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Generalized eruption in a young child
928 Self-Assessment examination
J AM ACAD DERMATOL NOVEMBER 2005
Generalized eruption in a young child Abigail J. Lee, MD, Steven Brett Sloan, MD, and David Yanase, MD San Antonio, Texas An 11-month-old boy presented with a 3-day history of a generalized, rapidly progressive eruption. It began with pruritic erythematous papules, and expanded into nonblanching ecchymotic urticarial plaques (Figs 4-6). There was no mucosal involvement. He had one episode of diarrhea on the day the rash appeared but was otherwise asymptomatic. His personal and family medical histories were noncontributory and he had taken no medications. 14. The most likely diagnosis is (Choose single best response.) a. Henoch-Scho¨nlein purpura b. acute hemorrhagic edema of infancy c. drug eruption d. erythema multiforme e. acute urticaria 15. This diagnosis is generally not made for children older than what age (Choose single best response.) a. 12 months b. 2 years c. 3 years d. 4 years e. 5 years 16. The characteristic histologic finding of this condition is (Choose single best response.) a. dermal hypersensitivity reaction b. superficial perivascular lymphocytic infiltrate with erythrocyte extravasation c. leukocytoclastic vasculitis d. interface dermatitis with necrotic keratinocytes e. normal skin 17. What type of perivascular immunoglobulin deposition is seen in one third of these cases (Choose single best response.) a. IgA b. IgD c. IgG d. IgE e. IgM 18. The most commonly seen sequela of this disease is (Choose single best response.) a. juvenile arthritis b. nephritis
c. recurrence d. gastrointestinal bleeding e. none Discussion Acute infantile hemorrhagic edema (AIHE) is also known as Finkelstein disease and Seidlmayer syndrome. Patients are usually younger than 2 years with onset typically preceded by infections, medication administration, or immunization. The disease is characterized by large purpuric lesions that have a cockade or targetoid appearance. Additional
J AM ACAD DERMATOL VOLUME 53, NUMBER 5
Self-Assessment examination 929
Millard T, Harris A, MacDonald D. Acute infantile hemorrhagic edema. J Am Acad Dermatol 1999;41:873-9. Saraclar Y, Tinaztepe K, Adalioglu G, Tuncer A. Acute hemorrhagic edema of infancy (AIHE)—a variant of Henoch-Scho¨nlein purpura or a distinct clinical entity? J Allergy Clin Immunol 1990;86:473-83.
Woman with multiple tender plaques with subsequent atrophy and scarring Karima Khechmoune, BS,a Shahbaz A. Janjua, MD,b and Amor Khachemoune, MD, CWSc Annaba, Algeria;a Lalmusa, Pakistan;b and Boston, Massachusettsc A 45-year-old woman presented with a several-year history of multiple painful and tender nodular swellings involving her face, breasts, and upper aspects of her arms followed by atrophic scarring of the affected areas (Fig 7). She also had chronic arthralgia, myalgia, and fatigue.
symptoms include nontender acral edema and low-grade fever. Occasionally diarrhea, melena, arthralgias, transient microscopic hematuria, and mild proteinuria are present; however, these complications are rare and transient. Laboratory studies may reveal leukocytosis, eosinophilia, thrombocytosis, an elevated erythrocyte sedimentation rate, or circulating immune complexes; however, laboratory results frequently produce normal findings. Histology will show a leukocytoclastic vasculitis with C3 and fibrinogen deposits. Perivascular IgA deposits are seen in roughly one third of the cases of AIHE. The prognosis for AIHE is good. Complete spontaneous resolution typically occurs within 3 weeks of onset with treatment being supportive only. Of interest is the striking similarity and frequent overlap of findings in AIHE and Henoch-Scho¨nlein purpura; however Henoch-Scho¨nlein purpura portents a much poorer prognosis because of a higher risk of internal organ involvement, a longer course (# 6 weeks), and a nearly 50% rate of relapse. For this series, the recommended choices are as follows: 14, b; 15, b; 16, c; 17, a; 18, e. BIBLIOGRAPHY Baselga E, Drolet BA, Esterly NB. Purpura in infants and children. J Am Acad Dermatol 1997;37:673-705. Crowe MA, Jonas PP. Acute hemorrhagic edema of infancy. Pediatr Dermatol 1998;62:65-6. Legrain V, Lejean S, Taieb A, Guillard J-M, Battin J, Maleville J. Infantile acute hemorrhagic edema of the skin: study of ten cases. J Am Acad Dermatol 1991;24:17-22.
