Clinical case: “Challenges in treating the Pemphigus vulgaris patients.”

Introduction: It is challenging to differentiate bullous dermatoses, there are several differential diagnoses. Pemphigus vulgaris (PV) usually begins with painful oral mucosal lesions. Cutaneous symptoms usually develop with flaccid bullae with transparent content on non-erythematous skin. The lesions also can be localized in seborrheic areas. [https://doi.org/10.1111/jdv.16752]
Case presentation: a 64-year-old woman presented with complaints: painful blisters in oral mucosa, scalp, and skin since April 2023. Complaints first appeared in scalp skin two years ago, she consulted with dermatologist. The skin punch biopsy revealed morphology of acantholytic dermatosis between Darier’s disease and Grover’s disease. There is not any history of skin disease previously. As the pathological condition of the skin progresses, the patient consults an immunologist, a plasmapheresis course of five procedures is started. The patient is hospitalized for additional examination and treatment.
Materials and methods: Videodermatoscopy in the hairy part of the head - a "strawberry" colored erythematous background with white keratin structures, hair follicles have disappeared, individual keratin "corks". In the distal third of the thighs, neck, back area, blisters, and erosions are observed, positive Nikolski's sign, painful ulcers in the gum mucosa are formed. A skin punch biopsy is taken from a fresh in blister on the back and hairy part of the head, the diagnosis of Pemphigus vulgaris is confirmed histologically.
Laboratory tests revealed leukocytopenia, thrombocytosis, elevated C-reactive protein, elevated alpha-1 globulin alpha-2 globulin, decreased gamma globulins and albumin, M-protein (paraprotein) was not found.
Therapy in the hospital consisted of pulse Methylprednisolone and Azathioprine. In outpatient treatment, Prednisolone and Azathioprine were continued after the scheme.
Conclusion: PV patients should be monitored regularly during therapy to observe efficacy and side effects of high dose of systemic glucocorticoids and immunosuppressive therapy. In this case PV should be differentiated from Paraneoplastic pemphigus and Pemphigus foliaceus. Great attention should be paid to accurate diagnosis and treatment, an interprofessional approach is necessary to optimize outcomes of disease. [Ingold CJ, Khan MAB. Pemphigus Vulgaris. [Updated 2023 Feb 11]]