PICTURE OF THE MONTH

A Boy with Sapphire Thumbnails: Lunulae Ceruleae

Mahesh Kamate & Gowda Parameshwar Prashanth &

Sunil Gandhi

Received: 19 August 2013 /Accepted: 2 January 2014 /Published online: 6 March 2014# Dr. K C Chaudhuri Foundation 2014

A 12-y-old boy presented with bluish discoloration of thumbnails for three months. There was no significant past or presentmedical history except one episode of jaundice of 1 mo dura-tion at 3 y of age. Blood investigations done during the episodewere unavailable. The boy was otherwise asymptomatic.

On examination, there was no pallor, icterus, or centralcyanosis. Bluish discoloration was limited to the lunular por-tion of nail on both thumbs and great toes (Fig. 1). There wasno clubbing. Per abdomen, liver was normally palpable 2 cmbelow right costal margin with a span of 6 cm. Spleen was notenlarged. Serum total bilirubin [1.8 mg/dL (normal: 0.2–1.0),direct bilirubin of 0.26 mg/dL (normal: 0.0–0.2)], liver enzymes[Alanine aminotransferase (ALT): 21 IU/L (normal: 15–27),Aspartate aminotransferase (AST): 26 IU/L (normal: 30–65)]and serum protein (5.5 g/dL) were normal. Serum ceruloplasminwas low-normal (0.28 g/L; normal: 0.22–0.61 g/L) with in-creased baseline urinary copper excretion [564 mcg/d (normal:32–64 mcg/d)]. No Kayser Fleischer (KF) ring was seenon slit-lamp examination. Considering the possibility ofWilson disease, oral D-penicillamine (20 mg/kg/d) withzinc acetate (75 mg/d) and pyridoxine (25 mg/d) werestarted [1]. High urinary-copper (1,286 mcg/d) was

observed during follow-up. Hepatic-copper estimationwas not feasible in the present set-up and hence Wilsondisease could not be confirmed. Incidentally, the femalesibling (asymptomatic and without nail-changes) alsoshowed high urinary-copper and low-normal ceruloplas-min. Both refused to undergo liver biopsy.

Blue discoloration of lunula is most commonly seen withdrug therapy like zidovudine (HIV infection) and chemother-apy (cyclophosphamide, vincristine, doxorubicin, andhydroxyuria) [2, 3]. Azure nails are classically described insilver and phenolphthalein toxicity. Systemic conditions man-ifesting with blue nails include acrolabial telengiectasis, met-hemoglobinemia (HbM disease), and Wilson disease [4].

Lunulae ceruleae or blue lunula in Wilson disease is notreported in pediatric literature till date. The pathogenesisremains to be elucidated [4, 5]. In the present case Wilsondisease, though not confirmed, is likely in view of the absenceof other causes of blue nails. Outward shift of the discolorednail portion owing to nail growth (Fig. 2) lead to it’s disappearance after 4 mo of therapy. The authors suggest thatthe clinicians be cognizant of the possibility of Wilson diseasein children presenting with bluish nail-discoloration.

Fig. 1 Pictures showing bluish discoloration of lunular portions ofthumbs and great toes. Also seen is the early involvement of other fingernails

M. KamateChild Developmental Clinic, Department of Pediatrics, JawaharlalNehru Medical College and KLES Prabhakar Kore Hospital andMedical Research Centre, Nehru Nagar, Belgaum, Karnataka, India

G. P. Prashanth (*)Pediatric Intensive Care Unit, Department of Pediatrics, JawaharlalNehru Medical College and KLES Prabhakar Kore Hospital andMedical Research Centre, Nehru Nagar, Belgaum 590010,Karnataka, Indiae-mail: dr.prashanth.gp@gmail.com

S. GandhiDepartment of Dermatology, Jawaharlal Nehru Medical College andKLES Prabhakar Kore Hospital and Medical Research Centre,Nehru Nagar, Belgaum, Karnataka, India

Indian J Pediatr (July 2014) 81(7):737–738DOI 10.1007/s12098-014-1341-7

Acknowledgments The authors would like to thank the parents of thechildren reported here for consenting to report the case.

Contributions GPP: Conceived the idea, did literature search, anddrafted the manuscript. MK: Reviewed the manuscript and will act asguarantor for this paper. MK and SG: Contributed equally in literature

search. All authors were involved in the management of the cases andapproved the final version of the manuscript.

Conflict of Interest None.

Role of Funding Source None.

References

1. Ferenci P, Czlonkowska A, Stremmel W, Houwen R, Rosenberg W,Schilsky M, et al. EASL Clinical Practice Guidelines: Wilson’s dis-ease. J Hepatol. 2012;56:671–85.

2. Greenberg RG, Berger TG. Nail and mucocutaneous hyperpig-mentation with azidothymidine therapy. J Am Acad Dermatol.1990;22:327–30.

3. Nixon DW. Alterations in nail pigment with cancer chemotherapy.Ann Intern Med. 1976;136:1117–8.

4. Cohen PR. The lunula. J Am Acad Dermatol. 1996;34:943–53.5. Bearn AG, McKusick VA. Azure lunulae: An unusual change in the

fingernails in two patients with hepatolenticular degeneration

(Wilson’s disease). JAMA. 1958;166:904–6.

Fig. 2 Resolution of lunular discoloration with d-Penicillamine therapy

738 Indian J Pediatr (July 2014) 81(7):737–738