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.

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.

One hundred and four biopsy specimens of various dermatoses were stained with Periodic Acid-Schiff and examined. The lesions were grouped into five groups; A. twenty two cases of vesicob-ullous diseases; B. twenty one cases of maculopapulosquamous diseases; C. eighteen cases of chronic granulomatous infection; D. seventeen cases of precancerosis and benign and malignant neoplasms; and E. twenty six cases of miscellaneous skin diseases. Special attention was given to the morph-ological changes of the dermoepidermal junction and the basement membrane. The results were as follows. 1. Among 10 cases of erythema multiforme, partial fraying appeared in 4 cases, partial thicken-ing in 4 and hyperchromasia in 4. Marked convolution was noted in the thickened basement membrane. PAS-positive material was scattered in the edemateus papillary dermis in some cases. 2. All of 6 cases of pemphigus erythematosus and pemphigus vulgaris showed normal basement membrane. 3, In bullous pemphigoid, partial absence in the areas of bulla formation appeared in 2 cases am-ong 3 cases. PAS positive material was present within the bullae and in the upper dermis. 4. All of 3 cases of dermatitis herpetiformis showed partial absence or fraying, 5. Half of 10 cases of psoriasis showed normal basement membrane. Among the remainder, fraying and partial thickening appeared in 3 and 2 cases respectively. 6. Among 6 cases of lichen planus, almsot total absence appeared in 2 cases and fraying in 3 cases. PAS positive material appeared focally in the upper dermis in some cases. 7. Four of 5 cases of pityriasis rubra pilaris showed diffuse thickening and marked convolution ef the basement membrane. 8. Half of 4 cases of lupus vulgaris showed normal basement membrane. Fraying and partial thickening appeared in 2 and 2 cases respectively. 9. All but 1 among 5 cases of lepromatous leprosy showed poor staining and poor visualization of the basement membrane. 10. One case of tuberculoid leprosy showed partial fraying, partial thickening and hyperchromasia with marked convolution, among 5 cases. il. Among 4 cases of condyloma latum, only one showed partial fraying. 12. One of 3 cases of senile keratosis showed partial fraying of the basemement membrane. 13. One case of Bowens disease showed partial thickening and hyperchromasia among 3 cases. PAS-positive material was present focally in the upper dermis in some cases. 14. All of 6 cases of basal cell carcinoma, showed normal basement membrane. PAS-positive ma-terial were present focally in the uppermoet dermis in some vases. 15. All of 3 cases of squamous cell carcinoma showed total absence of the basement membrane. 16. All of the 2 cases of keratoacanthoma showed partial fraying. 17. Among 10 cases of chronic discoid lupus erythematosus, 9 cases showed partial thickening and hyperchromasia with marked convolution of the basement membrane. Two cases showed partial fraying in areas of marked liquefaction degeneration of the basal cells. PAS positive material appe-ared in the uppermost dermis in some cases. 18. All of 3 cases of fixed drug eruption, 2 cases of seborrheic keratosis and 6 cases of verrucae showed normal basement membrane. 19. One case of poikiloderma atrophicans vasculare showed partial fraying, among 2 cases. 20. All of 2 cases of chronic radiodermatitis showed diffuse thickening and hyperchramasia. The thickened basement membrane showed marked convolution focally.
Basement Membrane* ; Biopsy ; Bowen's Disease ; Carcinoma, Basal Cell ; Carcinoma, Squamous Cell ; Dermatitis Herpetiformis ; Dermis ; Drug Eruptions ; Erythema Multiforme ; Keratoacanthoma ; Keratosis ; Keratosis, Seborrheic ; Leprosy, Lepromatous ; Leprosy, Tuberculoid ; Lichen Planus ; Lupus Erythematosus, Discoid ; Lupus Vulgaris ; Membranes ; Pemphigoid, Bullous ; Pemphigus ; Pityriasis Rubra Pilaris ; Psoriasis ; Radiodermatitis ; Skin Diseases ; Warts

No abstract available.
Pemphigus*

No abstract available.
Pemphigus

No abstract available.
Pemphigus

No abstract available.
Pemphigus

Pemphigus foliaceus (PF) is a chronic, benign, acquired autoimmune blistering disease characterized by fragile, superficial blisters and bullae.1 2 3 Patients commonly report blister formation on the skin, followed by localized lesions that typically begin on the trunk, face, or scalp.2 PF is a rare disease with a very low worldwide incidence and prevalence. However, it has a high incidence in endemic areas located in North Africa, Brazil, Colombia, and Peru.2 3 4 The disease affects both sexes equally, with symptoms typically appearing between the ages of 50 and 60 years.2 4 The universal type of PF that occurs sporadically is idiopathic, while the endemic type is linked exclusively to geographically-related environmental factors.
Pemphigus

No abstract available.
Pemphigus* ; Psoriasis*

No abstract available.
Pemphigus* ; Pregnancy*