Bullous pemphigoid (BP) is a rare autoimmune blistering disorder primarily affecting older adults, with limited occurrences in children. BP in children typically manifests as large, tense blisters on the skin, often on flexural areas. It also more often affects the oromucosal areas and the face in children than in adults. Diagnosis involves histopathological examination revealing eosinophilic spongiosis or subepidermal split, immunofluorescence tests highlighting immunoglobulin G (IgG) and C3 depositions, and immunological assays detecting BP180 and BP230 IgG autoantibodies. This report presents two cases of childhood BP (CBP) with atypical immunopathological findings. Clinically, the two cases had generalized plaques and bullae, including the face. The first case exhibited the characteristic linear deposits of IgG and C3 on the basement membrane through direct immunofluorescence (DIF) and revealed negative anti‑BP180 antibodies on enzyme‑linked immunosorbent assay (ELISA). In contrast, the second case showed negative DIF results, despite clinical suspicion, but had positive anti‑BP180 IgG antibodies on ELISA. It is, therefore, crucial to consider the complete clinical presentation of the patient, in conjunction with the histological findings and immunopathologic assessments to diagnose CBP.
Pemphigoid, Bullous

Bullous pemphigoid (BP) is a severe autoimmune blistering condition that characteristically presents as large tense bullae on a background of normal or erythematous skin. In atypical cases, presentation is widely variegated ranging from vesicular eruptions to erythrodermic presentations. This case highlights on a rare presentation of BP manifesting with varicella-like eruption. The recognition and early diagnosis of a unique presentation of this condition is of great importance as it may greatly influence the patient’s outcome and prognosis.

Bullous pemphigoid (BP) is an autoimmune blistering disorder characterized by tense vesicles and bullae, primarily affecting the elderly. It results from autoantibodies targeting hemidesmosomal proteins BP180 and BP230, leading to subepidermal blistering. BP often presents with widespread pruritic plaques and blisters on flexural surfaces, and mucosal involvement is rare. While BP typically occurs spontaneously, certain medications, such as linagliptin, have been implicated as triggers. Early diagnosis and treatment with systemic corticosteroids or immunosuppressive agents can significantly reduce morbidity.

Bullous pemphigoid (BP) is the most common autoimmune blistering disorder in the elderly but is rare in children. Pediatric BP often presents with dramatic features, including eczematous plaques and tense bullae, accompanied by intense pruritus and occasional mucosal involvement. Despite its rarity, BP should be considered in differential diagnoses due to its varied presentations.

An 18-year-old Filipino male presented with intermittent fever followed by multiple erythematous pruritic plaques on his extremities, which later spread to the head and trunk. Generalized tense vesicles and bullae developed on top of existing plaques, accompanied by severe pruritus. Nikolsky and Asboe-Hansen signs were negative. Lesions eventually covered the entire body, including the oral mucosa. Self-medication with herbal concoctions worsened the condition, causing erosion and plaque thickening. Lab tests showed leukocytosis with eosinophilia and extremely elevated serum IgE level. Histopathology revealed subepidermal split with eosinophils, and direct immunofluorescence confirmed BP with 1+ IgG and 2+C3 deposits in the basement membrane zone. Anti-BP180 ELISA was positive. The patient was treated with intravenous hydrocortisone, then transitioned to oral prednisone (40 mg daily, tapered), leading to complete resolution of lesions with no recurrence.

Pediatric bullous pemphigoid, rare in late childhood, mirrors adult cases but with more acral distribution and mucosal involvement. Elevated IgE levels may correlate with disease activity, prompting further study of risk factors and triggers. A collaborative approach is crucial for managing physical and social challenges, improving quality of life.

