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Gazeta Médica

Print version ISSN 2183-8135On-line version ISSN 2184-0628

Gaz Med vol.8 no.3 Queluz Sept. 2021  Epub Oct 01, 2021

https://doi.org/10.29315/gm.v1i1.492 

Imagens Médicas

Punctate Palmoplantar Keratoderma

Queratodermia Palmoplantar Punctate

1. Department of Pediatrics, Trás-os-Montes e Alto Douro Hospital Center, Vila Real, Portugal.

2. Department of Dermatology, Trás-os-Montes e Alto Douro Hospital Center, Vila Real, Portugal.


Keywords: Child; Keratoderma, Palmoplantar/diagnosis; Keratoderma, Palmoplantar/genetics; Keratoderma, Palmoplantar/therapy

Palavras-chave: Criança; Queratodermia Palmoplantar/diagnóstico; Queratodermia Palmoplantar/genética; Queratodermia Palmoplantar/tratamento

A 5-year-old caucasian boy, born of non-consanguineous parents, was referred to the dermatology department due to palmar hiperlinearity and multiple 2-3 mm hyperkeratotic circular lesions in the soles (Fig. 1). The remaining physical examination was unremarkable. There was a family history of palmoplantar hyperkeratosis in the mother and maternal grandfather. To spare the child, a punch skin biopsy was taken from his mother. Pathology revealed an epidermis with irregular acanthosis and orthokeratotic hyperkeratosis, without other morphologic alterations (Fig. 2). Clinical aspect of the lesions associated with a positive family history and histologic findings allowed the diagnosis of punctate palmoplantar keratoderma. Satisfactory results were achieved with urea 20% cream and emollient applied to the soles.

Figure 1: Hyperkeratotic circular lesions of 2-3 mm in diameter in the soles. 

Figure 2: Histological cut of the sole skin of the mother reveals epidermis with irregular acanthosis and orthokeratotic hyperkeratosis (hematoxylin-eosin, 20X). 

Palmoplantar keratodermas are a heterogeneous group of hereditary or acquired diseases, characterized by an excessive epidermal thickening of palmoplantar surfaces.1-5 One of its rare hereditary forms is punctate palmoplantar keratoderma (Brauer-Buschke-Fischer syndrome), that follows a pattern of autosomal dominant inheritance with variable penetrance, and usually appears in late childhood to adulhood.3 In our case the age of presentation was much earlier, which is uncommon. This genodermatosis, although benign, tends to suffer clinical worsening over time,3 and may be associated with the development of neoplasms (pancreas, colon, breast, kidney and lymphoma).2,4 There is no efficient treatment, although some improvement may be achieved with the use of emollients and topical keratolytics. Other treatments such as psoralen plus ultraviolet A (PUVA), systemic or topical retinoids or liquid nitrogen have also been used, with limited success.4

References

1. Bjorn R, Thomas BR, Edel A, O'Toole EA. Diagnosis and Management of Inherited Palmoplantar Keratodermas. Acta Derm Venereol. 2020; 100: adv00094:168-76. doi: 10.2340/00015555-3430. [ Links ]

2. Bukhari R, Alhawsawib W, Radin AA, Jan HD, Al Hawsawi K, Al Ahmadi M. Punctate Palmoplantar Keratoderma: A Case Report of Type 1 (Buschke-Fischer-Brauer Disease). Case Rep Dermatol. 2019;11:292-6. doi: 10.1159/000503337. [ Links ]

3. Guerra L, Castori M, Didona B, Castiglia D, Zambruno G. Hereditary palmoplantar keratodermas. Part I. Non-syndromic palmoplantar keratodermas: classification, clinical and genetic features. J Eur Acad Dermatol Venereol. 2018;32:704-19. doi: 10.1111/jdv.14902. [ Links ]

4. Sakiyama T, Kubo A. Hereditary palmoplantar keratoderma “clinical and genetic differential diagnosis”. J Dermatol. 2016;43:264-74. doi: 10.1111/1346-8138.13219. [ Links ]

5. Monteiro AF, Rato M, Tavares E. Multiple bilateral hyperkeratotic palmoplantar papules. Eur J Dermatol. 2019;29:242-3. doi: 10.1684/ejd.2019.3565. [ Links ]

Responsabilidades Éticas

Fontes de Financiamento: Não existiram fontes externas de financiamento para a realização deste artigo

Confidencialidade dos Dados: Os autores declaram ter seguido os protocolos da sua instituição acerca da publicação dos dados de doentes

Consentimento: Consentimento do doente para publicação obtido

Proveniência e Revisão por Pares: Não comissionado; revisão externa por pares

Ethical Disclosures

Financing Support: This work has not received any contribution, grant or scholarship

Confidentiality of Data: The authors declare that they have followed the protocols of their work center on the publication of data from patients

Patient Consent: Consent for publication was obtained

Provenance and Peer Review: Not commissioned; externally peer reviewed

Received: August 26, 2021; Accepted: September 17, 2021; Published: September 30, 2021

Susana Cláudia Teixeira [snteixeira@chtmad.min-saude.pt] Centro Hospitalar de Trás-os-Montes e Alto Douro, Avenida Noruega, 5000-508 Vila Real, Portugal ORCID iD: 0000-0001-5827-909X

Conflitos de Interesse:

Os autores declaram a inexistência de conflitos de interesse na realização do presente trabalho.

Conflicts of Interest:

The authors have no conflicts of interest to declare.

Creative Commons License This is an open-access article distributed under the terms of the Creative Commons Attribution License