Dermatitis herpetiformis

A chronic autoimmune subepidermal bullous disease characterized by grouped pruritic lesions such as papules, urticarial plaques, erythema, and herpetiform vesiculae, with a predominantly symmetrical distribution on extensor surfaces of the elbows (90%), knees (30%), shoulders, buttocks, sacral region, and face of children and adults. Erosions, excoriations and hyperpigmentation usually follow. It may also appear as a consequence of gluten intolerance.



Input patient's signs and symptoms


Narrow down the case reports



Total: 163 (papers)

  


(per page)
Matched Phenotype  Gene  Mutation  MeSH
Rank
(Similarity)
PMID
(PMCID)
1
(54.2%)
8792511
Dapsone in rheumatoid arthritis.
Chang DJ, Lamothe M, Stevens RM, Sigal LH.
Semin Arthritis Rheum. 1996;25(6):390-403.
Proteinuria Jaundice Hemolytic anemia
Anti-Inflammatory Agents, Non-Steroidal Antirheumatic Agents Females Homo sapiens Male Middle Aged Rheumatoid Arthritis
2
(49.8%)
17805039
A rare condition associated with celiac disease: Evans syndrome.
Yarali N, Demirceken F, Kondolat M, Ozkasap S, Kara A, Tunc B.
J Pediatr Hematol Oncol. 2007;29(9):633-5.
Diabetes mellitus Anemia Vitamin B12 deficiency Thyroiditis
Celiac Disease Duodenum Females Hemoglobin Homo sapiens Syndrome
3
(46.5%)
19778310
Dapsone hypersensitivity syndrome with circulating 190-kDa and 230-kDa autoantibodies.
Chun JS, Yun SJ, Kim SJ, Lee SC, Won YH, Lee JB.
Clin Exp Dermatol. 2009;34(8):e798-801.
Jaundice Purpura Fever Lymphadenopathy
Adult Autoantibodies Dermatologic Agents Drug Eruptions Females Homo sapiens Hypersensitivity Syndrome Vasculitis
4
(45.7%)
3970060
Pernicious anemia and giant cell myocarditis. New association.
Kloin JE.
Am J Med. 1985;78(2):355-60.
Diabetes mellitus Vitiligo Anemia Granulomatosis
Anemia, Pernicious Autoimmune Diseases Giant Cell Granuloma Homo sapiens Male Middle Aged Myocarditis Myocardium Necrosis
5
(44.6%)
2266352
Gall-bladder perforation after long-term dapsone therapy.
Choy AM, Lang CC.
J Intern Med. 1990;228(4):409-10.
Cholecystitis Heinz bodies
Abdomen, Acute Anemia, Hemolytic Cholecystitis Dermatitis Herpetiformis Homo sapiens Male Time Factors
6
(43.7%)
15887437
Cardiomyopathy associated with celiac disease.
Goel NK, McBane RD, Kamath PS.
Mayo Clin Proc. 2005;80(5):674-6.
Diabetes mellitus Iron deficiency anemia Malabsorption
Angiotensin II Type 1 Receptor Blockers Cardiomyopathies Celiac Disease Diet Differential Diagnosis Echocardiography Endoscopy, Gastrointestinal Follow-Up Studies Gluten Homo sapiens Male Radiography, Thoracic X-Ray Computed Tomography
7
(38.2%)
22106118
Leukocytoclastic vasculitis as the presenting feature of dermatitis herpetiformis.
Naylor E, Atwater A, Selim MA, Hall R, Puri PK.
Arch Dermatol. 2011;147(11):1313-6.
Petechiae Edema
Biopsy Dermatitis Herpetiformis Fluorescent Antibody Technique, Direct Gluten-Free Diet Homo sapiens Male Middle Aged Transglutaminases Vasculitis, Leukocytoclastic, Cutaneous
8
(38.0%)
9401285
Rapid progression of chronic myelomonocytic leukemia following diaminodiphenyl sulphone treatment for dermatitis herpetiformis.
Kanda Y, Chiba S, Hirano N, Hirai H, Yazaki Y.
Int J Hematol. 1997;66(3):383-5.
Neutropenia Hepatomegaly
Adult Dermatitis Herpetiformis Disease Progression Homo sapiens Leprostatic Agents Leukemia, Myelomonocytic, Chronic Male Time Factors
9
(37.8%)
20093649
Pathophysiologic mechanisms, diagnosis, and management of dapsone-induced methemoglobinemia.
Ashurst JV, Wasson MN, Hauger W, Fritz WT.
J Am Osteopath Assoc. 2010;110(1):16-20.
Methemoglobinemia Contact dermatitis
Anti-Allergic Agents Contact Dermatitis Females Histamine H1 Antagonists Homo sapiens Leprostatic Agents Methemoglobinemia
10
(37.5%)
24637913
Transverse myelitis associated with an itchy rash and hyperckemia: neuromyelitis optica associated with dermatitis herpetiformis.
Iyer A, Rathnasabapathi D, Elsone L, Mutch K, Terlizzo M, Footitt D, Jacob A.
JAMA Neurol. 2014;71(5):630-3.
Iron deficiency anemia Vomiting
AQP4
Creatine Kinase Dermatitis Herpetiformis Differential Diagnosis Exanthema Females Homo sapiens Middle Aged Myelitis, Transverse Neuromyelitis Optica Pruritus
        

