Test your knowledge with multiple-choice cases This month 9 cases: 1. Dark, Fuzzy Spots on the Back p.37 2. Thin, Concave Fingernails p.38 3. A Stye and Eyelid Swelling p.39 4. Discolouration of the Legs p.40 Copyright 5. Longitudinal Fingernail Bands p.41 6. Persistent, Pruritic Plaques p.42 7. White Cheek Patches p.43 8. Discolouration of the Shoulder p.44 9. Eruption on the Inner Bicep p.45 Not for Sale or Commercial Distribution Unauthorised use prohibited. Authorised users can download, display, view and print a single copy for personal use Case 1 Dark, Fuzzy Spots on the Back A 37-year-old woman presents with several dark and fuzzy spots on her back of several years duration. a. Seborrheic keratoses b. Compound nevi c. Pedunculated nevi d. Dysplastic nevi e. Lentigines Atypical nevi are moles with clinically unusual features. A biopsy with histopathology will provide the diagnosis of dysplastic nevi (answer d). The concern is that it can be hard to tell whether a dysplastic nevus might already be an early melanoma; hence, they are often biopsied or excised. They can occur sporadically or can be inherited. Atypical nevi are often asymmetric, have fuzzy or blurred borders, have variegated or multiple colours, are bigger than 5 mm in diameter, and/or may change in size or colour over time (these are referred to as the ABCDE rules). Sporadic, atypical nevi are much more common in fair-skinned persons, especially if they have had a fair bit of sun or tanning salon exposure. People with atypical or dysplastic nevi are at a slightly higher risk than the general population of developing melanoma (especially if they have five or more atypical nevi). These patients should be educated about sun protection and avoidance, and they should be taught how to examine their skin. They should also have their skin periodically reviewed by a physician. Benjamin Barankin, MD, FRCPC, is Medical Director and Dermatologist at the Toronto Dermatology Centre in The Canadian Journal of CME / February 2014 37
Case 2 Thin, Concave Fingernails An 89-year-old, Caucasian woman is admitted to the coronary care unit with a complaint of chest pain radiating to her back. She was recently diagnosed with an ascending aortic aneurysm. Physical examination is remarkable only for hypertension and thin fingernails that have a concave appearance. CT reveals progression of her ascending aneurysm and a new descending thoracic aortic dissection. The patient s aortic dissection is managed medically with improvement in her BP control, and a surgery is planned to repair her ascending aneurysm. a. Hyperthyroidism b. Iron deficiency anemia c. Psoriasis d. Endocarditis e. Bronchiectasis This patient exhibits koilonychia, commonly referred to as spoon nails, owing to the upward curvature of the distal nail plate, which gives the nail a spoon-like appearance. This abnormality is most often associated with iron deficiency anemia (answer b). There are other causes, including trauma, hemochromatosis, systemic lupus erythematosus, hypothyroidism, nail-patella syndrome, and occupational exposure to petroleumbased products. It is often a normal finding in infants. Koilonychia is a cutaneous manifestation of a systemic disease, rather than a primary nail abnormality. Iron deficiency anemia usually results from blood loss, most often from the gastrointestinal tract. Other gastrointestinal conditions commonly implicated include celiac sprue, gastritis, inflammatory bowel diseases, and diets deficient in iron. In women, one Figure 1: Thin Fingernails Figure 2: Thin Fingernails with a Distinguishable Concave Appearance should also consider gynecological causes of blood loss. Other causes include renal failure, pulmonary hemosiderosis, and intravascular hemolysis. Diagnosis is based on a thorough history and physical examination. Laboratory investigations consistent with iron deficiency anemia include a microcytic anemia, decreased reticulocyte count, low serum iron, increased total iron binding capacity, low transferrin saturation level, and low serum ferritin. Treatment should be aimed at correcting the underlying cause. The patient did acknowledge a past history of iron deficiency anemia. Brent M. McGrath, MD, MSc, PhD, is an Associate Chief Resident in the Department of Medicine at Saint John Regional Hospital in Saint John, New Brunswick, and Dalhousie University in Halifax, Nova Scotia. Nicholas A. Forward, MD, MSc, is a Resident in the Department of Medicine at Dalhousie University in Halifax, Nova Scotia. Corey Stevens, BSc, is a Medical Student at Dalhousie Medicine New Brunswick in Saint John, New Brunswick, and the Faculty of Medicine at Dalhousie University in Halifax, Nova Scotia. 38 The Canadian Journal of CME / February 2014
