Pemphigus Foliaceus in an Adolescent
A
13-year-old female presented with a 1-week history of a worsening diffuse, desquamating rash that began on her stomach but resultantly spread to involve her entire body including face, hands, and feet. She also developed bilateral conjunctivitis with purulent discharge. There was no history of fever, dysuria, or dysphagia. She denied any new exposures, including medications. At presentation, her examination was significant for a diffuse, tender, erythematous, desquamating rash on the trunk, face, and extremities. Although individual lesions were annular erosions with crusting, the rash had coalesced and was confluent on the trunk (Figure 1). Intact flaccid blisters with positive Nikolsky sign were noted. There was significant crusting around her eyes, nose, and mouth. Mucosal findings were limited to mild conjunctivitis. Initial management consisted of fluid resuscitation and initiation of antistaphylococcal (vancomycin) and antiviral (acyclovir) coverage. Dermatology was consulted and a skin biopsy was diagnostic for pemphigus foliaceus with positive IgG and C3 direct immunofluorescence (Figure 2; available at www.
jpeds.com). Definitive treatment was started with a 3-day course of high-dose pulse steroids (methylprednisolone 500 mg daily), which brought rapid improvement. Vancomycin and acyclovir were discontinued once respective cultures were negative. She was discharged home on oral prednisone (40 mg/d) and mycophenolate mofetil (30 mg/kg/d). After discharge she did well; prednisone was weaned over a 2-month period, and she remained on mycophenolate mofetil. Pemphigus foliaceus is an acquired, autoimmune, blistering disease whose target antigen is desmoglein 1 found in skin but not mucous membranes. It is a chronic form of pemphigus characterized by loss of intracellular adhesion of keratinocytes in the superficial epidermal layer of the skin, resulting in superficial blistering of the skin but sparing the oral mucosa.1,2 Differentiating pemphigus foliaceus from the more severe pemphigus vulgaris can be done clinically, with pemphigus vulgaris showing a more widespread distribution and propensity for mucous membrane involvement. Also, on direct immunofluorescence, pemphigus vulgaris stains throughout the epidermis displaying its antigenic targets as desmoglein 1 and 3, and pemphigus foliaceus stains only the upper epidermis.1 Treatment is guided by degree of involvement. In limited involvement, topical corticosteroids may be sufficient.3 In more severe cases, treatment with immunosuppressants may be necessary, including systemic corticosteroids, azathioprine, mycophenolate mofetil, cyclophosphamide, cyclosporin A, or rituximab.2-4 n Ameze Adah, MD Kelley Hill, MD Department of Pediatrics
Julie Wyatt, MD Department of Dermatology
Lisa Didion, MD Figure 1. Coalescing areas of erosion and crusted plaques, which are typical for pemphigus foliaceus, were noted on admission.
J Pediatr 2014;165:1062. 0022-3476/$ - see front matter. Copyright ª 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2014.07.023
1062
Division of General Pediatrics Blair E. Batson Children’s Hospital University of Mississippi Medical Center Jackson, Mississippi
References available at www.jpeds.com
Vol. 165, No. 5 November 2014
References 1. Joly P, Litrowski N. Pemphigus group (vulgaris, vegetans, foliaceus, herpetiformis, brasiliensis). Clin Dermatol 2011;29:432-6. 2. Connelly EA, Aber C, Kleiner G, Nousari C, Charles C, Schachner LA. Generalized erythrodermic pemphigus foliaceus in a child and its successful response to rituximab treatment. Pediatr Dermatol 2007;24:172-6.
3. Metry DW, Herbert AA, Jordon RE. Nonendemic pemphigus foliaceus in children. J Am Acad Dermatol 2002;46:419-22. 4. Frew JW, Martin LK, Murrell DF. Evidence-based treatments in pemphigus vulgaris and pemphigus foliaceus. Dermatol Clin 2011;29: 599-606.
