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.

Human ; Male ; Child: 6-12 Yrs Old ; Linear Iga Bullous Dermatosis ; Immunoglobulin A ; Skin Diseases

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.

BACKGROUND

Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are rare, chronic autoimmune blistering diseases primarily treated with systemic glucocorticoids. Long-term use of glucocorticoids can result in significant adverse effects, leading to the exploration of steroid-sparing adjuvants. This meta-analysis aimed to evaluate the efficacy and safety of glucocorticoids alone versus in combination with adjuvants for treating PV and PF.

The main objective of this study was to compare the efficacy and safety of oral glucocorticoids alone versus with steroid-sparing adjuvants in treatment of PV and PF.

This research was a systematic review and quantitative meta-analysis of randomized controlled trials (RCTs) that compared systemic glucocorticoids with or without steroid-sparing adjuvants in treating PV and PF. A total of 20 RCTs were included after meeting the inclusion criteria, comprising 1169 patients diagnosed with PV or PF. Primary outcomes were remission rates, while secondary outcomes included relapse rates, disease control, and the occurrence of adverse events.

Studies involving rituximab and cyclophosphamide demonstrated significantly higher remission rates compared to other adjuvants or glucocorticoid monotherapy. Adverse events were common, particularly with high-dose glucocorticoids.

The meta-analysis found that rituximab and cyclophosphamide were superior steroid-sparing adjuvants in the treatment of PV and PF. Other adjuvants, such as azathioprine and mycophenolate mofetil, also showed efficacy but had a higher incidence of adverse events. Further studies were needed to optimize treatment protocols and reduce long-term complications.

Hailey-Hailey Disease (HHD) is a rare, chronic skin condition characterized by relapsing episodes and spontaneous remissions, significantly affecting patients’ quality of life, especially in severe cases. Due to its rarity, there are no established evidence-based treatment guidelines or extensive clinical trials. This case report highlights the rapid improvement of HHD in a 55-year-old Filipino woman treated with a combination of 5-fluorouracil and excimer phototherapy, suggesting that this treatment approach may be an effective alternative for managing the disease.

Junctional Epidermolysis Bullosa (JEB) is a rare inherited blistering disorder characterized by extreme skin fragility. It is associated with COL17A1 mutation that is critical for dermoepidermal adhesion. Effective management in resource-limited settings is challenging and focuses on wound care and trauma prevention to prevent complications and improve quality of life.

A 27-year-old Filipino female presented with recurrent blisters and erosions since infancy, accompanied by diffuse alopecia and anonychia of the toenails. On physical examination, she presented with multiple, well-defined, erythematous tense vesicles and bullae on the trunk, upper and lower extremities which were triggered by minor trauma to the skin. Skin punch biopsy was done revealing a subepidermal blistering dermatosis while direct immunofluorescence (DIF) and Enzyme-Linked Immunosorbent Assay (ELISA) tests for BP180, BP230, and type VII collagen were negative. Genetic testing was done and revealed compound heterozygous mutations in the COL17A1 gene, consistent with JEB. She was managed with non-adhesive dressings, sterile aspiration of blisters, and nutritional support. Despite the severity of her condition, the patient experienced significant improvement in wound healing and quality of life through meticulous wound care, trauma prevention and nutritional support.

This case highlights the unusual case of a 27-year-old Filipino female with JEB who has survived into adulthood, a rarity given the typically poor prognosis associated with severe forms of the disease.

Pemphigus is a rare, chronic, life-threatening autoimmune blistering disease characterized by blisters and erosions of the skin and mucous membranes. Rituximab has been approved as a first-line treatment for moderate to severe pemphigus vulgaris. Despite of its efficacy in achieving remission, Rituximab can also lead to serious complications such as Hepatitis B reactivation.

We present a case of a 42-year-old Filipino male with severe pemphigus vulgaris on chronic immunosuppressive therapy. He had a 10-month history of multiple bullae and crusted erosions associated with pruritus and burning pain on the mouth resulting to odynophagia and dysphagia. He is a known case of chronic Hepatitis B with unrecalled vaccination status. On physical examination, Nikolsky and Asboe-Hansen signs were positive. Histopathology show intraepidermal split and row of suprabasal keratinocytes pattern. ELISA showed very high levels (>200 RU/ml) for both anti-desmoglein 1 & 3. DIF was positive for IgG & C3. Prior to Rituximab administration, Tenofovir 300 mg/tab/day was started as pre-emptive therapy. To lessen the dependence on systemic corticosteroids, two infusions of Rituximab 1g 2 weeks apart were then given. Notable improvement was seen, evidenced by absence of new bullae, reduction of affected BSA, from 19% to 5.3% and decreased PDAI (78 to 1) and ABSIS (46.5 to 2.75) four months after treatment. Maintained remission and undetectable Hepatitis B viral load 4 months following the last dose of Rituximab were noted, indicating a positive treatment response to both Rituximab and Tenofovir.

Rituximab represents a viable treatment option even for patients with chronic Hepatitis B. Pre-emptive therapy may be done prior to infusion to prevent hepatitis reactivation. Clinical evidence supports the efficacy and safety of Rituximab in this case where preventive measures are taken.

Bullous pemphigoid (BP) is a severe autoimmune blistering condition that characteristically presents as large tense bullae on a background of normal or erythematous skin. In atypical cases, presentation is widely variegated ranging from vesicular eruptions to erythrodermic presentations. This case highlights on a rare presentation of BP manifesting with varicella-like eruption. The recognition and early diagnosis of a unique presentation of this condition is of great importance as it may greatly influence the patient’s outcome and prognosis.

Toxic epidermal necrolysis (TEN) is a rare, life-threatening dermatological emergency characterized by extensive epidermal necrosis and detachment, along with significant mucous membrane erosions and systemic symptoms. While conventional medications such as sulfonamides and anticonvulsants are known triggers, recent reports suggest that herbal medications can also induce TEN. This case report highlights the development of TEN in a patient with invasive cervical cancer following the use of an herbal supplement.

A 48-year-old Filipino woman with newly diagnosed invasive cervical cancer developed TEN after taking an herbal supplement containing 23 botanical extracts. Despite discontinuing the supplement, her condition rapidly worsened, with vesicles and bullae progressing to epidermal detachment over 90% of her body surface area. A histopathological examination confirmed the diagnosis of TEN. The patient was treated with systemic corticosteroids and supportive care, achieving full recovery within 14 days.

This case underscores the potential risks associated with herbal medications, particularly in vulnerable patients such as those with cancer. Although conventional pharmaceuticals are the primary culprits for TEN, this report highlights the need for greater awareness of herbal medicine-induced adverse drug reactions. Underreporting of such events, due to lack of awareness among patients and healthcare providers, further complicates the issue. This case emphasizes the need for strengthened regulatory policies and enhanced education to safeguard public health amidst the increasing use of herbal remedies worldwide.

Bullous pemphigoid (BP) is an autoimmune blistering disorder characterized by tense vesicles and bullae, primarily affecting the elderly. It results from autoantibodies targeting hemidesmosomal proteins BP180 and BP230, leading to subepidermal blistering. BP often presents with widespread pruritic plaques and blisters on flexural surfaces, and mucosal involvement is rare. While BP typically occurs spontaneously, certain medications, such as linagliptin, have been implicated as triggers. Early diagnosis and treatment with systemic corticosteroids or immunosuppressive agents can significantly reduce morbidity.