The contents of these questions are taken from the Galderma Pre-Board Webinar. The Pre-Board Webinar is now an online course. For details, go to https://www.galdermausa.com/Our-Commitment/PreBoard-Webinar.aspx.
1. The diagnosis is:
a) Polymorphic light reaction
b) Acute cutaneous lupus erythematosus
c) Erythromelalgia
d) Porphyria cutanea tarda
e) Dermatomyositis
2. This child has:
a) Generalized epidermolysis bullosa simplex
b) Kawasaki disease
c) Staphylococcal scalded skin syndrome
d) Chronic bullous dermatosis of childhood
e) Infantile pemphigus vulgaris
To learn the answers, go to page 2
{{pagebreak}}
BOARD REVIEW ANSWERS
1. The diagnosis is:
e) Dermatomyositis
This clinical photograph illustrates the typical hand findings in dermatomyositis, including atrophic or hypertrophic violaceous papules (“Gottron papules”) arising over the proximal and distal interphalangeal and metacarpophalangeal joints, periungual telangiectases, or dilated capillary loops. Note: Gottron papules may sometimes be morphologically confused with localized lichen planus. These hand findings may be the most useful cutaneous diagnostic markers for dermatomyositis, particularly if truncal or periocular findings are subtle or absent.
Other hand findings may include markedly ragged cuticles, calcinosis cutis, and infrequently, scaliness and redness of the palms and digits (“mechanic’s hands”), the latter mimicking severe chronic hand eczema. Interstitial lung disease is an important complication of classic dermatomyositis, occurring in 10% to 40% of patients. About 20% to 25% of all patients with adult-onset dermatomyositis are at risk of developing an internal malignancy, although this increased risk appears to be mainly within the first 3 years of the onset of clinical disease. Approximately 20% to 30% of patients with cutaneous findings of dermatomyositis have clinically amyopathic disease (CADM; dermatomyositis sine myositis). Over time as many as one-third of patients with CADM will evolve into classic dermatomyositis. Whether these patients are also at increased risk of associated internal malignancy is still controversial.
References
Euwer RL, Sontheimer RD. Amyopathic dermatomyositis: a review. J Invest Dermatol. 1993;100(1):124S-127S.
Kovacs SO, Kovacs SC. Dermatomyositis. J Am Acad Dermatol. 1998;39(6):899-920.
Sontheimer RD. Cutaneous features of classic dermatomyositis and amyopathic dermatomyositis. Curr Opin Rheumatol. 1999;11(6):475-482.
Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet. 2003;362(9388):971-982.
Sontheimer RD. Dermatomyositis: an overview of recent progress with emphasis on dermatologic aspects. Dermatol Clin. 2002;20(3):387-408.
Krathen MS, Fiorentino D, Werth VP. Dermatomyositis. Curr Dir Autoimmun. 2008;10:313-332.
Sontheimer RD. Clinically amyopathic dermatomyositis: what can we now tell our patients? Arch Dermatol. 2010;146(1):76-80.
2. This child has:
c) Staphylococcal scalded skin syndrome
Staphylococcal scalded skin syndrome (SSSS) is caused by an exotoxin, epidermolysin, which is produced mainly by group II Staphylococcus aureus. This infectious disease causes a noninflammatory skin cleavage plane just beneath the stratum corneum, identical to the site of blister formation in pemphigus foliaceus. Recently been proven to be a serine protease that degrades desmoglein 1, the autoimmune target in pemphigus foliaceus. SSSS occurs almost exclusively in infants and very small children. Although only rarely seen in adults, it may occur in adults who are immunocompromised or who have markedly impaired renal function. Presumably the latter is a shared pathophysiological link with affected infants, since normal infants have reduced glomerular filtration rates and thus cannot readily excrete the exotoxin, thereby allowing toxic levels to accumulate within the skin. Characteristic features of SSSS are widespread erythema and peeling skin, with associated Nikolsky sign-positivity. In SSSS, a cutaneous source of bacterial infection is usually readily apparent.
References
Lyell A. The staphylococcal scalded skin syndrome in historical perspective: emergence of dermopathic strains of Staphylococcus aureus and discovery of the epidermolytic toxin. A review of events up to 1970. J Am Acad Dermatol. 1983;9(2):285-294.
Mander SM. Toxin-mediated streptococcal and staphylococcal disease. J Am Acad Dermatol. 1998;39(3):383-398.
Ladhani S, Evans RW. Staphylococcal scalded skin syndrome. Arch Dis Child. 1998;78(1):85-88.
Ladhani S. Recent developments in staphylococcal scalded skin syndrome. Clin Microbiol Infect. 2001;7(6):301-307.
Amagai M, Matsuyoshi N, Wang ZH, Andi C, Stanley JR. Toxin in bullous impetigo and staphylococcal scalded-skin syndrome targets desmoglein 1. Nat Med. 2000;6(11):1275-1277.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine is currently professor of medicine (dermatology) and pediatrics at Vanderbilt University School of Medicine in Nashville, TN.
Ron J. Feldman, MD, PhD, is assistant professor in the department of dermatology at Emory University School of Medicine in Atlanta, GA.
