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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 22  |  Issue : 3  |  Page : 244-246

Raynaud's phenomenon in an adolescent girl: A case report


Department of Paediatrics, Government Medical College, Nagpur, Maharashtra, India

Date of Submission26-Feb-2020
Date of Decision10-Sep-2020
Date of Acceptance04-Apr-2021
Date of Web Publication30-Jun-2021

Correspondence Address:
Rajkumar Motiram Meshram
Department of Paediatrics, Government Medical College, Nagpur, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_31_20

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  Abstract 


Introduction: Triphasic color change of the distal extremities in response to cold and emotional stress is known as Raynaud's phenomenon (RP). Fingers, toes, nose, and ears are the commonly affected organs. Secondary RP is usually associated with underlying connective tissue disorder. Case Report: An 11-year-old adolescent girl presented with bluish discoloration of digits and toes, which were more marked on cold exposure. She had a positive antinuclear antibody test. Her grandmother was a known case of rheumatoid arthritis. She was managed with nifedipine, counseling and avoidance of cold environment. Discussion: It is essential to search for any underlying causes especially connective tissue diseases in such cases. Effective education and clear explanation about the disease reduce the anxiety and improve the quality of life.

Keywords: Child; Female; Nifedopine; Rheumatic diseases; Antibodies, antinuclear


How to cite this article:
Meshram RM, Gajimwar VS, Gholap S, Jhanwar M. Raynaud's phenomenon in an adolescent girl: A case report. Indian J Paediatr Dermatol 2021;22:244-6

How to cite this URL:
Meshram RM, Gajimwar VS, Gholap S, Jhanwar M. Raynaud's phenomenon in an adolescent girl: A case report. Indian J Paediatr Dermatol [serial online] 2021 [cited 2021 Jul 24];22:244-6. Available from: https://www.ijpd.in/text.asp?2021/22/3/244/319945




  Introduction Top


Triphasic color change consisting of paleness, bluish discoloration, and redness of the distal extremities in response to cold and emotional stress is known as Raynaud's phenomenon (RP). It was demonstrated for the first time in an adult patient by Maurice Raynaud in 1862. The first description of RP in children appeared in 1967.[1],[2] RP is considered to be rare in children, and girls are affected more frequently.[3] Primary RP is more common in children. Secondary RP is usually associated with an underlying collagen vascular disease. Fingers, toes, nose, and ears are the commonly affected organs.[4] Usually, primary RP does not require any treatment, except reassurance and avoidance of precipitating factors; however, an effort should be made to ensure that it is not associated with irreversible ischemia, resulting into digital ulceration and gangrene due to scleroderma and related diseases. Here, we describe an adolescent girl with RP who responded to reassurance, avoidance of cold environment, and calcium channel blocker.


  Case Report Top


An 11-year-old girl was admitted to our institute with a history of bluish discoloration of the fingers of both hands for the past 1½ months. Initially, discoloration was in the index, middle, and ring finger of distal phalanx of both hands in a patchy manner, which gradually progressed to involve the entire distal phalanx and other fingers of both the hands and was associated with pain. On further inquiry, she experienced deepening of discoloration after exposure to cold environment and also had a history of difficulty in holding a pen while writing. She had no history of joint pain/swelling, rash, bleeding diathesis, cardiac complaints, or tingling and numbness in the limbs. There was no history of insect bite/local repeated trauma, drug exposure, use of naphthalene balls, recent vaccination, blood transfusion, and exposure to environmental toxins. Her past history and birth history were not significant. She had not achieved menarche. Her maternal grandfather died because of myocardial infarction, maternal grandmother is a diagnosed case of rheumatoid arthritis, and her mother also has history of joint pain and swelling.

On examination, her vitals were stable with oxygen saturation of at room air. All the peripheral pulses were well felt and blood pressure was within normal limit for age. Her growth and development were normal, and Tanner staging was prepubertal. There were no signs of vitamin deficiency, petechiae/purpura, malar rash, joint swelling, and cafe-au lait spots. There were no signs suggestive of scleroderma, Sjogren's syndrome, or lupus erythematosus. Systemic examination and ophthalmic examination were normal. Local examination revealed cold palms and bluish discoloration of the distal phalanges of all digits, and minimal discoloration of toes of both feet this discoloration were more marked on exposure to cold [Figure 1]a and [Figure 1]b and [Figure 2].
Figure 1: (a and b) Bluish discoloration of fingers