Case 3 A Stye and Eyelid Swelling A 14-year-old male presents with a stye on his right eyelid, which first appeared a few days ago. He later noticed swelling of the same eyelid. He denies trauma, blurry vision, or pain. a. Dacryocystitis b. Orbital cellulitis c. Periorbital cellulitis d. Angioedema e. Blepharitis Periorbital cellulitis (commonly known as preseptal cellulitis) (answer c) usually occurs from exogenous sources such as trauma, insect bites, and infections. It can also be an extension of an eyelid infection, such as a chalazion or hordeolum. On examination, patients with periorbital cellulitis will have an erythematous, swollen, and painful eyelid. Patients may also have a mild fever. The eyeball itself is usually unaffected, unless it extends to an orbital cellulitis. One way to differentiate between periorbital and orbital cellulitis is that the latter can be accompanied by a decrease in vision, less reactive pupils, pain with eye movement, chemosis (conjunctival edema), and even proptosis (bulging forward of the eyeball). Periorbital cellulitis is usually treated with a 7 to 10 day course of oral antibiotics, allowing for coverage of Staphylococcus aureus and Streptococcus pyogenes, which are the two most common pathogens involved. Antibiotic choices include cephalexin, clindamycin, and amoxicillin/clavulanate. Further investigation and treatment may be warranted if there is failure to improve within two to three days, and there should be close vigilance for the development of orbital cellulitis. Kimmy Goyal, MD, CCFP, ABFM, is a Family Doctor in Brampton, Ontario. Fenny Goyal, MD, is a Medical Graduate from the Windsor University School of Medicine in Cayon, Saint Kitts-Nevis. The Canadian Journal of CME / February 2014 39
Case 4 Discolouration of the Legs A 62-year-old female presents with reddish-brown discolouration on the right and left pretibial areas of her legs. On examination, there are nonblanching, red, pinpoint dots within the lesions. a. Pigmented purpuric dermatoses b. Thrombocytopenic purpura c. Stasis dermatitis d. Psoriasis Pigmented purpuric dermatoses (PPD) (answer a) are characterized by a reddish-brown discolouration of the skin that occurs primarily on the lower extremities and occasionally extends to the lower trunk and upper extremities. The four clinical variants of PPD are known as Schamberg disease, Majocchi disease, Gougerot-Blum disease, and Lichen Aureus. While the etiology is not entirely known, it is believed that the primary mechanism is cell-mediated immune injury of blood vessels with subsequent red blood cell extravasation. Degradation of the erythrocytes and the formation of hemosiderin are believed to be the primary causes of the characteristic brown discolouration. Other etiologic factors that increase the incidence of PPD are trauma, pressure, and certain drugs, such as NSAIDs and diuretics. Drug-induced cases of PPD often have a rapid onset and a quick recovery, upon discontinuation of the drug; however, the majority of PPD cases are idiopathic, insidious, and long lasting. Onset is more likely to happen between the ages of thirty and sixty, and males are affected more often than females. PPD can be cosmetically disfiguring, and patients often seek treatment for the lesions. Compression stockings may be help with PPD, and topical steroids may be helpful if dermatitis occurs. Ereni Neonakis is a Research Assistant in the Division of Dermatology at Dalhousie University in Halifax, Nova Scotia. Richard Langley, MD, FRCPC, is a Professor of Dermatology and Director of Research in the Division of Dermatology at Dalhousie University in Halifax, Nova Scotia. 40 The Canadian Journal of CME / February 2014
