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• W3108570203 abstract "Koilonychia, also called spoon nails, is a nail disease characterized by concave nail plates.1Chelidze K. Lipner S.R. The water-drop test for the diagnosis of koilonychia.J Am Acad Dermatol. 2017; 77: e157-e158Abstract Full Text Full Text PDF PubMed Scopus (4) Google Scholar It can be either hereditary or acquired, with the latter typically occurring in individuals with iron deficiency, trauma, occupational exposures, or diabetes mellitus.1Chelidze K. Lipner S.R. The water-drop test for the diagnosis of koilonychia.J Am Acad Dermatol. 2017; 77: e157-e158Abstract Full Text Full Text PDF PubMed Scopus (4) Google Scholar,2Walker J. Baran R. Vélez N. Jellinek N. Koilonychia: an update on pathophysiology, differential diagnosis and clinical relevance.J Eur Acad Dermatol Venereol. 2016; 30: 1985-1991Crossref PubMed Scopus (22) Google Scholar Rarely, it has also been associated with thyroid disorders or autoimmune diseases such as alopecia areata, psoriasis, or lichen planus.2Walker J. Baran R. Vélez N. Jellinek N. Koilonychia: an update on pathophysiology, differential diagnosis and clinical relevance.J Eur Acad Dermatol Venereol. 2016; 30: 1985-1991Crossref PubMed Scopus (22) Google Scholar This report presents the unusual case of koilonychia occurring secondary to primary Raynaud's phenomenon and the successful reversal of koilonychia with topical nitroglycerin. A 52-year-old woman with a thirty-year history of Raynaud's phenomenon presented with scooped nails on her bilateral hands. Over the past year, her nail plates had become increasingly brittle, ridged, and flat or scooped-shaped. She did not have ulcerations of her fingertips, but noted that she was developing frequent and severe Raynaud's phenomena with white and red color changes and pain in response to cold temperatures or stress on both hands and occasionally on her toes. The patient otherwise denied any hair loss, nail pitting, rashes, oral ulcers, joint pain, dysphagia, skin thickening, fever, fatigue, shortness of breath, chest pain, dry eyes or mouth, or cough. She had treated her Raynaud's phenomenon with behavioral modification. The patient also tried topical minoxidil on her dorsal hands and cannabidiol cream but saw little to no improvement in her nails and the frequency of her Raynaud's phenomenon with these interventions. She had no other medical conditions and only took biotin and multivitamin supplements. She did not work with petroleum-based products, and she had a predominantly vegetarian diet with occasional chicken and fish. Her physical examination was notable for proximal leukonychia, erythronychia, longitudinal ridging, ragged cuticles, and concave-shaped nail plates on multiple nails on her left and right hands (Fig 1, A and Fig 2, A). Nailfold capillary microscopy revealed regular vessel architecture, density, and capillary size without signs of hemorrhage, avascular regions, or loss of capillary loops. She did not have conjunctival pallor.Fig 2A, Proximal leukonychia, erythronychia, longitudinal ridging, ragged cuticles, and concave-shaped nail plates on multiple nails on the patient's left hand prior to initiation of nitroglycerin treatment. B, Interval improvement in nail dystrophy and cuticles after 5 months of treatment with topical nitroglycerin.