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.

Human ; Male ; Adult: 25-44 Yrs Old ; Hepatitis B ; Pemphigus Vulgaris ; Pemphigus ; Rituximab

MMP is a rare blistering disease manifesting with painful erosions or blisters on one or more mucosal surfaces. An estimate of one to two cases per million annually are diagnosed, with females more affected than males.

We present a case of a 73-year-old Filipino female with recurrent multiple mucosal lesions involving the oral mucosa and genitals, ocular symptoms of foreign-body sensation, and erosions on her scalp.

On physical exam, there were multiple well-defined white plaques on an erythematous base on the oral mucosa and the labia minora. There were multiple well-defined erythematous alopecic patches with erosions and milia on the scalp. Histopathology revealed subepidermal split, while DIF findings were consistent with the pemphigoid group. ELISA showed increased anti-BP180, the most common target antigen for MMP. The patient was managed as a case of MMP. Prednisone 25 mg/day was given, which improved her lesions and decreased her MMPDAI score from 44 (moderate) to 10 (mild). She is currently maintained on azathioprine 50 mg/tablet.

Mucous membrane pemphigoid, being rare, is often overlooked as a differential diagnosis. When presented with a patient with predominantly mucosal lesions, it is important to consider MMP to prevent consequences from delayed diagnosis.

Human ; Female ; Aged: 65-79 Yrs Old

Acute generalized exanthematous pustulosis (AGEP) and Stevens-Johnson Syndrome (SJS) are uncommon, severe cutaneous drug eruptions with distinct clinical and histopathological features. AGEP-SJS overlap is a rare and complicated cutaneous drug eruption. Neutrophilia, leukocytosis, and elevated liver enzymes can be seen in these patients. Currently, there are no available dermoscopic studies on AGEP overlapping SJS. The pathophysiology of overlapping drug reaction are mediated by T cells and delayed-type hypersensitivity. Management includes removal of offending drug and giving supportive measures like pain management, moist dressing and fluids.

Human ; Female ; Middle Aged: 45-64 Yrs Old ; Acute Generalized Exanthematous Pustulosis ; Stevens-johnson Syndrome

Introduction: Zinc deficiency is of high magnitude in developing countries such as the Philippines. Zinc deficiency dermatitis is recognized through characteristic cutaneous presentation supported by diagnostic workups which may not be feasible or practical in low-resource settings. Case report: A three-month-old Filipino male was brought in for erosions of three (3) weeks duration that were unrespon- sive to topical and systemic antimicrobial treatment. On examination, he had multiple erythematous erosions with yellowish to brownish, crusted borders with predilection on the face, inguinal and gluteal areas, flexures of the extremities, and digits. Workup revealed normal zinc levels, decreased alkaline phosphatase, and bacterial growth in cultures. Histopathology revealed intraepidermal vesiculobullous dermatitis. Given the clinicopathologic presentation, a diagnosis of zinc deficiency-associated dermatitis was made. Along with antimicrobials and topical care, oral zinc sulfate with elemental zinc at 3 mg/kg/day was started, with remarkable improvement within three (3) days and near-resolution after eight (8) days of zinc therapy. Zinc supplementation was administered for three (3) months with gradual tapering. The skin remained clear despite the withdrawal of zinc supplemen- tation. Response to treatment supported the impression of zinc deficiency, while sustained skin clearance upon withdrawal verified an acquired etiology. Conclusion Zinc deficiency-associated dermatitis is more common in areas where costly diagnostic modalities are not readily available. In clinically suspected zinc deficiency, response to treatment can serve as a retrospective diagnostic feature, and sus- tained clearance upon withdrawal may aid in identifying etiology. Trial of therapy may then be considered in optimizing the cost-ef- fective management of zinc deficiency-associated dermatitis.
Malnutrition