BOARD REVIEW ANSWERS
1. Cutaneous T-cell Lymphoma
Which form of this disease is typically associated with aggressive clinical behavior?
d) Primary cutaneous gamma/delta T-cell lymphoma
Primary cutaneous gamma/delta T-cell lymphoma is typically associated with aggressive clinical be- havior.The other forms of cutaneous T-cell lymphoma listed tend to run a more indolent clinical course.
References
Jawed SI, Myskowski PL, Horwitz S, Moskowitz A, Querfeld C. Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part I. Diagnosis: clinical and histopathologic features and new molecular and biologic markers. J Am Acad Dermatol. 2014;70(2)205.e1-16.
Wilcox RA. Cutaneous T-cell lymphoma: 2011 update on diagnosis, risk-stratification, and management. Am J Hematol. 2011; 86(11):928-948.
Hwang ST, Janik JE, Jaffe ES,Wilson WH. Mycosis fungoides and Sézary syndrome. Lancet. 2008;371(9616):945-957.
2. Dysplastic Nevus
The recurrence rate for these lesions after shave biopsy is approximately:
a) 4%
In a study of 195 moderately dysplastic nevi, the recurrence rate 2 years after biopsy was approximately 3.6%, roughly the same as the recurrence rate for 61 “benign” nevi (3.3%). Recurrence was associated with shave biopsy technique, but not with the histologic picture or margin involvement as noted on the initial pathology report.
References
Duffy K, Grossman D.The dysplastic nevus: from historical perspective to management in the modern era: part II. Molecular aspects and clinical management. J Am Acad Dermatol. 2012;67(1):19.e1-12.
Elston D. Practical advice regarding problematic pigmented lesions. J Am Acad Dermatol. 2012;67(1):148-155.
Goodson AG, Florell SR, Boucher KM, Grossman D.Low rates of clinical recurrence after biopsy of benign to moderately dysplastic melanocytic nevi. J Am Acad Dermatol. 2010;62(4): 591-596.
3. Atopic Dermatitis
Which statement is true regarding this condition?
c) Most of the genetic regions associated with the disease correspond to loci associated with psoriasis
Filaggrin mutations are not specific for atopic dermatitis and are highly associated with ichthyosis vulgaris as well. Unlike psoriasis, in which the Th1 predominates, the Th2 response is associated with atopic dermatitis. Surprisingly, most of the genetic regions associated with atopic dermatitis correspond to the loci associated with psoriasis, although the 2 diseases are rarely linked. The incidence of atopic dermatitis appears to be increasing, especially in industrialized countries.
References
Wolf R,Wolf D.Abnormal epidermal barrier in the pathogenesis of atopic dermatitis. Clin Dermatol. 2012;33(suppl1):S67-S69. Sabin BR, Peters N, Peters AT. Chapter 20:Atopic dermatitis. Allergy Asthma Proc. 2012; 33:67-69.
Bieber T.Atopic dermatitis. N Engl J Med. 2008;358(14):1483-1494.
4. Ichthyosis
A recent Ichthyosis Consensus Conference has proposed the term “keratinophathic ichthyosis” to replace which older designation? e) Epidermolytic ichthyosis
In the proposed nomenclature,“keratinopathic ichthyosis” is used to describe disease caused by keratin mutations, including epidermolytic ichthyosis, superficial epidermolytic ichthyosis and ichthyosis Curth-Macklin.“Autosomal recessive congenital ichthyosis” is proposed as the term to include harlequin ichthyosis, lamellar ichtyosis and congenital ichthyosiform erythroderma.
References
Chamcheu JC,Wood GS, Siddiqui IA, et al. Progress towards genetic and pharmacological therapies for keratin genodermatoses: current perspective and future promise. Exp Dermatol. 2012;21(7):481-489.
OjiV,Tadini G,Akiyama M, et al. Revised nomenclature and classification of inherited ichthyoses: results of the First Ichthyosis Consensus Conference in Sorèze 2009. J Am Acad Dermatol. 2010;63(4):607-641.
Bruce Thiers, MD, FAAD, is a professor with the Department of Dermatology and Dermatologic Surgery at the Medical University of South Carolina in Charleston, SC.
