Faculty Bibliography

OBJECTIVE: The aim was to assess the level of systemic involvement and character of renal disease in patients with chronic cutaneous lupus erythematosus of the discoid lupus variety (hereafter referred to as 'discoid lupus') and features of systemic lupus erythematosus (SLE). Clinical confusion with other types of cutaneous lupus erythematosus complicates interpretation of some previously reported studies. METHODS: Over three years, sixteen patients met the diagnostic criteria of discoid lupus, positive anti-nuclear-antibody, and at least one extracutaneous manifestation. RESULTS: Most patients (14/16) were female, between 26 to 66 years old. Arthritis was the most common extracutaneous manifestation followed by Raynaud's phenomenon. The anti-nuclear-antibody was speckled in ten patients with titers ranging from 1:40 to 1:1280 IU/mL. Elevated levels of double-stranded-DNA in low titers were found in four patients, anti-Smith-antibody in four; anti-Sjogren-syndrome-A-antibody in seven, and anti-ribonucleoprotein-antibody in seven. Renal function markers were transiently high in some patients but normalized over time. Hematuria and/or proteinuria were present at some time in seven patients. The highest BUN and creatinine levels were 42 mg/dL and 1.5 mg/dL, respectively. One patient had membranous glomerulonephropathy class 5; however, discoid lupus developed well after the onset of renal disease during a time when renal function had returned to normal. CONCLUSION: Our observational data supports previous reports suggesting that patients with active discoid lupus rarely have progressive renal insufficiency. The mechanism for the development of discoid lupus may involve an immunologic mechanism that differs from that which produces severe organ involvement, especially advanced immune-complex-mediated renal disease. Patients with discoid lupus rarely have sustained high levels of antibodies to double-stranded-DNA. Discoid lupus appears to be a marker for a more benign lupus course. This clinical observation lays the groundwork for a larger prospective, longitudinal cohort study for further validation.

Participation in ice-skating sports, particularly figure skating, ice hockey and speed skating, has increased in recent years. Competitive athletes in these sports experience a range of dermatological injuries related to mechanical factors: exposure to cold temperatures, infectious agents and inflammation. Part I of this two part review discussed the mechanical dermatoses affecting ice-skating athletes that result from friction, pressure, and chronic irritation related to athletic equipment and contact with surfaces. Here, in Part II, we review the cold-induced, infectious and inflammatory skin conditions observed in ice-skating athletes. Cold-induced dermatoses experienced by ice-skating athletes result from specific physiological effects of cold exposure on the skin. These conditions include physiological livedo reticularis, chilblains (pernio), Raynaud phenomenon, cold panniculitis, frostnip and frostbite. Frostbite, that is the literal freezing of tissue, occurs with specific symptoms that progress in a stepwise fashion, starting with frostnip. Treatment involves gradual forms of rewarming and the use of friction massages and pain medications as needed. Calcium channel blockers, including nifedipine, are the mainstay of pharmacological therapy for the major nonfreezing cold-induced dermatoses including chilblains and Raynaud phenomenon. Raynaud phenomenon, a vasculopathy involving recurrent vasospasm of the fingers and toes in response to cold, is especially common in figure skaters. Protective clothing and insulation, avoidance of smoking and vasoconstrictive medications, maintaining a dry environment around the skin, cold avoidance when possible as well as certain physical manoeuvres that promote vasodilation are useful preventative measures. Infectious conditions most often seen in ice-skating athletes include tinea pedis, onychomycosis, pitted keratolysis, warts and folliculitis. Awareness, prompt treatment and the use of preventative measures are particularly important in managing such dermatoses that are easily spread from person to person in training facilities. The use of well ventilated footgear and synthetic substances to keep feet dry, as well as wearing sandals in shared facilities and maintaining good personal hygiene are very helpful in preventing transmission. Inflammatory conditions that may be seen in ice-skating athletes include allergic contact dermatitis, palmoplantar eccrine hidradenitis, exercise-induced purpuric eruptions and urticaria. Several materials commonly used in ice hockey and figure skating cause contact dermatitis. Identification of the allergen is essential and patch testing may be required. Exercise-induced purpuric eruptions often occur after exercise, are rarely indicative of a chronic venous disorder or other haematological abnormality and the lesions typically resolve spontaneously. The subtypes of urticaria most commonly seen in athletes are acute forms induced by physical stimuli, such as exercise, temperature, sunlight, water or particular levels of external pressure. Cholinergic urticaria is the most common type of physical urticaria seen in athletes aged 30 years and under. Occasionally, skaters may develop eating disorders and other related behaviours some of which have skin manifestations that are discussed herein. We hope that this comprehensive review will aid sports medicine practitioners, dermatologists and other physicians in the diagnosis and treatment of these dermatoses.

