COMMON Features of the Disorder Sparse hair Blisters on soles of feet/palms of hand (palmoplantar keratosis) Skin infections Premature tooth loss (periodontitis) Inflammation around teeth Nail abnormalities PROGNOSIS Individuals with the disorder lose [evolvegene.com]

Nail abnormalities. Decreased sweating. [firstskinfoundation.org]

PPK as a feature of systemic disease – Hypothyroidism, myxedema, diabetes mellitus, and chronic lymphedema. [visualdx.com]

A few days before admission, the mother noted an abdominal mass associated with right upper quadrant pain. The pain, which was dull, achy, intermittent, but not radiating, was not associated with vomiting, diarrhea, or jaundice. [pediatrics.aappublications.org]

The pain, which was dull, achy, intermittent, but not radiating, was not associated with vomiting, diarrhea, or jaundice. On physical examination, the child was well developed and well nourished. [pediatrics.aappublications.org]

He was also referred to dental services for dental caries and delayed teething (Figure 3), and to an otolaryngologist to rule out nasal polyps. [cureus.com]

Premature loss of teeth and loss of alveolar bone had resulted in decreased facial height and a senile appearance in case 1 [Figure 2] b. [jidonline.com]

hyperkeratosis periodontal disease Descripción : The connection between palmar plantar hyperkeratosis and severe periodontal disease was first reported in 1924 by Papillon and Lefevre. [189.203.43.34]

(B) Plantar hyperkeratosis. Figure 1 (A) Severe destructive periodontitis. (B) Plantar hyperkeratosis. [academic.oup.com]

Anderson, The syndrome of palmar-plantar hyperkeratosis and premature periodontal destruction of the teeth, The Journal of Pediatrics, 1964, 65, 6, 895 CrossRef [onlinelibrary.wiley.com]

Patients affected with autosomal dominant palmoplantar keratosis often present with malignancies of the esophagus and/or other malignancies (lymphomas, pancreas, breast, etc.). [accessanesthesiology.mhmedical.com]

Papillon-Lefèvre Syndrome: A Report of Three Siblings Author: Muddathir, Abdelkhalig Abstract: We reported an 18 year old Sudanese female patient, Gaali tribe, and another 2 males, brothers ages 10 and 8 years old, presented with diffuse trangrediens palmoplantar [khartoumspace.uofk.edu]

Loss-of-function mutations in the cathepsin C gene result in periodontal disease and palmoplantar keratosis. Nat Genet 1999 ;23 (4):421-4. 16. Eickholz P, Kugel B, Pohl S, Naher H, Staehle HJ. [ayubmed.edu.pk]

Definition An autosomal recessive disorder characterized by palmoplantar keratosis and severe periodontitis affecting deciduous and permanent dentitions and resulting in premature tooth loss. [uniprot.org]

Keywords: Cathepsin C gene, Papillon-Lefevre syndrome, periodontitis INTRODUCTION Papillon-Lefevre syndrome belongs to a heterogeneous group of skin diseases that are characterized by hyperkeratosis of palms and soles. [ncbi.nlm.nih.gov]

The role of cathepsin C in the development of dermatological conditions in PLS is still unclear and may explain the nonsignificant correlation between the severity of skin disease and severity of oral infection [5, 7-9]. [karger.com]

Increased sweating ( hyperhidrosis ) is quite common and there is a tendency to fungal and bacterial infections of the feet. [dermnetnz.org]

On examination, he was found to have an erythematous hyperkeratotic skin plaques and papules with scales over the planter and palmar aspect of both hands with similar lesions observed on both feet, legs, scalp, and ears with nail pitting. [cureus.com]

Conclusion Even though PLS is an extremely rare condition it is associated with lifelong psychological and social impacts on the growing children such as depression including hopelessness, aimlessness, social phobia, and a fear of communicating with people [hindawi.com]

The most common organisms associated with dental and liver infections are anaerobic bacteria (such as Actinobacillus actinomycetem comitans) and Staphylococcus aureus (5, 6). [ijp.tums.pub]

Staphylococcus aureus, sensitive to cloxacillin and vancomycin and resistant to penicillin, was isolated from the liver abscess culture. [pediatrics.aappublications.org]

In these siblings, the inflamed gingiva was the likely point of entry of Staphylococcus aureus that led to bacteremia and subsequently the liver abscess (case 2). [jidonline.com]

Serum antibody test for Entamoeba histolytica and E granulosus was negative, as well as stool for ova and parasite. Ultrasound-guided drainage was performed, and 100 mL of thick, yellowish exudate was obtained. [pediatrics.aappublications.org]

Liver biopsy showed a granulomatous lesion caused by Mycobacterium tuberculosis that was confirmed by PCR. Contrary to this study, no serious cutaneous infection was observed. [ijp.tums.pub]