MetaADEDB 2.0 @ LMMD
Papillon-Lefevre Disease
(UMLS:C0030360)
Definition:
Rare, autosomal recessive disorder occurring between the first and fifth years of life. It is characterized by palmoplantar keratoderma with periodontitis followed by the premature shedding of both deciduous and permanent teeth. Mutations in the gene for CATHEPSIN C have been associated with this disease.
UMLS ID:
C0030360
MeSH ID:
D010214
Classification 1:
Name:
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
MeSH Tree Number(s):
C16.320.850.475.600
Classification 2:
Name:
Skin and Connective Tissue Diseases
MeSH Tree Number(s):
C17.800.428.435.600|C17.800.827.475.600
Synonym(s)
1.
Papillon-Lefevre Disease
2.
Juvenile periodontosis with hyperkeratosis
3.
PAPILLON-LEFEVRE SYNDROME
4.
Papillon Lefevre syndrome
5.
Papillon-Lefevre Syndrome
6.
Papillon-Lefevre syndrome
7.
Papillon-Lef猫vre syndrome
8.
Periodontosis-Papillon-Lefebvre syndrome
9.
papillon lefevre syndrome
10.
papillon-lefevre syndrome
Associated Drug(s)
NameNumber of ReportsReference(s)Data Source
1Acitretin16008657CTD
Similar ADE(s)
NameSemantic Similarity
1Porokeratosis44.3%
2Epidermolysis Bullosa37%
3Dermatitis, Atopic35.8%
4Cutis Laxa33.7%
5Xeroderma Pigmentosum32.6%
6Ectodermal Dysplasia30.9%
7Aplasia Cutis Congenita30.9%
8Pyoderma Gangrenosum29.5%
9Ichthyosis, X-Linked28%
10Pseudoxanthoma Elasticum27.2%
11Lupus Erythematosus, Cutaneous26.8%
12Panniculitis26.8%
13Systemic Scleroderma26.8%
14Morphea26.8%
15Xeroderma26.5%
16Albinism25.7%
17Sjogren-Larsson Syndrome25%
18Breast Diseases24.8%
19Dermatitis24.8%
20Exanthema24.8%
21Facial Dermatoses24.8%
22Hair Diseases24.8%
23Hand Dermatoses24.8%
24keratoacanthoma24.8%
25Nail Diseases24.8%
26Photosensitivity Disorders24.8%
27Prurigo24.8%
28Pyoderma24.8%
29Rosacea24.8%
30Sebaceous Gland Diseases24.8%
31Skin Diseases, Vesiculobullous24.8%
32Skin Ulcer24.8%
33Sweat Gland Diseases24.8%
34Skin Diseases, Papulosquamous24.8%
35Skin Diseases, Vascular24.8%
36Acneiform Eruptions24.8%
37Ocular Rosacea24.8%
38Subcorneal pustular dermatosis24.8%
39Erythematotelangiectatic Rosacea24.8%
40Papulopustular Rosacea24.8%
41Chronic Actinic Dermatitis24.8%
42Cholesteatoma24.4%
43Parakeratosis24.4%
44Porphyrias, Hepatic24.2%
45Acne Vulgaris23.5%
46Contact Dermatitis23.5%
47Eczema23.5%
48Erythema Multiforme23.5%
49Intertrigo23.5%
50Neurodermatitis23.5%
51Rhinophyma23.5%
52Perioral Dermatitis23.5%
53Acne23.5%
54Skin Abnormalities23.5%
55Hyperkeratosis, Epidermolytic21.9%
56Acrodermatitis21.3%
57Gianotti-Crosti Syndrome21.3%
58Foot Diseases20.7%
59Skin Diseases, Infectious20.1%
60Skin Neoplasms20.1%
61Pigmentation Disorders20.1%
62Dermatitis Herpetiformis19.9%
63Actinic keratosis19.9%
64Lipomatosis19.9%
65Pruritus19.9%
66Erythema19.9%
