- Email: [email protected]
Drug reaction with eosinophilia and systemic symptoms syndrome: A picture is worth a thousand words
J AM ACAD DERMATOL
1056 Letters
children newly diagnosed with cancer reported significantly greater distress and higher levels of family conflict than comparison samples.6 Signs and symptoms of posttraumatic stress in parents of children diagnosed with cancer last many years after the diagnosis.7 Concisely, labeling CTCL in a case of AM/FM that merely fulfills current diagnostic criteria for early CTCL is likely to induce parental distress, anxiety, and malfunctioning, rather than improve compliance. In summary, primum non nocere policy must be invariably applied to any patient. Based on my personal experience and literature review, I strongly advocate to cautiously weigh pros and cons before making early CTCL diagnosis in children, especially those with AM/FM. Sometimes we must attempt to protect our young patients from medical care with the same passion as we attempt to protect them with medical care. Alex Zvulunov, MD, MHA Faculty of Health Sciences, Medical School for International Health, Ben-Gurion University of the Negev, Research Authority, Southern District, Clalit Health Services, and Pediatric Dermatology Unit, Schneider Children’s Medical Center of Israel, Petah Tikva, Israel Funding sources: None. Conflicts of interest: None declared. Correspondence to: Alex Zvulunov, MD, MHA, Pediatric Dermatology Unit, Schneider Children’s Medical Center of Israel, 14 Kaplan St, Petah Tikva, Israel 49202 E-mail: [email protected]
REFERENCES 1. Zvulunov A, Shkalim V, Ben-Amitai D, Feinmesser M. Clinical and histopathologic spectrum of alopecia mucinosa/follicular mucinosis and its natural history in children. J Am Acad Dermatol 2012;67:1174-81. 2. Benton EC, Crichton S, Talpur R, Agar NS, Fields PA, Wedgeworth E, et al. A cutaneous lymphoma international prognostic index (CLIPi) for mycosis fungoides and Sezary syndrome. Eur J Cancer 2013;49:2859-68. 3. Hays DM, Landsverk J, Sallan SE, Hewett KD, Patenaude AF, Schoonover D, et al. Educational, occupational, and insurance status of childhood cancer survivors in their fourth and fifth decades of life. J Clin Oncol 1992;10:1397-406. 4. Gurney JG, Krull KR, Kadan-Lottick N, Nicholson HS, Nathan PC, Zebrack B, et al. Social outcomes in the childhood cancer survivor study cohort. J Clin Oncol 2009;27:2390-5. 5. Quillen J, Crawford E, Plummer B, Bradley H, Glidden R. Parental follow-through of neuropsychological recommendations for childhood-cancer survivors. Pediatr Oncol Nurs 2011;28: 306-10.
DECEMBER 2013
6. Pai AL, Greenley RN, Lewandowski A, Drotar D, Youngstrom E, Peterson CC. A meta-analytic review of the influence of pediatric cancer on parent and family functioning. J Fam Psychol 2007;21:407-15. 7. Norberg AL, Boman KK. Parent distress in childhood cancer: a comparative evaluation of posttraumatic stress symptoms, depression and anxiety. Acta Oncol 2008;47:267-74. http://dx.doi.org/10.1016/j.jaad.2013.08.004
Drug reaction with eosinophilia and systemic symptoms syndrome: A picture is worth a thousand words To the Editor: We read with great interest the excellent CME articles by Drs Husain, Reddy, and Schwartz on drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome in the May 2013 Journal of the American Academy of Dermatology.1,2 The authors did a very thorough job reviewing the literature and providing essential information to practicing dermatologists. We believe they appropriately covered all aspects of the disease’s manifestations, proposed etiopathogenesis and pathophysiology, diagnosis, management strategies, and the short- and long-term sequelae. However, we are concerned that the editor’s choice of mucositis as the cover photograph for that month’s issue will be potentially misleading to readers of the Journal of the American Academy of Dermatology. We believe it is important to point out that the cover photograph (Fig 2 in the first article), demonstrating a severe oral mucositis is a very rare clinical finding in patients with DRESS syndrome. It is our clinical impression that Figs 5 to 8 of the first CME article are far more representative of the typical cutaneous manifestations seen in most cases of DRESS syndrome. Severe mucositis, as demonstrated in the cover photograph, is more indicative of DRESS syndrome/Stevens-Johnson Syndrome overlap, which is a very rare clinical entity.3 Because of the exceptional quality of this CME article, we believe it is likely to be widely read and referenced. As a result, we think it is very important to point out to the readership that a widespread morbilliform eruption with prominent facial involvement, often accompanied by facial edemaebut without a severe mucositiseis in fact the far more commonly encountered cutaneous presentation of patients with DRESS syndrome. Misha Rosenbach, MD, Nicole Fett, MD, Karolyn A. Wanat, MD, and Robert I. Rudolph, MD Department of Dermatology, University of Pennsylvania, Philadelphia Funding sources: None.
