- Email: [email protected]
Exploring the prevalence of learning disabilities in children with cutaneous mastocytosis: A pilot cohort study
J AM ACAD DERMATOL
1254 Research Letters
DECEMBER 2016
Mengting Qiu,a Gordon H. Bae, BA,b,c Hasan Khosravi, BA,b,c and Susan J. Huang, MDb,c
4 3.5
Average Score
a
Harvard College, Cambridge, Harvard Medical Schoolb and Department of Dermatology, Beth Israel Deaconess Medical Center,c Boston, Massachusetts
Reading Subscale Scores
4.5
3
μ+1σ
2.5 2
μ
1.5 1
Authors Qiu and Bae are first co-authors.
0.5 0
Funding sources: None. Conflicts of interest: None reported. Correspondence to: Susan J. Huang, MD, 330 Brookline Avenue, Department of Dermatology, Shapiro 2, Boston, MA 02115 E-mail: [email protected] REFERENCES 1. Cohen JJ, Gabriel BA, Terrell C. The case for diversity in the health care workforce. Health Aff (Millwood). 2002;21:90-102. 2. Bae GH, Qiu M, Reese E, Nambudiri V, Huang S. Changes in sex and ethnic diversity in dermatology residents over multiple decades. JAMA Dermatol. 2016;152:92-94. 3. Association of American Medical Colleges. Faculty Roster: U.S. Medical School Faculty, 1995 through 2014. Available at: https://services.aamc.org/famous/. Accessed September 9, 2015. 4. Fang D, Moy E, Colburn L, Hurley J. Racial and ethnic disparities in faculty promotion in academic medicine. J Am Med Assoc. 2000;284:1085-1092. 5. Peterson NB, Friedman RH, Ash AS, Franco S, Carr PL. Faculty self-reported experience with racial and ethnic discrimination in academic medicine. J Gen Intern Med. 2004;19:259-265. 6. Price EG, Gozu A, Kern DE, et al. The role of cultural diversity climate in recruitment, promotion, and retention of faculty in academic medicine. J Gen Intern Med. 2005;20:565-571. 7. PL1 Carr, Gunn CM, Kaplan SA, Raj A, Freund KM. Inadequate progress for women in academic medicine: findings from the National Faculty Study. J Womens Health (Larchmt). 2015;24: 190-199. http://dx.doi.org/10.1016/j.jaad.2016.06.050
Exploring the prevalence of learning disabilities in children with cutaneous mastocytosis: A pilot cohort study To the Editor: Mast cell disease behaves differently in the pediatric and adult populations. Skin symptoms, such as pruritus and flushing, are similar in both age groups. Pediatric mast cell disease tends to be limited to the cutaneous form with spontaneous regression typically during adolescence. In contrast, the adult form frequently progresses to systemic disease without regression.1,2 Adults with mast cell disease report ‘‘brain fog,’’ trouble processing information, or changes in cognitive ability. Studies indicate that cognitive impairment is present in 38.6% of adult patients with
1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
Pa ent No CogniƟve Disorder
CognƟve Disorder
Fig 1. Cutaneous mastocytosis and learning disabilities. Average reading subscale scores by patient (max score of 5). Standard deviation (); mean ().
mastocytosis. The most common types are attention impairment and immediate auditory memory problems.1 Although some studies suggest that the prevalence of autism is increased, little is known about other cognitive impairments in children with mast cell disease.3 This pilot cohort study sought to investigate whether mast cell disease and cognitive disorders are associated in the pediatric population. A patient database identified 67 pediatric patients with cutaneous mastocytosis seen at an academic pediatric dermatology clinic from 1997 to 2010. Parents were contacted by phone to answer questions about their child’s development; these included the Colorado Learning Difficulties Questionnaire (CLDQ) a 20-item screening questionnaire for learning difficulties in children on a 5-point Likert scale, of which math and reading scales are validated. Authors of the CLDQ survey suggest that scores greater than 1 standard deviation (SD) above the mean indicate a possible learning disorder. The mean scores are 1.79 (SD ¼ 0.94) and 1.73 (SD ¼ 0.88) for reading and math subscales, respectively. This cutoff provides high sensitivity but low specificity for learning disorders.4 The parents of 17 children agreed to answer survey questions. Average age was 11.4 years (SD ¼ 4 years, range 5.4 to 17.4 years). Two children had a formal diagnosis of a cognitive disorder (attention deficit hyperactivity disorder: 1; autism: 1). Three children (17%) had used special services at school, and 4 children (23%) had needed physical, occupational, or speech therapy. Concerning the CLDQ, 2 children (12%) had scores 1 SD above the mean for reading, 1 child (6%) had a score 1 SD above the mean for math, and 1 child (6%) had scores 1 SD above the mean for both subscales (Figs 1 and 2). Both children with known cognitive disorders were above 1 SD in at least 1 area. Of the children without a known cognitive disorder, 2 (13%) had
J AM ACAD DERMATOL
Research Letters 1255
VOLUME 75, NUMBER 6
Math Subscale Scores 4.5
Average score
4.0 3.5 3.0
μ+1σ
2.5 2.0
4. Patrick KE, McCurdy MD, Chute DL, Mahone EM, Zabel TA, Jacobson LA. Clinical utility of the Colorado Learning Difficulties Questionnaire. Pediatrics. 2013;132:e1257-e1264. 5. Altarac M, Saroha E. Lifetime prevalence of learning disability among US children. Pediatrics. 2007;119(suppl 1): 77-83.
