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LETTER TO EDITOR
Year : 2021  |  Volume : 22  |  Issue : 3  |  Page : 282-283

Late onset Vitamin K deficiency bleeding disorder presenting as nodular purpura in a 3-month-old exclusively breastfed infant


1 Department of Pediatrics, Shree Guru Gobind Singh Tricentenary Medical College and Hospital, Gurugram, Haryana, India
2 Department of Dermatology and STD, Shree Guru Gobind Singh Tricentenary Medical College and Hospital, Gurugram, Haryana, India

Date of Submission23-Jul-2020
Date of Decision13-Aug-2020
Date of Acceptance30-Mar-2021
Date of Web Publication30-Jun-2021

Correspondence Address:
Shikhar Ganjoo
Associate Professor, Department of Dermatology and STD, Shree Guru Gobind Singh Tricentenary Medical College and Hospital, Gurugram, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_124_20

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How to cite this article:
Madan S, Ganjoo S, Richa, Sharma S. Late onset Vitamin K deficiency bleeding disorder presenting as nodular purpura in a 3-month-old exclusively breastfed infant. Indian J Paediatr Dermatol 2021;22:282-3

How to cite this URL:
Madan S, Ganjoo S, Richa, Sharma S. Late onset Vitamin K deficiency bleeding disorder presenting as nodular purpura in a 3-month-old exclusively breastfed infant. Indian J Paediatr Dermatol [serial online] 2021 [cited 2021 Jul 24];22:282-3. Available from: https://www.ijpd.in/text.asp?2021/22/3/282/319940



Sir,

A 3-month-old male infant presented to the pediatrics outpatient department with multiple bluish to skin-colored raised swellings over the front and back of trunk and behind left ear for 1 month [Figure 1] and [Figure 2]. The lesions were gradual in onset and progressive in nature. The onset of the swellings was associated with bluish discoloration, which gradually faded while the swelling persisted. This was not associated with melena or noticeable bleed from any other part of the body. There was no history of fever, jaundice, abnormal movements, swellings or restriction of movements of joints, abdominal distension, vomiting, breathlessness, or history of any drug intake.
Figure 1: Largest subcutaneous nodule over the back of right shoulder measuring 3 cm × 3 cm. It is soft to firm, noncollapsible, and nontender

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Figure 2: Subcutaneous nodules over the lumbosacral area

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The infant was born of nonconsanguineous marriage and at term in the same hospital. There were no perinatal complications. The infant was immediately breastfed after birth and was on exclusive breastfeeding ever since. The infant is immunized as per age and has one elder sibling who is healthy. The mother denied a history of any drug intake by the infant or herself. The father is on treatment with carbimazole and propranolol for the past 3 years for hyperthyroidism. Cutaneous examination revealed multiple soft to firm, noncollapsible, subcutaneous, nontender nodules, largest measuring 3 cm × 3 cm over the back of right shoulder. The other nodules were present over the lumbosacral region, abdomen, and one behind the left auricle. Only two of them had a persistent bluish discoloration.

General examination revealed a healthy infant, with all anthropometric parameters adequate as per age, and normal developmental milestones. Detailed systemic examination revealed no abnormalities. Routine hemogram and biochemistry profile and urine microscopy were within normal range. Bleeding time, clotting time, prothrombin time (PT) and activated partial thromboplastin time (aPTT), plasma fibrinogen, and C reactive protein were done at baseline and found to be deranged. PT was 25.3 seconds (age specific range: 11.5-15.3 seconds), INR was 2.3 (age specific range: 0.86-1.22) and aPTT was 61.0 seconds (age specific range: 35.1-46.3 seconds). There was no evidence of thrombocytopenia. Peripheral smear revealed microcytic hypochromic anemia with teardrop cells and pencil cells. No evidence of bleed could be found on neurosonogram and ultrasound abdomen and KUB. Serum Vitamin K was advised but was declined by the parents owing to the cost. A provisional diagnosis of late idiopathic Vitamin K deficiency was made, and the infant was given 5 mg Vitamin K1 once daily by intravenous route for 3 days. The infant improved, with no occurrence of any new lesion and regression of the existent lesions. The prolonged PT, INR, and aPTT normalized after 3 days on injection Vitamin K to 11.5 s, 1.0 s, and 28.8 s, respectively.