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VOLUME 69, NUMBER 6
Conflicts of interest: None declared. Correspondence to: Misha Rosenbach, MD, Department of Dermatology, University of Pennsylvania, 3600 Spruce St, 2nd Floor Maloney Bldg, Philadelphia, PA 19104-4283 E-mail: [email protected] REFERENCES 1. Husain Z, Reddy BY, Schwartz RA. DRESS syndrome, part I: clinical perspectives. J Am Acad Dermatol 2013;68: 693e1-14. 2. Husain Z, Reddy BY, Schwartz RA. DRESS syndrome, part II: management and therapeutics. J Am Acad Dermatol 2013;68: 709.e1-9. 3. Bouvresse S, Valeyrie-Allanore L, Ortonne N, Konstantinou MP, Kardaun SH, Bagot M, et al. Toxic epidermal necrolysis, DRESS, AGEP: do overlap cases exist? Orphanet J Rare Dis 2012;7:72. http://dx.doi.org/10.1016/j.jaad.2013.06.052
Drug reaction with eosinophilia and systemic symptoms syndrome and toxic epidermal necrolysis: An uncommon confounding oral mucosal overlap To the Editor: Rosenbach et al1 have provided discerning comments, with which we concur. The image utilized showing prominent lip erosions with hemorrhagic crusts should engender discussion, as it is neither subtle nor a typical finding in the drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome. On the other hand, conspicuous oral mucosal involvement is a quintessential component of toxic epidermal necrolysis. We had no input into the editor’s choice of this striking cover photograph, but fully support its use as an uncommon but salient aspect of the DRESS syndrome.2,3 After all, it emphasizes a confounding overlap between the DRESS syndrome and toxic epidermal necrosis,4,5 just as does the astute letter by Rosenbach and associates. Robert A. Schwartz, MD, MPH, DSc (Hon), FRCP Edin,a Zain Husain, MD,b and Bobby Y. Reddy, MD, PhDc Dermatology, Rutgers University New Jersey Medical School, Newark, New Jerseya; Dermatology, Georgetown University School of Medicine, Washington, DCb; and Dermatology, Columbia University College of Physicians and Surgeons, New York, New Yorkc Funding sources: None. Conflicts of interest: None declared. Correspondence to: Robert A. Schwartz, MD, MPH, DSc (Hon), FRCP Edin, Dermatology, Rutgers
University New Jersey Medical School, 185 S Orange Ave, MSB H-576, Newark, NJ 071032714 E-mail: [email protected] REFERENCES 1. Rosenbach M, Fett NM, Wanat KA, Rudolph R. DRESS: a picture is worth a thousand words. J Am Acad Dermatol 2013;69:1056-7. 2. Husain Z, Reddy BY, Schwartz RA. DRESS syndrome, part I: clinical perspectives. J Am Acad Dermatol 2013;68:693-705. 3. Husain Z, Reddy BY, Schwartz RA. DRESS syndrome, part II: management and therapeutics. J Am Acad Dermatol 2013;68:709-17. 4. Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis, part I: introduction, history, classification, clinical features, systemic manifestations, etiology, and immunopathogenesis. J Am Acad Dermatol 2013;69:173-84. 5. Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis, part II: prognosis, sequelae, diagnosis, differential diagnosis, prevention, and treatment. J Am Acad Dermatol 2013;69:187-202. http://dx.doi.org/10.1016/j.jaad.2013.07.046
