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Clinical appearance of Dyskeratosis Congenita. (a) Ridging and splitting of nails affecting all fingernails with varying severity. (b) Brown lacy reticular network of pigmentation on the neck and V area of the chest. (c) Leukokeratotic lesions over a background of hyperpigmentation on the dorsal aspect of the tongue. (d) Thickening of palmar skin with dyspigmentation d c
Contexts in source publication
Context 1
... Editor, A 23-year-old male presented to our clinic with complaints of progressive roughening, cracking, and fragility of finger and toenails since the age of 9 years. Examination revealed longitudinal ridging and splitting of all finger nails with mild dystrophy and onychoschizia of some nails [ Figure 1a]. The toenails were partially involved. ...
Context 2
... toenails were partially involved. A closer cutaneous evaluation revealed tan-brown mottled to reticulate pigmentation on the anterior aspect of the neck and V area of the chest [ Figure 1b]. The oral cavity showed asymptomatic leukokeratosis of the tongue [ Figure 1c]. ...
Context 3
... closer cutaneous evaluation revealed tan-brown mottled to reticulate pigmentation on the anterior aspect of the neck and V area of the chest [ Figure 1b]. The oral cavity showed asymptomatic leukokeratosis of the tongue [ Figure 1c]. The skin over the palms and soles was dyspigmented and thickened with the absence of dermatoglyphics [Figure 1d]. ...
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Dyskeratosis Congenita (DC) is a rare genetic syndrome characterized by mucocutaneous abnormalities, often complicating into progressive life-threatening systemic manifestations like bone marrow failure, increased rate of malignancy, lung, and liver diseases.
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Background
Dyskeratosis congenita (DKC), also known as Zinsser–Cole–Engman syndrome, is a progressive genetic disease with a triad of reticulate skin pigmentation, nail dystrophy, and leukoplakia. Approximately 8–10% of patients with DKC develop malignancies, and cases of colorectal cancer with DKC in young people have been reported previously.
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Citations
... Xeroderma pigmentosa [10] Asymmetrical blotch, atypical network, hyperpigmented follicular openings, pigmented rhomboidal areas. Dyskeratosis congenita [11] Pigmented lines, brown dots and globules in netlike pattern. Lichen planus pigmentosus [12] Brown pseudonetwork, hem-like pattern, speckled dots and globules in various patterns, perifollicular deposition. ...
Background:
Pediatric nail disorders encompass a broad range of conditions. This article aimed to consolidate current knowledge on pediatric nail disorders to enhance diagnostic proficiency and clinical management among healthcare professionals.
Summary:
Pediatric nail disorders present a diagnostic challenge due to their diverse nature. Non-syndromic congenital nail disorders encompass various anomalies such as anonychia/hyponychia, congenital malalignment of the great toenail, and racket thumbs, each with distinct clinical presentations and genetic associations. Syndromic congenital nail disorders, often part of complex syndromes, are characterized by unique features and associated abnormalities. Acquired nail diseases in children, like Beau's lines and onychomadesis, typically result from trauma or infection, while melanonychia, although rare in children, requires careful monitoring for potential malignant alterations, with consideration for biopsy in cases with concerning features.
Key messages:
(1) Pediatric nail disorders may pose diagnostic challenges and require a comprehensive understanding of nail anatomy and development. (2) Congenital nail disorders encompass isolated anomalies and syndromic associations, necessitating thorough evaluation for associated systemic conditions. (3) Acquired nail diseases may indicate underlying trauma or systemic illness and require careful assessment.(4) Melanonychia in children requires ongoing monitoring and evaluation, emphasizing the importance of consistent follow-up and histopathological examination when necessary.
