The content of these case studies, ideal to review during peer study groups, was developed by Chief Resident Robin Lewallen, MD under the guidance of dermatologist William W Huang, MD, MPH, FAAD, Assistant Professor of Dermatology, Wake Forest University School of Medicine.
Case Study Answers
1.) B.
The correct answer is B: Linear IgA Bullous Dermatosis.
Explanation/Literature Review
The patient in this case had a positive direct immunofluorescence for IgG and C3 consistent with pemphigoid gestationis, formerly known as herpes gestationis. The H&E findings of eosinophilic spongiosis have the “HAAPPIE†differential, which includes herpes gestationis, arthropod bite, allergic contact dermatitis, pemphigus, pemphigoid, incontinentia pigmenti, and erythema toxicum neonatorum. While linear IgA bullous dermatosis could be in the clinical differential, she did have this eruption prior to the administration of vancomycin and eosinophic spongiosis would not be seen with this predominately neutrophilic condition..
The patient in this case had a positive direct immunofluorescence for IgG and C3 consistent with pemphigoid gestationis, formerly known as herpes gestationis. The H&E findings of eosinophilic spongiosis have the “HAAPPIE†differential, which includes herpes gestationis, arthropod bite, allergic contact dermatitis, pemphigus, pemphigoid, incontinentia pigmenti, and erythema toxicum neonatorum. While linear IgA bullous dermatosis could be in the clinical differential, she did have this eruption prior to the administration of vancomycin and eosinophic spongiosis would not be seen with this predominately neutrophilic condition..
2.) A.
The correct answer is A: Acute hemorrhagic edema of infancy.
Explanation/Literature Review
Acute hemorrhagic edema of infancy (AHEI) is a form of cutaneous small vessel vasculitis seen in young children under the age of 2. The cutaneous lesions are plaques with varying degrees of hemorrhage and edema that can sometimes take on a targetoid appearance. AHEI was previously classified as a variant of Henoch-Schönlein purpura (HSP) but is now considered its own discinct clinical entitiy. Unlike HSP, these patients do not develop systemic involvement of the gastrointestinal or renal systems. Approximately 75% of patients with AHEI have associated infection, drug exposure, or immunization preceeding the development of cutaneous lesions.
Acute hemorrhagic edema of infancy (AHEI) is a form of cutaneous small vessel vasculitis seen in young children under the age of 2. The cutaneous lesions are plaques with varying degrees of hemorrhage and edema that can sometimes take on a targetoid appearance. AHEI was previously classified as a variant of Henoch-Schönlein purpura (HSP) but is now considered its own discinct clinical entitiy. Unlike HSP, these patients do not develop systemic involvement of the gastrointestinal or renal systems. Approximately 75% of patients with AHEI have associated infection, drug exposure, or immunization preceeding the development of cutaneous lesions.
3.) C.
The correct answer is A: Human immunodeficiency virus.
Explanation/Literature Review
Erythema elevatum diutinum (EED) is characterized by red-brown to violaceous papules, plaques and nodules typically distributed over extensor surfaces. EED has been described in association with a number of systemic diseases, most commonly HIV which presents with earlier onset. EED has also been associated with infections, autoimmune diseases, inflammatory conditions, and hematologic disorders especially IgA paraproteinemia. Immune complex deposition is thought to play a major role in this disease. Leukocytoclastic vasculitis is seen in early lesions, followed by fibrotic replacement of the dermis in older lesions. The majority of cases resolve over a period of 5 to 10 years. Dapsone, NSAIDs, niacinamide, tetracyclines, chloroquine, colchicine, low dose methotrexate and plasmapheresis have all been reported with success. Relapses are common with discontinuation of treatment. Intralesional corticosteroids may be helpful for mild cases.
Erythema elevatum diutinum (EED) is characterized by red-brown to violaceous papules, plaques and nodules typically distributed over extensor surfaces. EED has been described in association with a number of systemic diseases, most commonly HIV which presents with earlier onset. EED has also been associated with infections, autoimmune diseases, inflammatory conditions, and hematologic disorders especially IgA paraproteinemia. Immune complex deposition is thought to play a major role in this disease. Leukocytoclastic vasculitis is seen in early lesions, followed by fibrotic replacement of the dermis in older lesions. The majority of cases resolve over a period of 5 to 10 years. Dapsone, NSAIDs, niacinamide, tetracyclines, chloroquine, colchicine, low dose methotrexate and plasmapheresis have all been reported with success. Relapses are common with discontinuation of treatment. Intralesional corticosteroids may be helpful for mild cases.
