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Introduction
Pemphigus is a rare blistering disease that affects the skin and mucous membranes. It is an autoimmune disease characterized by production of pathogenic autoantibodies directed against desmosome proteins of keratinocytes in the epidermis [1]. There are two basic forms of pemphigus, viz. pemphigus vulgaris (PV) and pemphigus foliaceus (PF). The etiological factors associated with pemphigus are blunt trauma, ionizing radiation, stress, cocaine use, herpes simplex virus, Epstein–Barr virus, cytomegalovirus, and medication (e.g., hormones, vaccines, nonsteroidal antiinflammatory drugs) [2, 3, 4–5].
In PV, blisters develop above the stratum basale and are associated with autoantibodies against desmoglein (Dsg)3 (i.e., a cell surface adhesion molecule of keratinocytes). Meanwhile, in PF, blisters form under the stratum corneum and are associated with autoantibodies against Dsg1 [2]. PV diagnosis requires the following: (1) clinically compatible presentation, (2) lesions histopathologically compatible with direct immunofluorescence microscopy of perilesional skin, and (3) serological detection of autoantibodies against the surface of epithelial cells by indirect immunofluorescence microscopy and/or enzyme-linked immunosorbent assay (ELISA) [6]. Among the differential diagnoses for PV are PF, pemphigus erythematosus, pemphigus vegetans, and paraneoplastic pemphigus. Other dermatologic lesions with similar morphology include herpes simplex, bullous pemphigoid, dermatitis herpetiformis, erythema multiforme, and lichen planus [7]. We present the clinical case of a patient with ten relapses, probably secondary to cocaine use. The patient’s evolution was torpid and refractory to first-line treatment, despite reported adequate adherence to treatment and without presenting other triggering factors. This is the first case reported in literature in which a patient with multiple relapses secondary to continuous cocaine use is refractory to multiple therapeutic schemes, but ultimately has complete remission after suspension of cocaine use and establishment of an effective therapeutic scheme. Approval from the ethical committee was not required due to the nature of this case report. Abiding by the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards, patient anonymity was guaranteed. Upon hospital admission, the patient signed an informed consent form permitting use of their clinical file information for didactic and research purposes.
Clinical Presentation
A 33-year-old male arrived at the emergency department, presenting polymorphic-type dermatosis with 1 week of evolution, characterized by blisters, vesicles, and excoriations on the scalp and oral cavity that spread to the thorax (anterior thorax impetiginized and...