Dysphagia, a major early manifestation in DRESS syndrome To the Editor: I read with great interest the article entitled ‘‘DRESS syndrome Part I. Clinical perspectives.’’1 In this article the authors described the clinical manifestations of DRESS and proposed key points for clinical features. One of the major and early manifestations of DRESS (drug reaction with eosinophilia and systemic symptoms), dysphagia, was not sufficiently stressed. The authors stated that the eruption ‘‘can be associated with mucosal involvement, such as cheilitis, erosions, erythematous pharyngitis, and enlarged tonsils,’’ but this manifestation is very frequent. Oral mucosal involvement was observed in 52% of the 117 cases in a recent prospective RegiSCAR study.2 More interestingly dysphagia may appear before skin lesions.2 This manifestation needs to be well known because it can be misdiagnosed or overlooked. We reported that amoxicillin may be given at the beginning of DRESS with a false diagnosis of bacterial pharyngitis. Administration of amoxicillin may be detrimental for the course of DRESS and is associated with a flare of the disease (as observed in infectious mononucleosis).3 We demonstrated that amoxicillin may have a direct effect on human herpesvirus 6 replication (HHV-6) in vitro.3 The beginning of DRESS syndrome with pharyngitis is reminiscent of primary herpesvirus infection (HHV-6, Epstein-Barr virus [EBV], and cytomegalovirus [CMV]). In a recent study we demonstrated a shedding of HHV-6, HHV-7, EBV, and CMV in saliva at the early phase of DRESS.4 The DNA viral loads in blood and saliva
1056 Letters
children newly diagnosed with cancer reported significantly greater distress and higher levels of family conflict than comparison samples.6 Signs and symptoms of posttraumatic stress in parents of children diagnosed with cancer last many years after the diagnosis.7 Concisely, labeling CTCL in a case of AM/FM that merely fulfills current diagnostic criteria for early CTCL is likely to induce parental distress, anxiety, and malfunctioning, rather than improve compliance. In summary, primum non nocere policy must be invariably applied to any patient. Based on my personal experience and literature review, I strongly advocate to cautiously weigh pros and cons before making early CTCL diagnosis in children, especially those with AM/FM. Sometimes we must attempt to protect our young patients from medical care with the same passion as we attempt to protect them with medical care. Alex Zvulunov, MD, MHA Faculty of Health Sciences, Medical School for International Health, Ben-Gurion University of the Negev, Research Authority, Southern District, Clalit Health Services, and Pediatric Dermatology Unit, Schneider Children’s Medical Center of Israel, Petah Tikva, Israel Funding sources: None. Conflicts of interest: None declared. Correspondence to: Alex Zvulunov, MD, MHA, Pediatric Dermatology Unit, Schneider Children’s Medical Center of Israel, 14 Kaplan St, Petah Tikva, Israel 49202 E-mail: [email protected]
REFERENCES 1. Zvulunov A, Shkalim V, Ben-Amitai D, Feinmesser M. Clinical and histopathologic spectrum of alopecia mucinosa/follicular mucinosis and its natural history in children. J Am Acad Dermatol 2012;67:1174-81. 2. Benton EC, Crichton S, Talpur R, Agar NS, Fields PA, Wedgeworth E, et al. A cutaneous lymphoma international prognostic index (CLIPi) for mycosis fungoides and Sezary syndrome. Eur J Cancer 2013;49:2859-68. 3. Hays DM, Landsverk J, Sallan SE, Hewett KD, Patenaude AF, Schoonover D, et al. Educational, occupational, and insurance status of childhood cancer survivors in their fourth and fifth decades of life. J Clin Oncol 1992;10:1397-406. 4. Gurney JG, Krull KR, Kadan-Lottick N, Nicholson HS, Nathan PC, Zebrack B, et al. Social outcomes in the childhood cancer survivor study cohort. J Clin Oncol 2009;27:2390-5. 5. Quillen J, Crawford E, Plummer B, Bradley H, Glidden R. Parental follow-through of neuropsychological recommendations for childhood-cancer survivors. Pediatr Oncol Nurs 2011;28: 306-10.
