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Itchy Hyperkeratotic Acral Plaque in a Patient With Chronic Calcific Pancreatitis
ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58
Bilal Hameed, Uma Mahadevan, and Kay Washington, Section Editors
Itchy Hyperkeratotic Acral Plaque in a Patient With Chronic Calcific Pancreatitis Q2
T. P. Afra,1 Muhammed T. Razmi,1 and Saleem Suhail2 1 2
Department of Dermatology, IQRAA Aesthetics, IQRAA International Hospital and Research Centre, Calicut, Kerala, India; Department of Histopathology, Noble Diagnostics, Kannur, Kerala, India
Question: A woman in her 40s complained of itchy, thick plaque of 5 years duration on her left foot. She had had a history of chronic calcific pancreatitis for which she had undergone Roux-en-Y lateral pancreaticojejunostomy followed by the Frey procedure some years later. She recalled the presence of a rash on both feet during this period. Even though she was on pancreatic enzyme replacement, she complained of occasional claycolored stools. She was on topical steroids and oral antihistamines from multiple care providers for the cutaneous lesion with no significant improvement in her symptoms. On examination, we noticed hyperpigmented, thick keratotic plaque on her left hind foot near the sole margin (Figure A). Histopathology (Figure B; hematoxylin and eosin stain; original magnification 10) revealed acanthosis, diffuse parakeratosis, and necrotic keratinocytes tracking the acrosyringium (Figure B, green arrows) in the epidermis with lymphocytic infiltrates in the dermis. Her serum zinc level was 34.68 mg/dL (reference range, 54-151 mg/dL). Viral markers including hepatitis C virus (HCV) were nonreactive. The rest of the routine blood investigations were within normal limits. What is the diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.
Q1
Conflicts of interest The authors disclose no conflicts. © 2019 by the AGA Institute 0016-5085/$36.00 https://doi.org/10.1053/j.gastro.2019.05.050
Gastroenterology 2019;-:e1–e2 DIS 5.6.0 DTD YGAST62679 proof 4 October 2019 3:53 pm ce
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ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI 117 118 119 120 121 122 123 124 125 126 127 128 129 130 131 132 133 134 135 136 137 138 139 140 141 142 143 144 145 146 147 148 149 150 151 152 153 154 155 156 157 158 159 160 161 162 163 164 165 166 167 168 169 170 171 172 173 174
Answer to: Image 2: Necrolytic Acral Erythema Owing to Zinc Deficiency Associated with Pancreatic Insufficiency At the 3-month follow-up after oral replacement of zinc (50 mg/d), she had marked relief in the itching and the keratotic plaques subsided leaving hyperpigmentation. Necrolytic acral erythema (NAE), which is characterized by psoriasiform acral plaques with dusky peripheral erythema, has been considered as a cutaneous marker of HCV infection. However, there are reports of seronegative NAE, like those associated with zinc deficiency.1 In fact, zinc dysmetabolism induced by a virus has been attributed to be the cause of HCV related NAE. Zinc is required for proper epidermal keratinocyte proliferation and differentiation, and its deficiency may lead to keratinocyte necrosis. Cases of zinc deficiency have been reported with fat malabsorption as in cystic fibrosis. Pancreatic enzyme supplementation was found to improve zinc absorption in them. It has been postulated that zinc forms insoluble complexes with fat in the gut lumen leading to impairment in its absorption.2 A previous single report of NAE in chronic calcific pancreatitis was found to be associated with HCV induced zinc dysmetabolism.3 To the best of our knowledge, seronegative NAE associated with fat malabsorption in chronic calcific pancreatitis has not been reported. A possibility of NAE associated with zinc deficiency should be considered in patients with pancreatic insufficiency presenting with itchy, pigmented, hyperkeratotic acral plaques.
References 1. 2. 3.
Najarian DJ, Lefkowitz I, Balfour E, et al. Zinc deficiency associated with necrolytic acral erythema. J Am Acad Dermatol 2006;55:S108–S110. Easley D, Krebs N, Jefferson M, et al. Effect of pancreatic enzymes on zinc absorption in cystic fibrosis. J Pediatr Gastroenterol Nutr 1998;26:136–139. Li F, Kaffenberger B, Hart PA. An unusual rash during evaluation for chronic pancreatitis. Gastroenterology 2017; 152:e4–e5.
