Pemphigus vulgaris is an autoimmune bullous dermatosis presenting with flaccid blisters and erosions. The morphology of pemphigus reflects the more superficial intraepidermal level of split seen histologically in contrast with pemphigoid, where the level of split is deep below the epidermis. This is a case of a 58-year-old male clinically presenting with arcuate tense bullae, which are more characteristic of the pemphigoid group of disorders, which revealed an intraepidermal split and tombstoning pattern of the basal epidermis on histopathology. Direct immunofluorescence revealed intercellular IgG and C3 distribution. Although this patient presented clinically with tense bullae, the histopathology and direct immunofluorescence results were consistent with pemphigus vulgaris.
Pemphigus ; Fluorescent Antibody Technique, Direct

Hailey-Hailey disease (HHD) is an uncommon acantholytic disorder of the skin. This is a case of a 64-year-old Filipino female with a chronic history of painful and malodorous intertriginous plaques. Histopathologic evaluation showed overlapping features of pemphigus vulgaris and Hailey-Hailey disease. A negative direct immunofluorescence test clinched the diagnosis of Hailey-Hailey disease. The patient was advised regarding preventive measures and treated with topical antibiotics and corticosteroids with improvement of her lesions.
Pemphigus, Benign Familial ; Fluorescent Antibody Technique, Direct

Pemphigus foliaceous is a rare autoimmune blistering disease, while psoriasis is a common immune‑mediated inflammatory skin disease. The coexistence of psoriasis and pemphigus foliaceous has rarely been reported. We report a case of a 55‑year‑old Filipino female with an 8‑year history of chronic plaque‑type psoriasis biopsy‑proven. After 5 years, she developed generalized flaccid bullae and crusted erosions over the face, trunk, and extremities, with no mucous membrane involvement. Skin punch biopsy, direct immunofluorescence, and enzyme‑linked immunosorbent assay were consistent with pemphigus foliaceous. The combination of topical corticosteroids and oral methotrexate was selected as the therapeutic approach, leading to a notable improvement in the patient’s condition. This case report underscores the significance of identifying the simultaneous presence of psoriasis alongside autoimmune blistering diseases like pemphigus foliaceous. Examining predisposing and triggering factors, performing re‑biopsy, and further work‑up as the disease evolves may yield more profound insights. Nonetheless, effectively managing this condition poses a significant challenge.
Fluorescent Antibody Technique, Direct ; Methotrexate ; Psoriasis

Desquamative gingivitis is characterized by a diffuse erythema of the free and attached gingiva associated with areas of vesiculation, erosion, and desquamation. Desquamative gingivitis is not a distinct disease entity but represents a reaction pattern of the gingiva to various stimuli. Pemphigus vulgaris, cicatricial pemphigoid, and lichen planus may presents as desquamative gingivitis. We observed 3 patients whose disease was limited to the gingiva, and studied them by light and direct immunofluorescence microscope. We classified them according to clinical, histologic, and immunopathologic observations. Identification of the underlying causes of desquamative gingivitis is of utmost importance and is dependent upon clinical, histologic, and immunologic criteria.
Diagnosis, Differential ; Erythema ; Fluorescent Antibody Technique ; Fluorescent Antibody Technique, Direct ; Gingiva ; Gingivitis* ; Humans ; Lichen Planus ; Pemphigoid, Benign Mucous Membrane ; Pemphigus

We present a case of pemphigus vulgaris developed during pregnancy. The clinical features of this patient were similar to those of hetpes gestationis. By immunofluorescence studies we found that the patient's serum contained autoantibodies to intercellular substance of epidermis. Direct immunofluorescence microscopy of a perilesional skin also demontrated immunoglobulin deposition in the intercellular areas. Additionally we did in vitro C3 staining for further confirmation of the diagnosis but it was negative in basement membrane zone. This patient was initially treated with corticosteroid and azathioprine. Several months later gold compound was given instead of azathioprine and corticosteroid with good therapeutic response.
Autoantibodies ; Azathioprine ; Basement Membrane ; Diagnosis ; Epidermis ; Fluorescent Antibody Technique ; Fluorescent Antibody Technique, Direct ; Humans ; Immunoglobulins ; Microscopy ; Pemphigus* ; Pregnancy* ; Skin

BACKGROUND: The diagnosis of bullous pemphigoid is made based on clinical, histologic, and immunofluorescence features. OBJECTIVE: The purpose of this study was to analyze the factors that may affect the positivity and intensity of direct immunofluorescence (DIF) in patients with bullous pemphigoid. METHODS: We performed a retrospective review of 41 cases of bullous pemphigoid at Ilsan Paik Hospital between January 2008 and December 2014. We investigated the positivity of DIF, immunofluorescence intensity of C3, age, sex, biopsy sites, extent of disease, duration of disease, and the degree of inflammatory cell infiltration. RESULTS: Twenty-seven of 41 (65.9%) cases had positive DIF results for either IgG or C3, and 14 of 41 (34.1%) had negative DIF results for both IgG and C3. Twenty-one cases (51.2%) of IgG and 25 cases (61.0%) of C3 had characteristic linear C3 deposition on the dermo-epidermal junction. Disease duration influenced DIF positivity (p<0.05). Although a higher positive rate of DIF was observed in biopsy specimens taken from the upper extremities than in those from other sites, the difference was not statistically significant. Sex, age, extent of disease, and the degree of inflammatory cell infiltration were not significantly associated with the positivity of DIF. There was no relationship between fluorescence intensity of C3 and the degree of inflammatory cell infiltration. CONCLUSION: This study suggests that the long duration of disease (more than 10 days) may increase the positivity of DIF. Age, sex, biopsy site, extent of disease, and the degree of inflammatory cell infiltration had no influence on DIF positivity.
Biopsy ; Diagnosis ; Fluorescence ; Fluorescent Antibody Technique ; Fluorescent Antibody Technique, Direct* ; Humans ; Immunoglobulin G ; Pemphigoid, Bullous* ; Retrospective Studies ; Upper Extremity