67Necrolytic Migratory Erythema19.9%
68Cutaneous Fistula19.9%
69Nephrogenic Fibrosing Dermopathy19.9%
70Nephrogenic Systemic Fibrosis19.9%
71Sebaceous Gland Neoplasms19.7%
72Foot Ulcer19.5%
73Dermatitis, Phototoxic19.4%
74Pityriasis Lichenoides et Varioliformis Acuta19.4%
75Pityriasis Lichenoides19.4%
76Phototoxicity19.4%
77Dermatomyositis18.9%
78Necrobiosis Lipoidica Diabeticorum18.7%
79Panniculitis, Lupus Erythematosus18.6%
80Nail-Patella Syndrome17.2%
81Behcet Syndrome17.2%
82Dermatitis, Photoallergic16.6%
83Erythema Induratum16.5%
84Mastocytosis16.5%
85Urticaria Pigmentosa16.2%
86Variegate Porphyria15.7%
87Acute intermittent porphyria15.7%
88Porphyria Cutanea Tarda15.7%
89Lupus Erythematosus, Discoid15.7%
90Scleroderma, Limited15.7%
91Dermatitis, Irritant15.5%
92Acne Conglobata15.5%
93Connective Tissue Diseases15.4%
94Anhidrosis14.6%
95Pressure Ulcer14.6%
96Folliculitis14.6%
97Foot Dermatoses14.6%
98Gynecomastia14.6%
99Hypertrichosis14.6%
100Hypohidrosis14.6%
101Hypotrichosis14.6%
102Leg Ulcer14.6%
103Miliaria14.6%
104Parapsoriasis14.6%
105Pityriasis14.6%
106Psoriasis14.6%
107Sweet Syndrome14.6%
108Hidradenitis14.6%
109Onycholysis14.6%
110Miliaria Rubra14.6%
111Hyperpigmentation14.6%
112Lichenoid Eruptions14.6%
113Chloracne14.6%
114Erythema Infectiosum14.2%
115Exanthema Subitum14.1%
116Erythema Nodosum13.9%
117Dermatitis, Occupational13.4%
118Dermatitis, Allergic Contact13%
119Toxic Epidermal Necrolysis12.9%
120Stevens-Johnson Syndrome12.9%
121Tinea Capitis12.4%
122Onychomycosis12.4%
123Keratosis12.4%
124Ecthyma12.2%
125Granuloma Annulare12%
126Acantholysis11.9%
127Breast Cyst11.9%
128Pemphigoid, Benign Mucous Membrane11.9%
129Pemphigus11.9%
130Piedra11.9%
131Sunburn11.9%
132Argyria11.8%
133Hirsutism11.8%
134Lactation Disorder11.8%
135Mastitis11.8%
136Urticaria11.8%
137Adiposis Dolorosa11.7%
138Pruritus Vulvae11.7%
139Mastocytoma11.7%
140Pruritus Ani11.7%
141Diaper Rash11.5%
142Osteogenesis Imperfecta11.5%
143Angioedemas, Hereditary11.2%
144Menkes Kinky Hair Syndrome10.4%
145Neoplastic Syndromes, Hereditary10.3%
146Marfan Syndrome10%
147Dermatomycoses9.9%
148Mucocutaneous Lymph Node Syndrome9.9%
149Skin Diseases, Bacterial9.9%
150CHARGE Syndrome9.9%
151Multiple Epiphyseal Dysplasia9.9%
152Osteochondrodysplasias9.9%
153Pelger-Huet Anomaly9.9%
154Werner Syndrome9.9%
155Myasthenic Syndromes, Congenital9.9%
156Drug Eruptions9.8%
157Lipodystrophy9.8%
158Livedo Reticularis9.8%
159Malignant Atrophic Papulosis9.8%
160Keratoderma, Palmoplantar9.8%
161Cystic Fibrosis9.8%
162Hidradenitis Suppurativa9.8%
163Polyarteritis Nodosa9.7%
164Takayasu Arteritis9.7%
165Erythema Chronicum Migrans9.7%
166Lichen Planus9.7%
167Melanosis9.7%
168Chloasma9.7%
169Pityriasis Rosea9.7%
170Pityriasis Rubra Pilaris9.7%
