An unusual clinical form of pemphigus vulgaris]

A 36-year-old woman developed bullous skin lesions which clinically seemed most likely to be dermatitis herpetiformis or bullous pemphigoid. However, histological examinations as well as immunofluorescence and electron microscopic observations substantiated the diagnosis of pemphigus vulgaris.     

Macroglossia. An unusual presentation of pemphigus vulgaris

A 19-year-old woman presented with macroglossia of five months' duration and without bullae or erosions. A biopsy revealed a picture consistent with pemphigus vulgaris. The patient subsequently developed typical oral erosions. Her tongue enlargement and oral ulcerations improved dramatically with topical and systemic steroid therapy. Pemphigus vulgaris should be considered in the differential diagnosis of macroglossia.     

An unusual case of pemphigus vulgaris presenting as bilateral foot ulcers

We describe an unusual presentation of pemphigus vulgaris, an autoimmune intraepidermal blistering skin disease associated with autoantibodies to the desmosome glycoprotein, desmoglein 3. A 60-year-old man presented with bilateral ulceration on the dorsum of the feet. These clinical features persisted for 4 months before more characteristic signs of pemphigus vulgaris, including mouth ulceration and skin erosions, developed. The atypical presentation led to a delay in diagnosis and initiation of the appropriate treatment. Pemphigus vulgaris may have unusual manifestations, such as nail dystrophy, paronychia, or granulation tissue-like lesions, but this case of bilateral foot ulceration highlights a further, perhaps unique, clinical presentation of this autoimmune disease.     

Brain abscesses with pemphigus vulgaris: a rare association

Pemphigus is a rare group of skin disorders characterized by blistering because of loosening of desmosomal links between epidermal cells attributed to immunological attack. Pemphigus vulgaris is characterized by the presence of the split within the epidermis just above the basal layers (suprabasal). Central nervous system complications of the disorder have scantily been described in the English literature. We report what we believe to be the first case of cerebral abscesses in a 38-year-old male associated with pemphigus vulgaris and immunosuppressive therapy.     

A case of pemphigus vulgaris in association with Addisonian pernicious anaemia

A case is reported in which the diagnosis of pemphigus vulgaris was followed 4 months later by that of Addisonian pernicious anaemia. The lack of case reports of the co-existence of pemphigus vulgaris and other autoimmune diseases, with the exception of myasthenia gravis, is discussed.     

An unusual form of pemphigus vegetans

Numerous vegetating plaques developed in a 63-year-old man followed up for the management of pemphigus vulgaris of 5 years' duration. These lesions occurred over the trunk and right upper arm with no involvement in the intertriginous area, and were controlled with systemic steroid and ODT. Direct immunofluorescence test showed heavy deposition of fibrinogen in the papillary dermis. To our knowledge, the occurrence of pemphigus vegetans in the non-intertriginous area is rare.     

Neonatal pemphigus vulgaris with extensive mucocutaneous lesions from a mother with oral pemphigus vulgaris

The clinical phenotype of pemphigus is well explained by the combination of desmoglein (Dsg) 1 and Dsg3 distribution pattern and antiDsg autoantibody profile (Dsg compensation theory). It has been reported that neonatal skin has a similar Dsg distribution pattern to adult mucosal epithelia. We describe a newborn girl with mucocutaneous pemphigus vulgaris (PV) from a mother with mucosal dominant PV. The mother had had painful oral erosions for at least 7 months. Histopathological examination and direct and indirect immunofluorescence studies confirmed the diagnosis of PV and neonatal PV in the mother and daughter, respectively. The mother had a high titre of anti-Dsg3 IgG and a low titre of antiDsg1 IgG, while the neonate had only a high titre of anti-Dsg3 IgG, but no detectable antiDsg1 IgG. AntiDsg3 IgG, which caused the oral dominant phenotype in the mother, induced extensive oral as well as cutaneous lesions in the neonate. Our case provides clinical evidence for the Dsg compensation theory in neonatal PV.     

