Background

Scurvy is Vitamin C deficiency. It occupies an interesting place in the history of the world when English sailors became progressively ill after long voyages at sea. It was only with the observation that the consumption of fruit helped to ward off the devastating effects of scurvy did the sailors begin to transport limes with them. Hence, the term Limeys came to refer to English sailors.

Scurvy results because humans cannot synthesize vitamin C (ascorbic acid) in our bodies. We lack the enzyme L-gluconolactone which converts L-gluconogammalactone to L-ascorbic acid (vitamin C). Curiously, guinea pigs are the only other mammal lacking this enzyme. Vitamin C is essential for collagen synthesis. Any deficiency will affect any organ or structure that requires collagen. Patients may present with hyperkeratotic papules centered in follicles. Hair may grow in a corkscrew-like pattern and may be associated with hemorrhage, often with minor trauma. In chronic disease, ecchymoses, usually over pressure points such as the shins, may occur. The mouth may show gingival hypertrophy. Hemorrhage may occur in any mucosal surface including conjunctiva, subungua, and skin. In addition, hemorrhage in the skeletal muscle, intra-articular sites, and subperiosteal region of the epiphyses of the long bones may occur.

OUTLINE

Epidemiology
Disease Associations
Pathogenesis
Laboratory/Radiologic/ Other Diagnostic Testing
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Special Stains/ Immunohistochemistry/ Electron Microscopy
Differential Diagnosis
Prognosis
Treatment
Commonly Used Terms
Internet Links

 

EPIDEMIOLOGY	CHARACTERIZATION
SYNONYMS	Vitamin C deficiency
INCIDENCE	Rare, usually in poverty stricken areas
AGE RANGE-MEDIAN	Usually children but may affect any age Especially 6 months to 1 year

 

EPIDEMIOLOGIC ASSOCIATIONS	CHARACTERIZATION
Alcoholics
Dietary faddists
Esophageal reflux
Unsupplemented breast milk	If milk formula is unsupplemented by citrus fruits or vegetables

 

LABORATORY/ RADIOLOGIC/ OTHER TESTS	CHARACTERIZATION
Radiographs	Massive periosteal reaction with increase in the distance between tibia and fibula
CT scan and MRI
Laboratory Markers

 

CLINICAL APPEARANCE/ GROSS DISEASE

CHARACTERIZATION

An orange a day keeps the doctor away: scurvy in the year 2000. Weinstein M, Babyn P, Zlotkin S. Department of Paediatrics, Hospital for Sick Children and University of Toronto, Toronto, Canada.

Pediatrics 2001 Sep;108(3):E55 Abstract quote Scurvy has been known since ancient times, but the discovery of the link between the dietary deficiency of ascorbic acid and scurvy has dramatically reduced its incidence over the past half-century. Sporadic reports of scurvy still occur, primarily in elderly, isolated individuals with alcoholism. The incidence of scurvy in the pediatric population is very uncommon, and it is usually seen in children with severely restricted diets attributable to psychiatric or developmental problems. The condition is characterized by perifollicular petechiae and bruising, gingival inflammation and bleeding, and, in children, bone disease. We describe a case of scurvy in a 9-year-old developmentally delayed girl who had a diet markedly deficient in vitamin C resulting from extremely limited food preferences. She presented with debilitating bone pain, inflammatory gingival disease, perifollicular hyperkeratosis, and purpura. Severe hypertension without another apparent secondary cause was also present, which has been previously undescribed. The signs of scurvy and hypertension resolved after treatment with vitamin C. The diagnosis of scurvy is made on clinical and radiographic grounds, and may be supported by finding reduced levels of vitamin C in serum or buffy-coat leukocytes. The response to vitamin C is dramatic. Clinicians should be aware of this potentially fatal but easily curable condition that is still occasionally encountered among children.

 

HISTOLOGICAL TYPES	CHARACTERIZATION
General	Psoraisiform hyperplasia with pallor of the lower half of the epidermis Numerous extravasated rbcs with lymphocytic infiltrate in the upper dermis
VARIANTS

 

DIFFERENTIAL DIAGNOSIS	KEY DIFFERENTIATING FEATURES
Deficiency dermatitis (acrodermatitis enteropathica, pellagra, necrolytic migratory erythema, Hartnup's disease, kwashiokor)	These deficiency diseases usually show epidermal pallor in the upper part of the epidermis

Cutis 1980;26:375-377
Arch Dermatol 1984;120:1212-1214
Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.

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Last Updated 10/1/2001

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