9. Motegi S, Hattori M, Tago O, Nagai Y, Ishikawa O. Elevated serum levels of TARC/CCL17, eotaxin-3/CCL26 and vascular endothelial growth factor in a patient with non-episodic angioedema associated with eosinophilia and granulomatous cutaneous reaction. Eur J Dermatol 2013; 23: 524-6. 10. Iwagaki M. Nonepisodic angioedema associated with eosinophilia. Hifuka-no Rinsho (Japanese) 2002; 45: 1557-9. doi:10.1684/ejd.2014.2274
Jacquet erosive diaper dermatitis: a notso-rare syndrome Jacquet erosive diaper dermatitis (JED), also called dermatitis syphiloides posterosiva, is typically described as a severe irritant dermatitis affecting the genital and/or the perianal region. JED usually affects infants aged >6 months but can also arise in adults with urinary and/or fecal incontinence [1, 2]. Usually JED is characterized by 2-5 mm, well-demarcated, red-purple papules with central umbilication, which can evolve into punched-out erosions and ulcers. This form of dermatitis has become relatively rare. We present another case of JED in a child affected by spina bifida. A 6-year-old male was affected by spina bifida and he was operated on at birth for spinal cord untethering. The spinal defect led to urinary and fecal incontinence. About a month before, he had developed multiple papular-erosive itchy lesions, in some cases umbilicated, localized on the glans and on the foreskin (figures 1A-C). The child had no other symptoms or lymphadenopathy. Skin swabs were positive for E. coli. A diagnosis of JED was made. Response to topical treatment with zinc oxide cream and aqueous solution of eosin 2% was poor; also treatment with topical sucralfate 4% cream three times daily for 2 months led to only partial improvement. Thus, we suggested performing a surgical circumcision of the penis. The histology of the skin showed a non-specific inflammatory infiltrate and mild epidermal hyperplasia (figure 1D). Two months
A
B
C
D
after the surgery a complete resolution of the lesions was obtained. We report a case of JED treated with topical zinc oxide cream, aqueous solution of eosin 2% and topical sucralfate cream which achieved only little improvement, while removing the urinary stagnation by circumcision and using proper sanitary nappy rules led to a complete healing. JED is a form of diaper dermatitis usually characterized by 2-5 mm, umbilicated or eroded papules and nodules in the genital and perianal areas. Nevertheless, JED may occur with a heterogeneous pattern, some authors suggested that JED, perianal pseudoverrucous papules and nodules (PPPN) and granuloma gluteale infantum (GGI) could be all part of the same clinical spectrum, representing the result of chronic irritant contact dermatitis [3]. Probably all three diseases represent the same entity with different names, depending on the predominance of the clinical lesion: papules/erosions (JED), papules (PPPN) and nodules (GGI). One or another of these lesions may dominate at different times in the evolution of dermatitis or may be different inflammatory responses to various stimuli [4]. Some authors have performed many biopsies of these diseases and shown a histological overlap among JED, PPPN and GGI [5]. The differential diagnosis of JED includes: fungal and bacterial infections, cutaneous Crohn’s disease, Langerhans cell histiocytosis and acrodermatitis enteropatica. The pathogenesis involves several factors: urinary and fecal incontinence, infrequent diaper changing, persistence of detergent residues and use of rough toilet paper/diapers. The continued contact with urine increases the skin pH and makes it more susceptible to irritants [6], such as the fecal enzymes (proteases and lipases), which are activated by the alkaline pH [7]. The occlusion of the diaper and the friction of baby’s movements perpetuate the irritation process. Some authors attribute the increase of this disease to the growing use of reusable diapers, as they are considered more natural, ecologic and economic [8]. The diagnosis of JED is based on clinical features because histology shows only a non-specific polymorphous inflammatory infiltrate and sometimes mild epidermal hyperplasia. JED is not so uncommon but its recognition is important in order to avoid inappropriate treatment. The literature documents a poor efficacy of topical treatment with antibiotics, miconazole, zinc oxide and non-steroidal anti-inflammatory drugs, while some reports described successful treatment with topical sucralfate [9, 10]. However, full recovery is achieved only by removing the main irritating factors, as our case, in which topical treatment was ineffective and a surgical therapy was necessary. In our patient the spinal defect led to chronic urinary and fecal incontinence but the circumcision reduced the amount of urinary stagnation and we obtained the complete healing of the disease. Disclosure. Financial support: none. Conflict of interest: none. 1
Department of Dermatology, Department of Pediatric Science, Catholic University of Sacred Heart Largo A. Gemelli 8 00168 Rome, Italy 2 Health Services Research Unit, IDI-IRCCS, Rome, Italy 3
Figure 1. A-C) Multiple papular-erosive lesions, in some cases umbilicated, localized on the glans and on the foreskin; D) Histology of our case showed hyper-parakeratosis and a non-specific polymorphous inflammatory infiltrate in the interstitium.
