Ocular cicatricial pemphigoid (OCP) is a chronic bilateral, blinding, cicatrizing form of conjunctivitis with relapsing and remitting periods. It has strong evidence for an immune type II hypersensitivity that leads to subconjunctival fibrosis and extensive systemic bullae formation. To the best knowledge of the authors, this is the first reported case of direct immunofluorescence (DIF) assay-proven OCP in an elderly Filipino man.

A 68-year-old male presented with bilateral corneal conjunctivalization, symblepharon, ectropion, conjunctival hyperemia testing positive with conjunctival biopsy for basement membrane antibodies with DIF for the left eye, while turning out negative for the right eye. He was managed as a case of OCP, both eyes, and was given topical steroids and antibiotics. Oral Dapsone was started by Dermatology and Rheumatology Services.

OCP is a rare autoimmune and blinding disease. Early diagnosis and prompt treatment are vital as ocular complications permanently affect the quality of life of patients as seen in our patient. DIF assay remains the gold-standard for diagnosis. Systemic immunosuppression is the mainstay of treatment. Adjunctive supportive topical medication may be given to alleviate ocular discomfort. A multidisciplinary approach is essential to provide holistic care to each patient.

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

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 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

Immunoglobulin A (IgA) pemphigus is a rare variant of an autoimmune bullous disease with IgA antibodies. IgA pemphigus is divided into 2 major subtypes: the subcorneal pustular dermatosis (SPD) type and intraepidermal neutrophilic (IEN) dermatosis type. We documented a case of an 18-year-old woman with recurrent generalized blisters and pustules that were especially severe in the intertriginous areas. Some half-and-half blisters and coalesced pustules in an annular pattern with crusts were simultaneously observed. A biopsy specimen from one of the half-and-half blister lesions showed intraepidermal separation with multiple neutrophils. Direct immunofluorescence staining revealed lace-like intercellular deposition of IgA in the entire epidermis. IgA antibody deposits were also observed in the patient's serum. The eruptions cleared with systemic steroids and colchicine 0.6 mg for 1 week, and the patient remained in partial remission at the 8-month follow-up. Herein, we report a case of IEN-type IgA pemphigus, clinically mimicking SPD with half-and-half blisters.
Adolescent ; Antibodies ; Biopsy ; Blister ; Colchicine ; Epidermis ; Female ; Fluorescent Antibody Technique, Direct ; Follow-Up Studies ; Humans ; Immunoglobulin A ; Immunoglobulins ; Neutrophils ; Pemphigus ; Skin Diseases ; Skin Diseases, Vesiculobullous ; Steroids

Subepidermal blistering disorders (SBD) are diseases associated with antibodies that attack structural proteins of the skin. Blister formation with widespread distribution is common in these diseases. Diagnosis of SBD is established through the demonstration of immunoglobulin deposits in the dermoepidermal junction by direct immunofluorescence microscopy, and through the presence of circulating autoantibodies by serology. Systemic corticosteroids and other immunosuppressive drugs are used to treat SBD. We present the case of a 16-year old female with a 6-week history of intensely pruritic, erythematous plaques with generalized blister formation on the face, trunk, upper extremities, and inner thighs. We diagnosed the patient as having a subepidermal blistering disorder. We placed her on a course of prednisone and azathioprine, which successfully treated her lesions.
Autoimmune Diseases ; Pemphigoid, Bullous ; Epidermolysis Bullosa Acquisita ; Fluorescent Antibody Technique, Direct ; Adrenal Cortex Hormones

Subepidermal blistering disorders (SBD) are diseases associated with antibodies that attack structural proteins of the skin. Blister formation with widespread distribution is common in these diseases. Diagnosis of SBD is established through the demonstration of immunoglobulin deposits in the dermoepidermal junction by direct immunofluorescence microscopy, and through the presence of circulating autoantibodies by serology. Systemic corticosteroids and other immunosuppressive drugs are used to treat SBD. We present the case of a 16-year old female with a 6-week history of intensely pruritic, erythematous plaques with generalized blister formation on the face, trunk, upper extremities, and inner thighs. We diagnosed the patient as having a subepidermal blistering disorder. We placed her on a course of prednisone and azathioprine, which successfully treated her lesions.
Autoimmune Diseases ; Pemphigoid, Bullous ; Epidermolysis Bullosa Acquisita ; Fluorescent Antibody Technique, Direct ; Adrenal Cortex Hormones