171Vitiligo9.7%
172Lichen Nitidus9.7%
173Aicardi's syndrome9.3%
174Dwarfism8.6%
175Tinea Pedis8.4%
176Anemia, Sickle Cell8.4%
177Thalassemia8.4%
178Muscular Dystrophy, Duchenne8.4%
179Collagen Diseases8.3%
180Mixed Connective Tissue Disease8.3%
181Mucinoses8.3%
182Muscular Dystrophy8.2%
183Beckwith-Wiedemann Syndrome8.2%
184Wolf-Hirschhorn Syndrome8.2%
185Fragile X Syndrome8%
186Alopecia7.9%
187Sweating, Gustatory7.9%
188Androgenetic Alopecia7.9%
189Auriculotemporal Syndrome7.9%
190Lice Infestations7.8%
191Varicose Ulcer7.8%
192Breast Neoplasms, Male7.8%
193Triple Negative Breast Neoplasms7.8%
194Mycetoma7.8%
195Galactorrhea7.7%
196Peutz-Jeghers Syndrome7.7%
197Deformity7.7%
198Cockayne Syndrome7.7%
199Familial Mediterranean Fever7.3%
200Mucopolysaccharidosis II7.3%
201Acanthosis Nigricans7.2%
202Alopecia Areata7.2%
203Lentigo7.2%
204Autoimmune Lymphoproliferative Syndrome7.2%
205Smith-Magenis syndrome7.1%
206Cri-du-Chat Syndrome7%
207Down Syndrome7%
208Trisomy 217%
209Cartilage Diseases6.9%
210Rheumatism6.9%
211Chondromalacia6.9%
212Alstrom Syndrome6.8%
213Lupus Erythematosus, Systemic6.7%
214Overlap syndrome6.7%
215Homocystinuria6.6%
216Larva Migrans6.6%
217Leishmaniasis6.6%
218Molluscum Contagiosum6.6%
219Angioedema6.6%
220Wiskott-Aldrich Syndrome6.5%
221Onchocerciasis6.5%
222Chromoblastomycosis6.5%
223Dermatophytosis6.5%
224Sporotrichosis6.5%
225Tinea6.5%
226Tinea Versicolor6.5%
227Trichophytosis6.5%
228Hyalohyphomycosis6.5%
229Papillon-Lefevre Disease6.5%
230Acute Generalized Exanthematous Pustulosis6.5%
231Rett Syndrome6.4%
232Kartagener Syndrome6.4%
233Holoprosencephaly6.2%
234Fetal Diseases6.2%
235Prader-Willi Syndrome6.1%
236Kallmann Syndrome6%
237Lichen Planus, Oral5.9%
238Amino Acid Metabolism, Inborn Errors5.9%
239Carbohydrate Metabolism, Inborn Errors5.9%
240Metal Metabolism, Inborn Errors5.9%
241Progeria5.9%
242Lysosomal Storage Diseases5.9%
243Cytochrome-c Oxidase Deficiency5.9%
244Peroxisomal Disorders5.9%
245Myotonic Dystrophy5.9%
246Giant Cell Arteritis5.8%
247Temporal Arteritis5.8%
248CREST Syndrome5.7%
249Warts5.7%
250Candidiasis, Chronic Mucocutaneous5.6%
251Cutaneous Candidiasis5.6%
252Polycystic Kidney Diseases5.6%
253Williams Syndrome5.6%
254Erysipelas5.6%
255Rubinstein-Taybi Syndrome5.6%
256Serum Sickness5.6%
257Staphylococcal Skin Infections5.6%
258Erythrasma5.5%
259Carcinoma, Lobular5.5%
260Arthritis, Psoriatic5.5%
261Sickle Cell Trait5.5%
262Dupuytren Contracture5.5%
263Neuronal Ceroid-Lipofuscinoses5.5%
264Tuberous Sclerosis5.5%
265Primary Ciliary Dyskinesia5.4%
266Smith-Lemli-Opitz Syndrome5.4%
267Mucopolysaccharidosis III5.2%
268Noonan Syndrome5.1%
269Retinal Dysplasia5.1%
270Aniridia5%
271Ataxia Telangiectasia5%
272Retinitis Pigmentosa4.9%
273Pigmentary retinopathy4.9%