252
Francesco RICCI1 Andrea PARADISI2 Francesca PERINO1 Rodolfo CAPIZZI1 Valentina PAOLUCCI3 Claudia RENDELI3 Cristina GUERRIERO1
EJD, vol. 24, n◦ 2, March-April 2014
P = 0.522
A 70
P < 0.001
P = 0.037
P = 0.002
65 60
56.2
53.5
STAI
55
49.9 50
45.5
45 40 35 30
Serum IgE
100 ≤ (n = 255)
< 100 (n = 13)
< 100 (n = 13)
100 ≤ (n = 255)
TA
B
SA
P = 0.202
P < 0.001
70
P = 0.239
65
P = 0.086
60 55
STAI
1. Hara M, Watanabe M, Tagami H. Jacquet erosive diaper dermatitis in a young girl with urinary incontinence. Pediatr Dermatol 1991; 8: 160-1. 2. Virgili A, Corazza M, Califano A. Diaper dermatitis in an adult. A case of erythema papuloerosive of Sevestre and Jacquet. J Reprod Med 1998; 43: 949-51. 3. Van L, Harting M, Rosen T. Jacquet erosive diaper dermatitis: a complication of adult urinary incontinence. Cutis 2008; 82: 72-4. 4. Rodriguez-Poblador J, González-Castro U, Herranz-Martínez S, Luelmo-Aguilar J. Jacquet erosive diaper dermatitis after surgery for Hirschsprung disease. Pediatr Dermatol 1998; 15: 46-7. 5. Robson KJ, Maughan JA, Purcell SD, Petersen MJ, Haefner HK, Lowe L. Erosive papulonodular dermatosis associated with topical benzocaine: a report of two cases and evidence that granuloma gluteale, pseudoverrucous papules, and Jacquet’s erosive dermatitis are a disease spectrum. J Am Acad Dermatol 2006; 55: S74-80. 6. Buckingham KW, Berg RW. Etiologic factors in diaper dermatitis: the role of feces. Pediatr Dermatol 1986; 3: 107-12. 7. Berg RW, Buckingham KW, Stewart RL. Etiologic factors in diaper dermatitis: the role of urine. Pediatr Dermatol 1986; 3: 102-6. 8. Maruani A, Lorette G, Barbarot S, et al. Re-emergence of papulonodular napkin dermatitis with use of reusable diapers: report of 5 cases. Eur J Dermatol 2013; 23: 246-9. 9. Markham T, Kennedy F, Collins P. Topical sucralfate for erosive irritant diaper dermatitis. Arch Dermatol 2000; 136: 1199-200. 10. Paradisi A, Capizzi R, Ghitti F, Lanza-Silveri S, Rendeli C, Guerriero C. Jacquet erosive diaper dermatitis: a therapeutic challenge. Clin Exp Dermatol 2009; 34: e385-6.
