Indian Journal of Paediatric Dermatology

ORIGINAL ARTICLE
Year
: 2021  |  Volume : 22  |  Issue : 2  |  Page : 118--122

Scurvy in children: Down but not out


Sukhjot Kaur1, Jatinder Singh Goraya2,  
1 Department of Dermatology, Venereology, and Leprology, Dayanand Medical College and Hospital, Ludhiana, Punjab, India
2 Department of Pediatrics, Division of Pediatric Neurology, Dayanand Medical College and Hospital, Ludhiana, Punjab, India

Correspondence Address:
Jatinder Singh Goraya
Department of Pediatrics, Division of Pediatric Neurology, Dayanand Medical College and Hospital, Ludhiana, Punjab
India

Abstract

Background: Scurvy, a common disease of the past, has become rare in the modern era. Because of lack of awareness regarding its clinical and radiological manifestations, diagnosis of scurvy is often missed or delayed resulting in unnecessary and potentially harmful diagnostic and therapeutic interventions. Dermatological manifestations are a very important component of the clinical picture and can suggest the diagnosis to the unsuspecting physicians. The objectives were to describe the clinical presentation of scurvy in children in the modern era and to highlight its dermatological manifestations. Methods: This was a retrospective chart review of children diagnosed with scurvy over a period of 10 years at a tertiary care hospital. Results: Eleven children, 6 girls and 5 boys, aged from 21 months to 16 years with scurvy were identified. All the children had an underlying disorder that predisposed them to scurvy, with cerebral palsy in 10 and autism spectrum disorder with celiac disease in one child. All had presented with musculoskeletal complaints of irritability, limb pain, painful restriction of movements, and in some cases, swelling of the affected limb. Dermatological signs consisting of various combinations of perifollicular hyperkeratosis and hemorrhages, petechiae, ecchymosis, corkscrew hair, and bleeding gums were present in 10 children. Characteristic radiographic changes were seen in all the 8 children who underwent this examination. All children responded dramatically to treatment with oral Vitamin C. Conclusions: Awareness of clinical and radiographic signs of scurvy allows early diagnosis and treatment of scurvy. Dermatological manifestations are the most noticeable and helpful clues to the diagnosis of scurvy.



How to cite this article:
Kaur S, Goraya JS. Scurvy in children: Down but not out.Indian J Paediatr Dermatol 2021;22:118-122


How to cite this URL:
Kaur S, Goraya JS. Scurvy in children: Down but not out. Indian J Paediatr Dermatol [serial online] 2021 [cited 2021 Apr 18 ];22:118-122
Available from: https://www.ijpd.in/text.asp?2021/22/2/118/312828


Full Text



 Introduction



Scurvy, a Vitamin C deficiency disease, is an ancient disease with an interesting historical account. It occurred in epidemic proportions in the past, especially on sea voyages, and with the realization that its cure or prevention could be accomplished by oranges and lemons, it became an uncommon disease.[1],[2] Scurvy has not completely disappeared in the modern era, though. In children, it continues to be seen in certain high-risk groups such as those with neurodevelopmental disorders and those with poverty.[3],[4],[5],[6] Underlying mechanism in these conditions is an extremely restricted diet. In infants, scurvy occurs because of the use of heated milk as heating destroys Vitamin C in the milk. Medical conditions such as malabsorption, food allergies, iron overload conditions such as thalassemia, renal failure, alcoholism, and malnutrition also predispose individuals to Vitamin C deficiency.[3],[4],[6]

Scurvy in children usually presents with musculoskeletal symptoms and signs.[4],[6],[7],[8],[9] Cutaneous signs include bleeding and swollen gums, petechiae/ecchymoses, follicular hyperkeratosis, perifollicular hemorrhages, and “corkscrew” hair, and are very helpful in clinical diagnosis of scurvy.[8],[10],[11] Characteristic radiographic findings and rapid amelioration of symptoms following specific treatment with Vitamin C confirm the diagnosis.[2],[7],[10],[11],[12] Serum or white blood cell Vitamin C levels are helpful but not required for the diagnosis as the results are rarely available soon enough to guide treatment.[4],[6],[7],[8],[9] Since scurvy is now encountered only rarely, awareness about its various clinical and radiographic findings has diminished. As a result, diagnosis of scurvy may be delayed or missed altogether resulting in unnecessary and potentially harmful diagnostic and therapeutic interventions. In this report, we describe our experience with scurvy in children.

 Methods



This is a retrospective chart review of children diagnosed with scurvy in the department of Dermatology and Pediatrics at the Dayanand Medical College and Hospital, Ludhiana, Punjab, from February 2010 and May 2020, Cases were identified from inpatient and outpatient hospital records. Diagnosis of scurvy was made on the basis of clinical presentation, radiological findings, and response to treatment. Laboratory investigations were done to exclude alternative diagnoses where appropriate. Charts of the children thus identified were examined, and data on age, gender, clinical presentation, physical signs, underlying disease, laboratory and radiographic findings, and response to treatment with Vitamin C were extracted. One case has previously been reported.[13] The study was approved by the Institutional Ethics Committee (IEC 2014-13, dated 05.04.2014).