Bullous pemphigoid (BP) is the most common autoimmune blistering disease primarily characterized by tense blisters and occasionally with urticarial plaques, affecting the skin and mucous membranes. These are caused by autoantibodies against BP180 and BP230 which target antigens on the basement membrane zone. The diagnosis relies on the integration of clinical, histopathological, immunopathological, and serological findings. The management depends on the clinical extent and severity. We present in this article a literature review and the clinical consensus guidelines of the Immunodermatology Subspecialty Core Group of the Philippine Dermatological Society in the management of BP.
Pemphigoid, Bullous

China/epidemiology* ; Humans ; Pemphigoid, Bullous/epidemiology* ; Retrospective Studies

INTRODUCTION: Bullous pemphigoid (BP) is a chronic, relapsing autoimmune blistering disorder commonly found in adults older than 60 years of age. It is mediated by autoantibodies directed against the hemidesmosomal proteins BP180 and BP230, which trigger an inflammatory cascade leading to blister formation. BP may present with pruritus, followed by an erythematous plaque or urticaria, and subsequently by bullae formation with or without mucosal involvement. It develops sporadically but can also be triggered by ultraviolet light exposure, radiation therapy, and medications such as dipeptidyl peptidase-4 inhibitor (DPP4i). Since 2006, the increasing use of DPP4i (also known as gliptins) for their good safety profi le in treating Type II Diabetes Mellitus has led to a further increase in the incidence of bullous pemphigoid.

CASE REPORT: This is a case of a 65-year-old hypertensive and diabetic elderly Filipino female presenting DPP4i (linagliptin)-induced bullous pemphigoid with an atypical dyshidrosiform pattern, negative direct immunofluorescence (DIF), and Enzyme-linked immunosorbent assay (ELISA) that is negative for anti-BP180 antibodies but positive for anti-BP230 antibodies.

CONCLUSION: The increasing use of DPP4i for diabetes mellitus for its good safety profile may be an essential contributing factor to the increasing incidence of BP in elderly hypertensive and diabetic patients with a simultaneous increasing incidence of atypical BP presentations such as the dyshidrosiform variant. Inability to recognize these factors carries significant therapeutic implications, including prolonged multidrug immunosuppression and increased patient morbidity and mortality.

KEYWORDS: Bullous pemphigoid, gliptin, ELISA

Humans ; Pemphigoid, Bullous ; Pemphigus ; Pemphigus, Benign Familial

Bullous pemphigoid (BP) is a common autoimmune subepidermal bullous disease.The diagnosis of BP relies on clinical manifestation,histopathology,direct and indirect immunofluorescence,and serological assay.In the past two decades,topical corticosteroids and systemic and/or topical corticosteroids were the major therapeutic options for localized/mild/moderate and extensive/severe BP,respectively.In 2021,several experts from the French Study Group on Autoimmune Bullous Skin Diseases collaboratively issued the updated guidelines for the therapeutic management of BP based on evidence-based medicine.The guidelines fully detailed the updated therapeutic options for extensive BP,BP of limited extent,localized form of BP,corticosteroid-dependent BP,and drug-induced/associated BP.In particular,systemic corticosteroids are no longer the first-line treatment for extensive BP.We interpret the guidelines to assist dermatologists in the comprehensive management of BP and promote the standardization of BP treatment.
Humans ; Pemphigoid, Bullous/drug therapy* ; Autoimmune Diseases/drug therapy* ; Glucocorticoids/therapeutic use* ; Adrenal Cortex Hormones/therapeutic use*

Introduction: Bullous pemphigoid (BP) is an acquired autoimmune subepidermal bullous disease characterized by linear depo- sition of IgG and C3 along the basement membrane. It rarely occurs in childhood, especially in adolescence, with only 14 cases identified in literature. Treatment of choice is systemic corticosteroids but other treatment options such as anti-inflammatory antibacterials and methotrexate are available. Case report: A 16-year-old Filipino girl presented with a three-month history of generalized vesicles and bullae. Nikolsky and Asboe-Hansen signs were negative. Histopathology and direct immunofluorescence were consistent with BP. ELISA to BP180 au- toantibody levels was elevated at 135 IU (normal <9 IU). Complete blood count showed leukocytosis with increase in neutrophils. Chest x-ray revealed pulmonary tuberculosis. The patient was given quadruple anti-Koch’s medication (pyrazinamide, rifampi- cin, ethambutol, isoniazid), prednisone, oral erythromycin and topical clobetasol propionate. Complete remission was attained at 10 months and is sustained at the time of writing. Conclusion To establish a definitive diagnosis and appropriate management, BP requires clinical, histopathologic, and immuno- logical correlation. Childhood BP has good prognosis and rapid treatment response, with rare relapses.
Pemphigoid, Bullous