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Background: Pemphigus is associated with several autoimmune, dermatological, and psychiatric diseases. Previous studies have reported an increasing incidence of pemphigus in Finland, particularly pemphigus foliaceus and erythematosus., Objectives: The aim of this study was to determine the clinical presentation and associated comorbidities in pemphigus patients., Materials & Methods: We retrospectively assessed 66 pemphigus patients in Helsinki University Hospital and, with an age-standardised control group, performed a comparison of the studied comorbidities., Results: The patients displayed a 0.8 female:male distribution and a mean age of 57.4 years. Pemphigus vulgaris (41%), foliaceus (30%), and erythematosus (15%) were the most common subtypes. Hypertension (30%) and dyslipidaemia (21%) were the most prevalent comorbidities. We found a statistically significant association between pemphigus and a past history of, or concurrent malignancies and atopic dermatitis (p = 0.002 and p = 0.028, respectively). No significant difference was observed in the prevalence of cardiovascular disease, asthma, chronic obstructive lung disease, type I or II diabetes mellitus, inflammatory bowel disease, depression, or anxiety. Erosions (65%), bullae (59%), and crusted lesions (55%) were observed in most patients. Half of the patients experienced pruritus before or at diagnosis. Pruritus was associated with pemphigus without mucosal involvement (p = 0.01)., Conclusion: We found a significant association between pemphigus and atopic dermatitis and a history of malignancy. The clinical picture frequently included pruritus. These results support the findings of some recent studies of pruritus occurring more frequently in patients with pemphigus foliaceus and cutaneous pemphigus vulgaris.

IgA pemphigus is a rare variant of pemphigus. IgA pemphigus is subdivided into intraepidermal neutrophilic IgA dermatosis-type (IEN-type), whose target antigen is still an enigma, and subcorneal pustular dermatosis-type, whose target antigen is desmocollin 1 (Dsc1). We report a 56-year-old Japanese male with IgA pemphigus showing atypical erythema. One month after erythema developed, the patient visited his private physician, and was tentatively diagnosed as having erythema multiforme. The patient had been intermittently treated with a low dose of oral prednisolone for a year without benefit before visiting our hospital. Clinical examination revealed irregularly-shaped and partially edematous erythema over the trunk and extremities without mucosal involvement. Neither bullae nor pustules were seen during the course. Direct immunofluorescence showed IgA deposition on cell surfaces of keratinocytes in the upper two thirds of the epidermis. Indirect immunofluorescence of monkey esophagus sections revealed IgA and IgG anti-cell surface antibodies. Our new enzyme-linked immunosorbent assays using eukaryotic recombinant proteins of human Dsc 1-3 detected IgA antibodies to Dsc1 and Dsc2. Although no apparent bullae were observed, the diagnosis of IgA pemphigus was made. Prednisolone 30 mg daily was required to control erythematous lesions. Although the pathomechanism for the unique skin lesion is unknown, the possibility that IgA pemphigus has a prodromal phase and that early administration of low dose prednisolone suppressed the development of pustules or bullae were considered.

Localized pemphigus foliaceus (PF) clinically presents as red plaques with scale and crust due to acantholysis of the epidermis. The follicular infundibulum shows acantholysis, but usually with epidermal acantholysis or at least erosion. Localized PF exclusively involving the follicular infundibulum, which shows a peau d'orange appearance, has never been reported. We describe a patient with a red plaque and peau d'orange appearance on the cheek for 20 years. Dyskeratotic acantholysis in the follicular infundibulum was overlooked when the first biopsy specimen was obtained, and a disseminated lesion developed soon after, which led to the diagnosis. Although a peau d'orange appearance is a rare clinical manifestation of PF, PF should be considered as the differential diagnosis on the basis of this appearance, especially when the lesion persists on the face for long periods.

IgA pemphigus is a rare disease which is classified into two major types: subcorneal pustular dermatosis (SPD) and intraepidermal neutrophilic IgA dermatosis (IEN). The autoantigen of the SPD type was identified as desmocollin1 (Dsc1), while the antigen of the IEN type is still unknown. We report a case of IgA pemphigus possessing antibodies exclusively against desmoglein3 (Dsg3). The patient also had ulcerative colitis, clinically severe oral mucosal lesions with pustule formation, and the "sunflower-like" pustular skin lesions. Histopathology showed intraepidermal bullae with numerous neutrophils. Direct immunofluorescence showed IgA deposits in the keratinocyte cell surfaces only in the lower epidermis. Indirect immunofluorescence using normal human skin as a substrate showed circulating IgA anti-cell surface autoantibodies at a titer of 1:160. cDNA transfection test for Dsc1-3 was negative. IgA antibodies to Dsg3, but not Dsg1, were detected with IgA-ELISA for Dsgs. Addition of diaphenylsulfone (DDS) to prednisolone and cyclosporine A significantly improved the oral and skin lesions. There have been rare cases showing IgA antibodies to either Dsg1 or Dsg3. These cases can be designated as IgA pemphigus foliaceus or IgA pemphigus vulgaris, respectively, according to the autoantigens. We report here a case of IgA pemphigus vulgaris complicated by ulcerative colitis.