Evidence of an association between desmoglein 3 haplotypes and pemphigus vulgaris

BACKGROUND: Pemphigus vulgaris (PV, OMIM 169610) is a severe blistering disorder of the skin and mucous membranes, caused by the production of autoantibodies directed against the epithelial adhesive protein desmoglein 3. Although an association between PV and HLA class II alleles has been established, the genetic factors predisposing to the disease remain poorly understood, the rarity of PV hampering the recruitment of substantial patient cohorts. OBJECTIVES: To investigate DSG3 as a candidate PV susceptibility gene. METHODS: We examined five DSG3 single nucleotide polymorphisms (rs8085532, rs3911655, rs3848485, rs3794925 and rs1466379) in two case-control datasets respectively originating from the U.K. (62 PV patients, 154 controls) and northern India (28 patients, 98 controls). RESULTS: In the U.K. sample, we observed a significant association between PV and the DSG3*TCCTC haplotype (Fisher's exact test P = 0.002). A related haplotype (DSG3*TCCCC) was associated with PV in the Indian dataset (P = 0.002). We also found that all British and Indian patients bearing DSG3 risk haplotypes carried at least one copy of a PV-associated HLA allele. CONCLUSIONS: These results suggest that genetic variation of DSG3 may be an additive risk factor predisposing to PV and warrant further investigations of this gene.     

Treatment of pemphigus vulgaris and pemphigus foliaceus with mycophenolate mofetil

BACKGROUND: Mycophenolate mofetil is increasingly being used as a corticosteroid-sparing agent in immunosuppressive regimens. OBJECTIVE: To elucidate the effectiveness of mycophenolate as adjuvant therapy in the treatment of both pemphigus vulgaris and pemphigus foliaceus. DESIGN: Historical prospective study. SETTING: University hospital. PATIENTS: The study included 42 consecutive patients with pemphigus (31 with pemphigus vulgaris and 11 with pemphigus foliaceus) who had relapses during prednisone taper or had clinically significant adverse effects from previous drug therapy. RESULTS: Remission was achieved in 22 (71%) and 5 (45%) of patients with pemphigus vulgaris and pemphigus foliaceus, respectively. Partial remission was achieved in 1 (3%) and 4 (36%), respectively. The median time to achieve complete remission was 9 months (range, 1-13 months). The treatment was administered for a median of 22 months, and the median follow-up period was 22 months. Seventy-seven percent of patients had no adverse effect. Two patients had side effects severe enough to necessitate discontinuation of treatment, one because of symptomatic but reversible neutropenia and the other because of nausea. CONCLUSION: Mycophenolate is an effective and safe adjuvant in the treatment of both pemphigus vulgaris and pemphigus foliaceus.     

Neonatal pemphigus vulgaris associated with mild oral pemphigus vulgaris in the mother during pregnancy

We report a neonate with immunofluorescence-proven pemphigus vulgaris. The condition presented at birth with widespread skin erosions and ulceration of the oral mucosa. Histopathological and immunofluorescence studies confirmed pemphigus vulgaris. The mother had mild oral pemphigus vulgaris treated during pregnancy with topical corticosteroids. All the neonate's skin erosions had crust formation at day 2 but healed completely within 2 weeks.     

Neonatal pemphigus vulgaris

A male newborn with skin erosions was born to a 32-year-old woman who was under treatment for pemphigus vulgaris that had been diagnosed 16 months earlier. Antibodies to desmoglein (Dsg)1 and Dsg3 were analyzed by enzyme-linked immunosorbent assay. Index values of antibodies to Dsg1 and Dsg3 were 49 (normal index values, <14) and 121 (normal index values, <7), respectively. Those findings concluded a diagnosis of neonatal pemphigus vulgaris. No new vesicles or bullae appeared in the newborn after the birth. Non-corticosteroid ointments produced prompt epithelialization on the erosive lesions. All the eruptions disappeared in 3 weeks. The level of serum anti-Dsg3 autoantibodies when measured at the 76th day was negative (<5).