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JAAD Grand Rounds quiz∗
JAAD
GRAND ROUNDS
A note to our readers: You will notice the JAAD Grand Rounds series appears this month without an opportunity to earn CME credit. Unfortunately, the exhaustive underlying documentation required of journal articles that offer CME credit has become so burdensome that we are forced to abandon this benefit for this popular feature. Rest assured that CME credit is still available for the JAAD lead article.We will continue to strive to provide the best, most clinically relevant articles to assist you in the practice of dermatology.
JAAD Grand Rounds quiz* Melissa Tucker, MD, Paras Ramolia, MD, Michael J. Wells, MD, Parvathi Mudigonda, MD, Howard P. Ragland, MD, Andrea Murina, MD, April Taylor, BS, Roselynn H. Nguyen, BA, Donald A. Glass, II, MD, PhD, and Nnenna G. Agim, MD Instructions: In answering each question, refer to the specific directions provided. Because it is often necessary to provide information occurring later in a series that give away answers to earlier questions, please answer the questions in each series in sequence. ( J Am Acad Dermatol 2013;68:877-84.)
Neonate with extensive papulovesicles Melissa Tucker, MD, Paras Ramolia, MD, and Michael J. Wells, MD Lubbock, Texas A 2-month-old Hispanic female presented with hundreds of red papulovesicles and pustules (Figs 1 and 2) that began on the trunk and spread to the head and extremities with an onset at 2 weeks of age. The eruption was not particularly bothersome except for occasional pruritus. The infant is a full-term baby who underwent an uneventful delivery. She has met her developmental milestones and has had no issues with feeding. She has not had improvement with hydrocortisone 1% cream. At her initial visit, a 3-mm biopsy specimen was obtained from her abdomen (Figs 3 and 4). 1. What is the most likely diagnosis? a. Erythema toxicum neonatorum b. Eosinophilic folliculitis of infancy c. Transient neonatal pustular melanosis d. Acropustulosis of infancy e. Langerhans cell histiocytosis 2. Which of the following is the most common site for the lesions of this disorder? a. Trunk b. Extremities
The authors, editors, and peer reviewers have no relevant financial relationships. *The section editor for JAAD Grand Rounds is Erin E. Boh, MD. J AM ACAD DERMATOL
c. Scalp d. Palms and soles e. Neck 3. When does this disease typically have its onset? a. Within the first 24 hours of life b. Between the first 24 to 48 hours of life c. Between the first 3 days to 2 weeks of life d. Between the first 3 to 4 weeks of life e. Between the first month to the first year of life 4. What is the best treatment during outbreaks of this disorder? a. Topical corticosteroids b. Topical permethrin c. Topical antibiotics d. Systemic immunosuppressants e. Systemic antibiotics 5. When do the outbreaks usually subside? a. 1 to 5 days b. 1 to 3 months c. 3 months to 5 years d. 5 to 10 years e. More than 10 years Discussion Eosinophilic folliculitis was first described by Ofuji et al in 1965 in an adult who experienced recurrent crops of papulopustules involving the face, extremities, and trunk. The infantile form was first reported in 1984 by Lucky et al in a case series involving 5 infants. Eosinophilic folliculitis of infancy MAY 2013
877
878 JAAD grand rounds
is a rare, self-limiting disorder that presents with multiple pruritic erythematous perifollicular vesiculopapules. The infantile form primarily involves the scalp and brow region but can affect the neck, trunk, and extremities. The pustules are sterile, and their etiology is unknown. The lesions develop within the first few days to weeks of life, and the outbreaks are usually cyclic in nature with a lifespan of 3 months to 5 years. Eosinophilic folliculitis appears to be more common in boys. Histologically, the dermis contains a dense perifollicular infiltrate of eosinophils, histiocytes, and lymphocytes. The epidermis displays exocytosis of eosinophils with spongiosis and subcorneal pustules (Figs 3 and 4). The complete blood cell count can be normal or peripheral blood eosinophilia can be present, particularly during outbreaks. Eosinophilic folliculitis of infancy and erythema toxicum neonatorum share a similar histology and must be differentiated based on their natural histories. Erythema toxicum neonatorum is more widespread, appears within the first 48 hours of life, and resolves within 1 week. Transient neonatal pustular melanosis features neutrophils rather than eosinophils on histology and lesions on the face, neck, and lower extremities that are present at birth and resolve within 48 hours.
