Dyschromatosis

Samuelov, Liat; Sprecher, Eli

doi:10.1002/9781119142812.ch126

Cited by 2 publications

(3 citation statements)

References 203 publications

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“…In general, children with PLS are moderately built and their physical and intellectual development levels are within normal limits. A sub-type of PLS is Haim Munk syndrome characterized by PLS along with onychogryphosis, arachnodactyly and acro-osteolysis with out pyoderma and falx cerebri, seen in Jews of South Indian origin (4) .Diagnosis is based on clinical signs and symptoms, patient/family history, genetic tesing. Urine CTSC analysis in infants (complete absence) and absent of azurophilic granules in mature neutrophils but not in progenitor cells, indicating CTSC promotes protease degradation in immune cells.…”

Section: Discussion:-mentioning

confidence: 99%

“…Urine CTSC analysis in infants (complete absence) and absent of azurophilic granules in mature neutrophils but not in progenitor cells, indicating CTSC promotes protease degradation in immune cells. (4) Treatment goal is mainly halting the rapid devastating periodontal destruction and reducing the hyperkaratotic skin lesions by topical emollients and keratolytic agents and oral retinoids 0.5-1 mg/kg/day. (5)…”

Section: Discussion:-mentioning

confidence: 99%

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A Rare Case of Papillon-Lefevre Syndrome: A Case Report

Vidya¹,

Shabana²,

Boon³

et al. 2023

IJAR

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Papillon Lefevre syndrome is an extremely rare autosomal recessive disorder characterized by diffuse palmoplantar hyperkeratosis, rapidly progressive and devastating periodonitis, and pyodermas. The etiopathogenesis of the disorder is multifactorial, with genetic and immunological factors playing a major role. Consanguinity is a contributing factor. Genetic mutations of the gene 11q14- q21 encoding for cathepsin-c, a lysosomal protease that activates enzymes involved in a variety of inflammatory and immune processes. Management directed towards halting periodontal destruction using conventional periodontal treatment, systemic antibiotics, oral hygiene instructions, antiseptic mouth rinses. Palmoplantar hyperkeratosis usually treated with topical application of emollients, keratolytic agents, topical steriods and systemic retinoids.

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Section: Discussion:-mentioning

confidence: 99%

Section: Discussion:-mentioning

confidence: 99%

A Rare Case of Papillon-Lefevre Syndrome: A Case Report

Vidya¹,

Shabana²,

Boon³

et al. 2023

IJAR

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“…Acquired palmoplantar keratoderma (PPK) is a non-hereditary hyperkeratosis of the palms and soles covering ≥50% of the skin surface of involved acral area that can be caused by inflammatory disease, metabolic disease, or exposure to drugs, 1 , 2 such as chemotherapeutic agents, including capecitabine, doxorubicin, hydroxyurea, and tegafur. 2 , 3 The mechanism by which capecitabine causes PPK is still not clear.…”

Section: Introductionmentioning

confidence: 99%

A Rare Case on Capecitabine Induced Acquired Palmoplantar Keratoderma

Tsaqilah,

Mudia,

Usman

et al. 2023

CCID

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Acquired palmoplantar keratoderma (PPK) is a non-hereditary hyperkeratosis of the palms and soles that is caused by various factors, including chemotherapeutic agents. The purpose of this case report is to present a rare case of acquired PPK caused by the chemotherapeutic agent capecitabine. A 54-year-old female complained of painful erythematous plaques on her palms and soles with history of consuming capecitabine. Physical examination revealed scaly erythematous plaques on the palmoplantar surface and knuckle pads on both hands. Histopathological features showed hyperkeratosis, acanthosis, vasodilatation, and perivascular lymphocytic infiltration. Therefore, the patient was diagnosed with acquired PPK due to capecitabine. The dose of capecitabine was reduced and the patient was administered topical corticosteroid and emollient. Improvement of skin lesions was strongly observed after discontinuation of capecitabine. The underlying cause of PPK should be identified to determine the appropriate treatment. Dose reduction or drug discontinuation is the mainstay therapy for patients with acquired PPK caused by chemotherapeutic agents.

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Dyschromatosis

Cited by 2 publications

References 203 publications

A Rare Case of Papillon-Lefevre Syndrome: A Case Report

A Rare Case of Papillon-Lefevre Syndrome: A Case Report

A Rare Case on Capecitabine Induced Acquired Palmoplantar Keratoderma

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