e2 DIS 5.6.0 DTD YGAST62679 proof 4 October 2019 3:53 pm ce
175 176 177 178 179 180 181 182 183 184 185 186 187 188 189 190 191 192 193 194 195 196 197 198 199 200 201 202 203 204 205 206 207 208 209 210 211 212 213 214 215 216 217 218 219 220 221 222 223 224 225 226 227 228 229 230 231 232
Bilal Hameed, Uma Mahadevan, and Kay Washington, Section Editors
Itchy Hyperkeratotic Acral Plaque in a Patient With Chronic Calcific Pancreatitis Q2
T. P. Afra,1 Muhammed T. Razmi,1 and Saleem Suhail2 1 2
Department of Dermatology, IQRAA Aesthetics, IQRAA International Hospital and Research Centre, Calicut, Kerala, India; Department of Histopathology, Noble Diagnostics, Kannur, Kerala, India
Question: A woman in her 40s complained of itchy, thick plaque of 5 years duration on her left foot. She had had a history of chronic calcific pancreatitis for which she had undergone Roux-en-Y lateral pancreaticojejunostomy followed by the Frey procedure some years later. She recalled the presence of a rash on both feet during this period. Even though she was on pancreatic enzyme replacement, she complained of occasional claycolored stools. She was on topical steroids and oral antihistamines from multiple care providers for the cutaneous lesion with no significant improvement in her symptoms. On examination, we noticed hyperpigmented, thick keratotic plaque on her left hind foot near the sole margin (Figure A). Histopathology (Figure B; hematoxylin and eosin stain; original magnification 10) revealed acanthosis, diffuse parakeratosis, and necrotic keratinocytes tracking the acrosyringium (Figure B, green arrows) in the epidermis with lymphocytic infiltrates in the dermis. Her serum zinc level was 34.68 mg/dL (reference range, 54-151 mg/dL). Viral markers including hepatitis C virus (HCV) were nonreactive. The rest of the routine blood investigations were within normal limits. What is the diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.
Q1
Conflicts of interest The authors disclose no conflicts. © 2019 by the AGA Institute 0016-5085/$36.00 https://doi.org/10.1053/j.gastro.2019.05.050
Gastroenterology 2019;-:e1–e2 DIS 5.6.0 DTD YGAST62679 proof 4 October 2019 3:53 pm ce
59 60 61 62 63 64 65 66 67 68 69 70 71 72 73 74 75 76 77 78 79 80 81 82 83 84 85 86 87 88 89 90 91 92 93 94 95 96 97 98 99 100 101 102 103 104 105 106 107 108 109 110 111 112 113 114 115 116
ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI 117 118 119 120 121 122 123 124 125 126 127 128 129 130 131 132 133 134 135 136 137 138 139 140 141 142 143 144 145 146 147 148 149 150 151 152 153 154 155 156 157 158 159 160 161 162 163 164 165 166 167 168 169 170 171 172 173 174
Answer to: Image 2: Necrolytic Acral Erythema Owing to Zinc Deficiency Associated with Pancreatic Insufficiency At the 3-month follow-up after oral replacement of zinc (50 mg/d), she had marked relief in the itching and the keratotic plaques subsided leaving hyperpigmentation. Necrolytic acral erythema (NAE), which is characterized by psoriasiform acral plaques with dusky peripheral erythema, has been considered as a cutaneous marker of HCV infection. However, there are reports of seronegative NAE, like those associated with zinc deficiency.1 In fact, zinc dysmetabolism induced by a virus has been attributed to be the cause of HCV related NAE. Zinc is required for proper epidermal keratinocyte proliferation and differentiation, and its deficiency may lead to keratinocyte necrosis. Cases of zinc deficiency have been reported with fat malabsorption as in cystic fibrosis. Pancreatic enzyme supplementation was found to improve zinc absorption in them. It has been postulated that zinc forms insoluble complexes with fat in the gut lumen leading to impairment in its absorption.2 A previous single report of NAE in chronic calcific pancreatitis was found to be associated with HCV induced zinc dysmetabolism.3 To the best of our knowledge, seronegative NAE associated with fat malabsorption in chronic calcific pancreatitis has not been reported. A possibility of NAE associated with zinc deficiency should be considered in patients with pancreatic insufficiency presenting with itchy, pigmented, hyperkeratotic acral plaques.
References 1. 2. 3.
Najarian DJ, Lefkowitz I, Balfour E, et al. Zinc deficiency associated with necrolytic acral erythema. J Am Acad Dermatol 2006;55:S108–S110. Easley D, Krebs N, Jefferson M, et al. Effect of pancreatic enzymes on zinc absorption in cystic fibrosis. J Pediatr Gastroenterol Nutr 1998;26:136–139. Li F, Kaffenberger B, Hart PA. An unusual rash during evaluation for chronic pancreatitis. Gastroenterology 2017; 152:e4–e5.
e2 DIS 5.6.0 DTD YGAST62679 proof 4 October 2019 3:53 pm ce
175 176 177 178 179 180 181 182 183 184 185 186 187 188 189 190 191 192 193 194 195 196 197 198 199 200 201 202 203 204 205 206 207 208 209 210 211 212 213 214 215 216 217 218 219 220 221 222 223 224 225 226 227 228 229 230 231 232