DECEMBER 2013
6. Pai AL, Greenley RN, Lewandowski A, Drotar D, Youngstrom E, Peterson CC. A meta-analytic review of the influence of pediatric cancer on parent and family functioning. J Fam Psychol 2007;21:407-15. 7. Norberg AL, Boman KK. Parent distress in childhood cancer: a comparative evaluation of posttraumatic stress symptoms, depression and anxiety. Acta Oncol 2008;47:267-74. http://dx.doi.org/10.1016/j.jaad.2013.08.004
Drug reaction with eosinophilia and systemic symptoms syndrome: A picture is worth a thousand words To the Editor: We read with great interest the excellent CME articles by Drs Husain, Reddy, and Schwartz on drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome in the May 2013 Journal of the American Academy of Dermatology.1,2 The authors did a very thorough job reviewing the literature and providing essential information to practicing dermatologists. We believe they appropriately covered all aspects of the disease’s manifestations, proposed etiopathogenesis and pathophysiology, diagnosis, management strategies, and the short- and long-term sequelae. However, we are concerned that the editor’s choice of mucositis as the cover photograph for that month’s issue will be potentially misleading to readers of the Journal of the American Academy of Dermatology. We believe it is important to point out that the cover photograph (Fig 2 in the first article), demonstrating a severe oral mucositis is a very rare clinical finding in patients with DRESS syndrome. It is our clinical impression that Figs 5 to 8 of the first CME article are far more representative of the typical cutaneous manifestations seen in most cases of DRESS syndrome. Severe mucositis, as demonstrated in the cover photograph, is more indicative of DRESS syndrome/Stevens-Johnson Syndrome overlap, which is a very rare clinical entity.3 Because of the exceptional quality of this CME article, we believe it is likely to be widely read and referenced. As a result, we think it is very important to point out to the readership that a widespread morbilliform eruption with prominent facial involvement, often accompanied by facial edemaebut without a severe mucositiseis in fact the far more commonly encountered cutaneous presentation of patients with DRESS syndrome. Misha Rosenbach, MD, Nicole Fett, MD, Karolyn A. Wanat, MD, and Robert I. Rudolph, MD Department of Dermatology, University of Pennsylvania, Philadelphia Funding sources: None.
