During the 2025 Skin of Color Update, Prince Adotama, MD, FAAD, discussed management of bullous disease in patients with skin of color, with a focus on bullous pemphigoid as the most common autoimmune blistering dermatosis.
Clinical Spectrum of BP
Bullous pemphigoid (BP) is the most common autoimmune blistering disorder, typically affecting adults over the age of 60–70 years old. While the classic presentation of BP is tense bullae on an erythematous base, patients may present with non-bullous variants that include erythematous or urticarial plaques (~50%), papules or nodules (~20%), and occasionally annular, figurate, or even erythrodermic morphologies. Increasing evidence suggests non-bullous pemphigoid may represent a distinct subtype rather than a mere prodrome.
Mucosal involvement signals more severe disease. Beyond skin findings, BP patients often have significant comorbidities, including neurologic disorders such as dementia. Recognizing its diverse presentations across skin types and clinical subtypes is critical for timely diagnosis and optimal management. A recent review from Dr. Adotama and colleagues offers a useful diagnostic algorithm for clinicians.
BP in Skin of Color
In patients with skin of color, erythema may appear subtle or violaceous rather than bright pink-red, posing diagnostic challenges. Dyspigmentation is common both at presentation and during resolution, where perifollicular hyperpigmentation may be seen. Studies have shown that darker skinned patients often have higher disease activity and higher baseline BP180/BP230 antibody titers and peripheral eosinophilia. These patients also experience greater itch and disease burden, underscoring the need for early recognition and treatment.
Dr. Adotama discussed several cases to highlight the management and treatment of BP in patients with skin of color.
Case 1: Misdiagnosed Eczema in a 64-Year-Old Man
A 64-year-old African American male presented with a 3-month history of intensely pruritic rash, initially diagnosed as eczema. Appropriate next steps included performing a direct immunofluorescence (DIF) and ordering BP180/BP230 serologies. The DIF revealed IgG and C3 deposition along the dermoepidermal junction, confirming BP.
Diagnostic pearls:
- Use Michel’s medium for DIF samples.
- Sample perilesional normal skin for bullous lesions, or lesional skin if non-bullous lesions.
Serologic insights:
- BP180 antibody titers generally correlate with disease activity and are useful for monitoring.
- BP230 positivity may indicate milder, more localized disease.
- Indirect immunofluorescence can help distinguish BP from epidermolysis bullosa acquisita (EBA).
Case 2: Extensive BP and History of Coagulopathy
An 80-year-old man with biopsy-proven BP and a history of multiple deep venous thrombosis and pulmonary embolism events presented with extensive bullae on the trunk and limbs. Given his contraindications to systemic immunosuppression, omalizumab was selected. By blocking the FcεRI on mast cells, omalizumab mitigates IgE-driven inflammation. The peripheral eosinophilia seen in 50-60% of BP cases also supports the use of omalizumab in these patients.
A French study of 100 patients demonstrated complete remission in 77% within a median of 3 months, especially in those with anti-BP180 IgE antibodies. Urticarial lesions, total IgE levels, and eosinophil count were notably not predictive of response. Overall, omalizumab is an appealing choice for patients with malignancy-associated BP or in whom corticosteroids and immunosuppression are undesirable.
Case 3: Cost-Conscious Management
A 78-year-old African American woman with biopsy-proven BP presented post-COVID infection with extensive lesions on the trunk and thighs. The patient had insufficient insurance coverage and wanted to minimize medication expenses as much as possible. Doxycycline (100 mg twice daily) was initiated, and she had marked improvement at 6 weeks.
A study showed that doxycycline achieved disease control in 74% of patients, which was considered non-inferior to prednisolone. There were fewer adverse events (18%) compared to systemic corticosteroid treatment (36%). This supports doxycycline as safe and effective treatment for mild-to-moderate BP, particularly in uninsured or underinsured patients.
Case 4: Elderly Patient with Multiple Comorbidities and New-Onset Severe BP
A 93-year-old Asian female with multiple cardiopulmonary comorbidities presented with new-onset widespread bullae. She was not on any diuretics or other medications that can be associated with BP. Biopsy findings and serologies were consistent with active BP. Given elevated BP180/BP230 titers and eosinophilia, dupilumab was selected. She was started on high-dose prednisone and then transitioned to doxycycline. Eventually, she was stared on dupilumab due to refractory disease.
Targeting IL-4 and IL-13, dupilumab interrupts the Th2 immune axis involved in the pathogenesis of BP. A recent study reported 87% disease control at 4 weeks and 36% complete remission, with notable reductions in BP disease activity and itch scores. BP180 levels >50 predicted better response, while male sex predicted higher relapse rates.
Summary
- BP is heterogeneous, and can often be misdiagnosed as eczema or urticaria, especially in early or non-bullous phases.
- Skin of color patients require heightened clinical suspicion, as erythema may be subtle and dyspigmentation can be a prominent long-term sequela.
- DIF and serologies (BP180, BP230) remain the cornerstones for diagnosing BP
- Omalizumab, doxycycline, and dupilumab represent emerging, patient-tailored alternatives to traditional corticosteroids and immunosuppressants.
- Early recognition and individualized therapy can dramatically improve outcomes for this complex and increasingly heterogeneous disease.
References:
Obijiofor, Chinemelum E. et al. Insights into bullous pemphigoid: A comprehensive review of diagnostic modalities. JAAD Reviews, Volume 3, 26-36.
Chebani R, Lombart F, Chaby G, et al. Omalizumab in the treatment of bullous pemphigoid resistant to first-line therapy: a French national multicentre retrospective study of 100 patients. Br J Dermatol. 2024;190(2):258-265. doi:10.1093/bjd/ljad369
Williams HC, Wojnarowska F, Kirtschig G, et al. Doxycycline versus prednisolone as an initial treatment strategy for bullous pemphigoid: a pragmatic, non-inferiority, randomised controlled trial. Lancet. 2017;389(10079):1630-1638. doi:10.1016/S0140-6736(17)30560-3
Zhao L, Wang Q, Liang G, et al. Evaluation of Dupilumab in Patients With Bullous Pemphigoid. JAMA Dermatol. 2023;159(9):953-960. doi:10.1001/jamadermatol.2023.2428
This information was presented at the 2025 Skin of Color Update conference by Prince Adotama, MD, FAAD. The above highlights from this lecture were written and compiled by Riyad N.H. Seervai, MD, PhD.
