e592
Correspondence
Are patients with mild to moderate psoriasis more physically active than healthy controls? Comments on the study by Demirel et al.
Editor, It is not clear why the authors of the report entitled “Do patients with mild to moderate psoriasis really have a sedentary lifestyle?”1 chose to exclude individuals actively participating in a sport. Furthermore, it is not clear what their working definition of sport was. If you are going to study physical activity and use criteria so obviously tied to physical activity, the provision of a clear definition of that parameter is warranted. Individuals with psoriasis are more likely to avoid participating in sporting activities,2 but the use of such participation as an exclusion criterion may exclude a greater number of potential controls, resulting in a relatively inactive control group. The use of psoriatic arthritis as an exclusion criterion is also an important point of discussion. The decision to exclude patients with psoriatic arthritis could potentially be justified, but there is little explanation of why this was done. Given that a significant proportion of psoriasis patients, including those with mild skin manifestations, have psoriatic arthritis,3,4 Demirel et al.1 excluded a significant subgroup based on a relatively common manifestation of the disease. Although it may seem obvious that psoriatic arthritis decreases physical activity, the data available to confirm that suspicion are sparse. Equally important are the questions of whether control subjects were similarly scrutinized when they were recruited and whether they were excluded for other types of arthritis diagnosed by a physician. Again, the authors1 should provide more information on the participants; simply stating the exclusion criteria is not sufficient to allow the reader to evaluate whether the patients and controls were selected properly. Specifically, race/ethnicity, socioeconomic status, and time of year are several examples of factors that might have a significant effect on physical activity levels.5–7 I do not imply that the physical activity data are invalid but, rather, that it is difficult to interpret the data without access to such critical information. I agree with Demirel, et al that their selected physical activity assessment tool is a valid measure and that they used an appropriate length of data collection (3 days). This, however, does not help the reader to interpret and apply the findings if information on the sample population is scarce. Compounded with a small sample size (which the authors candidly acknowledge1), the study leaves many unanswered questions on the relationship between psoriasis severity and physical activity.
International Journal of Dermatology 2014, 53, e578–e596
Patrick B. Wilson, MS School of Kinesiology University of Minnesota Minneapolis MN, USA E-mail: [email protected]
References 1 Demirel R, Genc A, Ucok K, et al. Do patients with mild to moderate psoriasis really have a sedentary lifestyle? Int J Dermatol 2013; 52: 1129–1134. 2 Ramsay B, OReagan M. A survey of the social and psychological effects of psoriasis. Br J Dermatol 1988; 118: 195–201. 3 Gladman DD, Antoni C, Mease P, et al. Psoriatic arthritis: epidemiology, clinical features, course, and outcome. Ann Rheum Dis 2005; 64: 14–17. 4 Jones SM, Armas JB, Cohen MG, et al. Psoriatic arthritis: outcome of disease subsets and relationship of joint disease to nail and skin disease. Br J Rheumatol 1994; 33: 834– 839. 5 Troiano RP, Berrigan D, Dodd KW, et al. Physical activity in the United States measured by accelerometer. Med Sci Sports Exerc 2008; 40: 181–188. 6 Tucker P, Gilliland J. The effect of season and weather on physical activity: a systematic review. Public Health 2007; 121: 909–922. 7 Van Dyck D, Cardon G, Deforche B, et al. Neighborhood SES and walkability are related to physical activity behavior in Belgian adults. Prev Med 2010; 50(Suppl.): 74–79.