274Hepatolenticular Degeneration4.9%
275Chronic granulomatous disease4.9%
276Scleromyxedema4.9%
277Amyloid Neuropathies, Familial4.9%
278Leishmaniasis, Cutaneous4.9%
279Welander Distal Myopathy4.9%
280Eczema Herpeticum4.9%
281Adrenoleukodystrophy4.8%
282Fusariosis4.8%
283Cellulitis4.7%
284Refsum Disease4.6%
285Acute Chest Syndrome4.4%
286Blastomycosis4.4%
287HIV-Associated Lipodystrophy Syndrome4.3%
288Duane Retraction Syndrome4.3%
289Fabry Disease4.3%
290Galactosemias4.3%
291Urea Cycle Disorders, Inborn4.3%
292Tyrosinemias4.3%
293Meconium Aspiration Syndrome4.2%
294Myotonia Congenita4.2%
295Thrombasthenia4.2%
296Antithrombin III Deficiency4.2%
297Protein C Deficiency4.2%
298Brain Diseases, Metabolic, Inborn4.2%
299Afibrinogenemia4.2%
300Dystonia Musculorum Deformans4.2%
301Factor VII Deficiency4.2%
302Factor X Deficiency4.2%
303Factor XII Deficiency4.2%
304Hemophilia A4.2%
305Activated Protein C Resistance4.2%
306Factor II deficiency4.2%
307Factor VIII Deficiency4.2%
308Factor V deficiency4.2%
309Factor XI Deficiency4.2%
310Hypoprothrombinemias4.2%
311Epidermodysplasia Verruciformis4.2%
312Herpes Labialis4.2%
313Fanconi Anemia4.2%
314Diabetic Foot4.2%
315Lafora Disease4.2%
316Unverricht-Lundborg Syndrome4.2%
317Anemia, Diamond-Blackfan4.1%
318Abdominal Cramps4.1%
319Abnormalities, Drug-Induced4.1%
320Amniotic Band Syndrome4.1%
321Asphyxia Neonatorum4.1%
322Infant, Premature, Diseases4.1%
323Situs Inversus4.1%
324Infantile Colic4.1%
325Staphylococcal Scalded Skin Syndrome4.1%
326Hereditary Angioedema Type III4%
327Hereditary Angioedema Types I and II4%
328Rhinoscleroma4%
329Mesenteric Panniculitis4%
330Panniculitis, Peritoneal4%
331Tietze's Syndrome4%
332Orbital Cellulitis4%
333Myxoid cyst4%
334Canavan Disease4%
335Alexander Disease4%
336Myxedema3.9%
337Glycogen storage disease type II3.9%
338Optic Atrophy, Hereditary, Leber3.9%
339Alkaptonuria3.9%
340Glycogen Storage Disease3.9%
341Hypophosphatasia3.9%
342Propionic acidemia3.9%
343Dihydropyrimidine Dehydrogenase Deficiency3.9%
344Cystinosis3.9%
345Hyperphosphaturia3.9%
346Multiple Endocrine Neoplasia3.8%
347Laryngomalacia3.8%
348Mobius Syndrome3.8%
349Pectus carinatum3.8%
350Zellweger Syndrome3.8%
351Congenital Hypothyroidism3.7%
352Condylomata Acuminata3.7%
353Polycystic Kidney, Autosomal Dominant3.7%
354Furunculosis3.7%
355furuncle3.7%
356Impetigo3.6%
357Classical Lissencephalies and Subcortical Band Heterotopias3.5%
358Eye Abnormalities3.4%
359Cardiovascular Abnormalities3.4%
360carbuncle3.4%
361Leigh Disease3.4%
362Lymphatic Abnormalities3.4%
363Congenital Microtia3.4%
364Osteochondritis3.4%
365Polymyalgia Rheumatica3.4%
366Anemia, Neonatal3.3%
367Persistent Fetal Circulation Syndrome3.3%
368Congenital diaphragmatic hernia3.3%