J AM ACAD DERMATOL
MAY 2013
Acropustulosis of infancy appears mostly on the hands and feet of infants and are not perifollicular, but a relationship has been suggested between this disease and eosinophilic folliculitis because of their similar clinical courses. Langerhans cell histiocytosis, notably the congenital self-healing reticulohistiocytoisis and LetterereSiwe types, affects infants but differs in its histology from eosinophilic folliculitis with the presence of S-100 and CD1a1 Langerhans cells. Congenital self-healing reticulohistiocytosis presents in the first few days of life as red-brown papules that crust and then self-resolve. Letterere Siwe presents as papulopustules favoring a seborrheic distribution and has internal organ involvement. Impetigo, candida, neonatal herpes simplex, and scabies can be distinguished from eosinophilic folliculitis by the presence of positive cultures or scrapings in the former conditions. Treatment should include reassurance of the benign yet cyclical nature of eosinophilic folliculitis of infancy. Topical corticosteroids and oral antihistamines are considered first-line treatment and used during outbreaks to help treat pruritus. Second-line treatment usually starts with indomethacin, which reportedly works via the inhibition of cyclooxygenase in the arachidonic acid pathway. Dapsone, oxyphenbutazone, aspirin, minocycline, colchicine,
J AM ACAD DERMATOL VOLUME 68, NUMBER 5
JAAD grand rounds 879
and glycylrrhizin have been used in adults but, with the exception of dapsone, are generally not recommended for children. Eosinophilic folliculitis of infancy was reported to be associated with HIV in 1 case. This 8-month-old male presented with the typical lesions of eosinophilic folliculitis but also had generalized lymphadenopathy, oral thrush, pallor, and hepatomegaly. The main differences on physical examination can include discrete, erythematous, urticarial follicular papules with crusting and excoriations. HIVassociated eosinophilic folliculitis is a marker of AIDS in these patients. Their CD4 count is \300 cells/mm3, they may have eosinophilia, and their leukocyte count is variable. The disease is usually more widely distributed and more persistent than eosinophilic folliculitis without the HIV association. Treatment includes topical corticosteroids and highly active antiretroviral therapy. For this series, the recommended choices are: 1, b; 2, c; 3, c; 4, a; 5, c. BIBLIOGRAPHY Buckley DA, Munn SE, Higgins EM. Neonatal eosinophilic pustular folliculitis. Clin Exp Dermatol 2001;26:251-5. Lucky AW, Esterly NB, Heskel N, Krafchik BR, Solomon LM. Eosinophilic pustular folliculitis in infancy. Pediatr Dermatol 1984;1:202-6. Ofuji S. Eosinophilic pustular folliculitis. Dermatologica 1987;174: 53-6. Ramdial PD, Morar B, Dlova NC, Aboabaker J. HIV-associated eosinophilic folliculitis in an infant. Am J Dermatopathol 1999; 21:241-6.
Posttreatment lesional hyperpigmentation Parvathi Mudigonda, MD, Howard P. Ragland, MD, and Andrea Murina, MD New Orleans, Louisiana A 63-year-old man presented to the dermatology clinic with a 1-year history of numerous erythematous, asymptomatic, annular plaques on his abdomen, arms, and legs (Fig 5). His social history included a long career as a trapper in the south Louisiana marshland; none of his close contacts had a similar outbreak. He reported no sensory loss; however, on physical examination, he had a stocking pattern of numbness that extended from his feet to his ankles. Laboratory studies, including an antinuclear antibody panel, a rapid plasma reagin test, rheumatoid factor, and erythrocyte sedimentation rate, were within normal limits. Biopsy specimens with special stains are shown below (Fig 6). Three months after initiating treatment with minocycline and rifampin, he presented with patches of blue-black pigmentation limited to lesional areas (Fig 7), in both sun-
exposed and covered sites. Biopsy specimens were obtained from the pigmented areas. Fontana-Masson and Perls stains are shown in Fig 8 and 9. 6. What is the most likely diagnosis? a. Arcuate dermal erythema b. Morphea c. Hansen disease d. Lupus vulgaris e. Tinea corporis 7. What is the most likely causative agent of the patient’s subsequent hyperpigmentation? a. Dapsone b. Bactrim c. Rifampin d. Minocycline e. Amiodarone 8. What is the type of hyperpigmentation associated with the causative agent that is deposited at locations of inflammation and scarring? a. Type I b. Type II c. Type III d. Type A e. Type C 9. In what manner does type II hyperpigmentation generally stain? a. Positive Perls stain, negative Fontana-Masson stain
GRAND ROUNDS
A note to our readers: You will notice the JAAD Grand Rounds series appears this month without an opportunity to earn CME credit. Unfortunately, the exhaustive underlying documentation required of journal articles that offer CME credit has become so burdensome that we are forced to abandon this benefit for this popular feature. Rest assured that CME credit is still available for the JAAD lead article.We will continue to strive to provide the best, most clinically relevant articles to assist you in the practice of dermatology.
