Pemphigus vulgaris in a postmenopausal diabetic woman

INTRODUCTION

Pemphigus vulgaris (PV) is a rare, potentially life-threatening autoimmune blistering disorder characterized by the formation of mucocutaneous lesions due to the production of autoantibodies directed against desmogleins—adhesion proteins within the epidermis. The disruption of these proteins leads to acantholysis, resulting in intraepidermal blistering.¹ The global incidence of PV is estimated to range between 0.1 and 0.5 cases per 100,000 individuals annually, with a peak onset typically observed in individuals aged 40–60 years.² The disease often presents with painful erosions on the skin and mucous membranes, and if left untreated, it can result in severe complications, including secondary infections, fluid and electrolyte imbalances, and impaired nutritional status.³

Autoimmune bullous diseases like PV carry increased morbidity, particularly in postmenopausal women. Hormonal changes associated with menopause, such as decreased estrogen levels, can alter immune function and contribute to immune dysregulation.⁴ Additionally, immunosenescence—an age-related decline in immune competence—may exacerbate disease severity and delay recovery.⁵ The presence of chronic comorbid conditions, such as type 2 diabetes mellitus (T2DM), further complicates disease management due to an increased risk of infections, delayed wound healing, and heightened sensitivity to the adverse effects of immunosuppressive therapy.⁶

This case report underscores the complexity of managing PV in a 60-year-old postmenopausal woman with diabetes mellitus. The patient’s treatment regimen involved systemic corticosteroids for immunosuppression, targeted antibiotic therapy to address methicillin-resistant Staphylococcus aureus (MRSA) infection, and supportive interventions led by the clinical pharmacy team.⁷ The involvement of clinical pharmacists proved pivotal in optimizing therapy, managing drug-related adverse effects, and ensuring safe pharmacological practices. Recommendations included calcium supplementation to mitigate steroid-induced bone demineralization, adjustment of insulin dosage for glycemic control, and identification of potential drug-drug and drug-food interactions.⁸

Overall, this case illustrates the importance of a multidisciplinary approach in the management of autoimmune bullous disorders, particularly in patients with complex clinical profiles. Timely diagnosis, individualized immunosuppressive therapy, proactive monitoring, and integrated pharmaceutical care are essential to improve clinical outcomes and enhance the quality of life in affected individuals.⁹

CASE REPORT

A 60-year-old postmenopausal female with a BMI (body mass index) of 24.57 kg/m² presented with a 1-month history of skin ulcers on the trunk and limbs, along with multiple spreading, fluid-filled blisters. She had a 7-year history of T2DM and had been receiving hormone replacement therapy with tibolone (2.5 mg OD [once daily]) for the past 6 months. Her regular medications included human insulin (4-4-0 U), tibolone 2.5 mg OD, and calcium gluconate 600 mg BD. On examination, the patient was drowsy but responsive to pain. Vital signs showed a blood pressure of 120/70 mmHg, a pulse rate of 149 bpm, a respiratory rate of 20 bpm, and an SpO₂ of 98%. Clinical examination revealed erythematous crusted plaques, hemorrhagic plaques over the lips and chin, purulent eye discharge, and conjunctival congestion. Laboratory investigations indicated macrocytic anemia showed an elevated mean corpuscular volume (MCV) of 120 fL (normal range is 80–100 fL), indicating the presence of larger-than-normal red blood cells, consistent with macrocytic anemia, and culture reports confirmed MRSA infection. Diagnostic tests revealed a positive Nikolsky sign, acantholytic cells on Tzanck smear, and a fishnet pattern of IgG on direct immunofluorescence, suggestive of PV. The treatment plan is divided into three phases, each tailored to the patient’s condition and response. In the acute phase (Days 1–3), the patient receives IV fluids (2% NS + 1% RL at 100 ml/h) along with broad-spectrum antibiotics: Piperacillin-Tazobactam 4.5 g IV TID, Metronidazole 500 mg IV TID, Ceftriaxone 1 g IV BD (twice daily), and Meropenem 1 g IV TID (added from Day 2). Dexamethasone 8 mg IV BD is used to control inflammation. For glycemic control, human insulin 6-6-6 units SC TID (three times a day) is administered. Supportive medications include ranitidine 50 mg IV BD, 10% calcium gluconate 10 ml IV QID (four times a day), and 1% topical SSD (silver sulfadiazine) cream. In the transition phase (Days 4–5), antibiotic therapy is adjusted to cefoperazone + sulbactam 1.5 g IV BD and linezolid 600 mg IV BD. Dexamethasone is continued at 8 mg IV OD, and pantoprazole 40 mg IV OD replaces ranitidine. Insulin is slightly reduced to 6-6-4 units SC TID, while calcium gluconate and topical treatments (including gentamicin eye drops and mupirocin ointment) continue. In the maintenance phase (Days 6–19), the patient is transitioned to oral Prednisolone 5 mg with a tapering schedule: QID (Days 1–4), TID (Days 5–8), BD (Days 9–12), OD (Days 13–17), and 5 mg or 2.5 mg OD (Days 18–19). The patient was prescribed a 19-day tapering course of prednisolone following the NIH 2024 protocol, beginning with a high dose of 20 mg daily for Days 1–4, then tapering to 15 mg (Days 5–8), 10 mg (Days 9–12), 5 mg (Days 13–17), and finally 2.5 mg on Days 18–19. This gradual reduction is designed to safely withdraw corticosteroids and prevent adrenal insufficiency. However, due to the high initial dose, the patient experienced steroid-induced gastritis on the 4th day of admission, a known side effect of corticosteroids. Steroids, such as prednisolone, can irritate the gastric lining by increasing acid secretion and reducing protective mucus, which can lead to inflammation and discomfort. Following a pharmacist intervention, calcium gluconate was added to counter steroid-induced calcium depletion. Additional pharmacist contributions included managing steroid-induced gastritis and adverse drug reactions (ADRs), recommending calcium supplementation for osteoporosis prevention, and identifying drug-drug and drug-food interactions, such as the potential interaction between prednisolone and sodium.