A case of tinea cruris of the amputation stump: an example of an immunocompromised district
A 17-year-old landmine amputee girl presented with two itchy erythematous scaly patches with central resolution, which had first appeared one month earlier on the amputation stump and secondarily extended to the groin (Fig. 1). Skin cultures positive for Trichophyton rubrum supported the clinically suspected diagnosis of tinea cruris. The patient was successfully treated with topical and systemic terbinafine (250 mg/d), achieving full recovery after eight weeks. One-third of amputees are known to experience skin disorders of the amputation stump, which are another important cause of morbidity in these patients.1 Among these disorders, contact dermatitis is the most frequent, although many others have been described, such as epidermoid cysts, follicular hyperkeratosis, verrucous hyperplasia, psoriasis, eczema, and infections. In particular, T. rubrum infection has been rarely reported.1
ª 2014 The International Society of Dermatology
Correspondence
the case of a patient where an ICD subsequent to a burn scar harbored both a condition of immune suppression (skin cancer) and immune activation (discoid lupus erythematosus).7 The spectrum of ICD is progressively growing and expanding to an even larger group of diseases. The application of the ICD to amputation stumps probably is the unifying concept able to explain such a variegate possibility of skin disorders affecting amputees’ stumps. Figure 1 Erythematous scaly patches with central resolution affecting the amputation stump and the homolateral inguinal fold
The major frequency of skin disorders on amputation stumps has been previously attributed to the heat, humidity, friction, and pressure encountered within limb prosthesis.1 Although obvious, this hypothesis seems to be simplistic if related to the large spectrum of diseases occurring on the amputated limb. In our opinion, most skin disorders in amputees are examples of immunocompromised district (ICD). ICD is defined as a site in which there is an obstacle to the normal trafficking of immunocompetent cells through lymphatic channels, and/or an interference with the signals that the neuropeptides and neurotransmitters, released by peripheral nerves, send to cell membrane receptors of immunocompetent cells.2 Different conditions have been blamed for potentially causing ICD, such as chronic lymphedema, herpetic infections (Wolf’s isotopic response), vaccinations, physical injuries,2 and more lately neurological impairment.3,4 The ICD behaves as a locus minoris resistentiae, with propensity to harbor a secondary opportunistic disease (infections, tumors, immune skin disorders), which can occur after an extremely variable lapse of time.2 It must be clarified that the term immunocompromised does not exclusively refer to a reduction of local immune response but also to an increase in it.2 The amputation stump is a well-known site of lymph stasis, circulatory disorders, and peripheral nervous system impairment, which creates the full conditions for a local neuroimmunocutaneous destabilization and dysregulation of neuropeptide release, thus featuring a clear example of ICD. The different expression of cutaneous disorders on the amputation stump would depend on the prevalence of immunosuppressive or immunostimulating neuropeptides, accounting for the onset of infections1 (such as tinea cruris of our patient) and tumors5 in the case of immune suppression, or contact dermatitis, psoriasis, eczema,6 discoid lupus erythematosus, and bullous pemphigoid in the case of immune activation. It has even been reported that ª 2014 The International Society of Dermatology
Vincenzo Piccolo, MD Adone Baroni, MD, PhD Teresa Russo, MD Marco Adriano Chessa, MD Eleonora Ruocco, MD, PhD Department of Dermatology and Venereology Second University of Naples Naples Italy Vincenzo Piccolo, MD c/o II Policlinico, Edificio 9, Primo piano Via Pansini 5, 80131 Napoli, Italy E-mail: [email protected] Conflicts of interest: None.
References 1 Lyon CC, Kulkarni J, Zimerson E, et al. Skin disorders in amputees. J Am Acad Dermatol 2000; 42: 501–507. 2 Ruocco V, Brunetti G, Puca RV, et al. The immunocompromised district: a unifying concept for lymphoedematous, herpes-infected and otherwise damaged sites. J Eur Acad Dermatol Venereol 2009; 23: 1364– 1373. 3 Baroni A, Piccolo V, Russo T, et al. Recurrent blistering of the fingertips as a sign of carpal tunnel syndrome: an effect of nerve compression. Arch Dermatol 2012; 148: 545–546. 4 Piccolo V, Russo T, Baroni A. Unilateral bullous pemphigoid in hemiplegic patients: an instance of immunocompromised district. J Dermatol 2012; 40: 64–65. 5 Baroni A, Russo T, Piccolo V, et al. Opportunistic metastatic porocarcinoma after saphenous venectomy for coronary bypass surgery. Clin Exp Dermatol 2013; 38: 507–510. International Journal of Dermatology 2014, 53, e578–e596
e593
e594
Correspondence
6 Baroni A, Piccolo V, Russo T. A possible explanation for the high frequency of contact sensitisation in chronic venous ulcers. Int Wound J 2013. doi:10.1111/iwj.12108. 7 Baroni A, Brunetti G, Ruocco E. Coexistence of malignancy (skin cancer) and immune disorder (discoid lupus erythematosus) on a burn scar: a concrete example of immunocompromised district. Br J Dermatol 2011; 164: 673–675. Characteristic “pebbling” skin eruption as a presenting sign of Hunter syndrome
