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Localized penile bullous pemphigoid of childhood
Journal of Pediatric Urology (2008) 4, 395e397
CASE REPORT
Localized penile bullous pemphigoid of childhood Moben Mirza*, Ismael Zamilpa, Jason M. Wilson Division of Urology, Department of Surgery, University of New Mexico, MSC 105610, 1 University of New Mexico, Albuquerque, NM 87131, USA Received 3 February 2008; accepted 29 February 2008 Available online 25 April 2008
KEYWORDS Circumcision; Bullous pemphigoid; Autoimmune; Penile blisters; Skin basement membrane
Abstract Bullous pemphigoid is an acquired autoimmune bullous condition that is uncommon in childhood. Genital involvement is extremely rare. We present a case of a 7-year-old boy with bullous lesions confined to the glans penis. Precise diagnosis is based on clinical presentation and specific histopathologic testing. Oral corticosteroid therapy is the treatment of choice. Published by Elsevier Ltd on behalf of Journal of Pediatric Urology Company.
Bullous pemphigoid (BP) is a chronic, autoimmune, blistering disease characterized by autoantibodies targeting the skin basement membrane zone (BMZ) [1]. An accurate diagnosis of BP requires direct immunofluorescence (IF) and in some cases identification of the target antigen [2]. This condition is more commonly seen in the elderly. Childhood presentation is rare, but BP is still the most common IgGmediated subepidermal bullous disease in children [3]. Childhood BP is characterized by tense bullae developing on normal or erythematous skin with a predilection for flexural areas [4]. Although genital involvement is uncommon, there have been several reports of lesions localized to the vulva in female children [5,6].
* Corresponding author. Tel.: þ1 505 272 5505; fax: þ1 505 272 3699. E-mail address: [email protected] (M. Mirza).
Case report A 7-year-old boy was referred for evaluation of phimosis and painful urination. Past medical history was unremarkable. Family history was non-contributory. On examination the patient had severe phimosis. There were no signs of an active infectious process. He was taken to the operating room and a circumcision was performed. Foreskin and glans demonstrated chronic inflammatory changes. No other lesions were noted. Several weeks following the procedure, the patient developed vesicles on the glans penis (Fig. 1). These lesions were approximately 1 cm in the greatest diameter. The lesions were tense and filled with a clear fluid. Examination of the skin and mucous membranes revealed no other lesions. Following eruption, minimal inflammatory changes were noted at the site of the vesicles. The lesions recurred despite a course of mild topical steroids. The patient was taken to the operating room for biopsies of the blisters.
1477-5131/$34 Published by Elsevier Ltd on behalf of Journal of Pediatric Urology Company. doi:10.1016/j.jpurol.2008.02.008
396
Figure 1 Patient’s penis at time of biopsy. Tense, clear, fluid-filled vesicle is shown at distal aspect. Area of ruptured vesicle is seen at the dorsal aspect.
The area of interface between the blisters and the normal skin was sampled. Pathology revealed cell-poor subepidermal blisters consistent with BP. IgG and complement (C3) IF stains localized to the epidermal side of the bullae (Figs. 2 and 3). A collagen IV immunostain revealed type IV collagen present at the base of the blister. Underlying dermis appeared inflamed with an admixture of lymphocytes, plasma cells and rare eosinophils. Corticosteroid therapy was initiated once the diagnosis was obtained and remission was achieved.
Discussion Approximately 50 cases of childhood BP have been reported in the literature [4]. Mucous membranes may be affected in 72% of childhood cases as compared to 10e40% in adults [7]. A characteristic feature of childhood BP is the marked involvement of the palms and soles, especially in infants [4]. Vesicles are rarely
Figure 2 Direct IF, IgG: 2þ (of 4) linear deposition at the dermal/epidermal junction. IgM: negative. IgA: negative.
M. Mirza et al.
Figure 3 Direct IF, C3: 2þ linear deposition at the dermal/ epidermal junction. Fibrinogen: 3þ non-specific perivascular and interstitial deposition. Testing was performed with an appropriate positive control for each antibody.
confined to the genitals. Localized vulvar BP is believed to be a distinct variant of childhood BP responding well to topical steroids [8]. Eight cases of BP with lesions localized to the vulva have been reported [6]. To the best of our knowledge, there are no reports in the literature describing BP exclusively localized to the glans penis. Overall, the course of childhood BP is described as benign in comparison to pemphigus vulgaris or epidermolysis bullosa. Remissions occur within 1 year of treatment [2]. In their series, Edwards et al. [8] reported a range of disease duration of 7 monthse3 years. Martinez-De Pablo et al. [3] suggest the following criteria to assist with diagnosis: (1) age less than 18 years with bullous skin lesions with or without mucous membrane involvement; (2) histopathologic features revealing subepidermal bullae with variable amount of eosinophils; and (3) direct IF showing linear deposition of IgG and/or C3 at the BMZ, or a positive indirect IF demonstrating IgG antibodies reacting with the BMZ. Type IV collagen has been seen to line the floor of blister cavities [2]. Although oral corticosteroid therapy is the treatment of choice (1e2 mg/kg/day), other regimens have been proposed especially in refractory cases. Addition of dapsone or sulfapyridine as adjunctive therapy to steroids may be useful. Erythromycin alone has also shown good results [4]. Lebeau et al. [5] reported successful treatment of vulvar BP with topical tacrolimus ointment 0.1%. High-dose intravenous immunoglobulin has been a promising agent for the treatment of BP as well, especially in those cases refractory to conventional therapy [7,9]. Intravenous methylprednisolone therapy has also been shown to be effective [2]. Other drugs used in combination with systemic steroids include nicotinamide and mycophenolate mofetil [7]. Childhood BP is a rare condition. Although lesions can be widely distributed, localized genital vesicles can occur. Long-term prognosis appears favorable, but quick and accurate diagnosis is imperative to implement effective therapy and achieve remission.