 Results



Eleven children, Six girls and five boys, aged between 21 months and 16 years were diagnosed with scurvy over a period of 10 years. The diagnosis of scurvy was made on the basis of combination of characteristic clinical (n = 11) and radiographic findings (n = 8) and rapid response to treatment with Vitamin C (n = 11). The demographics and clinical findings of the children with scurvy are summarized in [Table 1]. All the children had an underlying neurodevelopmental disorder including cerebral palsy in ten children and autism spectrum disorder in one child. Case summaries of three cases are described. Case 5 included in this series has already been reported.[13]{Table 1}

Case 1

This 16-year-old boy developed left shoulder swelling and painful restriction of movements six months prior to presentation to our hospital. Osteomyelitis was suspected at a local hospital, and treatment with antibiotics was initiated. Incision and drainage revealed only hemorrhagic fluid. Shoulder pain did not subside, and a new swelling of right elbow with discoloration was noted. Needle aspiration was attempted, and hemorrhagic fluid was obtained. Malignancy was suspected and the child was referred. A review of history uncovered a previous diagnosis of cerebral palsy and intellectual disability secondary to perinatal hypoxic-ischemic injury. The child had significant swallowing dysfunction; as a result, his diet was extremely limited and consisted largely of liquids and semisolids. There was little intake of fruits and vegetables, and milk was given after heating. Physical examination revealed wasted appearance and spastic quadriplegia with limb contractures. Tender costochondral rosary was present on chest examination. Perifollicular hyperkeratosis with petechial bleeds and corkscrew hair were observed on lower extremities. Oral cavity examination revealed red spongy gums with easy bleeding. Examination of the right upper limb revealed tender swelling around the elbow and distal arm with tenderness, petechiae, and ecchymoses affecting the distal arm and proximal forearm. Movements at the elbow were painful. X-ray of the affected arm revealed scorbutic changes of ground-glass appearance, pencil-thin cortex, periosteal reaction, and ringed epiphyses. An old healing fracture of proximal humerus was also seen [Figure 1]. Complete blood counts, PT/INR, and APTT were normal. Diagnosis of scurvy was made, and treatment with Vitamin C 500 mg/day resulted in complete resolution of symptoms over 3 weeks.{Figure 1}

Case 8

This 13-year-old girl with cerebral palsy with epilepsy controlled on sodium valproate presented with painful restriction of movements of both the lower extremities. She had fever and cough for one day prior to presentation. There was no history of rash or trauma. Examination revealed tenderness at knees and painful restriction of movements. She was anemic and her gums were red and swollen. The skin of the lower extremities was dry and showed follicular hyperkeratosis and corkscrew hair deformity [Figure 2]. Hemogram revealed microcytic anemia (hemoglobin: 6.8 g/dL, mean corpuscular volume: 59.9 fl) with normal platelet count (platelets: 178 × 103/μl). Serum calcium was 7.2 mg/dL (normal: 8.6–10.2 mg/dL), phosphorus 1.0 mg/dL (normal: 2.7–4.5 mg/dL), and alkaline phosphatase 511 (40–300). Serum 25-hydroxy Vitamin D was 0.94 ng/mL (normal: 20–40). Coagulogram was normal. Radiographic examination of bilateral knees showed both rachitic and scorbutic changes. She was treated with oral Vitamin C 500 mg daily for 7 days followed by 250 mg/day for 4 weeks. Complete recovery occurred in 2 weeks. She was also supplemented with iron and Vitamin D.{Figure 2}

Case 11

This 6-year-old girl with cerebral palsy and epilepsy presented with a 3-week history of irritability, generalized pain, and bleeding gums. There was no history of fever, rash, or trauma. Her diet was extremely limited because of chewing and swallowing difficulties caused by cerebral palsy. Examination revealed tender red and swollen gums with bleeding on touch [Figure 3]. Movements of legs were painful and restricted, especially at knee. Follicular hyperkeratosis and perifollicular petechial hemorrhages were also noted on lower extremities [Figure 4]. Radiographic examination of bilateral thighs and knees revealed changes consistent with scurvy. She was treated with oral Vitamin C 500 mg daily for 4 weeks with complete recovery.{Figure 3}{Figure 4}