Hunter syndrome, or mucopolysaccharidosis type II (MPS-II), is a rare lysosomal storage disease caused by a deficiency of iduronate-2-sulfate (I2S), which breaks down glycosaminoglycans.1 The disease is characterized by distinctive cutaneous lesions of ivory-white papules.2 We herein report a patient with Hunter syndrome presenting with characteristic skin manifestations. A 6-year-old boy presented with multiple asymptomatic skin-colored papules on the scapulae and lateral aspects of both upper arms and thighs. The lesions first appeared two years earlier and gradually increased in number. The child delivered normally and had no significant past medical history. There was no associated family history. Physical examination revealed multiple, symmetrical, firm, skin-colored, 0.5 cm sized papules over the scapulae and lateral aspects of the upper arms and thighs (Fig. 1a,c). A persistent Mongolian spot was noted on the buttocks,
along with mild hepatomegaly, puffy eyelids, and clawhand deformities. Histologic examination showed deposition of pale bluish material between separated collagen bundles in the dermis (Fig. 2a). No specific abnormalities were noted in the epidermis or appendages. Alcian blue staining (pH 2.5) suggested that the pale bluish material between collagen bundles was mucin (Fig. 2b). Laboratory tests, including thyroid function tests, were normal. An enzyme assay showed that the blood leukocytes were deficient in I2S, confirming a diagnosis of Hunter syndrome. The patient was treated with intravenous recombinant idursulfase replacement therapy for 17 weeks (0.5 mg/kg per week), after which the skin lesions decreased in size (Fig. 1b,d). Mucopolysaccharidosis (MPS) comprises a group of metabolic disorders caused by inherited defects of lysosomal enzymes that break down glycosaminoglycans.1,2 Hunter syndrome is an X-linked recessive disorder, in which dermatan sulfate and heparan sulfate accumulate in various tissues due to a defect of I2S.3 The estimated prevalence is between 1/100,000 and 150,000 male births in USA.2,4 Hunter syndrome comprises two different subtypes, which are distinguishable by the age of onset and severity of neurodegeneration. MPS-IIB is the mild form, which results in skeletal abnormalities with normal intelligence and survival into adulthood.1
(b)
(a)
(c)
(d)
Figure 1 (a) Firm, ivory-white papules were observed on both scapulae before enzyme replacement therapy. (b) The number and size of the papules on both scapulae decreased after 17 weeks of therapy. (c) Papules on the lateral aspect of the right upper arm before enzyme replacement therapy. (d) The papules almost completely disappeared after 17 weeks of therapy International Journal of Dermatology 2014, 53, e578–e596
ª 2014 The International Society of Dermatology
This document is a scanned copy of a printed document. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material.
Correspondence
Are patients with mild to moderate psoriasis more physically active than healthy controls? Comments on the study by Demirel et al.
Editor, It is not clear why the authors of the report entitled “Do patients with mild to moderate psoriasis really have a sedentary lifestyle?”1 chose to exclude individuals actively participating in a sport. Furthermore, it is not clear what their working definition of sport was. If you are going to study physical activity and use criteria so obviously tied to physical activity, the provision of a clear definition of that parameter is warranted. Individuals with psoriasis are more likely to avoid participating in sporting activities,2 but the use of such participation as an exclusion criterion may exclude a greater number of potential controls, resulting in a relatively inactive control group. The use of psoriatic arthritis as an exclusion criterion is also an important point of discussion. The decision to exclude patients with psoriatic arthritis could potentially be justified, but there is little explanation of why this was done. Given that a significant proportion of psoriasis patients, including those with mild skin manifestations, have psoriatic arthritis,3,4 Demirel et al.1 excluded a significant subgroup based on a relatively common manifestation of the disease. Although it may seem obvious that psoriatic arthritis decreases physical activity, the data available to confirm that suspicion are sparse. Equally important are the questions of whether control subjects were similarly scrutinized when they were recruited and whether they were excluded for other types of arthritis diagnosed by a physician. Again, the authors1 should provide more information on the participants; simply stating the exclusion criteria is not sufficient to allow the reader to evaluate whether the patients and controls were selected properly. Specifically, race/ethnicity, socioeconomic status, and time of year are several examples of factors that might have a significant effect on physical activity levels.5–7 I do not imply that the physical activity data are invalid but, rather, that it is difficult to interpret the data without access to such critical information. I agree with Demirel, et al that their selected physical activity assessment tool is a valid measure and that they used an appropriate length of data collection (3 days). This, however, does not help the reader to interpret and apply the findings if information on the sample population is scarce. Compounded with a small sample size (which the authors candidly acknowledge1), the study leaves many unanswered questions on the relationship between psoriasis severity and physical activity.