μ
1.5
http://dx.doi.org/10.1016/j.jaad.2016.07.004
1.0 0.5 0.0 1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
Pa ent No CogniƟve Disorder
Clinical simulators of cysts
CogniƟve Disorder
Fig 2. Cutaneous mastocytosis and learning disabilities. Average math subscale scores by patient (max score of 5). Standard deviation (); mean ().
scores above the cutoff. This percentage is compared with 5.4% of well developing children in the general population who have a learning disorder.5 Due to the poor specificity of the CLDQ, we can only conclude that the children with scores above the cutoff could possibly have a learning disorder. Our study more convincingly demonstrates that 76% of the children with cutaneous mastocytosis were unlikely to have a cognitive impairment given the high sensitivity but low specificity of the CLDQ. Further investigation of a larger sample size is needed to determine the true prevalence of cognitive disorders in pediatric patients with cutaneous mastocytosis. This information may contribute to optimal management and symptom control. Alexandra Seamens, MD,a Brian Taussig, BA,a Katherine Penziner, BA,a Aimee Smidt, MD,b and Leslie P. Lawley, MDa Department of Dermatology, Emory University,a Atlanta, Georgia, and Department of Dermatology, University of New Mexico,b Albuquerque Funding sources: None. Conflicts of interest: None declared. Correspondence to: Leslie P. Lawley, MD, Department of Dermatology, Emory University, 1525 Clifton Rd NE, 1st Floor, Atlanta, GA 30322 E-mail: [email protected]
REFERENCES 1. Moura DS, Sultan S, Georgin-Lavialle S, et al. Evidence for cognitive impairment in mastocytosis: prevalence, features and correlations to depression. PLoS One. 2012;7: e39468. 2. Frieri M, Quershi M. Pediatric mastocytosis: a review of the literature. Pediatr Allergy Immunol Pulmonol. 2013;26:175-180. 3. Theoharides TC. Autism spectrum disorders and mastocytosis. Int J Immunopathol Pharmacol. 2009;22:859-865.
To the Editor: Cutaneous cysts are commonly encountered in clinical practice, yet dermatologists’ ability to successfully identify these cysts is largely unknown due to paucity of studies on the subject. Reports of malignancies misdiagnosed as cysts highlight the importance of correct recognition of cysts by clinicians.1-3 In the present study, lesions incorrectly diagnosed as cysts were selected and the number and type of lesions misdiagnosed were analyzed. Further detailed analyses of selected entities were performed to identify trends that could aid the clinician in differentiating these lesions prior to biopsy. A retrospective review of a pathology database containing data on 51,985 specimens biopsied over a 10-year period by dermatologists at Jefferson Dermatology Associates was performed. True cysts, defined as an epithelial lined space, represented 2747 (5%) of the specimens in the database. There were 2098 lesions identified by the clinical diagnosis of ‘‘cyst.’’ Of the 2098 lesions, 265 lesions met inclusion criteria as having a clinical diagnosis of ‘‘cyst,’’ but a histologic diagnosis of an entirely different entity (Tables I and II). Any histopathologic diagnosis of a true cyst was excluded (eg, follicular cysts, steatocystoma, and hidrocystoma). Of note, 72 of the 265 lesions (27%) included a differential diagnosis, with 35 of these 72 (49%) listing the correct diagnosis. However, all differentials listed ‘‘cyst’’ as the first and primary diagnosis. The misdiagnosed cases consisted of a wide spectrum of mostly dermal skin lesions that were, reassuringly, mostly benign (93%). As a category, benign adnexal neoplasms were the most common mimicker of a cyst with pilomatricomas representing the most frequently misdiagnosed lesion, an expected finding in that they often behave like a cyst. Fibrosing processes (scar and dermatofibroma) also mimicked a cyst, with dermatofibroma representing the single most common simulator. Superficial calcification, having similar coloration to a cyst, simulated a cyst relatively frequently. While superficial vascular lesions are readily recognized in a naked-eye examination, they may be misdiagnosed as cysts when situated in the deep dermis, an
1254 Research Letters
DECEMBER 2016
Mengting Qiu,a Gordon H. Bae, BA,b,c Hasan Khosravi, BA,b,c and Susan J. Huang, MDb,c
4 3.5
Average Score
a
Harvard College, Cambridge, Harvard Medical Schoolb and Department of Dermatology, Beth Israel Deaconess Medical Center,c Boston, Massachusetts
Reading Subscale Scores
4.5
3
μ+1σ
2.5 2
μ
1.5 1
Authors Qiu and Bae are first co-authors.