Localized penile bullous pemphigoid
Conflicts of interest statement None of the listed authors have any conflict of interests to report.
Acknowledgements Monica Romero, MD, Resident Physician, Department of Dermatology, University of New Mexico; Jessica Spies, MD, Staff Pathologist, Presbyterian Medical Center, Albuquerque, New Mexico; Curtis Thompson, MD, and Steve Poole, MD, Curtis T. Thompson and Associates, Dermatopathologists, Tigard, Oregon.
References [1] Walsh SR. Bullous pemphigoid: from bench to bedside. Drugs 2005;65:905e26.
397 [2] Fisler RE. Childhood bullous pemphigoid: a clinicopathologic study and review of the literature. Am J Dermatopathol 2003; 25:183e9. [3] Martinez-De Pablo MI. Childhood bullous pemphigoid: clinical and immunological findings in a series of 4 cases. Arch Dermatol 2007;143:215e20. [4] Saenz AM. Childhood bullous pemphigoid: a case report and 10-year follow up. Int J Dermatol 2007;46:508e10. [5] Lebeau S. Localized childhood vulval pemphigoid treated with tacrolimus ointment. Dermatology 2004;208:273e5. [6] Schoeffler A. Vulvar cicatricial pemphigoid of childhood. Pediatr Dermatol 2004;21:51e3. [7] Sugawara N. Infantile bullous pemphigoid treated with intravenous immunoglobulin therapy. J Am Acad Dermatol 2007;57: 1084e9. [8] Edwards S. Bullous pemphigoid and epidermolysis bullosa acquisita: presentation, prognosis, and immunopathology in 11 children. Pediatr Dermatol 1998;15:184e90. [9] Xiao T. Childhood bullous pemphigoid treated by intravenous immunoglobulin. J Dermatol 2007;34:650e3.
CASE REPORT
Localized penile bullous pemphigoid of childhood Moben Mirza*, Ismael Zamilpa, Jason M. Wilson Division of Urology, Department of Surgery, University of New Mexico, MSC 105610, 1 University of New Mexico, Albuquerque, NM 87131, USA Received 3 February 2008; accepted 29 February 2008 Available online 25 April 2008
KEYWORDS Circumcision; Bullous pemphigoid; Autoimmune; Penile blisters; Skin basement membrane
Abstract Bullous pemphigoid is an acquired autoimmune bullous condition that is uncommon in childhood. Genital involvement is extremely rare. We present a case of a 7-year-old boy with bullous lesions confined to the glans penis. Precise diagnosis is based on clinical presentation and specific histopathologic testing. Oral corticosteroid therapy is the treatment of choice. Published by Elsevier Ltd on behalf of Journal of Pediatric Urology Company.
Bullous pemphigoid (BP) is a chronic, autoimmune, blistering disease characterized by autoantibodies targeting the skin basement membrane zone (BMZ) [1]. An accurate diagnosis of BP requires direct immunofluorescence (IF) and in some cases identification of the target antigen [2]. This condition is more commonly seen in the elderly. Childhood presentation is rare, but BP is still the most common IgGmediated subepidermal bullous disease in children [3]. Childhood BP is characterized by tense bullae developing on normal or erythematous skin with a predilection for flexural areas [4]. Although genital involvement is uncommon, there have been several reports of lesions localized to the vulva in female children [5,6].
* Corresponding author. Tel.: þ1 505 272 5505; fax: þ1 505 272 3699. E-mail address: [email protected] (M. Mirza).
Case report A 7-year-old boy was referred for evaluation of phimosis and painful urination. Past medical history was unremarkable. Family history was non-contributory. On examination the patient had severe phimosis. There were no signs of an active infectious process. He was taken to the operating room and a circumcision was performed. Foreskin and glans demonstrated chronic inflammatory changes. No other lesions were noted. Several weeks following the procedure, the patient developed vesicles on the glans penis (Fig. 1). These lesions were approximately 1 cm in the greatest diameter. The lesions were tense and filled with a clear fluid. Examination of the skin and mucous membranes revealed no other lesions. Following eruption, minimal inflammatory changes were noted at the site of the vesicles. The lesions recurred despite a course of mild topical steroids. The patient was taken to the operating room for biopsies of the blisters.