International Journal of Dermatology 2014, 53, e578–e596
Patrick B. Wilson, MS School of Kinesiology University of Minnesota Minneapolis MN, USA E-mail: [email protected]
References 1 Demirel R, Genc A, Ucok K, et al. Do patients with mild to moderate psoriasis really have a sedentary lifestyle? Int J Dermatol 2013; 52: 1129–1134. 2 Ramsay B, OReagan M. A survey of the social and psychological effects of psoriasis. Br J Dermatol 1988; 118: 195–201. 3 Gladman DD, Antoni C, Mease P, et al. Psoriatic arthritis: epidemiology, clinical features, course, and outcome. Ann Rheum Dis 2005; 64: 14–17. 4 Jones SM, Armas JB, Cohen MG, et al. Psoriatic arthritis: outcome of disease subsets and relationship of joint disease to nail and skin disease. Br J Rheumatol 1994; 33: 834– 839. 5 Troiano RP, Berrigan D, Dodd KW, et al. Physical activity in the United States measured by accelerometer. Med Sci Sports Exerc 2008; 40: 181–188. 6 Tucker P, Gilliland J. The effect of season and weather on physical activity: a systematic review. Public Health 2007; 121: 909–922. 7 Van Dyck D, Cardon G, Deforche B, et al. Neighborhood SES and walkability are related to physical activity behavior in Belgian adults. Prev Med 2010; 50(Suppl.): 74–79.
A case of tinea cruris of the amputation stump: an example of an immunocompromised district
A 17-year-old landmine amputee girl presented with two itchy erythematous scaly patches with central resolution, which had first appeared one month earlier on the amputation stump and secondarily extended to the groin (Fig. 1). Skin cultures positive for Trichophyton rubrum supported the clinically suspected diagnosis of tinea cruris. The patient was successfully treated with topical and systemic terbinafine (250 mg/d), achieving full recovery after eight weeks. One-third of amputees are known to experience skin disorders of the amputation stump, which are another important cause of morbidity in these patients.1 Among these disorders, contact dermatitis is the most frequent, although many others have been described, such as epidermoid cysts, follicular hyperkeratosis, verrucous hyperplasia, psoriasis, eczema, and infections. In particular, T. rubrum infection has been rarely reported.1
ª 2014 The International Society of Dermatology
Correspondence
the case of a patient where an ICD subsequent to a burn scar harbored both a condition of immune suppression (skin cancer) and immune activation (discoid lupus erythematosus).7 The spectrum of ICD is progressively growing and expanding to an even larger group of diseases. The application of the ICD to amputation stumps probably is the unifying concept able to explain such a variegate possibility of skin disorders affecting amputees’ stumps. Figure 1 Erythematous scaly patches with central resolution affecting the amputation stump and the homolateral inguinal fold
The major frequency of skin disorders on amputation stumps has been previously attributed to the heat, humidity, friction, and pressure encountered within limb prosthesis.1 Although obvious, this hypothesis seems to be simplistic if related to the large spectrum of diseases occurring on the amputated limb. In our opinion, most skin disorders in amputees are examples of immunocompromised district (ICD). ICD is defined as a site in which there is an obstacle to the normal trafficking of immunocompetent cells through lymphatic channels, and/or an interference with the signals that the neuropeptides and neurotransmitters, released by peripheral nerves, send to cell membrane receptors of immunocompetent cells.2 Different conditions have been blamed for potentially causing ICD, such as chronic lymphedema, herpetic infections (Wolf’s isotopic response), vaccinations, physical injuries,2 and more lately neurological impairment.3,4 The ICD behaves as a locus minoris resistentiae, with propensity to harbor a secondary opportunistic disease (infections, tumors, immune skin disorders), which can occur after an extremely variable lapse of time.2 It must be clarified that the term immunocompromised does not exclusively refer to a reduction of local immune response but also to an increase in it.2 The amputation stump is a well-known site of lymph stasis, circulatory disorders, and peripheral nervous system impairment, which creates the full conditions for a local neuroimmunocutaneous destabilization and dysregulation of neuropeptide release, thus featuring a clear example of ICD. The different expression of cutaneous disorders on the amputation stump would depend on the prevalence of immunosuppressive or immunostimulating neuropeptides, accounting for the onset of infections1 (such as tinea cruris of our patient) and tumors5 in the case of immune suppression, or contact dermatitis, psoriasis, eczema,6 discoid lupus erythematosus, and bullous pemphigoid in the case of immune activation. It has even been reported that ª 2014 The International Society of Dermatology
Vincenzo Piccolo, MD Adone Baroni, MD, PhD Teresa Russo, MD Marco Adriano Chessa, MD Eleonora Ruocco, MD, PhD Department of Dermatology and Venereology Second University of Naples Naples Italy Vincenzo Piccolo, MD c/o II Policlinico, Edificio 9, Primo piano Via Pansini 5, 80131 Napoli, Italy E-mail: [email protected] Conflicts of interest: None.