JAAD Grand Rounds quiz* Melissa Tucker, MD, Paras Ramolia, MD, Michael J. Wells, MD, Parvathi Mudigonda, MD, Howard P. Ragland, MD, Andrea Murina, MD, April Taylor, BS, Roselynn H. Nguyen, BA, Donald A. Glass, II, MD, PhD, and Nnenna G. Agim, MD Instructions: In answering each question, refer to the specific directions provided. Because it is often necessary to provide information occurring later in a series that give away answers to earlier questions, please answer the questions in each series in sequence. ( J Am Acad Dermatol 2013;68:877-84.)
Neonate with extensive papulovesicles Melissa Tucker, MD, Paras Ramolia, MD, and Michael J. Wells, MD Lubbock, Texas A 2-month-old Hispanic female presented with hundreds of red papulovesicles and pustules (Figs 1 and 2) that began on the trunk and spread to the head and extremities with an onset at 2 weeks of age. The eruption was not particularly bothersome except for occasional pruritus. The infant is a full-term baby who underwent an uneventful delivery. She has met her developmental milestones and has had no issues with feeding. She has not had improvement with hydrocortisone 1% cream. At her initial visit, a 3-mm biopsy specimen was obtained from her abdomen (Figs 3 and 4). 1. What is the most likely diagnosis? a. Erythema toxicum neonatorum b. Eosinophilic folliculitis of infancy c. Transient neonatal pustular melanosis d. Acropustulosis of infancy e. Langerhans cell histiocytosis 2. Which of the following is the most common site for the lesions of this disorder? a. Trunk b. Extremities
The authors, editors, and peer reviewers have no relevant financial relationships. *The section editor for JAAD Grand Rounds is Erin E. Boh, MD. J AM ACAD DERMATOL
c. Scalp d. Palms and soles e. Neck 3. When does this disease typically have its onset? a. Within the first 24 hours of life b. Between the first 24 to 48 hours of life c. Between the first 3 days to 2 weeks of life d. Between the first 3 to 4 weeks of life e. Between the first month to the first year of life 4. What is the best treatment during outbreaks of this disorder? a. Topical corticosteroids b. Topical permethrin c. Topical antibiotics d. Systemic immunosuppressants e. Systemic antibiotics 5. When do the outbreaks usually subside? a. 1 to 5 days b. 1 to 3 months c. 3 months to 5 years d. 5 to 10 years e. More than 10 years Discussion Eosinophilic folliculitis was first described by Ofuji et al in 1965 in an adult who experienced recurrent crops of papulopustules involving the face, extremities, and trunk. The infantile form was first reported in 1984 by Lucky et al in a case series involving 5 infants. Eosinophilic folliculitis of infancy MAY 2013
877
878 JAAD grand rounds
is a rare, self-limiting disorder that presents with multiple pruritic erythematous perifollicular vesiculopapules. The infantile form primarily involves the scalp and brow region but can affect the neck, trunk, and extremities. The pustules are sterile, and their etiology is unknown. The lesions develop within the first few days to weeks of life, and the outbreaks are usually cyclic in nature with a lifespan of 3 months to 5 years. Eosinophilic folliculitis appears to be more common in boys. Histologically, the dermis contains a dense perifollicular infiltrate of eosinophils, histiocytes, and lymphocytes. The epidermis displays exocytosis of eosinophils with spongiosis and subcorneal pustules (Figs 3 and 4). The complete blood cell count can be normal or peripheral blood eosinophilia can be present, particularly during outbreaks. Eosinophilic folliculitis of infancy and erythema toxicum neonatorum share a similar histology and must be differentiated based on their natural histories. Erythema toxicum neonatorum is more widespread, appears within the first 48 hours of life, and resolves within 1 week. Transient neonatal pustular melanosis features neutrophils rather than eosinophils on histology and lesions on the face, neck, and lower extremities that are present at birth and resolve within 48 hours.