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Laboratory tests were negative for antinuclear antibodies, centromere antibody, anti-Smith antibody, anti-RNP antibody, anti-LA antibody, anti-RO antibody, and anti-SCL70 antibody. The patient's hemoglobin, ferritin, iron, total iron binding capacity, unsaturated iron binding capacity, fasting blood glucose, and thyroid-stimulating hormone levels were all within the normal limits. Based on her history, examination, and laboratory findings, a clinical diagnosis of koilonychia and nail dystrophy secondary to primary Raynaud's phenomenon was made. The patient was started on 2% nitroglycerin ointment on the bilateral nail folds and web spaces daily. She was also advised to continue to keep her core warm to increase warming of her extremities. The patient reliably followed both of these recommendations, applying the nitroglycerin ointment 2% daily and keeping her core and hands warm. After five months of treatment, she had near resolution of her nail dystrophy and koilonychia (Fig 1, B and Fig 2, B). This case highlights the rare occurrence of koilonychia in a patient with primary Raynaud's phenomenon who was able to successfully reverse her koilonychia by treating her Raynaud's phenomenon with topical nitroglycerin. Raynaud's phenomenon has been associated with several nail findings, including parrot beak nails, brittle nails, and longitudinal ridging, but reports about koilonychia are sparse in the literature.3Solomons H.D. Raynaud's phenomenon.Cardiovasc J Afr. 2011; 22: 233PubMed Google Scholar,4Chen S.X. Cohen P.R. Parrot beak nails revisited: case series and comprehensive review.Dermatol Ther (Heidelb). 2018; 8: 147-155Crossref PubMed Scopus (1) Google Scholar While the pathogenesis of koilonychia is unclear, the nail disease is attributed to diminished digital blood flow disrupting the growth of subungual connective tissue, hyperkeratosis of the nail bed (as in psoriasis), and capillary shunting, all of which may cause a depression in the distal matrix and lead to concave nail plates.2Walker J. Baran R. Vélez N. Jellinek N. Koilonychia: an update on pathophysiology, differential diagnosis and clinical relevance.J Eur Acad Dermatol Venereol. 2016; 30: 1985-1991Crossref PubMed Scopus (22) Google Scholar,5Stone O.J. Clubbing and Koilonychia.Dermatol Clin. 1985; 3: 485-490Abstract Full Text PDF PubMed Google Scholar,6Rathod D.G. Sonthalia S. Spoon nails (Koilonychia).StatPearls. StatPearls Publishing, 2020http://www.ncbi.nlm.nih.gov/books/NBK559311/Google Scholar In this patient's case, we hypothesize that the frequent vasoconstriction of peripheral vessels in the fingers from Raynaud's led to poor blood flow and hypoxia of the nail matrix, leading to altered formation of the distal nail matrix. Koilonychia can be accompanied by thin and brittle nails.2Walker J. Baran R. Vélez N. Jellinek N. Koilonychia: an update on pathophysiology, differential diagnosis and clinical relevance.J Eur Acad Dermatol Venereol. 2016; 30: 1985-1991Crossref PubMed Scopus (22) Google Scholar It is classically associated with iron deficiency, which may be due to nutritional deficiencies, gastrointestinal bleeding, or Plummer-Vinson syndrome.2Walker J. Baran R. Vélez N. Jellinek N. Koilonychia: an update on pathophysiology, differential diagnosis and clinical relevance.J Eur Acad Dermatol Venereol. 2016; 30: 1985-1991Crossref PubMed Scopus (22) Google Scholar Interestingly, koilonychia has also been reported in up to 49% of patients with hemochromatosis, although phlebotomy does not appear to reverse the condition.7Chevrant-Breton J. Simon M. Bourel M. Ferrand B. Cutaneous manifestations of idiopathic hemochromatosis. Study of 100 cases.Arch Dermatol. 1977; 113: 161-165Crossref PubMed Scopus (76) Google Scholar Koilonychia is also associated with endocrinopathies such as thyroid disorders and diabetes; nutritional deficiencies in amino acids, vitamin C, zinc, copper, and selenium; occupational exposures to petroleum-based products, ammonium thioglycolate, and mineral oils; and living at a high altitude.2Walker J. Baran R. Vélez N. Jellinek N. Koilonychia: an update on pathophysiology, differential diagnosis and clinical relevance.J Eur Acad Dermatol Venereol. 