The contents of these questions are taken from the Galderma Pre-Board Webinar. The Pre-Board Webinar is now an online course. For details, go to https://www.galdermausa.com/Our-Commitment/PreBoard-Webinar.aspx.
1. The diagnosis is:
a) Polymorphic light reaction
b) Acute cutaneous lupus erythematosus
c) Erythromelalgia
d) Porphyria cutanea tarda
e) Dermatomyositis
2. This child has:
a) Generalized epidermolysis bullosa simplex
b) Kawasaki disease
c) Staphylococcal scalded skin syndrome
d) Chronic bullous dermatosis of childhood
e) Infantile pemphigus vulgaris
To learn the answers, go to page 2
{{pagebreak}}
BOARD REVIEW ANSWERS
1. The diagnosis is:
e) Dermatomyositis
This clinical photograph illustrates the typical hand findings in dermatomyositis, including atrophic or hypertrophic violaceous papules (“Gottron papules”) arising over the proximal and distal interphalangeal and metacarpophalangeal joints, periungual telangiectases, or dilated capillary loops. Note: Gottron papules may sometimes be morphologically confused with localized lichen planus. These hand findings may be the most useful cutaneous diagnostic markers for dermatomyositis, particularly if truncal or periocular findings are subtle or absent.
Other hand findings may include markedly ragged cuticles, calcinosis cutis, and infrequently, scaliness and redness of the palms and digits (“mechanic’s hands”), the latter mimicking severe chronic hand eczema. Interstitial lung disease is an important complication of classic dermatomyositis, occurring in 10% to 40% of patients. About 20% to 25% of all patients with adult-onset dermatomyositis are at risk of developing an internal malignancy, although this increased risk appears to be mainly within the first 3 years of the onset of clinical disease. Approximately 20% to 30% of patients with cutaneous findings of dermatomyositis have clinically amyopathic disease (CADM; dermatomyositis sine myositis). Over time as many as one-third of patients with CADM will evolve into classic dermatomyositis. Whether these patients are also at increased risk of associated internal malignancy is still controversial.
References
Euwer RL, Sontheimer RD. Amyopathic dermatomyositis: a review. J Invest Dermatol. 1993;100(1):124S-127S.
Kovacs SO, Kovacs SC. Dermatomyositis. J Am Acad Dermatol. 1998;39(6):899-920.
Sontheimer RD. Cutaneous features of classic dermatomyositis and amyopathic dermatomyositis. Curr Opin Rheumatol. 1999;11(6):475-482.
Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet. 2003;362(9388):971-982.
Sontheimer RD. Dermatomyositis: an overview of recent progress with emphasis on dermatologic aspects. Dermatol Clin. 2002;20(3):387-408.
Krathen MS, Fiorentino D, Werth VP. Dermatomyositis. Curr Dir Autoimmun. 2008;10:313-332.
Sontheimer RD. Clinically amyopathic dermatomyositis: what can we now tell our patients? Arch Dermatol. 2010;146(1):76-80.
2. This child has:
c) Staphylococcal scalded skin syndrome
Staphylococcal scalded skin syndrome (SSSS) is caused by an exotoxin, epidermolysin, which is produced mainly by group II Staphylococcus aureus. This infectious disease causes a noninflammatory skin cleavage plane just beneath the stratum corneum, identical to the site of blister formation in pemphigus foliaceus. Recently been proven to be a serine protease that degrades desmoglein 1, the autoimmune target in pemphigus foliaceus. SSSS occurs almost exclusively in infants and very small children. Although only rarely seen in adults, it may occur in adults who are immunocompromised or who have markedly impaired renal function. Presumably the latter is a shared pathophysiological link with affected infants, since normal infants have reduced glomerular filtration rates and thus cannot readily excrete the exotoxin, thereby allowing toxic levels to accumulate within the skin. Characteristic features of SSSS are widespread erythema and peeling skin, with associated Nikolsky sign-positivity. In SSSS, a cutaneous source of bacterial infection is usually readily apparent.
References
Lyell A. The staphylococcal scalded skin syndrome in historical perspective: emergence of dermopathic strains of Staphylococcus aureus and discovery of the epidermolytic toxin. A review of events up to 1970. J Am Acad Dermatol. 1983;9(2):285-294.
Mander SM. Toxin-mediated streptococcal and staphylococcal disease. J Am Acad Dermatol. 1998;39(3):383-398.
Ladhani S, Evans RW. Staphylococcal scalded skin syndrome. Arch Dis Child. 1998;78(1):85-88.
Ladhani S. Recent developments in staphylococcal scalded skin syndrome. Clin Microbiol Infect. 2001;7(6):301-307.
Amagai M, Matsuyoshi N, Wang ZH, Andi C, Stanley JR. Toxin in bullous impetigo and staphylococcal scalded-skin syndrome targets desmoglein 1. Nat Med. 2000;6(11):1275-1277.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine is currently professor of medicine (dermatology) and pediatrics at Vanderbilt University School of Medicine in Nashville, TN.
Ron J. Feldman, MD, PhD, is assistant professor in the department of dermatology at Emory University School of Medicine in Atlanta, GA.
