LETTER TO THE EDITOR
A Case of Burn-Induced Bullous Pemphigoid
To the Editor: Bullous pemphigoid (BP) is an autoimmune subepidermal blistering skin disease characterized by circulating autoantibodies directed against the basement membrane zone of stratified squamous epithelia. Most cases are idiopathic but in rare instances, some physical factors such as trauma, thermal burns, ultraviolet, ionizing, and x-ray irradiation can be at the origin of BP lesions.1 We report a case of BP induced by a severe thermal injury. A 73-year-old woman, with a medical history of hypertension and diabetes mellitus, sustained a thermal burn with boiling water to her left foot. Her superficial second-degree burn healed slowly and was followed by residual hyperpigmentation. Six weeks after the thermal burn, bullous lesions appeared on the formerly damaged skin. These changes have been considered as postburn bacterial infection and were treated with surgical debridement and broad-spectrum antibiotics. After 2 weeks of treatment, bullous lesions and a pruritic erythema appeared on the contralateral foot (Figure 1A). Subsequently, generalized eruption appeared at the low extremities, and several single lesions at the trunk and upper extremities (Figure 1B). There were no mucosal involvements. A biopsy specimen showed a subepidermal blister containing neutrophils and eosinophils with perivascular eosinophils and lymphocytes (Figure 2A). Direct immunofluorescence microscopy revealed deposition of linear IgG along the basement membrane (Figure 2B). Thus the diagnosis of BP was confirmed. Treatment with prednisone 50 mg/day led to complete healing of the disease. The dose of oral prednisolone was tapered according to the patient’s improvement.
Address correspondence to Katerina Damevska M.D., M.Sci., Clinic of Dermatology, Medical Faculty, University “Ss Cyrilius and Methodius,” Bul. Vodnjanska 31, Skopje 1000, Republic of Macedonia. Email: [email protected] Copyright © 2013 by the American Burn Association 1559-047X/2014 DOI: 10.1097/BCR.0b013e3182901124
Figure 1. A. Erosions and bullae at the site of the thermal injury; the first separated lesions appeared at the dorsum of the contralateral foot. B. Large, confluent, erythematous patches with clusters of tense blisters and erosions on the lower extremities.
Burn-induced BP is extremely rare, with only 11 cases being described in the literature to date. Time interval from a thermal injury to the onset of bullous changes ranged from 1 to 8 weeks.2 The culprit agents were boiled water, thermal burn, gas fire, and hot fat. In two cases, the bullous lesions were localized only at the burn area, whereas in the other cases, the bullae developed subsequently elsewhere.2,3 BP is induced by the effect of autoantibodies on the cutaneous basement membrane zone. Primary tissue loss in burn injury occurs as a result of protein denaturation. Damage to cell membranes results in a dynamic cascade of inflammatory mediators that exacerbate already abnormal cell-to-cell permeability and systemic inflammatory responses.4 BP antigens may be released and become available to the immune system through these mechanisms. It should be taken into consideration that the emergence of the spreading of the BP might be caused by to the “epitope spreading” whereby tissue damage from a primary inflammatory process causes the release of a previously “sequestered’ antigen, e281
Journal of Burn Care & Research July/August 2014
e282 Damevska et al
leading to an autoimmune response against the newly released antigen.5 This report describes an unusual case of burninduced BP. It is noteworthy to mention that, the first separated lesions appeared at the dorsum of the contralateral foot, followed by generalized eruption. Diagnosis of BP should be discussed when a bullous eruption develops at the area of recent burn. REFERENCES 1. Di Zenzo G, Marazza G, Borradori L. Bullous pemphigoid: physiopathology, clinical features and management. Adv Dermatol 2007;23:257–88. 2. Bachmeyer C, Cabanne-Hamy A, Moguelet P, Doizi S, Callard P. Bullous pemphigoid after boiling water burn. South Med J 2010;103:1175–7. 3. Vassileva S, Mateev G, Tsankov N. Burn-induced bullous pemphigoid. Int J Dermatol 1995;34:516–7. 4. Singh V, Devgan L, Bhat S, Milner SM. The pathogenesis of burn wound conversion. Ann Plast Surg 2007;59: 109–15. 5. Chan LS, Vanderlugt CJ, Hashimoto T, et al. Epitope spreading: lessons from autoimmune skin diseases. J Invest Dermatol 1998;110:103–9.
Figure 2. A. Skin biopsy specimen: subepidermal bulla and dermal infiltrate containing numerous eosinophils (hematoxylin and eosin; ×20). B. Direct immunofluorescence showing linear deposits of IgG along the basement membrane zone.
