorescence including rat bladder showed intercellular antibodies. Immunoblotting disclosed antibodies to envoplakin (210 kd protein) and periplakin (190 kd protein); in addition, immunoprecipitation detected antibodies to desmoplakin I (250 kd protein). Antibodies to Dsg3 (pemphigus vulgaris antigen) were detected by specific enzyme-linked immunosorbent assay. Myasthenia gravis was controlled by drugs; however, mucocutaneous changes were not fully responsive to corticosteroids and cyclophosphamide pulses, cyclosporine, and intravenous immunoglobulins. The surgical removal of Castleman tumor did not change the course of the disease. The fatal outcome was the result of bronchiolitis obliterans that occurred after the surgery and was only transitionally controlled by plasmapheresis. Conclusion: This is the first case of paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis, and bronchiolitis obliterans. Despite a benign character of the tumor the patient died, as do all patients with bronchiolitis obliterans. Massive plasmapheresis has only a transient effect. We confirmed the presence of antibodies to Dsg 3, in addition to the set of specific paraneoplastic pemphigus antibodies against various proteins of plakin family. (J Am Acad Dermatol 1999;41:393-400.)

Paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis and bronchiolitis obliterans

Background There are a number of reports of pemphigus with clinical shifting between pemphigus foliaceus (PF) and pemphigus vulgaris (PV). On the other hand, a novel enzyme-linked immunosorbent assay (ELISA) against recombinant baculoproteins of desmoglein 1 (Dsg1) (PF antigen) and Dsg3 (PV antigen) has been established and found to be extremely sensitive and specific.



Objectives To characterize the change in the antibody profiles in a series of pemphigus cases with mixed features of PF and PV by various methods, including the novel ELISA.



Patients/methods Sera were obtained from eight cases undergoing a shift between PF and PV and three cases of coexistent PF and PV. The autoantigens were analysed by ELISA, as well as by immunofluorescence using normal human skin sections and immunoblotting using normal human epidermal extracts.



Results The results of the ELISA, immunofluorescence and immunoblotting studies showed that the transition between PF and PV correlates well with the changes of autoantibodies against either Dsg1 or Dsg3.



Conclusions The clinical phenotype at each stage is defined by the anti-Dsg antibody profile in the serum of these pemphigus patients showing mixed features of PF and PV. In addition, ELISA using recombinant baculoproteins was particularly useful in distinguishing PF and PV.

The clinical transition between pemphigus foliaceus and pemphigus vulgaris correlates well with the changes in autoantibody profile assessed by an enzyme-linked immunosorbent assay

A Subset of Pemphigus Foliaceus Patients Exhibits Pathogenic Autoantibodies Against Both Desmoglein-1 and Desmoglein-3

Paraneoplastic pemphigus is a relatively rare but highly significant acquired mucocutaneous disorder. The condition typically presents in patients with previously diagnosed lymphoreticular disease, primarily malignancies (non-Hodgkin's lymphoma; chronic lymphocytic leukemia). Multiple sites of involvement are common, with the skin affected by a polymorphous eruption that includes lichenoid plaques as well as bullae. The oral lesions occur in all cases and present as painful widespread shallow ulcers with hemorrhagic crusting of the lips. Conjunctival involvement is also seen and may result in scarring. Pulmonary lesions are an ominous sign. Histopathologically, intraepithelial and subepithelial clefting associated with interface dermatitis are seen. A variety of autoantibodies directed against desmoplakins and desmogleins can be identified with immunoprecipitation studies. Treatment with immunosuppressive agents may result in some resolution of the disease, but the prognosis for paraneoplastic pemphigus is considered to be poor.

Paraneoplastic pemphigus: a review of the literature

We present a case of the transformation of pemphigus vulgaris (PV) into pemphigus foliaceus (PF), with clinical, histological and immunological characteristics of the two major forms of the disease. At the beginning, the patient had oral involvement and cutaneous vegetating lesions typical of PV. Later, and in addition, flaccid bullae with a histological pattern characteristic of PF, in some areas with suprabasal acantholysis characteristic of PV, developed. Intercellular antibodies, studied repeatedly by immunoblot, recognized both PV (130 kD) and PF (160 kD) antigens. To our knowledge, this is the first case of well-documented transition of PV into PF, and their coexistence. This case may be considered as an experiment of nature, which provides evidence that the clinical and histological expression of the disease is directly related to the specificity of IC antibodies.

Pemphigus vulgaris transforming into pemphigus foliaceus and their coexistence

We describe a case of a pical pemphigus associated with malignant thymoma and myasthenia gravis with clinical features suggestive of emphigus foliaceus, but with a histological pattern characteristic of emphigus vulgaris. Direct immunofluorescence revealed intercellular and basement membrane zone IgG and C3 deposits. The circulating antibodies were of unique specificity, restricted to the 170 kD antigen, as found by immunoprecipitation assay. Our case provides evidence that pemphigus associated with malignancy may have divergent clinical features and immune responses, such as to the 170 kD antigen, one of the characteristic polypeptides of paraneoplastic pemphigus (PNP). Thus, this case may present a variant of PNP

Atypical pemphigus associated with malignant thymoma and autoimmune response restricted to 170 kD antigen: Is it a variant of paraneoplastic pemphigus?

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Pemphigus vulgaris transforming into pemphigus foliaceus and their coexistence

Atypical pemphigus associated with malignant thymoma and autoimmune response restricted to 170 kD antigen: Is it a variant of paraneoplastic pemphigus?