J AM ACAD DERMATOL
MAY 2013
Acropustulosis of infancy appears mostly on the hands and feet of infants and are not perifollicular, but a relationship has been suggested between this disease and eosinophilic folliculitis because of their similar clinical courses. Langerhans cell histiocytosis, notably the congenital self-healing reticulohistiocytoisis and LetterereSiwe types, affects infants but differs in its histology from eosinophilic folliculitis with the presence of S-100 and CD1a1 Langerhans cells. Congenital self-healing reticulohistiocytosis presents in the first few days of life as red-brown papules that crust and then self-resolve. Letterere Siwe presents as papulopustules favoring a seborrheic distribution and has internal organ involvement. Impetigo, candida, neonatal herpes simplex, and scabies can be distinguished from eosinophilic folliculitis by the presence of positive cultures or scrapings in the former conditions. Treatment should include reassurance of the benign yet cyclical nature of eosinophilic folliculitis of infancy. Topical corticosteroids and oral antihistamines are considered first-line treatment and used during outbreaks to help treat pruritus. Second-line treatment usually starts with indomethacin, which reportedly works via the inhibition of cyclooxygenase in the arachidonic acid pathway. Dapsone, oxyphenbutazone, aspirin, minocycline, colchicine,
J AM ACAD DERMATOL VOLUME 68, NUMBER 5
JAAD grand rounds 879
and glycylrrhizin have been used in adults but, with the exception of dapsone, are generally not recommended for children. Eosinophilic folliculitis of infancy was reported to be associated with HIV in 1 case. This 8-month-old male presented with the typical lesions of eosinophilic folliculitis but also had generalized lymphadenopathy, oral thrush, pallor, and hepatomegaly. The main differences on physical examination can include discrete, erythematous, urticarial follicular papules with crusting and excoriations. HIVassociated eosinophilic folliculitis is a marker of AIDS in these patients. Their CD4 count is \300 cells/mm3, they may have eosinophilia, and their leukocyte count is variable. The disease is usually more widely distributed and more persistent than eosinophilic folliculitis without the HIV association. Treatment includes topical corticosteroids and highly active antiretroviral therapy. For this series, the recommended choices are: 1, b; 2, c; 3, c; 4, a; 5, c. BIBLIOGRAPHY Buckley DA, Munn SE, Higgins EM. Neonatal eosinophilic pustular folliculitis. Clin Exp Dermatol 2001;26:251-5. Lucky AW, Esterly NB, Heskel N, Krafchik BR, Solomon LM. Eosinophilic pustular folliculitis in infancy. Pediatr Dermatol 1984;1:202-6. Ofuji S. Eosinophilic pustular folliculitis. Dermatologica 1987;174: 53-6. Ramdial PD, Morar B, Dlova NC, Aboabaker J. HIV-associated eosinophilic folliculitis in an infant. Am J Dermatopathol 1999; 21:241-6.
Posttreatment lesional hyperpigmentation Parvathi Mudigonda, MD, Howard P. Ragland, MD, and Andrea Murina, MD New Orleans, Louisiana A 63-year-old man presented to the dermatology clinic with a 1-year history of numerous erythematous, asymptomatic, annular plaques on his abdomen, arms, and legs (Fig 5). His social history included a long career as a trapper in the south Louisiana marshland; none of his close contacts had a similar outbreak. He reported no sensory loss; however, on physical examination, he had a stocking pattern of numbness that extended from his feet to his ankles. Laboratory studies, including an antinuclear antibody panel, a rapid plasma reagin test, rheumatoid factor, and erythrocyte sedimentation rate, were within normal limits. Biopsy specimens with special stains are shown below (Fig 6). Three months after initiating treatment with minocycline and rifampin, he presented with patches of blue-black pigmentation limited to lesional areas (Fig 7), in both sun-
exposed and covered sites. Biopsy specimens were obtained from the pigmented areas. Fontana-Masson and Perls stains are shown in Fig 8 and 9. 6. What is the most likely diagnosis? a. Arcuate dermal erythema b. Morphea c. Hansen disease d. Lupus vulgaris e. Tinea corporis 7. What is the most likely causative agent of the patient’s subsequent hyperpigmentation? a. Dapsone b. Bactrim c. Rifampin d. Minocycline e. Amiodarone 8. What is the type of hyperpigmentation associated with the causative agent that is deposited at locations of inflammation and scarring? a. Type I b. Type II c. Type III d. Type A e. Type C 9. In what manner does type II hyperpigmentation generally stain? a. Positive Perls stain, negative Fontana-Masson stain