A rare case of recurrent pyogenic liver abscess since childhood: A case of Papillon-Lefèvre syndrome

Das, Somak Kumar; Nath, Tanusree; Ganguly, P.K.; Jana, Chanchal Kumar

doi:10.4103/0975-2870.108659

Cited by 4 publications

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“…Progressive periodontitis leading to premature loss of teeth, gingival inflammation, and rapid destruction of periodontium is the hallmark manifestation ( 3 ). Loss of teeth usually occurs during the teenage years, but the third molar may be spared ( 4 ). Periodontitis and loss of teeth were, however, not observed in both patients.…”

Section: Discussionmentioning

confidence: 99%

“…Periodontitis and loss of teeth were, however, not observed in both patients. Strict oral hygiene and careful management of infections may be required to prevent the development of this complication ( 4 ).…”

Section: Discussionmentioning

confidence: 99%

“…Hyperhidrosis may also be seen. Skin histopathology may reveal non-specific hyperkeratosis, focal parakeratosis, dilated tortuous capillaries in dermal papillae, and lymphocytic infiltration in the superficial layer ( 4 ). Palmoplantar keratoderma was observed in both patients in the present series for which topical corticosteroids, emollients, and calcitriol ointment were advised ( 5 ).…”

Section: Discussionmentioning

confidence: 99%

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Case report: Corticosteroids as an adjunct treatment for the management of liver abscess in Papillon–Lefèvre syndrome: A report on two cases

et al. 2022

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Papillon–Lefèvre syndrome (PLS) is a rare autosomal recessive disorder characterized clinically by palmoplantar keratoderma, periodontitis, and recurrent pyogenic infections. Liver abscess is rarely reported in patients. The use of corticosteroids for the treatment of liver abscess akin to chronic granulomatous disease (CGD) has not been reported previously. Here, we report 2 cases of liver abscess in PLS that responded to corticosteroids.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Case report: Corticosteroids as an adjunct treatment for the management of liver abscess in Papillon–Lefèvre syndrome: A report on two cases

et al. 2022

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“…In addition to palmoplantar keratoderma and early periodontal destruction of primary and permanent dentition, other features may be associated with PLS, i.e., increased susceptibility to infection [16], increased risk of pyogenic liver abscess [40][41][42][43], calcification of the falx cerebri of the dura mater [44], excessive sweating (hyperhidrosis), and growth of fine body hair with the development of dirty-colored skin on the affected areas [45]. Recently, the first case of multiple brain abscesses in a child with PLS was reported [46].…”

Section: Secondary Features Associated With Plsmentioning

confidence: 99%

Syndromes That Include Both Palmoplantar Keratoderma And Severe Periodontitis: A Review

Dababneh¹

2013

Dentistry

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Palmoplantar keratodermas (PPK) include a heterogeneous group of disorders with overlapping clinical features. The main aspect of PPK is thickening and hyperkeratosis of the palmar and plantar skin, that may be hereditary or acquired; diffuse, focal, or punctuate; and transgrediens or progrediens. PPKs are further distinguished by their mode of inheritance and by the presence of certain associated clinical features. Periodontitis was reported in association with more than one syndrome characterized by PPK. An extensively reported one is the Papillon-Lefévre syndrome (PLS) which is characterized by early onset of PPK and periodontitis affecting the primary and secondary dentitions. In addition to PLS, Haim-Munk, HOPP, Variant Carvajal and Weary-Kindler are other syndromes manifested by PPK and reported in association with severe periodontitis. Atypical cases of PLS were also reported, such as partial expression or a late presentation of the syndrome. The aim of this article is to critically review the literature concerned with Papillon-Lefévre syndrome in its typical and atypical clinical presentation, in addition to other syndromes manifested at the same time by PPK and severe periodontitis.Thorough history and medical examination, together with periodontal, dermatologic, and genetic counseling, are important to exclude other existing medical conditions or other syndromes that might need special attention and care.

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Complications of Odontogenic and Non-odontogenic Infections

Chigurupati

Shemkus²

2020

Complications in Cranio-Maxillofacial and Oral Surgery

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A rare case of recurrent pyogenic liver abscess since childhood: A case of Papillon-Lefèvre syndrome

Cited by 4 publications

References 1 publication

Case report: Corticosteroids as an adjunct treatment for the management of liver abscess in Papillon–Lefèvre syndrome: A report on two cases

Case report: Corticosteroids as an adjunct treatment for the management of liver abscess in Papillon–Lefèvre syndrome: A report on two cases

Syndromes That Include Both Palmoplantar Keratoderma And Severe Periodontitis: A Review

Complications of Odontogenic and Non-odontogenic Infections

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