J AM ACAD DERMATOL
Letters 1057
VOLUME 69, NUMBER 6
Conflicts of interest: None declared. Correspondence to: Misha Rosenbach, MD, Department of Dermatology, University of Pennsylvania, 3600 Spruce St, 2nd Floor Maloney Bldg, Philadelphia, PA 19104-4283 E-mail: [email protected] REFERENCES 1. Husain Z, Reddy BY, Schwartz RA. DRESS syndrome, part I: clinical perspectives. J Am Acad Dermatol 2013;68: 693e1-14. 2. Husain Z, Reddy BY, Schwartz RA. DRESS syndrome, part II: management and therapeutics. J Am Acad Dermatol 2013;68: 709.e1-9. 3. Bouvresse S, Valeyrie-Allanore L, Ortonne N, Konstantinou MP, Kardaun SH, Bagot M, et al. Toxic epidermal necrolysis, DRESS, AGEP: do overlap cases exist? Orphanet J Rare Dis 2012;7:72. http://dx.doi.org/10.1016/j.jaad.2013.06.052
Drug reaction with eosinophilia and systemic symptoms syndrome and toxic epidermal necrolysis: An uncommon confounding oral mucosal overlap To the Editor: Rosenbach et al1 have provided discerning comments, with which we concur. The image utilized showing prominent lip erosions with hemorrhagic crusts should engender discussion, as it is neither subtle nor a typical finding in the drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome. On the other hand, conspicuous oral mucosal involvement is a quintessential component of toxic epidermal necrolysis. We had no input into the editor’s choice of this striking cover photograph, but fully support its use as an uncommon but salient aspect of the DRESS syndrome.2,3 After all, it emphasizes a confounding overlap between the DRESS syndrome and toxic epidermal necrosis,4,5 just as does the astute letter by Rosenbach and associates. Robert A. Schwartz, MD, MPH, DSc (Hon), FRCP Edin,a Zain Husain, MD,b and Bobby Y. Reddy, MD, PhDc Dermatology, Rutgers University New Jersey Medical School, Newark, New Jerseya; Dermatology, Georgetown University School of Medicine, Washington, DCb; and Dermatology, Columbia University College of Physicians and Surgeons, New York, New Yorkc Funding sources: None. Conflicts of interest: None declared. Correspondence to: Robert A. Schwartz, MD, MPH, DSc (Hon), FRCP Edin, Dermatology, Rutgers
University New Jersey Medical School, 185 S Orange Ave, MSB H-576, Newark, NJ 071032714 E-mail: [email protected] REFERENCES 1. Rosenbach M, Fett NM, Wanat KA, Rudolph R. DRESS: a picture is worth a thousand words. J Am Acad Dermatol 2013;69:1056-7. 2. Husain Z, Reddy BY, Schwartz RA. DRESS syndrome, part I: clinical perspectives. J Am Acad Dermatol 2013;68:693-705. 3. Husain Z, Reddy BY, Schwartz RA. DRESS syndrome, part II: management and therapeutics. J Am Acad Dermatol 2013;68:709-17. 4. Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis, part I: introduction, history, classification, clinical features, systemic manifestations, etiology, and immunopathogenesis. J Am Acad Dermatol 2013;69:173-84. 5. Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis, part II: prognosis, sequelae, diagnosis, differential diagnosis, prevention, and treatment. J Am Acad Dermatol 2013;69:187-202. http://dx.doi.org/10.1016/j.jaad.2013.07.046
Dysphagia, a major early manifestation in DRESS syndrome To the Editor: I read with great interest the article entitled ‘‘DRESS syndrome Part I. Clinical perspectives.’’1 In this article the authors described the clinical manifestations of DRESS and proposed key points for clinical features. One of the major and early manifestations of DRESS (drug reaction with eosinophilia and systemic symptoms), dysphagia, was not sufficiently stressed. The authors stated that the eruption ‘‘can be associated with mucosal involvement, such as cheilitis, erosions, erythematous pharyngitis, and enlarged tonsils,’’ but this manifestation is very frequent. Oral mucosal involvement was observed in 52% of the 117 cases in a recent prospective RegiSCAR study.2 More interestingly dysphagia may appear before skin lesions.2 This manifestation needs to be well known because it can be misdiagnosed or overlooked. We reported that amoxicillin may be given at the beginning of DRESS with a false diagnosis of bacterial pharyngitis. Administration of amoxicillin may be detrimental for the course of DRESS and is associated with a flare of the disease (as observed in infectious mononucleosis).3 We demonstrated that amoxicillin may have a direct effect on human herpesvirus 6 replication (HHV-6) in vitro.3 The beginning of DRESS syndrome with pharyngitis is reminiscent of primary herpesvirus infection (HHV-6, Epstein-Barr virus [EBV], and cytomegalovirus [CMV]). In a recent study we demonstrated a shedding of HHV-6, HHV-7, EBV, and CMV in saliva at the early phase of DRESS.4 The DNA viral loads in blood and saliva