Case 5 Longitudinal Fingernail Bands A 20-year-old female is concerned about white and red longitudinal bands on her fingernails as well as some flat-topped papules in the periungual areas. She also has some greasy, hyperkeratotic papules on her trunk. Her father has a similar eruption on his back and legs. a. Seborrheic dermatitis b. Tuberous sclerosis c. Melanonychia striata d. Grover s disease e. Darier s disease Darier disease (answer e), also known as keratosis follicularis, is an autosomal dominant disorder characterized by the loss of intercellular adhesion (acantholysis) and abnormal keratinization of the epidermis, nails, and mucous membranes. The disease is caused by mutations in the ATP2A2 gene that encodes a sarco/endoplasmic reticulum Ca 2+ adenosine triphosphate isoform 2 protein (SERCA2). Patients typically present when they are between 6- and 20- years-of-age; onset peaks between 11- and 15-yearsof-age. Both sexes are equally affected, although males tend to be more severely affected than females. More than 95% of patients have nail changes that may include white and red longitudinal stripes, distal notches, splinter hemorrhages, and subungual hyperkeratosis; these changes may precede other signs of the disease. Skin changes are characterized by greasy, discrete, flat-topped, keratotic papules and plaques that occur in seborrheic areas. Lesions are itchy; yellow-brown, brown, or skin-coloured; and feel like coarse sandpaper. Over time, lesions become malodorous secondary to bacterial colonization or infection. Papules, fissures, and ulcers may develop on the palate, buccal mucosa, or tongue. Ocular features include keratotic plaques on the eyelid and Sjögren s syndrome. The disease is chronic and unremitting. Treatment options include emollients, topical corticosteroids, topical calcineurin inhibitors, topical 5-fluorouracil, topical retinoids, oral retinoids, dermabrasion, excision, electrodessication, cryosurgery, ablative laser, electron beam therapy, and photodynamic therapy. Alexander K.C. Leung, MBBS, FRCPC, FRCP(UK&Irel), FRCPCH, is a Clinical Professor of Pediatrics at the University of Calgary in Calgary, Alberta. Benjamin Barankin, MD, FRCPC, is Medical Director and Dermatologist at the Toronto Dermatology Centre in The Canadian Journal of CME / February 2014 41
Case 6 Persistent, Pruritic Plaques A 41-year-old male presents with a history of progressive and persistent plaques on his lower legs that are intensely pruritic, violaceous, and pink in colour. a. Psoriasis b. Lichen simplex chronicus c. Lichen planus d. Pityriasis rosea Lichen planus (answer c) is an inflammatory dermatosis that can affect the skin, hair, nails, and mucous membranes, and it can last for months to years. Lichen planus is characterized by the five Ps: purple, polygonal, planar, pruritic, and papular. Papules are often flat-topped, and it is common for them to coalesce into plaques. Lesions often have white lines on the surface, also referred to as Wickham s striae. Lichen planus can have oropharyngeal involvement in 40 to 60% of affected individuals. The risk of oral squamous cell carcinoma is increased and is believed to be in the order of 5%. Lichen planus can also affect hair follicles and nails. Initial management options include topical or intralesional glucocorticoids. In nonresponsive cases, phototherapy may be required; in refractory cases, referral for consideration of systemic therapies, such as cyclosporine and systemic retinoids, may be considered. Richard Langley, MD, FRCPC, is a Professor of Dermatology and Director of Research in the Division of Dermatology at Dalhousie University in Halifax, Nova Scotia. 42 The Canadian Journal of CME / February 2014
Case 7 White Cheek Patches A 12-year-old, Asian boy presents with white patches on his cheeks. The lesions were first noted a year ago. The child is asymptomatic and healthy. a. Tinea versicolor b. Nummular eczema c. Nevus depigmentosus d. Pityriasis alba e. Tuberous sclerosis Pityriasis alba (answer d) is characterized by hypopigmented, round or oval macules or patches with fine, loosely adherent scales and indistinct margins. The lesions appear mainly on the face, especially or the malar areas, and occasionally on the arms or elsewhere. The lesions range from 0.5 to 5 cm in diameter. Confluent lesions can give the appearance of larger, more amorphous lesions. Most lesions are asymptomatic. Pityriasis alba occurs predominantly in children between the ages of 3- and 16-years. The sex incidence is approximately equal. The condition is noted in up to 40% of dark-skinned children in the susceptible age group. Pityriasis alba is observed more commonly in atopic patients and during the spring and summer. Xerosis is an important pathogenic factor. The condition is self-limited and usually lasts two to three years. Moisturizing the skin is advised. Topical calcineurin inhibitors, and, less preferably, mild topical steroids can be used for two to three weeks to expedite resolution (though it can take a few months after treatment for the hypopigmentation to resolve completely). Tinea versicolor rarely presents on the face (trunk predominant), is