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Figure 2: Bluish discoloration of toes

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Laboratory analysis at the time of admission showed hemoglobin 11.1 gm%, white blood cell count 11,100/mm3, and platelet 297,000/mm3 with granulocyte count 76.9% without band cells. The erythrocyte sedimentation rate was 43 at the end of 1st hour with C-reactive protein and tuberculin test being negative. Her liver, kidney, and thyroid functions were within normal limit. Coagulation profile, lipid profile, and D-dimer assay were normal with an International Normalized Ratio of 0.8. Serum calcium, phosphorus, uric acid, sodium, and potassium were within normal limits. Cold agglutinin antibody test, Australia antigen, hepatitis C as well as HIV serology were all negative. Antinuclear antibody (ANA) titer was 1:100 by indirect immunofluorescence assay, but anti-dsDNA antibody, phospholipid antibody, anti-Smith antibody, and rheumatoid factor were negative. Chest radiography, ultrasound abdomen, two-dimensional echocardiography, and Doppler study of the upper and lower limbs did not detect any abnormality. Nail-fold capillaroscopy could not be performed due to unavailability of instrument in our institute. On the basis of clinical presentation and laboratory evidence, a diagnosis of RP was made and calcium channel blocker (nifedipine - 1mg/kg/day in two divided doses i.e. 15 mg two times a day) was started. Digital color changes gradually decreased, and she was discharged with counselling about protection from cold environment. She was on monthly follow-up initially and later on every 3 monthly. After follow-up of 1 year, she had no signs of systemic illness.


  Discussion Top


RP is highly heterogeneous and relatively uncommon in children. The prevalence of this disorder in general population is 4.85% and the annual incidence is 0.25%.[5] Although it is difficult to estimate the prevalence of RP in children, Jones et al reported 14.9% of patients between 12 to 15 years of age who had symptoms consistent with RP. In the school going children of United Kingdom, 18% of girls and 12% boys reported color changes in finger during cold climate.[6] Though RP is also reported in infants, a retrospective study done by Nigrovic et al reported that features of RP in 80% of the girls in their study had a mean age of symptoms onset between 12.3±4.3 years, while the mean age at diagnosis was 13.4±4.0 years.[6],[7],[8],[9] Majority of children who have primary RP patients with secondary RP are usually associated with connective tissue disorder. Multiple etiological factors are responsible for this condition.[10] Cold climate, female sex, positive family history, and rheumatological disorders such as systemic lupus erythematosus and systemic sclerosis are associated with RP.[11] Infectious agents (parvovirus, Helicobacter pylori), hematologic conditions (cryoglobulins, cold agglutinin disease, hyperhomocystinemia), toxins (jellyfish sting), and drugs such as beta blockers, clonidine, ergot alkaloids, selective serotonin re-uptake inhibitors, and sympathomimetic drugs are responsible for this phenomenon.[12],[13]

Irrespective of the underlying etiology, the main mechanism of RP is vasospasm of the arteries and arterioles due to cold-induced vasoconstriction and release of catecholamine, endothelin-1, and 5-hydroxytryptamine.[10] In RP, fingers, toes, and nose are typically involved due to anatomic peculiarity of high density of arteriovenous anastomosis, resulting into less capillary blood flow. Even though the triphasic (pallor, cyanosis, and erythema) color changes of RP were originally described by Maurice Raynaud, only a fraction of patients have this typical color change pattern and most of the patients of primary or secondary RP experience isolated bluish discoloration of digits. Chronic chilblains (Pernio) is a painful localized inflammatory skin lesion aggravated by cold exposure; it is one of the differential diagnoses of RP should be kept in mind which is characterized by prolonged capillary refill time and an abnormal Allen test. Another condition, primary erythromelalgia, mimics the hyperemic phase of RP which is worsened by warm temperatures and relieved by cold.[11]

A major concern in managing this condition is to determine the cause as well as the potential for serious complications and deterioration in quality of life. RP may be one of the early presentations of an underlying disease. One study revealed that 37.2% of individuals who had RP were subsequently diagnosed with a connective tissue disease.[14] Abnormal nail-fold capillaries and presence of ANA has strong association with secondary RP. In our case, capillaroscopy could not be performed due to unavailability of facility, but erythrocyte sedimentation rate was elevated with positive ANA (titer 1:100). Effective cold avoidance and stress reduction constitute the main treatment plan in primary RP and are also major factors in treating patients with secondary RP. Current evidence supports the use of a calcium channel blocker or synthetic prostacycline analogue (iloprost), but robust evidence is lacking for other agents. In our patient, symptoms were well controlled and a good quality of life was established by restriction of exposure to cold and use of a calcium channel blocker (nifedipine).