369Umbilical hernia3.3%
370Basal Cell Nevus Syndrome3.3%
371Keloid3.2%
372Charcot-Marie-Tooth Disease3.2%
373Antley-Bixler Syndrome Phenotype3.2%
374Hypolipoproteinemias3.2%
375Hyperlipidemia, Familial Combined3.2%
376Hyperlipoproteinemia Type III3.2%
377Gaucher Disease3%
378Huntington Disease2.9%
379Urogenital Abnormalities2.9%
380Glycogen Storage Disease Type I2.9%
381Glycogen Storage Disease Type V2.9%
382Carbamoyl-Phosphate Synthase I Deficiency Disease2.9%
383Rheumatoid Arthritis2.9%
384Juvenile arthritis2.9%
385Neonatal Abstinence Syndrome2.8%
386Familial Periodic Paralysis2.8%
387Bronchomalacia2.8%
388Tracheomalacia2.8%
389Lactose Intolerance2.8%
390Lupus Nephritis2.8%
391Hyperhomocysteinemia2.8%
392Fetal Growth Retardation2.8%
393Renal Aminoacidurias2.8%
394Fanconi Syndrome2.8%
395Pseudohypoaldosteronism2.8%
396Liddle Syndrome2.8%
397Fetal Hypoxia2.7%
398Congenital nystagmus2.7%
399Fetal Alcohol Spectrum Disorders2.7%
400Hyperlipoproteinemia Type IV2.7%
401Hyperlipoproteinemia Type V2.7%
402Toxoplasmosis, Congenital2.7%
403Anencephaly2.7%
404Syphilis, Congenital2.7%
405Nephroblastoma2.7%
406Tay-Sachs Disease2.5%
407Gout2.5%
408Niemann-Pick Disease, Type C2.5%
409Hydrops Fetalis2.5%
410Multiple Endocrine Neoplasia Type 12.4%
411Pseudohypoparathyroidism2.4%
412Prune Belly Syndrome2.4%
413Twins, Conjoined2.4%
414Waardenburg Syndrome2.4%
415Friedreich Ataxia2.4%
416Adenomatous Polyposis Coli2.4%
417Renal tubular acidosis2.4%
418Klinefelter Syndrome2.4%
419Hypoalphalipoproteinemias2.4%
420Congenital Hyperinsulinism2.4%
421Chorioamnionitis2.4%
422Maxillofacial Abnormalities2.4%
423Dural Arteriovenous Fistula2.4%
424Dextrocardia2.3%
425Turner Syndrome2.2%
426Rheumatoid Nodule2.2%
427Hypokalemic periodic paralysis2.1%
428Abnormalities, Radiation-Induced2.1%
429Cystinuria2.1%
430Anophthalmos2%
431Anus, Imperforate2%
432Hydranencephaly2%
433Microphthalmos2%
434Neural Tube Defects2%
435Paralysis, Obstetric2%
436Retinopathy of Prematurity2%
437Tethered Cord Syndrome2%
438Iniencephaly2%
439Craniorachischisis2%
440Limb Deformities, Congenital2%
441Exencephaly2%
442Septo-Optic Dysplasia2%
443Craniofacial Abnormalities2%
444Cortical Dysplasia2%
445Malformations of Cortical Development2%
446Anorectal Malformations2%
447Bloom Syndrome2%
448Bronchopulmonary Dysplasia1.9%
449Glycosuria, Renal1.9%
450Familial Hypophosphatemic Rickets1.9%
451Scimitar Syndrome1.9%
452Felty Syndrome1.9%
453Arthritis, Gouty1.8%
454Ophthalmia Neonatorum1.8%
455Blepharophimosis1.7%
456Laryngostenosis1.7%
457Mouth Abnormalities1.7%
458Respiratory Distress Syndrome, Newborn1.7%
459Esophageal Atresia1.7%
460Intestinal Atresia1.7%
461Horseshoe Kidney1.7%
462Pectus excavatum1.7%
463Klippel-Feil Syndrome1.6%
464Gastroschisis1.6%
465Synostosis1.6%