Despite its recognition as a distinct granulomatous disease for over a century, the etiology of sarcoidosis remains to be defined. Since the early 1900s, infectious agents have been suspected in causing sarcoidosis. For much of this time, mycobacteria were considered a likely culprit, yet until recently, the supporting evidence has been tenuous at best. In this review, we evaluate the reported association between mycobacteria and sarcoidosis. Historically, mycobacterial infection has been investigated using histologic stains, cultures of lesional tissue or blood, and identification of bacterial nucleic acids or bacterial antigens. More recently, advances in biochemical, molecular, and immunological methods have produced a more rigorous analysis of the antigenic drivers of sarcoidosis. The result of these efforts indicates that mycobacterial products likely play a role in at least a subset of sarcoidosis cases. This information, coupled with a better understanding of genetic susceptibility to this complex disease, has therapeutic implications

Lupus erythematosus tumidus (LET) is an uncommon variant of cutaneous lupus erythematosus. Lupus erythematodes tumidus is characterized by smooth, erythematous plaques on sun-exposed areas without surface changes, such as follicular plugs, atrophy, or scale. Histopathologic features include a lymphocytic, perivascular and periadnexal infiltrate with abundant interstitial, superficial, and deep dermal mucin without appreciable epidermal and vacuolar changes. Typically, LET is responsive to treatment with systemic antimalarials. We present a unique case of alopecia associated with LET, which was partially responsive to hydroxychloroquine. We also note that the plaque of LET is adjacent to the plaque of discoid lupus erythematosus.

We report the case of a 63-year-old obese man with a rapid-onset of widespread acanthosis nigricans (AN) in the setting of having recently initiated treatment with niacin for dyslipidemia. Although obesity and insulin-resistance are risk factors for AN, AN associated with endocrine dysfunction tends to have a more gradual onset and limited involvement. Owing to our patient's age, the rapid onset, and extensive distribution of his eruption, we initially were concerned about paraneoplastic AN. However, an evaluation for a malignant condition was negative. The timing of the onset of our patient's eruption within several months of starting niacin therapy is consistent with niacin-induced AN. Niacin is known to cause rapidly progressive, widespread AN that is reversible upon discontinuation of the medication. We discuss the pathogenesis of AN, which is thought to be the final common manifestation of stimulation of different subtypes of tyrosine kinase receptors by various epidermal growth factors.

A 49-year-old man with advanced HIV/AIDS on anti-retroviral therapy (HAART) and trimethoprim-sulfamethoxazole (TMP-SMX) presented with a several-month history of pruritic, erythematous, lichenified papules that coalesced into hyperkeratotic plaques on the trunk and extremities in a sun-exposed distribution. He shortly thereafter developed a progressive depigmentation over more than 80 percent of his body surface area. A biopsy specimen of an erythematous plaque on the trunk showed a superficial and mid-dermal infiltrate of lymphocytes with eosinophils, most consistent with either chronic lichenoid drug eruption or atypical lymphoproliferative disorder (ACLD) of HIV. The patient's lichenoid skin disease has persisted despite discontinuation of TMP-SMX, although it has improved partially with administration of topical glucocorticoids and acitretin. His depigmentation has continued to progress. We discuss the overlapping diagnostic entities which may be comprised by this patient's clinical disease, and highlight a unique presentation of the complex interaction between HIV infection and the skin.

Gorlin syndrome is an autosomal dominantly inherited disorder that results in numerous basal cell carcinomas as well as a number of other facial and skeletal findings. We present a patient with many classic features and review some of the treatment options available for these patients.

A 36-year-old African man from Guinea with a history of albinism presented with a many-year history of scaling and erythema of the face, neck, and arms. The patient had light eyes, hair, and skin. Physical examination showed extensive photodamage. A skin biopsy specimen from the posterior aspect of the lower leg showed a squamous-cell carcinoma in situ. The most common types of oculocutaneous albinism (OCA), OCA 1 and OCA 2, are autosomal recessive disorders of pigmentation that commonly affect the skin, hair, eyes, and ears. Photodamage and skin cancers plague patients with albinism. In Africa, where albinism is prevalent, albinos face a myriad of social and medical issues. Skin cancer surveillance is an important consideration for albinos, and sun protection is paramount.

We present a 31-year-old woman with a ten-year history of a progressive macular eruption; one of her macules demonstrated a wheal when rubbed. A biopsy was consistent with telangiectasia macularis eruptiva perstans. There were no signs and symptoms of systemic involvement despite the large body surface area of involvement.

Anetoderma is an uncommon dermatosis that manifests as discrete foci of well-circumscribed, atrophic skin. The condition can be idiopathic or can be secondary to a number of associated cutaneous diseases. Whereas the pathophysiologic mechanisms remain unknown, anetoderma results from diminished elastic fibers in the dermis. We present an unusual case of localized, lenticular anetoderma in a man with HIV, a history of syphilis, and lichen planus. Both of these infections have been associated with anetoderma. Although his lesions are vaguely reminiscent of a variant of syphilitic anetoderma described in the 1930s, they are confined to a smaller anatomic distribution, differ in size, and have a papular appearance. As anetoderma can develop in the context of infectious disease, a diagnosis of anetoderma should trigger a thorough examination and evaluation for treatable concomitant illnesses.