PURPOSE: Respiratory syncytial virus (RSV) is the major cause of acute lower respiratory tract infection (LRTI) in infants and children. We investigated the association of meteorological conditions and air pollution with the prevalence of RSV infection. METHODS: Between January 2005 and December 2012, a total of 9,113 nasopharyngeal swab specimens from children under 3 years of age who were admitted to the hospital with acute LRTI were tested for RSV antigens using a direct immunofluorescence kit. Meteorological data (mean temperature, precipitation, wind speed, and relative humidity) and air pollutant levels including PM₁₀ (particulate matter with a median aerodynamic diameter less than or equal to 10 µm in diameter), nitrogen dioxide (NO₂), sulfur dioxide (SO₂), and carbon monoxide (CO) in Seoul during the study period were collected from the national monitoring system. The correlations of the monthly incidence of RSV infection with climate factors and air pollutant levels were analyzed. RESULTS: RSV infection mainly occurred between October and February, and showed the peak in November. The prevalence of RSV infection had a moderate negative correlation with mean temperature (r=−0.60, P < 0.001), a weak negative correlation with relative humidity (r=−0.26, P=0.01), and precipitation (r=−0.34, P=0.001). Regarding air pollutants, RSV activity moderately correlated with NO₂ (r=0.40, P < 0.001), SO₂ (r=0.41, P < 0.001), and CO (r=0.58, P < 0.001). In the RSV peak season in Korea (between October and February), RSV epidemics showed a weak positive correlation with relative humidity (r=0.35, P=0.03) and precipitation (r=0.38, P=0.02). CONCLUSION: Meteorological factors and air pollutant levels may be associated with RSV activity. Therefore, further nationwide large-scaled intensive evaluations to prove factors affecting RSV activity are warranted.
Air Pollutants ; Air Pollution* ; Carbon Monoxide ; Child* ; Climate* ; Fluorescent Antibody Technique, Direct ; Humans ; Humidity ; Incidence ; Infant ; Korea ; Meteorological Concepts ; Nitrogen Dioxide ; Prevalence ; Respiratory Syncytial Viruses* ; Respiratory Tract Infections ; Seasons ; Seoul ; Sulfur Dioxide ; Wind

Linear immunoglobulin (Ig) A bullous dermatosis (LABD) is a rare subepidermal autoimmune blistering disease characterized by linear IgA deposits at the basement membrane zone visualized with direct immunofluorescence (DIF). Most cases of LABD are idiopathic, but some are drug-induced with vancomycin being the most common causative agent. We herein report a patient presenting with blisters and erosive lesions, primarily in the intertriginous and flexor areas, consistent with a diagnosis of piperacillin-tazobactam-induced LABD based on the patient's clinical course and histopathology, DIF, and in vitro T-cell activation assay (TAA) findings. Only one case of piperacillin-tazobactam-induced LABD has been previously reported. In addition to its rarity, our case was also unique in that the skin lesions occurred in the intertriginous and flexor areas, uncommon locations for typical adult patients with LABD, and TAA strongly suggested an association with the causative drug.
Adult ; Basement Membrane ; Blister ; Diagnosis ; Fluorescent Antibody Technique, Direct ; Humans ; Immunoglobulin A ; Immunoglobulins ; In Vitro Techniques ; Linear IgA Bullous Dermatosis* ; Skin ; Skin Diseases ; T-Lymphocytes* ; Vancomycin

Pemphigus vulgaris is an autoimmune bullous disorder characterized by the production of autoantibodies against the intercellular space of the epithelium. It has rarely been reported in association with inflammatory bowel disease. Ulcerative colitis is one of the forms of inflammatory bowel disease. A 62-year-old woman who had been treated for ulcerative colitis for 16 years developed pruritic bullae on the skin of her face and body. Histological findings and direct immunofluorescence examination of the skin showed pemphigus vulgaris. She was treated with systemic steroids, mesalazine, and azathioprine. Her cutaneous lesions have remained in remission and her ulcerative colitis has remained well-controlled. The relationship between pemphigus vulgaris and ulcerative colitis is unclear. An autoimmune response has been suspected in the pathogenesis of ulcerative colitis. Pemphigus vulgaris is also associated with an autoimmune mechanism. To our knowledge, this is the first case of ulcerative colitis associated with pemphigus vulgaris reported in Korea. The association may be causal.
Autoantibodies ; Autoimmunity ; Azathioprine ; Colitis, Ulcerative ; Epithelium ; Extracellular Space ; Female ; Fluorescent Antibody Technique, Direct ; Humans ; Inflammatory Bowel Diseases ; Korea ; Mesalamine ; Middle Aged ; Pemphigus ; Skin ; Steroids ; Ulcer