52.5
50.0
50
48.8 45.6
45
doi:10.1684/ejd.2014.2279
40 35 30
Serum IgE
Psychological aspects of patients with intrinsic atopic dermatitis
< 200 (n = 28)
200 ≤ (n = 240)
TA
EJD, vol. 24, n◦ 2, March-April 2014
200 ≤ (n = 240)
SA
P = 0.295
C
P = 0.024 P = 0.032
70
P = 0.121 65 60
57.3
55
STAI
Atopic dermatitis (AD) is classified into the IgE-high extrinsic and IgE-normal intrinsic types. While extrinsic AD is the classical type with high prevalence, intrinsic AD occupies approximately 20% of total AD with female predominance [1]. The skin barrier is perturbed in the extrinsic, but not intrinsic type [1], and thus filaggrin gene mutations are not a feature of intrinsic AD [2]. While the two types have comparable frequencies of circulating Th2 cells, intrinsic AD shows a significantly increased frequency of circulating interferon-␥-producing Th1 cells and lesional Th17 cells [2, 3]. We have shown that metal allergy may induce such an immunological state in intrinsic AD [1, 4]. In addition, we speculated that some itch-related factors, such as anxiety, might exacerbate intrinsic AD because of the refractory pruritus. The pathogenic role of the psychological factors in patients with AD remains controversial [5, 6]. There has been no report focusing on the impact of anxiety on intrinsic AD. To reveal the anxiety profile, the state-trait anxiety inventory (STAI) [7] is a useful questionnaire, which enables us to evaluate both state anxiety (SA) and trait anxiety (TA). We have previously shown that anxiety is significantly higher among subjects with AD than normal subjects, and TA was higher than SA in the AD group [8, 9]. Here, we sought to investigate the relationship between anxiety and intrinsic AD. Enrolled in this study were 268 Japanese AD patients, most of whom received our inpatient AD educational program. The diagnosis of AD was made according to the criteria of
< 200 (n = 28)
49.0
50
51.6 45.9
45 40 35 30
Serum IgE
< 100 (n = 8)
100 ≤ (n = 118)
TA
< 100 (n = 8)
100 ≤ (n = 118)
SA
Figure 1. Trait anxiety (TA) and State anxiety (SA) in the two groups of AD patients. The patients were divided into: (A) the IgE levels
Jacquet erosive diaper dermatitis: a notso-rare syndrome Jacquet erosive diaper dermatitis (JED), also called dermatitis syphiloides posterosiva, is typically described as a severe irritant dermatitis affecting the genital and/or the perianal region. JED usually affects infants aged >6 months but can also arise in adults with urinary and/or fecal incontinence [1, 2]. Usually JED is characterized by 2-5 mm, well-demarcated, red-purple papules with central umbilication, which can evolve into punched-out erosions and ulcers. This form of dermatitis has become relatively rare. We present another case of JED in a child affected by spina bifida. A 6-year-old male was affected by spina bifida and he was operated on at birth for spinal cord untethering. The spinal defect led to urinary and fecal incontinence. About a month before, he had developed multiple papular-erosive itchy lesions, in some cases umbilicated, localized on the glans and on the foreskin (figures 1A-C). The child had no other symptoms or lymphadenopathy. Skin swabs were positive for E. coli. A diagnosis of JED was made. Response to topical treatment with zinc oxide cream and aqueous solution of eosin 2% was poor; also treatment with topical sucralfate 4% cream three times daily for 2 months led to only partial improvement. Thus, we suggested performing a surgical circumcision of the penis. The histology of the skin showed a non-specific inflammatory infiltrate and mild epidermal hyperplasia (figure 1D). Two months
A
B
C
D