uncommon in childhood, and has a distinct margin. In case of doubt, tinea versicolor can be excluded with a potassium hydroxide preparation. The lesions of nummular eczema are usually plaque-like, sharply circumscribed, and pruritic. Nevus depigmentosus is characterized by a nonprogressive, well-circumscribed macule or patch of hypopigmentation and the appearance of the lesion before three-years-of-age. The hypopigmented lesions of tuberous sclerosis are usually present at birth or develop during the first two-years of life and have the appearance of an ashleaf. Alexander K.C. Leung, MBBS, FRCPC, FRCP(UK&Irel), FRCPCH, is a Clinical Professor of Pediatrics at the University of Calgary in Calgary, Alberta. Benjamin Barankin, MD, FRCPC, is Medical Director and Dermatologist at the Toronto Dermatology Centre in The Canadian Journal of CME / February 2014 43
Case 8 Discolouration of the Shoulder A 10-year-old boy presents with an asymptomatic, white lesion on his right shoulder that has been present since birth. The lesion has been stable in shape, distribution, texture, and relative size since then. Stroking the lesion induces erythema. Wood s lamp examination of the patch shows an off-white accentuation without fluorescence. a. Vitiligo b. Nevus depigmentosus c. Hypomelanosis of Ito d. Tuberous sclerosis complex e. Pityriasis alba Nevus depigmentosus (answer b) is characterized by a congenital, asymptomatic, nonprogressive, hypopigmented macule or patch that is stable in shape, distribution, texture, and relative size throughout life. The border of the lesion is not hyperpigmented. Sites of predilection include the trunk, lower abdomen, and proximal extremities. In most patients, the condition is asymptomatic. Three morphological variants of nevus depigmentosus are recognized: isolated, segmental, and systematized. An isolated circular or rectangular macule/patch involving a small, localized part of the body is the most common presentation. The margin is often serrated. The majority of patients with nevus depigmentosus have one lesion. The segmental variant presents as a unilateral streak or patch that appears in a segment or along Blaschko s lines, as is illustrated in the present case. The systematized variant presents with multiple whorls and streaks. This variant is very rare and may be associated with extracutaneous manifestations, such as seizures, mental retardation, hemihypertrophy, and yellow scalp hair. The commonly used clinical criteria for nevus depigmentosus are leukoderma, presentation at birth or early in life, no alternation in distribution of leukoderma throughout life, no alternation in texture or change of sensation in the affected area, and absence of a hyperpigmented border. With Wood s lamp examination, the lesion shows an off-white accentuation without fluorescence. In addition, dermoscopy, use of a pigment measuring device, and in vivo reflectance confocal microscopy are helpful in evaluating the impaired function of melanocytes. Naturally, melanin pigment decreases in lesional skin. Alexander K.C. Leung, MBBS, FRCPC, FRCP(UK&Irel), FRCPCH, is a Clinical Professor of Pediatrics at the University of Calgary in Calgary, Alberta. Benjamin Barankin, MD, FRCPC, is Medical Director and Dermatologist at the Toronto Dermatology Centre in 44 The Canadian Journal of CME / February 2014
Case 9 Eruption on the Inner Bicep This 45-year-old woman noted pain and numbness of her bicep three days prior to an otherwise asymptomatic eruption. The eruption is localized to only the inner bicep and the ulnar aspect of her wrist. a. Herpes simplex b. Herpes zoster c. Impetigo d. Wasp stings e. Nummular eczema Herpes zoster (answer b) is the reactivation of the varicella zoster virus found in sensory dorsal root ganglion cells. Annual incidence is 1:1,000 in patients 45-yearsof-age and four times greater in those over 75-years-ofage. Women are slightly more likely to be involved. Immunosuppression increases the risk of occurrence and, along with age, increases the likelihood of greater severity and duration of symptoms. In younger individuals, findings are minimal with more rapid involution as opposed to the more dramatic picture of blisters, crusting, and confluence that is seen in older populations. This patient presents with a lesion demonstrating only minimal redness with fine vesicles already involuting. The diagnosis, therefore, can be easily missed or confused with eczema. Stanley J. Wine, MD, FRCPC, is a Dermatologist in The Canadian Journal of CME / February 2014 45