Introduction: Epidermolysis Bullosa (EB) is a rare genodermatosis characterized by fragility of the skin and mucous membranes, manifested by blistering with little or no trauma. There are three subtypes: EB Simplex, Junctional EB, and Dystrophic EB. Each type of EB has its own specific genetic defect. We report a case of a 13-year-old girl who presented with multiple tense blisters and eroded plaques since birth on the entire body. Case summary: This is a 13-year-old-girl who presented with solitary tense blister on her right thigh three days after birth, which gradually affected the scalp, trunk, and upper and lower extremities, particularly on the trauma prone areas. There was nail dystrophy and multiple brownish dental pits at three years of age. A 4 mm lesional skin punch biopsy showed subepidermal blisters containing fibrin, lymphocytes and few red blood cells. PAS showed basement membrane zone beneath the blister, compatible with EB. Immunofluorescence mapping showed decreased immunofluorescence (+1) on keratin 5/6, (+2) on keratin 14, and absence of immunofluorescence on alpha 6 / beta 4 integrins. Final diagnosis is EB Simplex. Conclusion Early detection is important in managing this case, to detect systemic involvement and provide palliative care. Genetic counseling is recommended for prospective parents who have a family history of any form of epidermolysis bullosa. The prognosis of Inherited EB is very variable and the mortality is usually due to complications of systemic involvement. A multidisciplinary approach in the supportive management of this case is necessary as there is still no cure for this condition.
Fluorescent Antibody Technique ; Integrins

No abstract available.
Chlamydia* ; Fluorescent Antibody Technique, Indirect*

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

No abstract available.
Child* ; Cytomegalovirus* ; Fluorescent Antibody Technique* ; Humans ; Prevalence*

BACKGROUND: Behcet's disease has features consistent with an immunopathogenic mechanism, but the autoreactivity in pathogenesis is unclear. OBJECTIVE: This study was to investigate the association of antinuclear antibodies (ANA) with Behcet's disease. METHODS: The patients in this study were diagnosed at Severance Hospital Behcet's Disease Specialty Clinic from May, 1998 to May, 2002. We evaluated the frequency, titers and immunofluorescence patterns of ANA in patients with Behcet's disease, and compared the frequency with a healthy control group. According to the positivity of ANA, we compared the frequency of minor symptoms to investigate the association of the severity of disease with ANA. RESULTS: 1. Of the 554 cases of Behcet's disease, 46 cases (8.3%) were ANA positive, however, of the 271 cases of healthy control group, only 5 cases (1.8%) were ANA positive. (p=0.0003) 2. In ANA titers 38 cases (82.6%) showed low titer (1: 40+, 1: 160-), 5 cases (10.9%) intermediate titer (1: 160+, 1: 640-), and 3 cases high titer (>1: 640+). There was no significant difference in intermediate and high titers between complete (17.9%) and incomplete type (14.3%). 3. In immunofluorescence patterns of ANA, 17 cases (37%) were speckled pattern, 5 cases (10.9%) homogeneous pattern, 3 cases (6.5%) centromere pattern, 2 cases (4.3%) nucleolar pattern and 19 cases (41.3%) unknown pattern. 4. Of 508 cases with negative ANA patients, 272 cases (53.5%) had minor symptoms, however, of 46 cases with positive ANA patients, 14 cases (30.4%) had minor symptoms (p=0.0027). CONCLUSION: From this study ANA was more prevalent in Behcet's disease. However, it was not related to severity of disease and most of them were low titer. ANA, herein, might play a minor role in pathogenesis of Behcet's disease.
Antibodies, Antinuclear* ; Centromere ; Fluorescent Antibody Technique ; Humans

No abstract available.
Adult* ; Cytomegalovirus* ; Fluorescent Antibody Technique* ; Humans*

No abstract available.
Adult* ; Cytomegalovirus* ; Fluorescent Antibody Technique* ; Humans*

21 patients aged 2-76 years old (7 males, 14 females) with blistering dermatosis were diagnosed and treated in HCM city- dermatovenerology Hospital from May 1996 to May 1997- control group included subjects without blister. Primary diagnosis, routine tests, biopsy of blister, patho-anotomical examination and direct immunofluorescence assays, antibodies were conducted on the skin areas near by the blisters. Results showed that by direct IF assays, antibodies were detects as immunoflobulines and complements adhered on the skin, which got diagnostic values, to help to defimite and to differenciate in blistering dermatoses, dpecially in difficult cases.
Diagnosis ; Skin Diseases ; Fluorescent Antibody Technique

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