after the surgery a complete resolution of the lesions was obtained. We report a case of JED treated with topical zinc oxide cream, aqueous solution of eosin 2% and topical sucralfate cream which achieved only little improvement, while removing the urinary stagnation by circumcision and using proper sanitary nappy rules led to a complete healing. JED is a form of diaper dermatitis usually characterized by 2-5 mm, umbilicated or eroded papules and nodules in the genital and perianal areas. Nevertheless, JED may occur with a heterogeneous pattern, some authors suggested that JED, perianal pseudoverrucous papules and nodules (PPPN) and granuloma gluteale infantum (GGI) could be all part of the same clinical spectrum, representing the result of chronic irritant contact dermatitis [3]. Probably all three diseases represent the same entity with different names, depending on the predominance of the clinical lesion: papules/erosions (JED), papules (PPPN) and nodules (GGI). One or another of these lesions may dominate at different times in the evolution of dermatitis or may be different inflammatory responses to various stimuli [4]. Some authors have performed many biopsies of these diseases and shown a histological overlap among JED, PPPN and GGI [5]. The differential diagnosis of JED includes: fungal and bacterial infections, cutaneous Crohn’s disease, Langerhans cell histiocytosis and acrodermatitis enteropatica. The pathogenesis involves several factors: urinary and fecal incontinence, infrequent diaper changing, persistence of detergent residues and use of rough toilet paper/diapers. The continued contact with urine increases the skin pH and makes it more susceptible to irritants [6], such as the fecal enzymes (proteases and lipases), which are activated by the alkaline pH [7]. The occlusion of the diaper and the friction of baby’s movements perpetuate the irritation process. Some authors attribute the increase of this disease to the growing use of reusable diapers, as they are considered more natural, ecologic and economic [8]. The diagnosis of JED is based on clinical features because histology shows only a non-specific polymorphous inflammatory infiltrate and sometimes mild epidermal hyperplasia. JED is not so uncommon but its recognition is important in order to avoid inappropriate treatment. The literature documents a poor efficacy of topical treatment with antibiotics, miconazole, zinc oxide and non-steroidal anti-inflammatory drugs, while some reports described successful treatment with topical sucralfate [9, 10]. However, full recovery is achieved only by removing the main irritating factors, as our case, in which topical treatment was ineffective and a surgical therapy was necessary. In our patient the spinal defect led to chronic urinary and fecal incontinence but the circumcision reduced the amount of urinary stagnation and we obtained the complete healing of the disease. Disclosure. Financial support: none. Conflict of interest: none. 1
Department of Dermatology, Department of Pediatric Science, Catholic University of Sacred Heart Largo A. Gemelli 8 00168 Rome, Italy 2 Health Services Research Unit, IDI-IRCCS, Rome, Italy 3
Figure 1. A-C) Multiple papular-erosive lesions, in some cases umbilicated, localized on the glans and on the foreskin; D) Histology of our case showed hyper-parakeratosis and a non-specific polymorphous inflammatory infiltrate in the interstitium.
252
Francesco RICCI1 Andrea PARADISI2 Francesca PERINO1 Rodolfo CAPIZZI1 Valentina PAOLUCCI3 Claudia RENDELI3 Cristina GUERRIERO1
EJD, vol. 24, n◦ 2, March-April 2014
P = 0.522
A 70
P < 0.001
P = 0.037
P = 0.002
65 60
56.2
53.5
STAI
55
49.9 50
45.5
45 40 35 30
Serum IgE
100 ≤ (n = 255)
< 100 (n = 13)
< 100 (n = 13)
100 ≤ (n = 255)
TA
B
SA
P = 0.202
P < 0.001
70
P = 0.239
65
P = 0.086
60 55
STAI
