Aster CMI hospital Hebbal, Bengaluru, Karnataka,560092.
Pemphigus (from the Greek "Pemphix" meaning blister) foliaceous is an auto immune illnesses in which autoantibodies attack certain hemidesmosome proteins, resulting in blisters on the skin and mucous membranes. Whereas the epidemiology of pemphigus varies by ethnicity and there are 21.7 cases per million people worldwide each year. A 57-year-old male patient presented with a six-month history of pruritic, erythematous plaques and crusted erosions on the scalp, face, chest, and upper back. Clinical examination revealed multiple well-demarcated, erythematous plaques with overlying crusts and scales. Histopathology showed sub corneal acantholysis, and direct immunofluorescence revealed intercellular IgG and C3 deposits in the epidermis. Indirect immunofluorescence detected circulating autoantibodies against desmoglein1. Based on clinical presentation, histopathological findings, and immunofluorescence studies, a diagnosis of pemphigus foliaceus was confirmed. The patient was treated with systemic corticosteroids and azathioprine. Significant improvement was noted within two weeks, and follow-up talk about how to effectively handle Pemphigus foliaceus with early detection and immunosuppressive medication. Even if the illness might be recurrent and persistent, the right care can significantly improve the situation. For the best patient outcomes and management, interdisciplinary care is crucial.
Pemphigus foliaceus primarily affects the skin, presenting as well-defined erythematosquamous or erythematous-costly plaques with a seborrheic appearance and distribution (face, neck, and trunk). Mucous membranes are typically unaffected [7] and the condition is occasionally characterized by a burning sensation when exposed to sunlight, Fogosalvagem variant [8]. Pemphigus is a mucocutaneous blistering autoimmune disease with two main subtypes. In addition to the skin, mucosal membranes are affected by the more prevalent Pemphigus vulgaris (PV), which is distinguished by intraepidermal vesicles, IgG and C3 deposition on the cell surface of the whole epidermis, and autoantibodies to both desmoglein’s 1 and 3[1]. In Pemphigus foliaceus, an acquired autoimmune blistering disease, the body's immune system generates IgG autoantibodies that target the intercellular adhesion glycoprotein desmoglein-1, which is primarily expressed in the granular layer of the epidermis. This leads to acantholysis, or the breakdown of intercellular connections between keratinocytes, and the development of subcorneal blisters within the epidermis [2]. PF patients' sera solely contain antibodies to desmoglein 1 . We must stress that PF is clinically different from PV and that its incidence is far lower than PV's. There is evidence that the epidemiology of pemphigus varies by ethnicity. there are 21.7 cases per million people worldwide each year [5]. In central Europe, pemphigus is thought to affect two people out of every million each year. In the Americas, Brazil and Colombia are home to endemic pemphigus. [9] . Despite being an uncommon condition, it is the most common type of autoimmune blistering illness. All age groups have seen it, moreover older persons have been found to be more likely to have it [5]. A mix of clinical and histopathologic signs, as well as direct immunofluorescence methods, are used to diagnosis this type of lesion, mainly on perilesional biopsies of affected tissue [6]. Pemphigus foliaceus is treated with systemic immunosuppressive drugs, which reduce inflammation and moderate the inflammatory response. The cornerstone of treatment is corticosteroids, which are often used in conjunction with steroid-sparing drugs such mycophenolate mofetil (MMfs), azathioprine, or methotrexate. One biologic medication that targets B-cells and has shown promise in treating patients who are resistant to treatment is rituximab . Adjunctive therapy IVIG, antibiotic and antifungal for managing secondary infection[4,13][14]. Forecast The proper treatment can greatly improve the clinical state of patients with pemphigus foliaceus. However, the ailment may be chronic and recurrent, requiring constant care and monitoring.
Case Presentation
A 57 years old male patient came to the Emergency department (ED) with complaints of Crusted erosions over face, scalp, trunk and torso since last 2 weeks. Patient has similar symptoms for almost a 3-4 month back for which he was evaluated at Manipal Hospital and was treated with Azathioprine and Steroids and patient was well controlled. Patient was on maintenance dose of oral azathioprine 50 mg and was well maintained. Patient developed new lesions 2 weeks after stopping it then shifted to Hassan HIMS and was treated with inj. Rituximab 1st dose lesions did not subside pedal edema developed along with crushing Lesion. Patient was seen by dermatology team and shifted to medicine intensive care unit (MICU), Aster CMI hospital Hebbal banglore,560091 for further management Vital are normal. All necessary laboratory investigations were done.