The contents of these questions are taken from the Galderma Pre-Board Webinar. The Pre-Board Webinar is now an online course. For details, go to https://www.galdermausa.com/Our-Commitment/PreBoard-Webinar.aspx.
1. The diagnosis is:
a) Polymorphic light reaction
b) Acute cutaneous lupus erythematosus
c) Erythromelalgia
d) Porphyria cutanea tarda
e) Dermatomyositis
2. This child has:
a) Generalized epidermolysis bullosa simplex
b) Kawasaki disease
c) Staphylococcal scalded skin syndrome
d) Chronic bullous dermatosis of childhood
e) Infantile pemphigus vulgaris
To learn the answers, go to page 2
{{pagebreak}}
BOARD REVIEW ANSWERS
1. The diagnosis is:
e) Dermatomyositis
This clinical photograph illustrates the typical hand findings in dermatomyositis, including atrophic or hypertrophic violaceous papules (“Gottron papules”) arising over the proximal and distal interphalangeal and metacarpophalangeal joints, periungual telangiectases, or dilated capillary loops. Note: Gottron papules may sometimes be morphologically confused with localized lichen planus. These hand findings may be the most useful cutaneous diagnostic markers for dermatomyositis, particularly if truncal or periocular findings are subtle or absent.
Other hand findings may include markedly ragged cuticles, calcinosis cutis, and infrequently, scaliness and redness of the palms and digits (“mechanic’s hands”), the latter mimicking severe chronic hand eczema. Interstitial lung disease is an important complication of classic dermatomyositis, occurring in 10% to 40% of patients. About 20% to 25% of all patients with adult-onset dermatomyositis are at risk of developing an internal malignancy, although this increased risk appears to be mainly within the first 3 years of the onset of clinical disease. Approximately 20% to 30% of patients with cutaneous findings of dermatomyositis have clinically amyopathic disease (CADM; dermatomyositis sine myositis). Over time as many as one-third of patients with CADM will evolve into classic dermatomyositis. Whether these patients are also at increased risk of associated internal malignancy is still controversial.
References
Euwer RL, Sontheimer RD. Amyopathic dermatomyositis: a review. J Invest Dermatol. 1993;100(1):124S-127S.
Kovacs SO, Kovacs SC. Dermatomyositis. J Am Acad Dermatol. 1998;39(6):899-920.
Sontheimer RD. Cutaneous features of classic dermatomyositis and amyopathic dermatomyositis. Curr Opin Rheumatol. 1999;11(6):475-482.
Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet. 2003;362(9388):971-982.
Sontheimer RD. Dermatomyositis: an overview of recent progress with emphasis on dermatologic aspects. Dermatol Clin. 2002;20(3):387-408.
Krathen MS, Fiorentino D, Werth VP. Dermatomyositis. Curr Dir Autoimmun. 2008;10:313-332.
Sontheimer RD. Clinically amyopathic dermatomyositis: what can we now tell our patients? Arch Dermatol. 2010;146(1):76-80.
2. This child has:
c) Staphylococcal scalded skin syndrome
Staphylococcal scalded skin syndrome (SSSS) is caused by an exotoxin, epidermolysin, which is produced mainly by group II Staphylococcus aureus. This infectious disease causes a noninflammatory skin cleavage plane just beneath the stratum corneum, identical to the site of blister formation in pemphigus foliaceus. Recently been proven to be a serine protease that degrades desmoglein 1, the autoimmune target in pemphigus foliaceus. SSSS occurs almost exclusively in infants and very small children. Although only rarely seen in adults, it may occur in adults who are immunocompromised or who have markedly impaired renal function. Presumably the latter is a shared pathophysiological link with affected infants, since normal infants have reduced glomerular filtration rates and thus cannot readily excrete the exotoxin, thereby allowing toxic levels to accumulate within the skin. Characteristic features of SSSS are widespread erythema and peeling skin, with associated Nikolsky sign-positivity. In SSSS, a cutaneous source of bacterial infection is usually readily apparent.
References
Lyell A. The staphylococcal scalded skin syndrome in historical perspective: emergence of dermopathic strains of Staphylococcus aureus and discovery of the epidermolytic toxin. A review of events up to 1970. J Am Acad Dermatol. 1983;9(2):285-294.
Mander SM. Toxin-mediated streptococcal and staphylococcal disease. J Am Acad Dermatol. 1998;39(3):383-398.
Ladhani S, Evans RW. Staphylococcal scalded skin syndrome. Arch Dis Child. 1998;78(1):85-88.
Ladhani S. Recent developments in staphylococcal scalded skin syndrome. Clin Microbiol Infect. 2001;7(6):301-307.
Amagai M, Matsuyoshi N, Wang ZH, Andi C, Stanley JR. Toxin in bullous impetigo and staphylococcal scalded-skin syndrome targets desmoglein 1. Nat Med. 2000;6(11):1275-1277.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine is currently professor of medicine (dermatology) and pediatrics at Vanderbilt University School of Medicine in Nashville, TN.
Ron J. Feldman, MD, PhD, is assistant professor in the department of dermatology at Emory University School of Medicine in Atlanta, GA.