1. Hara M, Watanabe M, Tagami H. Jacquet erosive diaper dermatitis in a young girl with urinary incontinence. Pediatr Dermatol 1991; 8: 160-1. 2. Virgili A, Corazza M, Califano A. Diaper dermatitis in an adult. A case of erythema papuloerosive of Sevestre and Jacquet. J Reprod Med 1998; 43: 949-51. 3. Van L, Harting M, Rosen T. Jacquet erosive diaper dermatitis: a complication of adult urinary incontinence. Cutis 2008; 82: 72-4. 4. Rodriguez-Poblador J, González-Castro U, Herranz-Martínez S, Luelmo-Aguilar J. Jacquet erosive diaper dermatitis after surgery for Hirschsprung disease. Pediatr Dermatol 1998; 15: 46-7. 5. Robson KJ, Maughan JA, Purcell SD, Petersen MJ, Haefner HK, Lowe L. Erosive papulonodular dermatosis associated with topical benzocaine: a report of two cases and evidence that granuloma gluteale, pseudoverrucous papules, and Jacquet’s erosive dermatitis are a disease spectrum. J Am Acad Dermatol 2006; 55: S74-80. 6. Buckingham KW, Berg RW. Etiologic factors in diaper dermatitis: the role of feces. Pediatr Dermatol 1986; 3: 107-12. 7. Berg RW, Buckingham KW, Stewart RL. Etiologic factors in diaper dermatitis: the role of urine. Pediatr Dermatol 1986; 3: 102-6. 8. Maruani A, Lorette G, Barbarot S, et al. Re-emergence of papulonodular napkin dermatitis with use of reusable diapers: report of 5 cases. Eur J Dermatol 2013; 23: 246-9. 9. Markham T, Kennedy F, Collins P. Topical sucralfate for erosive irritant diaper dermatitis. Arch Dermatol 2000; 136: 1199-200. 10. Paradisi A, Capizzi R, Ghitti F, Lanza-Silveri S, Rendeli C, Guerriero C. Jacquet erosive diaper dermatitis: a therapeutic challenge. Clin Exp Dermatol 2009; 34: e385-6.
52.5
50.0
50
48.8 45.6
45
doi:10.1684/ejd.2014.2279
40 35 30
Serum IgE
Psychological aspects of patients with intrinsic atopic dermatitis
< 200 (n = 28)
200 ≤ (n = 240)
TA
EJD, vol. 24, n◦ 2, March-April 2014
200 ≤ (n = 240)
SA
P = 0.295
C
P = 0.024 P = 0.032
70
P = 0.121 65 60
57.3
55
STAI
Atopic dermatitis (AD) is classified into the IgE-high extrinsic and IgE-normal intrinsic types. While extrinsic AD is the classical type with high prevalence, intrinsic AD occupies approximately 20% of total AD with female predominance [1]. The skin barrier is perturbed in the extrinsic, but not intrinsic type [1], and thus filaggrin gene mutations are not a feature of intrinsic AD [2]. While the two types have comparable frequencies of circulating Th2 cells, intrinsic AD shows a significantly increased frequency of circulating interferon-␥-producing Th1 cells and lesional Th17 cells [2, 3]. We have shown that metal allergy may induce such an immunological state in intrinsic AD [1, 4]. In addition, we speculated that some itch-related factors, such as anxiety, might exacerbate intrinsic AD because of the refractory pruritus. The pathogenic role of the psychological factors in patients with AD remains controversial [5, 6]. There has been no report focusing on the impact of anxiety on intrinsic AD. To reveal the anxiety profile, the state-trait anxiety inventory (STAI) [7] is a useful questionnaire, which enables us to evaluate both state anxiety (SA) and trait anxiety (TA). We have previously shown that anxiety is significantly higher among subjects with AD than normal subjects, and TA was higher than SA in the AD group [8, 9]. Here, we sought to investigate the relationship between anxiety and intrinsic AD. Enrolled in this study were 268 Japanese AD patients, most of whom received our inpatient AD educational program. The diagnosis of AD was made according to the criteria of
< 200 (n = 28)
49.0
50
51.6 45.9
45 40 35 30
Serum IgE
< 100 (n = 8)
100 ≤ (n = 118)
TA
< 100 (n = 8)
100 ≤ (n = 118)
SA
Figure 1. Trait anxiety (TA) and State anxiety (SA) in the two groups of AD patients. The patients were divided into: (A) the IgE levels