0.5 0
Funding sources: None. Conflicts of interest: None reported. Correspondence to: Susan J. Huang, MD, 330 Brookline Avenue, Department of Dermatology, Shapiro 2, Boston, MA 02115 E-mail: [email protected] REFERENCES 1. Cohen JJ, Gabriel BA, Terrell C. The case for diversity in the health care workforce. Health Aff (Millwood). 2002;21:90-102. 2. Bae GH, Qiu M, Reese E, Nambudiri V, Huang S. Changes in sex and ethnic diversity in dermatology residents over multiple decades. JAMA Dermatol. 2016;152:92-94. 3. Association of American Medical Colleges. Faculty Roster: U.S. Medical School Faculty, 1995 through 2014. Available at: https://services.aamc.org/famous/. Accessed September 9, 2015. 4. Fang D, Moy E, Colburn L, Hurley J. Racial and ethnic disparities in faculty promotion in academic medicine. J Am Med Assoc. 2000;284:1085-1092. 5. Peterson NB, Friedman RH, Ash AS, Franco S, Carr PL. Faculty self-reported experience with racial and ethnic discrimination in academic medicine. J Gen Intern Med. 2004;19:259-265. 6. Price EG, Gozu A, Kern DE, et al. The role of cultural diversity climate in recruitment, promotion, and retention of faculty in academic medicine. J Gen Intern Med. 2005;20:565-571. 7. PL1 Carr, Gunn CM, Kaplan SA, Raj A, Freund KM. Inadequate progress for women in academic medicine: findings from the National Faculty Study. J Womens Health (Larchmt). 2015;24: 190-199. http://dx.doi.org/10.1016/j.jaad.2016.06.050
Exploring the prevalence of learning disabilities in children with cutaneous mastocytosis: A pilot cohort study To the Editor: Mast cell disease behaves differently in the pediatric and adult populations. Skin symptoms, such as pruritus and flushing, are similar in both age groups. Pediatric mast cell disease tends to be limited to the cutaneous form with spontaneous regression typically during adolescence. In contrast, the adult form frequently progresses to systemic disease without regression.1,2 Adults with mast cell disease report ‘‘brain fog,’’ trouble processing information, or changes in cognitive ability. Studies indicate that cognitive impairment is present in 38.6% of adult patients with
1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
Pa ent No CogniƟve Disorder
CognƟve Disorder
Fig 1. Cutaneous mastocytosis and learning disabilities. Average reading subscale scores by patient (max score of 5). Standard deviation (); mean ().
mastocytosis. The most common types are attention impairment and immediate auditory memory problems.1 Although some studies suggest that the prevalence of autism is increased, little is known about other cognitive impairments in children with mast cell disease.3 This pilot cohort study sought to investigate whether mast cell disease and cognitive disorders are associated in the pediatric population. A patient database identified 67 pediatric patients with cutaneous mastocytosis seen at an academic pediatric dermatology clinic from 1997 to 2010. Parents were contacted by phone to answer questions about their child’s development; these included the Colorado Learning Difficulties Questionnaire (CLDQ) a 20-item screening questionnaire for learning difficulties in children on a 5-point Likert scale, of which math and reading scales are validated. Authors of the CLDQ survey suggest that scores greater than 1 standard deviation (SD) above the mean indicate a possible learning disorder. The mean scores are 1.79 (SD ¼ 0.94) and 1.73 (SD ¼ 0.88) for reading and math subscales, respectively. This cutoff provides high sensitivity but low specificity for learning disorders.4 The parents of 17 children agreed to answer survey questions. Average age was 11.4 years (SD ¼ 4 years, range 5.4 to 17.4 years). Two children had a formal diagnosis of a cognitive disorder (attention deficit hyperactivity disorder: 1; autism: 1). Three children (17%) had used special services at school, and 4 children (23%) had needed physical, occupational, or speech therapy. Concerning the CLDQ, 2 children (12%) had scores 1 SD above the mean for reading, 1 child (6%) had a score 1 SD above the mean for math, and 1 child (6%) had scores 1 SD above the mean for both subscales (Figs 1 and 2). Both children with known cognitive disorders were above 1 SD in at least 1 area. Of the children without a known cognitive disorder, 2 (13%) had
J AM ACAD DERMATOL
Research Letters 1255
VOLUME 75, NUMBER 6
Math Subscale Scores 4.5
Average score
4.0 3.5 3.0
μ+1σ
2.5 2.0
4. Patrick KE, McCurdy MD, Chute DL, Mahone EM, Zabel TA, Jacobson LA. Clinical utility of the Colorado Learning Difficulties Questionnaire. Pediatrics. 2013;132:e1257-e1264. 5. Altarac M, Saroha E. Lifetime prevalence of learning disability among US children. Pediatrics. 2007;119(suppl 1): 77-83.