Katerina Damevska, MD, MS Gorgi Gocev, MD Suzana Nikolovska, MD, PhD Clinic of Dermatology, Medical Faculty Ss. Cyrilius and Methodius University of Skopje Skopje, Republic of Macedonia
A Case of Burn-Induced Bullous Pemphigoid
To the Editor: Bullous pemphigoid (BP) is an autoimmune subepidermal blistering skin disease characterized by circulating autoantibodies directed against the basement membrane zone of stratified squamous epithelia. Most cases are idiopathic but in rare instances, some physical factors such as trauma, thermal burns, ultraviolet, ionizing, and x-ray irradiation can be at the origin of BP lesions.1 We report a case of BP induced by a severe thermal injury. A 73-year-old woman, with a medical history of hypertension and diabetes mellitus, sustained a thermal burn with boiling water to her left foot. Her superficial second-degree burn healed slowly and was followed by residual hyperpigmentation. Six weeks after the thermal burn, bullous lesions appeared on the formerly damaged skin. These changes have been considered as postburn bacterial infection and were treated with surgical debridement and broad-spectrum antibiotics. After 2 weeks of treatment, bullous lesions and a pruritic erythema appeared on the contralateral foot (Figure 1A). Subsequently, generalized eruption appeared at the low extremities, and several single lesions at the trunk and upper extremities (Figure 1B). There were no mucosal involvements. A biopsy specimen showed a subepidermal blister containing neutrophils and eosinophils with perivascular eosinophils and lymphocytes (Figure 2A). Direct immunofluorescence microscopy revealed deposition of linear IgG along the basement membrane (Figure 2B). Thus the diagnosis of BP was confirmed. Treatment with prednisone 50 mg/day led to complete healing of the disease. The dose of oral prednisolone was tapered according to the patient’s improvement.
Address correspondence to Katerina Damevska M.D., M.Sci., Clinic of Dermatology, Medical Faculty, University “Ss Cyrilius and Methodius,” Bul. Vodnjanska 31, Skopje 1000, Republic of Macedonia. Email: [email protected] Copyright © 2013 by the American Burn Association 1559-047X/2014 DOI: 10.1097/BCR.0b013e3182901124
Figure 1. A. Erosions and bullae at the site of the thermal injury; the first separated lesions appeared at the dorsum of the contralateral foot. B. Large, confluent, erythematous patches with clusters of tense blisters and erosions on the lower extremities.
Burn-induced BP is extremely rare, with only 11 cases being described in the literature to date. Time interval from a thermal injury to the onset of bullous changes ranged from 1 to 8 weeks.2 The culprit agents were boiled water, thermal burn, gas fire, and hot fat. In two cases, the bullous lesions were localized only at the burn area, whereas in the other cases, the bullae developed subsequently elsewhere.2,3 BP is induced by the effect of autoantibodies on the cutaneous basement membrane zone. Primary tissue loss in burn injury occurs as a result of protein denaturation. Damage to cell membranes results in a dynamic cascade of inflammatory mediators that exacerbate already abnormal cell-to-cell permeability and systemic inflammatory responses.4 BP antigens may be released and become available to the immune system through these mechanisms. It should be taken into consideration that the emergence of the spreading of the BP might be caused by to the “epitope spreading” whereby tissue damage from a primary inflammatory process causes the release of a previously “sequestered’ antigen, e281
Journal of Burn Care & Research July/August 2014
e282 Damevska et al
leading to an autoimmune response against the newly released antigen.5 This report describes an unusual case of burninduced BP. It is noteworthy to mention that, the first separated lesions appeared at the dorsum of the contralateral foot, followed by generalized eruption. Diagnosis of BP should be discussed when a bullous eruption develops at the area of recent burn. REFERENCES 1. Di Zenzo G, Marazza G, Borradori L. Bullous pemphigoid: physiopathology, clinical features and management. Adv Dermatol 2007;23:257–88. 2. Bachmeyer C, Cabanne-Hamy A, Moguelet P, Doizi S, Callard P. Bullous pemphigoid after boiling water burn. South Med J 2010;103:1175–7. 3. Vassileva S, Mateev G, Tsankov N. Burn-induced bullous pemphigoid. Int J Dermatol 1995;34:516–7. 4. Singh V, Devgan L, Bhat S, Milner SM. The pathogenesis of burn wound conversion. Ann Plast Surg 2007;59: 109–15. 5. Chan LS, Vanderlugt CJ, Hashimoto T, et al. Epitope spreading: lessons from autoimmune skin diseases. J Invest Dermatol 1998;110:103–9.
Figure 2. A. Skin biopsy specimen: subepidermal bulla and dermal infiltrate containing numerous eosinophils (hematoxylin and eosin; ×20). B. Direct immunofluorescence showing linear deposits of IgG along the basement membrane zone.
Katerina Damevska, MD, MS Gorgi Gocev, MD Suzana Nikolovska, MD, PhD Clinic of Dermatology, Medical Faculty Ss. Cyrilius and Methodius University of Skopje Skopje, Republic of Macedonia