  Conclusion Top


Avoidance of cold exposure is the most effective way to relieve this condition. Effective education and clear explanation about the disease reduce anxiety, and providing reassurance can help alleviate the severity of disease. In addition, follow-up assessment over time is essential to detect development of any underlying disease.

Declaration of consent

The authors certify that they have obtained all appropriate consent forms, duly signed by the parent(s) of the patient. In the form the parent(s) has/have given his/her/their consent for the images and other clinical information of their child to be reported in the journal. The parents understand that the names and initials of their child will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Raynaud M. On local asphyxia and symmetrical gangrene of the extremities [1862]. New researches on the nature and treatment of local asphyxia of the extremities [1874]. In: Barlow T, editor. Trans Selected monographs. London: New Sydenham Society; 1988.  Back to cited text no. 1
    
2.
Guntheroth WG, Morgan BC, Harbinson JA, Mullins GL. Raynaud's disease in children. Circulation 967;36:724-9.  Back to cited text no. 2
    
3.
Goldman RD. Raynaud phenomenon in children. Can Fam Physician 2019;65:264-5.  Back to cited text no. 3
    
4.
Pain CE, Constantin T, Toplak N, Moll M, Iking-Konert C, Piotto DP, et al. Raynaud's syndrome in children: Systemic review and development of recommendations for assessment and monitoring. Clin Exp Rheumatol 2016;34:1-7.  Back to cited text no. 4
    
5.
Garner R, Kumari R, Lanyon P, Doherty M, Zhang W. Prevalence, risk factors and associations of primary Reynaud's phenomenon: Systematic review and meta-analysis of observational studies. BMJ Open 2015;5:e006389.  Back to cited text no. 5
    
6.
Jones GT, Herrik AL, Woodham SE, Baildam EM, Macfarlane GJ, Silman AJ. Occurrence of Raynaud's phenomenon in children age 12-15 years: Prevalence and association with other common symptoms. Artritis Rheum 2003;48:3581-21.  Back to cited text no. 6
    
7.
Nigrovic PA, Fuhlbrigge RC, Sundel RP. Raynaud's phenomenon in children: A retrospective review of 123 patients. Pediatrics 2003;111:715-21.  Back to cited text no. 7
    
8.
Sharathkumar AA, Castillo-Caro P. Primary Raynaud's phenomenon in an infant: A case report and review of literature. Pediatr Rheumatol Online J 2011;9:16.  Back to cited text no. 8
    
9.
Kobayashi M, Takano KI, Kamizono J, Ichikawa K. A serious case of primary Raynaud's phenomenon in an infant. Clin Case Rep 2018;6:2089-91.  Back to cited text no. 9
    
10.
Wigley FM, Flavahan NA. Raynaud's phenomenon. N Engl J Med 2016;375:556-65.  Back to cited text no. 10
    
11.
Rigante D, Fastiggi M, Ricci F, D'Errico F, Bracci B, Guerriero C. Handy hints about Raynaud's phenomenon in children: A critical review. Pediatr Dermatol 2017;34:235-9.  Back to cited text no. 11
    
12.
Khouri C, Blaise S, Carpentier P, Villier C, Cracowski JL, Roustit M. Drug induced Raynaud's phenomenon beyond beta-adrenocepter blockers. Br J Clin Pharmacol 2016;82:6-12.  Back to cited text no. 12
    
13.
Binnetoglu FK, Kizildag B, Topaloglu N, Kasapcopur O. Severe digital necrosis in a 4-year-old boy: Primary Raynaud's or jellyfish sting. BMJ Case Rep 2013;2013:bcr2013201478.  Back to cited text no. 13
    
14.
Pavlov-Dolijanovic SR, Damjanov NS, Vujasinovic Stupar NZ, Baltic S, Babic DD. The value of pattern capillary changes and antibodies to predict the development of systemic sclerosis in patients with primary Raynaud's phenomenon. Rheumatol Int 2013;33:2967-73.  Back to cited text no. 14
    


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