Phenotype(s) retrieved from Orphanet

    Total: 14

HPO ID Term Frequency
HP:0000989 Pruritus Very frequent (99-80%)
HP:0001025 Urticaria Very frequent (99-80%)
HP:0001935 Microcytic anemia Very frequent (99-80%)
HP:0002024 Malabsorption Very frequent (99-80%)
HP:0002757 Recurrent fractures Very frequent (99-80%)
HP:0002960 Autoimmunity Very frequent (99-80%)
HP:0008066 Abnormal blistering of the skin Very frequent (99-80%)
HP:0010783 Erythema Very frequent (99-80%)
HP:0012733 Macule Very frequent (99-80%)
HP:0200037 Skin vesicle Very frequent (99-80%)
HP:0000964 Eczema Frequent (79-30%)
HP:0000969 Edema Occasional (29-5%)
HP:0002653 Bone pain Occasional (29-5%)
HP:0100725 Lichenification Occasional (29-5%)


Phenotype(s) retrieved from case reports

    Total: 73

HPO ID Term # of case reports
HP:0010783 Erythema 10
HP:0000819 Diabetes mellitus 6
HP:0000112 Nephropathy 5
HP:0001045 Vitiligo 5
HP:0001025 Urticaria 4
HP:0000099 Glomerulonephritis 3
HP:0000967 Petechiae 3
HP:0002090 Pneumonia 3
HP:0011473 Villous atrophy 3
HP:0032282 Contact dermatitis 3
HP:0100646 Thyroiditis 3
HP:0000836 Hyperthyroidism 2
HP:0001271 Polyneuropathy 2
HP:0001370 Rheumatoid arthritis 2
HP:0012234 Agranulocytosis 2
HP:0025615 Abscess 2
HP:0030731 Carcinoma 2
HP:0100242 Sarcoma 2
HP:0200034 Papule 2
HP:0000083 Renal insufficiency 1
HP:0000100 Nephrotic syndrome 1
HP:0000230 Gingivitis 1
HP:0000763 Sensory neuropathy 1
HP:0000771 Gynecomastia 1
HP:0000821 Hypothyroidism 1
HP:0000829 Hypoparathyroidism 1
HP:0000846 Adrenal insufficiency 1
HP:0000952 Jaundice 1
HP:0000964 Eczema 1
HP:0000969 Edema 1
HP:0000988 Skin rash 1
HP:0001101 Iritis 1
HP:0001251 Ataxia 1
HP:0001369 Arthritis 1
HP:0001396 Cholestasis 1
HP:0001508 Failure to thrive 1
HP:0001638 Cardiomyopathy 1
HP:0001701 Pericarditis 1
HP:0001733 Pancreatitis 1
HP:0001744 Splenomegaly 1
HP:0001891 Iron deficiency anemia 1
HP:0001903 Anemia 1
HP:0002013 Vomiting 1
HP:0002014 Diarrhea 1
HP:0002019 Constipation 1
HP:0002024 Malabsorption 1
HP:0002027 Abdominal pain 1
HP:0002243 Protein-losing enteropathy 1
HP:0002254 Intermittent diarrhea 1
HP:0002570 Steatorrhea 1
HP:0002583 Colitis 1
HP:0002862 Bladder carcinoma 1
HP:0002900 Hypokalemia 1
HP:0002960 Autoimmunity 1
HP:0003198 Myopathy 1
HP:0004322 Short stature 1
HP:0005523 Lymphoproliferative disorder 1
HP:0009830 Peripheral neuropathy 1
HP:0011837 Partial IgA deficiency 1
HP:0012180 Cystic medial necrosis 1
HP:0012378 Fatigue 1
HP:0012393 Allergy 1
HP:0012538 Gluten intolerance 1
HP:0012722 Heart block 1
HP:0025084 Folliculitis 1
HP:0031364 Ecchymosis 1
HP:0031500 Abdominal mass 1
HP:0040075 Hypopituitarism 1
HP:0100279 Ulcerative colitis 1
HP:0100614 Myositis 1
HP:0100699 Scarring 1
HP:0100753 Schizophrenia 1
HP:0100820 Glomerulopathy 1


Causative gene(s) retrieved from Orphanet

    Total: 0

Gene Symbol Gene Name Entrez Gene ID