19. The most likely diagnosis is (Choose single best response.) a. discoid lupus erythematosus b. lupus profundus c. lupus tumidus d. lupus pernio e. erythema nodosum 20. A deep excisional biopsy specimen revealed a lobular panniculitis with a dense infiltrate of lymphocytes. The differential diagnosis includes the following except (Choose single best response.) a. traumatic panniculitis b. lupus panniculitis c. erythema nodosum d. pancreatic panniculitis e. erythema induratum of Bazin 21. Lupus profundus occurs in what percentage of patients with cutaneous lupus erythematosus? (Choose single best response.) a. 1% to 3% b. 5% to 10% c. 20% to 40% d. 40% to 60% e. 60% to 80% 22. Successful treatment of lupus profundus include the following except (Choose single best response.) a. dapsone b. hydroxychloroquine c. systemic glucocorticoids d. azathioprine e. systemic retinoids
J AM ACAD DERMATOL NOVEMBER 2005
Generalized eruption in a young child Abigail J. Lee, MD, Steven Brett Sloan, MD, and David Yanase, MD San Antonio, Texas An 11-month-old boy presented with a 3-day history of a generalized, rapidly progressive eruption. It began with pruritic erythematous papules, and expanded into nonblanching ecchymotic urticarial plaques (Figs 4-6). There was no mucosal involvement. He had one episode of diarrhea on the day the rash appeared but was otherwise asymptomatic. His personal and family medical histories were noncontributory and he had taken no medications. 14. The most likely diagnosis is (Choose single best response.) a. Henoch-Scho¨nlein purpura b. acute hemorrhagic edema of infancy c. drug eruption d. erythema multiforme e. acute urticaria 15. This diagnosis is generally not made for children older than what age (Choose single best response.) a. 12 months b. 2 years c. 3 years d. 4 years e. 5 years 16. The characteristic histologic finding of this condition is (Choose single best response.) a. dermal hypersensitivity reaction b. superficial perivascular lymphocytic infiltrate with erythrocyte extravasation c. leukocytoclastic vasculitis d. interface dermatitis with necrotic keratinocytes e. normal skin 17. What type of perivascular immunoglobulin deposition is seen in one third of these cases (Choose single best response.) a. IgA b. IgD c. IgG d. IgE e. IgM 18. The most commonly seen sequela of this disease is (Choose single best response.) a. juvenile arthritis b. nephritis
c. recurrence d. gastrointestinal bleeding e. none Discussion Acute infantile hemorrhagic edema (AIHE) is also known as Finkelstein disease and Seidlmayer syndrome. Patients are usually younger than 2 years with onset typically preceded by infections, medication administration, or immunization. The disease is characterized by large purpuric lesions that have a cockade or targetoid appearance. Additional
J AM ACAD DERMATOL VOLUME 53, NUMBER 5
Self-Assessment examination 929
Millard T, Harris A, MacDonald D. Acute infantile hemorrhagic edema. J Am Acad Dermatol 1999;41:873-9. Saraclar Y, Tinaztepe K, Adalioglu G, Tuncer A. Acute hemorrhagic edema of infancy (AIHE)—a variant of Henoch-Scho¨nlein purpura or a distinct clinical entity? J Allergy Clin Immunol 1990;86:473-83.
Woman with multiple tender plaques with subsequent atrophy and scarring Karima Khechmoune, BS,a Shahbaz A. Janjua, MD,b and Amor Khachemoune, MD, CWSc Annaba, Algeria;a Lalmusa, Pakistan;b and Boston, Massachusettsc A 45-year-old woman presented with a several-year history of multiple painful and tender nodular swellings involving her face, breasts, and upper aspects of her arms followed by atrophic scarring of the affected areas (Fig 7). She also had chronic arthralgia, myalgia, and fatigue.
symptoms include nontender acral edema and low-grade fever. Occasionally diarrhea, melena, arthralgias, transient microscopic hematuria, and mild proteinuria are present; however, these complications are rare and transient. Laboratory studies may reveal leukocytosis, eosinophilia, thrombocytosis, an elevated erythrocyte sedimentation rate, or circulating immune complexes; however, laboratory results frequently produce normal findings. Histology will show a leukocytoclastic vasculitis with C3 and fibrinogen deposits. Perivascular IgA deposits are seen in roughly one third of the cases of AIHE. The prognosis for AIHE is good. Complete spontaneous resolution typically occurs within 3 weeks of onset with treatment being supportive only. Of interest is the striking similarity and frequent overlap of findings in AIHE and Henoch-Scho¨nlein purpura; however Henoch-Scho¨nlein purpura portents a much poorer prognosis because of a higher risk of internal organ involvement, a longer course (# 6 weeks), and a nearly 50% rate of relapse. For this series, the recommended choices are as follows: 14, b; 15, b; 16, c; 17, a; 18, e. BIBLIOGRAPHY Baselga E, Drolet BA, Esterly NB. Purpura in infants and children. J Am Acad Dermatol 1997;37:673-705. Crowe MA, Jonas PP. Acute hemorrhagic edema of infancy. Pediatr Dermatol 1998;62:65-6. Legrain V, Lejean S, Taieb A, Guillard J-M, Battin J, Maleville J. Infantile acute hemorrhagic edema of the skin: study of ten cases. J Am Acad Dermatol 1991;24:17-22.
19. The most likely diagnosis is (Choose single best response.) a. discoid lupus erythematosus b. lupus profundus c. lupus tumidus d. lupus pernio e. erythema nodosum 20. A deep excisional biopsy specimen revealed a lobular panniculitis with a dense infiltrate of lymphocytes. The differential diagnosis includes the following except (Choose single best response.) a. traumatic panniculitis b. lupus panniculitis c. erythema nodosum d. pancreatic panniculitis e. erythema induratum of Bazin 21. Lupus profundus occurs in what percentage of patients with cutaneous lupus erythematosus? (Choose single best response.) a. 1% to 3% b. 5% to 10% c. 20% to 40% d. 40% to 60% e. 60% to 80% 22. Successful treatment of lupus profundus include the following except (Choose single best response.) a. dapsone b. hydroxychloroquine c. systemic glucocorticoids d. azathioprine e. systemic retinoids