Different environmental factors have been implicated in the pathogenesis of pemphigus vulgaris (PV), including drugs, diet, burns, X-rays, ultraviolet radiation, neoplasms, and infections. Several reports described the manifestation or aggravation of PV due to herpes simplex virus (HSV), varicella-zoster virus, Epstein-Barr virus, cytomegalovirus and human herpesvirus-8 infections. In the present study, we correlated secondary HSV1 infection in 3 PV patients on immunosuppressive treatment with the titers of IgG autoantibodies against desmoglein 1 (DSG1) and 3 (DSG3) over a follow-up period of at least 18 months. In these patients, the detection of HSV1 and clinical flare-up of PV did not correlate with a significant increase of DSG-specific IgG. Thus, secondary cutaneous HSV infections should be considered in patients with chronic PV with atypical sudden relapses or resistance to sufficient immunosuppressive treatment who do not show an increase of DSG-specific IgG autoantibodies.

We report a case of pemphigus vulgaris (PV) associated with Hashimoto's thyroiditis. Direct immunofluorescent studies revealed intercellular deposits of IgG and C3. Both anti-desmoglein (Dsg) 1 and Dsg 3 antibodies were positive in the patient's serum by enzyme-linked immunosorbent assays. HLA genotyping was performed from the patient's peripheral blood leukocytes by polymerase chain reaction. HLA-A*02011, -A*2402101, -DQA1*0104, -DQB1*05031 and -DRB1*1405 alleles, which confer strong susceptibility to Japanese patients with PV, were found. HLA-DQA1*0303 which is strongly associated with Hashimoto's thyroiditis in the Japanese population was also determined.

We describe a 19-year-old male patient who presented with recalcitrant erosions limited to the orolabial and genital mucosa for 18 months. The clinicopathologic diagnosis of paraneoplastic pemphigus was confirmed by indirect immunofluorescence staining of murine bladder epithelium, and the presence of IgG autoantibodies against envoplakin, periplakin, and 170 kDa protein. Enzyme-linked immunosorbent assay (ELISA) with desmoglein 3 (Dsg3) and desmoglein 1 (Dsg1) recombinant proteins showed that the patient's sera were positive against Dsg3. Castleman's tumour was found in the pelvic cavity and resected completely. The orogenital erosions disappeared slowly after the resection of Castleman's tumour with the concomitant decrease in intercellular antibody titre and index values of Dsg ELISA. Although the patient was unaware of dyspnea on exertion, a notable air flow obstruction persisted over 17 months. The expiratory images of high resolution computed tomography showed air trapping, indicating the presence of asymptomatic but gradually progressive bronchiolitis obliterans.

We report on a sixty-seven year old miner with pemphigus vulgaris characterised clinically by a three month history of relapsing oral lesions and blisters/erosions on the trunk, axillae and extremities, histologically by suprabasal cleavage due to acantholysis, immunologically by the epidermal intercellular net-like pattern due to deposits of IgG- and IgM-antibodies and complement C3 in the direct immunofluorescence as well as by serum antibodies to desmoglein 3 (130 KD) and plakoglobin (85 KD) by immunoblotting analysis. Silicosis has already been known for 6 years. In addition, antinuclear antibodies, anti-ssDNA-antibodies and anti-topoisomerase antibodies were found. Clinical improvement and clearing of skin symptoms could be achieved by systemic steroids in combination with cyclophosphamide. However, the patient died of sepsis deriving from recalcitrant pneumonia. Although the association of silicosis with various autoimmune diseases such as systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis and dermatomyositis has been reported many times, our patient is, to the best of our knowledge, the second case with features of the two diseases: pemphigus vulgaris and silicosis.

Pemphigus vulgaris is an autoimmune bullous disorder involving the skin and sometimes the mucosa. Koebner's phenomenon is encountered when the typical features of a dermatosis are observed on a part of the skin previously subject to friction or trauma. A few cases of pemphigus vulgaris developing after damage to the skin and especially scars have been described. To the best of our knowledge, we report the first case where typical lesions of pemphigus vulgaris appeared simultaneously on a recent and an old scar as the sign of the reactivation of the disease.

Several cases of psoriasis associated with bullous diseases have been reported in the literature. Bullous pemphigoid is the most frequent association observed, while pemphigus-related disorders are less frequently described. The authors report a case of a patient with long-standing psoriasis who developed pemphigus foliaceous and discuss the possible relationship between the diseases.