Linear IgA bullous dermatosis (LABD) is an autoimmune subepidermal blistering disease characterized on direct immunofluorescence of perilesional skin by the linear deposition of IgA at the basement membrane zone. The pathogenesis of LABD is not known, but rar ely some cases of LABD have been reported in association with drug exposure. We report a case of drug-induced LABD showing linear deposits of IgA and IgG at the basement membrane zone on direct immunofluorescence of perilesional skin as well as typical clinical and histologic characteristics, although the causative agent could not be specified.
Basement Membrane ; Blister ; Fluorescent Antibody Technique, Direct ; Immunoglobulin A ; Immunoglobulin G ; Linear IgA Bullous Dermatosis* ; Skin

Although psoriasis and bullous diseases are considered to be completely different disease entities, the literature has reported a few cases of psoriasis associated with bullous diseases, most of which are bullous pemphigoid. In limited cases, pemphigus foliaceus has also been reported in association with psoriasis. In most of them, pemphigus lesions usually developed on an untreated patient with a chronic history of psoriasis. Herein, we report a case of 53-year-old male with a chronic history of psoriasis who first developed generalized erosive lesions after 26 cycles of narrow-band ultraviolet B (NBUVB) therapy. A diagnosis of pemphigus foliaceus was made based on skin biopsy and direct immunofluorescence assay. Pemphigus lesions were well controlled with combination therapy of oral steroid and azathioprine. This is the first case where pemphigus foliaceus co-occurred with psoriasis during NBUVB therapy.
Azathioprine ; Biopsy ; Fluorescent Antibody Technique, Direct ; Humans ; Male ; Middle Aged ; Pemphigoid, Bullous ; Pemphigus ; Psoriasis ; Skin

BACKGROUND: Total T lymphocytes can be measured by CD3-fluorescein isothiocyanate (FITC)/CD4-phycoerythrin (PE) and CD3-FITC/CD8-PE. The difference in the CD3 percentages between these two determinations was evaluated. And, we characterized the CD3(+)CD4(-)CD8(-) T lymphocytes subset using the monoclonal antibody that detects gamma delta T lymphocytes receptors. METHODS: The T lymphocyte subset assay was performed on 221 samples. A two-color direct immunofluorescence flow cytometric assay was done using a Simultest IMK-Lymphocyte kit (Bec-ton- Dickinson, San Jose, CA, USA). If the difference between the CD3 determinations were greater than 3%, the entire procedure was reviewed and the flow cytograms were reanalyzed. In 71 among 221 samples the proportion of gamma delta T lymphocytes was determined. RESULTS: The difference between the CD3-FITC/CD4-PE tube and CD3-FITC/CD8-PE tube was 3.0%, 3.6%, 3.0%, 3.4%, and 2.4% in normal subjects, patients with chronic liver disease, patients with cancer, patients with other diseases, and children, respectively. The between-tube differences for CD3 exceeding 3% were found in 69 samples (31.2%). The proportion of gamma delta T lymphocytes was 0.81%, 2.46%, 2.50%, and 0.85% in normal controls, patients with chronic liver disease, patients with cancer and patients with other diseases, respectively. No correlation between gamma delta T lymphocytes and CD3(+)CD4(-)CD8(-) T lymphocytes was observed. CONCLUSIONS: The reproducibility of the total T lymphocytes should be improved because of the between-tube difference exceeding 3% in about one third of the cases. Additionally, CD3(+)CD4(-)CD8(-) T lymphocytes were composed of heterogeneous subsets including gamma delta T lymphocytes and their proportion might be considered to be related to individual variation.
Child ; Fluorescent Antibody Technique, Direct ; Humans ; Liver Diseases ; Lymphocyte Subsets* ; Lymphocytes* ; T-Lymphocytes

Majocchi's disease is one of chronic vascular disorders of the skin, histologically showing capillaritis with lymphoid cell infiltration. Although the hypothesis of delayed type hypersensitivity reaction against foreign antigen is proposed as the mechanism producing this condition, there are much yet to be defined. We tried to examine the lesional skin of five patients with Majocchi's disease by the direct immunofluorescence with anti-human immunoglobulins, C 3, and fibrin. As a result we found the deposition of immunoglobulins (M, and G) in papillary blood vessels in four patients, and C 3 and fibrin were found in all five patients. C3 was also found at the dermoepidermal junction in three patients. These findings suggest the posssibility that antibody mediated immunologic processes may alsobe involved in the pathogenesis of this disorder. With this observation Majocchis disease could be considered as a chronic immune vasculitis.
Blood Vessels ; Fibrin ; Fluorescent Antibody Technique, Direct ; Humans ; Hypersensitivity ; Immunoglobulins ; Lymphocytes ; Skin ; Vasculitis