DISCUSSION

This case highlights the significant interplay between menopausal hormonal changes, chronic comorbidities such as T2DM, and the development and management of PV, a rare autoimmune bullous disorder. The patient’s postmenopausal status, characterized by estrogen deficiency, along with immune dysregulation, may have played a key role in the pathogenesis and exacerbation of the disease. Advanced age further compounded the immunological vulnerability, increasing the risk of both disease onset and treatment-related complications. Management primarily involved immunosuppressive therapy with systemic corticosteroids, which remains the cornerstone of PV treatment.¹⁰ However, in patients with diabetes, such therapy necessitates meticulous monitoring to avoid steroid-induced hyperglycemia and other metabolic disturbances.¹¹ In this case, the patient’s insulin regimen was appropriately adjusted to maintain glycemic control throughout the course of steroid therapy.

Additionally, the risk of corticosteroid-induced complications, including osteoporosis and gastritis, was effectively mitigated through pharmacist-led interventions. The clinical pharmacist played a vital role by recommending calcium supplementation, identifying potential drug-drug and drug-food interactions—such as the interaction between prednisolone and sodium—and addressing ADRs. These interventions were instrumental in enhancing therapeutic safety and improving the overall clinical outcome.¹² The prompt addition of calcium gluconate, following pharmacist evaluation, also addressed concerns of steroid-induced calcium depletion, which is particularly relevant in postmenopausal women already at risk for bone loss.⁸

Ultimately, this case underscores the critical importance of early diagnosis and a comprehensive, multidisciplinary approach to care. It illustrates how collaborative management involving dermatologists, endocrinologists, and clinical pharmacists can lead to individualized treatment strategies that enhance efficacy while minimizing risk. The favorable outcome observed in this patient demonstrates the value of tailored therapy, proactive ADR management, and continuous monitoring in optimizing care for patients with autoimmune bullous diseases such as PV.⁹

Informed consent: Written informed consent was obtained from the patient’s legal representative prior to inclusion in the study. Patient confidentiality has been maintained, and no identifiable information has been disclosed.

CONCLUSION

Effective management of PV in postmenopausal patients with comorbid diabetes mellitus necessitates a comprehensive and balanced therapeutic approach that addresses both the autoimmune pathology and the underlying metabolic disturbances. Systemic corticosteroids, the cornerstone of PV treatment, are essential for immunosuppression; however, their use in diabetic patients can lead to significant complications such as hyperglycemia, increased infection risk, and steroid-induced osteoporosis. Therefore, individualized treatment planning that includes glycemic control, infection surveillance, and prophylactic measures against ADRs is crucial.

The integration of clinical pharmacy services into the multidisciplinary care team significantly enhances the safety, efficacy, and continuity of treatment in such complex cases. Clinical pharmacists contribute to patient care by monitoring for potential drug-drug and drug-food interactions, managing corticosteroid-related side effects, and optimizing medication regimens to enhance adherence and therapeutic outcomes. Their role in recommending adjunct therapies—such as calcium and vitamin D supplementation to counteract steroid-induced bone loss—and in providing patient education on medication use further supports long-term disease control and improves overall quality of life.