Yellow nails following hemodialysis in chronic renal failure: Is it yellow nail syndrome or a variant?
Department of Skin and STD, Kasturba Medical College, Manipal, India. email@example.com
Yellow nail syndrome (YNS) is triad of yellow nails, lymphedema, and respiratory tract involvement. The exact pathogenesis of nail changes in YNS is unknown. We present a case of yellow nails and localized lymphedema secondary to artificial AV fistula in a 55-year-old chronic renal failure patient on hemodialysis for 5 years. To the best of our knowledge, this is the first case of yellow nail syndrome reported in association with artificial AV fistula.
A 55-year-old female with chronic renal failure on hemodialysis for the past five years presented with swelling of the left hand and a nail deformity of seven months duration. The nail deformity began with a yellowish discoloration followed by a progressive increase in horizontal curvature. Further clinical evaluation revealed hyperpigmentation of the left upper limb up to the cubital fossa and an arteriovenous (AV) fistula on the left antecubital fossa. The overlying skin was shiny with peripheral limb edema. All the nails of the left hand showed yellowish discoloration with increased longitudinal and horizontal curvatures. Subungal hyperkeratosis was evident with the absence of lunulae. The right hand nails were unaffected (Figs. 1 & 2). Apart from the abnormal renal parameters and anemia, her other laboratory parameters were normal. In view of the peripheral limb edema and yellowish discoloration of the nails, she was provisionally considered to be a case of yellow nail syndrome (YNS) probably secondary to the AV fistula created for hemodialysis.
The syndrome of yellow nails and lymphedema was first described by Samman and White in 1964 . Later Emerson added pleural effusion as a frequent feature of this disease . Yellow nail syndrome is a disorder of thickened yellow nails and lymphedema in which a number of associations have been described, the most common being that with respiratory tract diseases . The characteristic nail changes described are thickening, hardening, longitudinal over-curvature, transverse ridging, total or distal yellow discoloration, loss of cuticle and lunulae, and onycholysis. Rarely erythema and edema of the proximal nail fold and dermatophytic superinfection may be seen. Virtually all the nails are affected and the nail growth is 0.1mm to 0.25mm/week compared to 0.5mm to 2mm/week in normal adult fingernails .
The exact pathogenesis of nail changes in YNS is unknown. However, it is currently considered to be due to an underlying lymphatic abnormality, either anatomical or functional . The reduced lymphatic drainage could be the culprit in causing the peripheral edema as well as the nail changes. DeCoste et al. hypothesized that primary sclerosis of the stroma led to lymphatic obstruction and the slow ungal growth rate causing brownish discoloration of the nail (Advanced Maillard reaction) .
The diagnosis of YNS is based on the characteristic triad of yellow nails, lymphedema and respiratory tract involvement. Two of these three symptoms are required for diagnosis as all the three features may not be present at any one time . According to Tosti et al. all three alterations of YNS were simultaneously seen in only 27 percent of cases . Of the 12 patients reported from the Mayo clinic, the presenting manifestation always was either lymphedema or yellow nails, pleural effusion appearing somewhat later in all . The YNS has been reported in association with thyroid disease, hypogammaglobulinemia, protein losing enteropathy, obstructive sleep apnea, xanthogranulomatous pyelonephritis, and nephrotic syndrome . It may even represent a paraneoplastic phenomenon due to its association with a variety of malignancies . A case of bucillamine-induced yellow nails has been reported by Ishizaki et al . The treatment options in YNS include intralesional triamcinolone injections, oral antifungals, oral zinc, and vitamin E. Spontaneous remission has been observed in 30 percent of cases .
Our patient presented with yellow nails and localized lymphedema secondary to her artificial AV fistula, accounting for two of the three components of YNS. There was no history of any previous skin eruption at the same site. Hence a postinflammatory cause was ruled out. The distinct hyperpigmentation of the left upper limb could be attributed to the artificial AV fistula that was created for the purpose of dialysis. The resulting venous congestion and swelling also could be the culprit in causing the lymphatic obstruction and overlying surface changes in the skin. Although lymphedema may be associated with YNS, Bull et al. in their study suggested that lymphatic involvement is secondary and probably functional in nature . There was no history of respiratory tract involvement and fungal infection of the nails was ruled out by a negative KOH mount of the nail clipping. To the best of our knowledge, this is the first case of probable acquired yellow nail syndrome reported in association with artificial AV fistula.
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