Vitamin K is an essential fat-soluble vitamin of the human body. It contains a 2-methyl-1,4-naphthoquinone nucleus with a lipophilic side chain. It plays a major role in the coagulation pathway as it is required for the activity of coagulation factors VII, IX, X and prothrombin and for the natural anticoagulants, Protein C and S. It also acts a cofactor for osteocalcin and matrix Gla protein, involved in bone mineralization. Dietary Vitamin K1 (phylloquinone) is found in high concentration in green vegetables such as broccoli and spinach. Vitamin K2 (menaquinone) is found in meat, cheese, soybean, and egg. It is also synthesized by microflora in the gut.[1]

Newborns are at high risk for Vitamin K deficiency due to immaturity of their liver which cannot utilize Vitamin K efficiently. They also have low Vitamin K stores due to low Vitamin K content of breast milk, sterile gut at birth and due to poor placental transfer of Vitamin K. The risk of Vitamin K deficiency is increased manifold in newborns with maternal ingestion of antibiotics, anticonvulsants, warfarin, or other coumarin such as anticoagulants during pregnancy. Deficiency of Vitamin K in newborns presents as bleeding from mucosal surfaces, umbilicus, gastrointestinal tract, circumcision site, intracranial hemorrhage (ICH), or as cutaneous bruises. Early-onset Vitamin K deficiency bleeding (VKDB) occurs within the first 24 h of life and is usually associated with maternal ingestion of drugs that block Vitamin K action such as anticonvulsants (carbamazepine, phenytoin, and barbiturates), antibiotics (cephalosporins), tuberculostatic agents (rifampicin and isoniazid), and Vitamin K antagonists (warfarin). Around 25% of such babies present with ICH.[2]

Classical VKDB begins in the 1st week excluding the first 24 h, usually between days 3 and 5 and in babies with delayed or inadequate feeding, can present till 3–4 weeks of life. Bleeding is usually from the umbilicus, the gastrointestinal tract, and skin punctures and may cause significant blood loss. Surgical procedures such as circumcision unmask subclinical cases of Vitamin K deficiency. ICH is a rare but can cause of significant morbidity or death. This can be prevented largely by administration of Vitamin K at birth.[3]

Late-onset VKDB develops between 3 weeks and 8 months of age. It can be either idiopathic or secondary. The idiopathic form is described as an acquired prothrombin complex deficiency seen with exclusively breastfed infants while the secondary form is usually associated with malabsorption of Vitamin K (diarrhea, celiac disease, and cystic fibrosis) or decreased synthesis of Vitamin K due to hepatic dysfunction (biliary atresia, hepatitis, and alpha 1 antitrypsin deficiency). Majority of the cases (50%) usually present with life-threatening ICH and other central nervous system manifestations such as vomiting or seizures. Some have “warning bleeds” such as mild bruises, nose bleeds, or umbilical oozing as the first manifestation of VKDB, followed (sometimes days later) by ICH.[4]

Only a small proportion of cases presenting solely as nodular purpura has been seen.[5]

To conclude, the etiology of purpura in infants should thus be investigated in time and considered as a warning bleed. Injection Vitamin K 5–10 mg given to these infants results in improvement of symptoms and rapid correction of PT and partial thromboplastin time. Timely management of such cases can prevent the occurrence of life-threatening ICH.

The parents were consented on the publication of the images.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Furie B, Bouchard BA, Furie BC. Vitamin K-dependent biosynthesis of gamma-carboxyglutamic acid. Blood 1999;93:1798-808.  Back to cited text no. 1
    
2.
Volpe JJ. Intracranial hemorrhage in early infancy – Renewed importance of Vitamin K deficiency. Pediatr Neurol 2014;50:545-6.  Back to cited text no. 2
    
3.
American Academy of Pediatrics Committee on Fetus and Newborn. Controversies concerning Vitamin K and the newborn. American Academy of Pediatrics Committee on Fetus and Newborn. Pediatrics 2003;112:191-2.  Back to cited text no. 3
    
4.
Sutor AH, Dagres N, Niederhoff H. Late form of Vitamin K deficiency bleeding in Germany. Klin Padiatr 1995;207:89-97.  Back to cited text no. 4
    
5.
Gahalaut P, Chauhan S. Vitamin K deficiency bleeding presenting as nodular purpura in infancy: A rare and life-threatening entity. Indian J Dermatol 2013;58:407.  Back to cited text no. 5
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