466Lymphangiectasis, Intestinal1.6%
467Rheumatoid Vasculitis1.6%
468Craniosynostosis1.6%
469Syndactyly1.6%
470Brachycephaly1.6%
471Microcephaly1.6%
472Macrocephaly1.6%
473MELAS Syndrome1.6%
474Denys-Drash Syndrome1.6%
475Acrocephalosyndactylia1.5%
476Arthrogryposis1.4%
477Choanal Atresia1.4%
478Tracheobronchomegaly1.4%
479Sicca Syndrome1.4%
480Sjogren's Syndrome1.4%
481Laryngocele1.4%
482Choledochal Cyst1.4%
483Hermaphroditism1.4%
484Disorders of Sex Development1.4%
485Cryptorchidism1.4%
486Hypospadias1.4%
487Central Nervous System Cysts1.4%
488Dandy-Walker Syndrome1.4%
489Leukomalacia, Periventricular1.4%
490POEMS Syndrome1.4%
491Nesidioblastosis1.4%
492Lupus Vasculitis, Central Nervous System1.4%
493Porencephaly1.4%
494Amelia1.3%
495Arachnodactyly1.3%
496Ectopia Cordis1.3%
497Ectromelia1.3%
498Hemimelia1.3%
499Meningomyelocele1.3%
500Phocomelia1.3%
501Sirenomelia1.3%
502Spina Bifida1.3%
503Polydactyly1.3%
504Brachydactyly1.3%
505Plagiocephaly1.3%
506Lower Extremity Deformities, Congenital1.3%
507Upper Extremity Deformities, Congenital1.3%
508Single umbilical artery1.3%
509Cleft Palate1.3%
510Jaw Abnormalities1.3%
511Bladder Exstrophy1.2%
512Epispadias1.2%
513Multicystic Dysplastic Kidney1.2%
514Poland Syndrome1.2%
515Kernicterus1.1%
516Aortic coarctation1.1%
517Cor Triatriatum1.1%
518Coronary Vessel Anomalies1.1%
519Dental Enamel Hypoplasia1.1%
520Patent ductus arteriosus1.1%
521Ebstein Anomaly1.1%
522Heart Septal Defects1.1%
523Hyaline Membrane Disease1.1%
524Hypodontia1.1%
525Macrostomia1.1%
526Meningocele1.1%
527Microstomia1.1%
528Tetralogy of Fallot1.1%
529Transposition of Great Vessels1.1%
530Hypoplastic Left Heart Syndrome1.1%
531May-Thurner Syndrome1.1%
532Encephalocele1.1%
533Craniofacial Dysostosis1.1%
534Wolff-Parkinson-White Syndrome1.1%
535Micrognathism1%
536Pierre Robin Syndrome1%
537Dermal Sinus1%
538Spina Bifida Cystica1%
539Spina Bifida Occulta1%
540Lissencephaly1%
541Polymicrogyria1%
542Pachygyria1%
543Schizencephaly1%
544Periventricular Nodular Heterotopia1%
545Long QT Syndrome0.9%
546Platybasia0.9%
547Tricuspid Atresia0.9%
548Arrhythmogenic Right Ventricular Dysplasia0.9%
549Gonadal Dysgenesis0.9%
550Adrenogenital Syndrome0.9%
551Ovotesticular Disorders of Sex Development0.9%
552Retrognathia0.9%
553Hyperandrogenism0.9%
554Prognathism0.8%
555Aortopulmonary Septal Defect0.8%
556Double Outlet Right Ventricle0.8%
557Endocardial Cushion Defects0.8%
558Myocardial bridging0.8%
559Aorticopulmonary Septal Defect0.8%
560Mandibulofacial Dysostosis0.7%
561Romano-Ward Syndrome0.7%
562Truncus Arteriosus, Persistent0.7%
563Goldenhar Syndrome0.7%
564Talipes0.6%
565Arteriovenous fistula0.5%
566Congenital clubfoot0.5%
567Vertical Talus0.5%
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