Figure 2. On light microscopy, examination of the skin showed a subcorneal blister filled with acantholytic keratinocytes and scattered neutrophils. On the direct immunofluorescence study, above, all of the acantholytic cells, as well as the intact adjacent cells, showed marked fluorescence with lgG, C3, and fibrinogen consistent with pemphigus foliaceus (C3 and lgG shown). The classic finding of intercellular fluorescence or “chicken wire” pattern was not seen here. This alteration was thought to be due to biopsy of a late stage blister. 1062.e1
A
13-year-old female presented with a 1-week history of a worsening diffuse, desquamating rash that began on her stomach but resultantly spread to involve her entire body including face, hands, and feet. She also developed bilateral conjunctivitis with purulent discharge. There was no history of fever, dysuria, or dysphagia. She denied any new exposures, including medications. At presentation, her examination was significant for a diffuse, tender, erythematous, desquamating rash on the trunk, face, and extremities. Although individual lesions were annular erosions with crusting, the rash had coalesced and was confluent on the trunk (Figure 1). Intact flaccid blisters with positive Nikolsky sign were noted. There was significant crusting around her eyes, nose, and mouth. Mucosal findings were limited to mild conjunctivitis. Initial management consisted of fluid resuscitation and initiation of antistaphylococcal (vancomycin) and antiviral (acyclovir) coverage. Dermatology was consulted and a skin biopsy was diagnostic for pemphigus foliaceus with positive IgG and C3 direct immunofluorescence (Figure 2; available at www.
jpeds.com). Definitive treatment was started with a 3-day course of high-dose pulse steroids (methylprednisolone 500 mg daily), which brought rapid improvement. Vancomycin and acyclovir were discontinued once respective cultures were negative. She was discharged home on oral prednisone (40 mg/d) and mycophenolate mofetil (30 mg/kg/d). After discharge she did well; prednisone was weaned over a 2-month period, and she remained on mycophenolate mofetil. Pemphigus foliaceus is an acquired, autoimmune, blistering disease whose target antigen is desmoglein 1 found in skin but not mucous membranes. It is a chronic form of pemphigus characterized by loss of intracellular adhesion of keratinocytes in the superficial epidermal layer of the skin, resulting in superficial blistering of the skin but sparing the oral mucosa.1,2 Differentiating pemphigus foliaceus from the more severe pemphigus vulgaris can be done clinically, with pemphigus vulgaris showing a more widespread distribution and propensity for mucous membrane involvement. Also, on direct immunofluorescence, pemphigus vulgaris stains throughout the epidermis displaying its antigenic targets as desmoglein 1 and 3, and pemphigus foliaceus stains only the upper epidermis.1 Treatment is guided by degree of involvement. In limited involvement, topical corticosteroids may be sufficient.3 In more severe cases, treatment with immunosuppressants may be necessary, including systemic corticosteroids, azathioprine, mycophenolate mofetil, cyclophosphamide, cyclosporin A, or rituximab.2-4 n Ameze Adah, MD Kelley Hill, MD Department of Pediatrics
Julie Wyatt, MD Department of Dermatology
Lisa Didion, MD Figure 1. Coalescing areas of erosion and crusted plaques, which are typical for pemphigus foliaceus, were noted on admission.
J Pediatr 2014;165:1062. 0022-3476/$ - see front matter. Copyright ª 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2014.07.023
1062
Division of General Pediatrics Blair E. Batson Children’s Hospital University of Mississippi Medical Center Jackson, Mississippi
References available at www.jpeds.com
Vol. 165, No. 5 November 2014
References 1. Joly P, Litrowski N. Pemphigus group (vulgaris, vegetans, foliaceus, herpetiformis, brasiliensis). Clin Dermatol 2011;29:432-6. 2. Connelly EA, Aber C, Kleiner G, Nousari C, Charles C, Schachner LA. Generalized erythrodermic pemphigus foliaceus in a child and its successful response to rituximab treatment. Pediatr Dermatol 2007;24:172-6.
3. Metry DW, Herbert AA, Jordon RE. Nonendemic pemphigus foliaceus in children. J Am Acad Dermatol 2002;46:419-22. 4. Frew JW, Martin LK, Murrell DF. Evidence-based treatments in pemphigus vulgaris and pemphigus foliaceus. Dermatol Clin 2011;29: 599-606.
Figure 2. On light microscopy, examination of the skin showed a subcorneal blister filled with acantholytic keratinocytes and scattered neutrophils. On the direct immunofluorescence study, above, all of the acantholytic cells, as well as the intact adjacent cells, showed marked fluorescence with lgG, C3, and fibrinogen consistent with pemphigus foliaceus (C3 and lgG shown). The classic finding of intercellular fluorescence or “chicken wire” pattern was not seen here. This alteration was thought to be due to biopsy of a late stage blister. 1062.e1