2016; 30: 1985-1991Crossref PubMed Scopus (22) Google Scholar Early childhood koilonychias are often secondary to tight-fitting shoes or finger sucking, both of which resolve with behavior modification.2Walker J. Baran R. Vélez N. Jellinek N. Koilonychia: an update on pathophysiology, differential diagnosis and clinical relevance.J Eur Acad Dermatol Venereol. 2016; 30: 1985-1991Crossref PubMed Scopus (22) Google Scholar,8Starace M. Alessandrini A. Piraccini B.M. Nail disorders in children.Skin Appendage Disord. 2018; 4: 217-229Crossref PubMed Scopus (7) Google Scholar Hereditary cases of the disease also occur in an autosomal dominant pattern. Rarely, koilonychia has been observed in patients with systemic lupus erythematous and Raynaud's phenomenon, typically involving abnormal proximal nail fold capillary loops, splinter hemorrhages, and red lunulae—signs that were not present in our case.2Walker J. Baran R. Vélez N. Jellinek N. Koilonychia: an update on pathophysiology, differential diagnosis and clinical relevance.J Eur Acad Dermatol Venereol. 2016; 30: 1985-1991Crossref PubMed Scopus (22) Google Scholar,9García-Patos V. Bartralot R. Ordi J. Baselga E. de Moragas J.M. Castells A. Systemic lupus erythematosus presenting with red lunulae.J Am Acad Dermatol. 1997; 36: 834-836Abstract Full Text Full Text PDF PubMed Google Scholar The diagnosis of koilonychia is often clinical, requiring a complete history, review of systems, and physical examination.2Walker J. Baran R. Vélez N. Jellinek N. Koilonychia: an update on pathophysiology, differential diagnosis and clinical relevance.J Eur Acad Dermatol Venereol. 2016; 30: 1985-1991Crossref PubMed Scopus (22) Google Scholar While some causes of koilonychia are idiopathic, the workup of the disease should include iron concentration studies, complete blood counts, and when an autoimmune disease is suspected, inflammatory markers and autoimmune disease antibodies in order to identify and manage underlying causes of koilonychia. Onychomycosis and inflammatory skin diseases (eg, psoriasis, alopecia areata, lichen planus) should also be considered.2Walker J. Baran R. Vélez N. Jellinek N. Koilonychia: an update on pathophysiology, differential diagnosis and clinical relevance.J Eur Acad Dermatol Venereol. 2016; 30: 1985-1991Crossref PubMed Scopus (22) Google Scholar Because the concavity of nails may be difficult to detect by visual inspection, the water-drop test, whereby a few drops of water are placed on the patient's nail, may facilitate the diagnosis of koilonychia.1Chelidze K. Lipner S.R. The water-drop test for the diagnosis of koilonychia.J Am Acad Dermatol. 2017; 77: e157-e158Abstract Full Text Full Text PDF PubMed Scopus (4) Google Scholar If the water droplets pool on the nail plate without sliding off, the concave shape of the nails is verified.1Chelidze K. Lipner S.R. The water-drop test for the diagnosis of koilonychia.J Am Acad Dermatol. 2017; 77: e157-e158Abstract Full Text Full Text PDF PubMed Scopus (4) Google Scholar As Raynaud's phenomenon affects an estimated 11% of middle-aged women and 8% of middle-aged men,10Suter L.G. Murabito J.M. Felson D.T. Fraenkel L. The incidence and natural history of Raynaud's phenomenon in the community.Arthritis Rheum. 2005; 52: 1259-1263Crossref PubMed Scopus (94) Google Scholar clinicians should be aware that koilonychia may be a potentially reversible manifestation of Raynaud's phenomenon. This case serves as an important reminder that nail changes are often manifestations of systemic disease. Further research is needed to elucidate the pathophysiology of koilonychia, but this case suggests that diminished digital blood flow to the nail matrix may be contributory. With proper detection and treatment, koilonychia secondary to Raynaud's phenomenon can be managed successfully." @default.
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• W3108570203 title "Koilonychia secondary to Raynaud's phenomenon: A rare co-occurrence" @default.
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