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Pemphigus Vulgaris in a Black Patient: Early Recognition of Disease Saves Lives

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eMediNexus    29 July 2022

There is a prominent shortage of racial diversity in medical textbooks and literature causing race-based healthcare inequalities. A recent case report describes a black puertorrican 58-year-old female who developed a painful nonpruritic blistering ulcer in the inner oral mucosa with associated erythema 6 months before the evaluation.

She was misdiagnosed many times leading to a rapid progression of the disease, and subsequently, her death. The scarcity of images in medical textbooks and shortage of literature explaining the initial presentation per-skin-tone of pemphigus vulgaris (PV) in patients with dark skin color caused misdiagnosis, delay in treatment and thus, a catastrophic outcome. 

Thus, a recent study explains the appearance of PV in patients with dark skin tone which will serve as an educational resource by providing images of this rare skin disease in people with dark skin and fill major gaps in medical literature, highlighting the importance of timely recognizing PV in patients with dark skin, and creating awareness among physicians.

Mucosal manifestation in PV most frequently involves the oral cavity, particularly the buccal and palatine mucosa, which may be the only site of involvement for 5 months to 1 year without any other skin manifestations. There is about 10-month delay of diagnosis, if the ulcer remains despite antibiotic, antifungal or antiviral topical medication. Differential diagnosis of erosive oral lesions includes infectious diseases (herpes simplex, impetigo), drug-induced (Stevens-Johnson syndrome, toxic epidermal necrolysis), vasculitis (Bechet disease) or autoimmune diseases (systemic lupus erythematosus or oral lichen planus), etc.

In people with white skin, PV’s skin lesions appear as scattered superficial erythematous flaccid, fragile blisters that grow into scaly, crusted lesions and erythematous ulcers on the mucosal surfaces. Whereas in darker skin tones, blisters may lack erythema and appear as hyperpigmented plaques with superficial erosion and hemorrhagic crusting, this may be due to reactive hyperpigmentation. This clinical feature makes recognizing erythema difficult thus masking a clinical diagnosis. Furthermore, this hyperpigmentation gives a purple shade to the lesion causing confusion and delay in diagnosis. When the disease advances and coalesces to cover large body areas, plaques appear with a pink base and peripheral hyperpigmentation.

Pemphigus vulgaris must be suspected when a patient complains of a nonpruritic poor healing ulcer that began as a flaccid blister that may or may not be over an erythematous base. During the physical exam, it is compulsory to verify and assess for palatine or buccal mucosa involvement. Further, it must be remembered that black skin tone patients are more prone to post-inflammatory hyperpigmentation, hence altering “the classic” appearance of PV.

Source: Rodríguez-Santiago MA, García-Marín J, Lamela-Domenech A, et al. Pemphigus vulgaris in a Black patient: early recognition of disease saves lives. J Dermatol Skin Sci. 2021;3(2):5-8.

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