1477-5131/$34 Published by Elsevier Ltd on behalf of Journal of Pediatric Urology Company. doi:10.1016/j.jpurol.2008.02.008
396
Figure 1 Patient’s penis at time of biopsy. Tense, clear, fluid-filled vesicle is shown at distal aspect. Area of ruptured vesicle is seen at the dorsal aspect.
The area of interface between the blisters and the normal skin was sampled. Pathology revealed cell-poor subepidermal blisters consistent with BP. IgG and complement (C3) IF stains localized to the epidermal side of the bullae (Figs. 2 and 3). A collagen IV immunostain revealed type IV collagen present at the base of the blister. Underlying dermis appeared inflamed with an admixture of lymphocytes, plasma cells and rare eosinophils. Corticosteroid therapy was initiated once the diagnosis was obtained and remission was achieved.
Discussion Approximately 50 cases of childhood BP have been reported in the literature [4]. Mucous membranes may be affected in 72% of childhood cases as compared to 10e40% in adults [7]. A characteristic feature of childhood BP is the marked involvement of the palms and soles, especially in infants [4]. Vesicles are rarely
Figure 2 Direct IF, IgG: 2þ (of 4) linear deposition at the dermal/epidermal junction. IgM: negative. IgA: negative.
M. Mirza et al.
Figure 3 Direct IF, C3: 2þ linear deposition at the dermal/ epidermal junction. Fibrinogen: 3þ non-specific perivascular and interstitial deposition. Testing was performed with an appropriate positive control for each antibody.
confined to the genitals. Localized vulvar BP is believed to be a distinct variant of childhood BP responding well to topical steroids [8]. Eight cases of BP with lesions localized to the vulva have been reported [6]. To the best of our knowledge, there are no reports in the literature describing BP exclusively localized to the glans penis. Overall, the course of childhood BP is described as benign in comparison to pemphigus vulgaris or epidermolysis bullosa. Remissions occur within 1 year of treatment [2]. In their series, Edwards et al. [8] reported a range of disease duration of 7 monthse3 years. Martinez-De Pablo et al. [3] suggest the following criteria to assist with diagnosis: (1) age less than 18 years with bullous skin lesions with or without mucous membrane involvement; (2) histopathologic features revealing subepidermal bullae with variable amount of eosinophils; and (3) direct IF showing linear deposition of IgG and/or C3 at the BMZ, or a positive indirect IF demonstrating IgG antibodies reacting with the BMZ. Type IV collagen has been seen to line the floor of blister cavities [2]. Although oral corticosteroid therapy is the treatment of choice (1e2 mg/kg/day), other regimens have been proposed especially in refractory cases. Addition of dapsone or sulfapyridine as adjunctive therapy to steroids may be useful. Erythromycin alone has also shown good results [4]. Lebeau et al. [5] reported successful treatment of vulvar BP with topical tacrolimus ointment 0.1%. High-dose intravenous immunoglobulin has been a promising agent for the treatment of BP as well, especially in those cases refractory to conventional therapy [7,9]. Intravenous methylprednisolone therapy has also been shown to be effective [2]. Other drugs used in combination with systemic steroids include nicotinamide and mycophenolate mofetil [7]. Childhood BP is a rare condition. Although lesions can be widely distributed, localized genital vesicles can occur. Long-term prognosis appears favorable, but quick and accurate diagnosis is imperative to implement effective therapy and achieve remission.
Localized penile bullous pemphigoid
Conflicts of interest statement None of the listed authors have any conflict of interests to report.
Acknowledgements Monica Romero, MD, Resident Physician, Department of Dermatology, University of New Mexico; Jessica Spies, MD, Staff Pathologist, Presbyterian Medical Center, Albuquerque, New Mexico; Curtis Thompson, MD, and Steve Poole, MD, Curtis T. Thompson and Associates, Dermatopathologists, Tigard, Oregon.
References [1] Walsh SR. Bullous pemphigoid: from bench to bedside. Drugs 2005;65:905e26.
397 [2] Fisler RE. Childhood bullous pemphigoid: a clinicopathologic study and review of the literature. Am J Dermatopathol 2003; 25:183e9. [3] Martinez-De Pablo MI. Childhood bullous pemphigoid: clinical and immunological findings in a series of 4 cases. Arch Dermatol 2007;143:215e20. [4] Saenz AM. Childhood bullous pemphigoid: a case report and 10-year follow up. Int J Dermatol 2007;46:508e10. [5] Lebeau S. Localized childhood vulval pemphigoid treated with tacrolimus ointment. Dermatology 2004;208:273e5. [6] Schoeffler A. Vulvar cicatricial pemphigoid of childhood. Pediatr Dermatol 2004;21:51e3. [7] Sugawara N. Infantile bullous pemphigoid treated with intravenous immunoglobulin therapy. J Am Acad Dermatol 2007;57: 1084e9. [8] Edwards S. Bullous pemphigoid and epidermolysis bullosa acquisita: presentation, prognosis, and immunopathology in 11 children. Pediatr Dermatol 1998;15:184e90. [9] Xiao T. Childhood bullous pemphigoid treated by intravenous immunoglobulin. J Dermatol 2007;34:650e3.