μ
1.5
http://dx.doi.org/10.1016/j.jaad.2016.07.004
1.0 0.5 0.0 1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
Pa ent No CogniƟve Disorder
Clinical simulators of cysts
CogniƟve Disorder
Fig 2. Cutaneous mastocytosis and learning disabilities. Average math subscale scores by patient (max score of 5). Standard deviation (); mean ().
scores above the cutoff. This percentage is compared with 5.4% of well developing children in the general population who have a learning disorder.5 Due to the poor specificity of the CLDQ, we can only conclude that the children with scores above the cutoff could possibly have a learning disorder. Our study more convincingly demonstrates that 76% of the children with cutaneous mastocytosis were unlikely to have a cognitive impairment given the high sensitivity but low specificity of the CLDQ. Further investigation of a larger sample size is needed to determine the true prevalence of cognitive disorders in pediatric patients with cutaneous mastocytosis. This information may contribute to optimal management and symptom control. Alexandra Seamens, MD,a Brian Taussig, BA,a Katherine Penziner, BA,a Aimee Smidt, MD,b and Leslie P. Lawley, MDa Department of Dermatology, Emory University,a Atlanta, Georgia, and Department of Dermatology, University of New Mexico,b Albuquerque Funding sources: None. Conflicts of interest: None declared. Correspondence to: Leslie P. Lawley, MD, Department of Dermatology, Emory University, 1525 Clifton Rd NE, 1st Floor, Atlanta, GA 30322 E-mail: [email protected]
REFERENCES 1. Moura DS, Sultan S, Georgin-Lavialle S, et al. Evidence for cognitive impairment in mastocytosis: prevalence, features and correlations to depression. PLoS One. 2012;7: e39468. 2. Frieri M, Quershi M. Pediatric mastocytosis: a review of the literature. Pediatr Allergy Immunol Pulmonol. 2013;26:175-180. 3. Theoharides TC. Autism spectrum disorders and mastocytosis. Int J Immunopathol Pharmacol. 2009;22:859-865.
To the Editor: Cutaneous cysts are commonly encountered in clinical practice, yet dermatologists’ ability to successfully identify these cysts is largely unknown due to paucity of studies on the subject. Reports of malignancies misdiagnosed as cysts highlight the importance of correct recognition of cysts by clinicians.1-3 In the present study, lesions incorrectly diagnosed as cysts were selected and the number and type of lesions misdiagnosed were analyzed. Further detailed analyses of selected entities were performed to identify trends that could aid the clinician in differentiating these lesions prior to biopsy. A retrospective review of a pathology database containing data on 51,985 specimens biopsied over a 10-year period by dermatologists at Jefferson Dermatology Associates was performed. True cysts, defined as an epithelial lined space, represented 2747 (5%) of the specimens in the database. There were 2098 lesions identified by the clinical diagnosis of ‘‘cyst.’’ Of the 2098 lesions, 265 lesions met inclusion criteria as having a clinical diagnosis of ‘‘cyst,’’ but a histologic diagnosis of an entirely different entity (Tables I and II). Any histopathologic diagnosis of a true cyst was excluded (eg, follicular cysts, steatocystoma, and hidrocystoma). Of note, 72 of the 265 lesions (27%) included a differential diagnosis, with 35 of these 72 (49%) listing the correct diagnosis. However, all differentials listed ‘‘cyst’’ as the first and primary diagnosis. The misdiagnosed cases consisted of a wide spectrum of mostly dermal skin lesions that were, reassuringly, mostly benign (93%). As a category, benign adnexal neoplasms were the most common mimicker of a cyst with pilomatricomas representing the most frequently misdiagnosed lesion, an expected finding in that they often behave like a cyst. Fibrosing processes (scar and dermatofibroma) also mimicked a cyst, with dermatofibroma representing the single most common simulator. Superficial calcification, having similar coloration to a cyst, simulated a cyst relatively frequently. While superficial vascular lesions are readily recognized in a naked-eye examination, they may be misdiagnosed as cysts when situated in the deep dermis, an