 Discussion



Scurvy has become extremely rare in healthy children and is now largely seen in children with underlying neurodevelopmental disorders (cerebral palsy, intellectual disability, autism spectrum disorder, etc.) and other risk factors (food allergies, malabsorption syndromes, malnutrition, iron overload conditions, and renal failure).[3],[4],[6],[9],[12],[14] As a result, most of the recent descriptions of scurvy have been restricted to case reports or case series of small number of patients. None of the children were previously healthy; ten children had cerebral palsy and one had autism spectrum disorder. Children with cerebral palsy frequently have comorbid chewing and swallowing difficulties due to oro-motor dysfunction. As a result, their diet is devoid of solid foods and is nutritionally insufficient.[3],[4],[6] Children with autism spectrum disorder have limited food repertoire and are more prone to restrictive diets because of behavioral and sensory issues. In our series, the child with autism not only had restricted diet but also suffered from celiac disease. Animal milk is prominent component of diets in Indian children, but the common Indian practice of boiling milk before feeding destroys its Vitamin C content. Moreover, if there are no fresh fruits and vegetables in their diet, Vitamin C deficiency may ensue. In addition to scurvy, many of our patients had multiple other nutritional deficiencies including but not limited to wasting, anemia, and Vitamin D and Vitamin B12 deficiency [Table 1]. This is underestimation as full laboratory evaluations were not undertaken in all the children because of financial constraints. Perkins et al. also found deficiencies of Vitamin D and Vitamin B12 in some of their patients with scurvy.[6]

Children with scurvy most commonly present with musculoskeletal symptoms with limping, pain, swelling, and pseudoparalysis.[2],[5],[6],[9],[12] All the children in our study had musculoskeletal presentation. Musculoskeletal symptoms arise from hemorrhages into soft tissues, muscles, subperiosteal regions of the bone, and joints. Constitutional symptoms of failure to thrive, irritability, and anorexia are common. In the absence of clinical suspicion, however, patients may be thought of having infectious, malignant, or a rheumatological condition leading to an extensive but unproductive diagnostic odyssey.[5],[6] For want of correct diagnosis, one of our patients (Case 1) underwent unnecessary invasive surgical procedures, treatment with antibiotics, and referral for suspected musculoskeletal malignancy.

Hallmark of scurvy is petechiae or ecchymotic lesions. They are generally more prominent on legs and have perifollicular distribution. Perifollicular hyperkeratosis is also noted, which in the presence of perifollicular hemorrhages may be misinterpreted as palpable purpura of vasculitis.[10],[11] At the same time, the combination of follicular hyperkeratosis and perifollicular hemorrhage is pathognomonic and occurs early in the course of scurvy.[11] Hydrostatic and other mechanical factors may account for crural predominance of hemorrhages in the skin as well as for the variable leg edema which is sometimes seen in patients with scurvy. Hemorrhage can also be seen in the periungual and periorbital areas as well as elsewhere in the skin. Hair may have coiled appearance, so-called “corkscrew” hair. Hair may fracture easily.[8],[10],[11]

Oral cavity is frequently affected, especially the gums. Gum changes are seen in only nonedentulous patients and begin at interdental and marginal gingiva. Gingivae become red, smooth, swollen, and shiny and bleed easily. Gums may turn purplish or even black from necrosis.[8],[10],[11] In advanced cases, teeth loss may occur from ligamental loosening and alveolar bone absorption secondary to scurvy. Poor oral-dental hygiene, which is always an issue in children with neurodevelopmental disorders, predisposes to gum changes of scurvy.[11]

The diagnosis of scurvy is generally made on clinical grounds based on characteristic presentation. Dietary history of inadequate Vitamin C intake is important but is generally obtained in retrospect after the clinical diagnosis has been suspected. Therefore, a very high index of clinical suspicion is required by the physician who is knowledgeable of various clinical manifestations of scurvy. Radiographic findings help to corroborate the clinical suspicion as well as exclude other causes.[4],[7],[10],[12] Plasma Vitamin C levels are generally not helpful as they are affected by dietary intake. Detection of reduced Vitamin C in platelets or leucocytes is not easily available, technically difficult, and turn over time may be too long to be of practical use in treatment decisions. Instead, dramatic clinical response to treatment with Vitamin C provides the best evidence for the diagnosis of scurvy.[2],[10],[11],[15] All our patients showed dramatic response to oral Vitamin C and they all became completely symptom free in 2–3 weeks.

As scurvy has become an uncommon disease, awareness among physicians regarding its clinical manifestations is decreasing, resulting in misdiagnosis and inappropriate interventions. Once suspected, the clinical diagnosis of scurvy can easily be established on the basis of clinical signs, radiological findings, and gratifying response to treatment with Vitamin C. Common misdiagnoses of scurvy include accidental or nonaccidental trauma, osteomyelitis, septic arthritis, connective tissue disorders, hematological disorders, and malignancy.[2],[7],[8]

Major limitation of this study is its retrospective nature. Most patients were evaluated and treated on outpatient basis with very limited resort to laboratory investigations beyond radiographic examinations. Vitamin C was not measured in any of the patients because of lack of in-house laboratory testing and financial constraints. However, the clinical and radiographic findings were quite suggestive of scurvy and a rapid gratifying response to treatment with Vitamin C was enough to settle the diagnosis. In all our patients, to our advantage, we were able to achieve the correct diagnosis with limited investigations and without much delay.

 Conclusions



Scurvy is a diagnosis that is “down but not out.” While dermatologists may not be involved in the direct care, they may be asked to opine on skin findings. Awareness among dermatologists of various cutaneous findings of scurvy, is important to prevent missing the diagnosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials 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.

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