History of laboratory investigation:
|
|
|
Day1 |
Day4 |
Day8 |
Day12 |
Day14 |
|
Neutrophils |
90.10% |
82.80% |
86.20% |
74.00% |
85.60% |
|
|
Lymphocyte |
4.40% |
8.40% |
5.70% |
15.20% |
7.30% |
|
|
Hemoglobin |
13.4g/dl |
12.1g/Dl |
12.7g/dl |
12.2g/dl |
12.5g/dl |
|
|
Absolute neutrophils |
8.1K/UL |
6.2K/UL |
7.7K/UL |
6.9K/UL |
8.8K/UL |
|
|
Absolute lymphocyte |
0.4K/UL |
0.6K/UL |
0.5K/UL |
1.4K/UL |
0.7K/UL |
|
|
PCV |
|
|
36.20% |
37.90% |
36.30% |
37.7 |
|
Albumin |
3.45g/dl |
|
3.2g/dl |
3.51g/dl |
3.29g/dl |
|
|
SGOT |
|
52.9U/L |
|
36U/L |
37.5U/L |
28.4U/L |
|
SGPT |
|
50.2U/L |
|
100U/L |
77.5U/L |
72.5U/L |
|
PUS CELLS |
124/hpf |
|
|
|
2/hpf |
|
|
CRP |
|
54.8mg/dl |
|
17mg/dl |
|
8.79mg/dl |
Dermatologist opinion taken, skin biopsy was done and reports confirm with superficial intra epidermal blister with presence of acantholytic cells. The upper dermis shows a mild perivascular lymphatic inflammatory infiltrate and Direct immunofluorescence report shows IgG Showed 3+fishnet, C3 showed 1+ fishnet pattern of positivity in the epidermis features are in favors of pemphigus foliaceous. he was given 2nd dose of Inj Rituximab 1gm IV on 14/06/2024, he withstood the transfusion well-no reaction. Once his condition was stabilized. He was shifted to ward. Patient currently presents with flare of lesions with pedal edema. Patient was seen by Dermatology team and admitted to MICU for further management. The treatment was initiated with antibiotic therapy Piperacillin-Tazobactam 4.5 g IV every 8 hours for 10 days to prevent secondary bacterial infections. Teicoplanin 400 mg IV once daily for 7 days was also initiated due to the immunosuppressive nature of his condition and the risk of infections. Given the diagnosis of pemphigus foliaceus, the patient was treated with Immunotherapy Rituximab 1 g IV on Day 1 and Day 14 to deplete B-cells and control the autoimmune process. Additionally, Immunosuppressive Therapy Mycophenolate Mofetil (MMF) 360 mg twice daily for 5 days was prescribed to suppress the ongoing autoimmune activity. To manage inflammation, corticosteroid Prednisolone 10 mg twice daily was started later tapered as per clinical response. Furosemide 40 mg twice daily was administered to control peripheral edema, while anticoagulant Dalteparin 5000 units subcutaneously daily was used as thromboprophylaxis due to the patient's prolonged hospitalization. Supportive therapies included Pantoprazole 40 mg once daily for gastric protection, Hydroxyzine 10 mg three times daily to alleviate pruritus, and lactulose Syrup 15 mL at bedtime for constipation. Topical management included Beclomethasone (0.025% w/w) + Fusidic Acid (2% w/w) Ointment applied twice daily to the affected areas, Fluocinolone acetonide (0.01% w/v)Shampoo twice weekly, and Cetaphil Gentle Cleanser along with Liquid Paraffin Oil for moisturizing. After a period of improvement, the patient was transitioned to oral medications upon discharge. He was prescribed Cefuroxime 500 mg twice daily for 5 days to cover any potential infections. Prednisolone was tapered, starting at 10 mg twice daily for 9 days, followed by 10 mg once daily for 2 weeks. Furosemide was continued with the directive to hold if the blood pressure fell below 110/60 mmHg. Telmisartan 40 mg once daily was continued for blood pressure control, and Calcium Supplementation 500 mg once daily was prescribed for 1 month to prevent osteopenia. Continue with Mycophenolate Mofetil (MMF) 360 mg twice daily till review. The patient was also instructed to continue Fluocinolone acetonide (0.01% w/v),Cetaphil Gentle Cleanser, and Liquid Paraffin Oil as part of his daily skin care routine for the next 2 weeks. The treatment strategy focused on controlling the autoimmune flare, preventing infections, and managing the symptoms of pemphigus foliaceus while ensuring supportive care for overall stability. The patient was advised to follow up regularly with the dermatology team for further management.
DISCUSSION
The severe autoimmune blistering disease Pemphigus foliaceus is caused by autoantibodies that attack desmoglein 1, resulting in crusted lesions and superficial erosions. Pathophysiology and Immunity of the loss of keratinocyte adhesion results from acantholysis, which is caused by the formation of IgG autoantibodies against desmoglein 1, an essential adhesion molecule in the upper epidermis. Both genetic susceptibility and external stimuli, such as drugs or viruses, may cause this autoimmune reaction. Clinical Indications and Signs of The scalp, face, chest, and upper back are common seborrheic sites where patients usually present with crusted erosions and erythematous scaly plaques. One important difference from pemphigus vulgaris is that there is no mucosal involvement. Diseases can vary in intensity from localized to broad [10]. Diagnosis of p Immunological, histological, and clinical analyses are essential. Skin biopsies frequently reveal sub corneal acantholysis. While direct immunofluorescence (DIF) verifies intercellular IgG and C3 deposits, indirect immunofluorescence (IIF) finds circulating autoantibodies. Treatment success depends on an accurate and timely diagnosis [11]. Management of Chronic and recurrent Pemphigus foliaceus. In order to control disease activity and lessen immunosuppressive medication side effects, long-term monitoring is crucial. Dermatologists, immunologists, and primary care doctors must all work together in a multidisciplinary manner. Psychosocial Impact of the physical, emotional, and social well-being of a patient is greatly impacted by Pemphigus foliaceus. Visible skin lesions can result in low self-esteem, social rejection, and mental anguish. It is essential to provide counseling and psychological support in addition to medical care [12].
CONCLUSION
Pemphigus foliaceus is an uncommon but severe autoimmune blistering illness that needs to be diagnosed very away and treated thoroughly. The use of Rituximab and corticosteroids as effective treatment for disease. Numerous individuals now have better prognoses thanks to developments in the understanding of the disease's pathophysiology and the creation of tailored medicines. In order to support patients in reaching the highest quality of life possible and to manage this chronic condition, multidisciplinary care and ongoing follow-up are essential.
REFRENCES
Rohmotullah Mondal*, Mohd Ahteshamuddin, Praveen Kumar, Rohith A., Pemphigus Foliaceus: A Rare Case Report Highlighting Diagnostic and Therapeutic Challenges, Int. J. of Pharm. Sci., 2025, Vol 3, Issue 2, 1637-1641. https://doi.org/10.5281/zenodo.14892924
10.5281/zenodo.14892924