References 1 Lyon CC, Kulkarni J, Zimerson E, et al. Skin disorders in amputees. J Am Acad Dermatol 2000; 42: 501–507. 2 Ruocco V, Brunetti G, Puca RV, et al. The immunocompromised district: a unifying concept for lymphoedematous, herpes-infected and otherwise damaged sites. J Eur Acad Dermatol Venereol 2009; 23: 1364– 1373. 3 Baroni A, Piccolo V, Russo T, et al. Recurrent blistering of the fingertips as a sign of carpal tunnel syndrome: an effect of nerve compression. Arch Dermatol 2012; 148: 545–546. 4 Piccolo V, Russo T, Baroni A. Unilateral bullous pemphigoid in hemiplegic patients: an instance of immunocompromised district. J Dermatol 2012; 40: 64–65. 5 Baroni A, Russo T, Piccolo V, et al. Opportunistic metastatic porocarcinoma after saphenous venectomy for coronary bypass surgery. Clin Exp Dermatol 2013; 38: 507–510. International Journal of Dermatology 2014, 53, e578–e596
e593
e594
Correspondence
6 Baroni A, Piccolo V, Russo T. A possible explanation for the high frequency of contact sensitisation in chronic venous ulcers. Int Wound J 2013. doi:10.1111/iwj.12108. 7 Baroni A, Brunetti G, Ruocco E. Coexistence of malignancy (skin cancer) and immune disorder (discoid lupus erythematosus) on a burn scar: a concrete example of immunocompromised district. Br J Dermatol 2011; 164: 673–675. Characteristic “pebbling” skin eruption as a presenting sign of Hunter syndrome
Hunter syndrome, or mucopolysaccharidosis type II (MPS-II), is a rare lysosomal storage disease caused by a deficiency of iduronate-2-sulfate (I2S), which breaks down glycosaminoglycans.1 The disease is characterized by distinctive cutaneous lesions of ivory-white papules.2 We herein report a patient with Hunter syndrome presenting with characteristic skin manifestations. A 6-year-old boy presented with multiple asymptomatic skin-colored papules on the scapulae and lateral aspects of both upper arms and thighs. The lesions first appeared two years earlier and gradually increased in number. The child delivered normally and had no significant past medical history. There was no associated family history. Physical examination revealed multiple, symmetrical, firm, skin-colored, 0.5 cm sized papules over the scapulae and lateral aspects of the upper arms and thighs (Fig. 1a,c). A persistent Mongolian spot was noted on the buttocks,
along with mild hepatomegaly, puffy eyelids, and clawhand deformities. Histologic examination showed deposition of pale bluish material between separated collagen bundles in the dermis (Fig. 2a). No specific abnormalities were noted in the epidermis or appendages. Alcian blue staining (pH 2.5) suggested that the pale bluish material between collagen bundles was mucin (Fig. 2b). Laboratory tests, including thyroid function tests, were normal. An enzyme assay showed that the blood leukocytes were deficient in I2S, confirming a diagnosis of Hunter syndrome. The patient was treated with intravenous recombinant idursulfase replacement therapy for 17 weeks (0.5 mg/kg per week), after which the skin lesions decreased in size (Fig. 1b,d). Mucopolysaccharidosis (MPS) comprises a group of metabolic disorders caused by inherited defects of lysosomal enzymes that break down glycosaminoglycans.1,2 Hunter syndrome is an X-linked recessive disorder, in which dermatan sulfate and heparan sulfate accumulate in various tissues due to a defect of I2S.3 The estimated prevalence is between 1/100,000 and 150,000 male births in USA.2,4 Hunter syndrome comprises two different subtypes, which are distinguishable by the age of onset and severity of neurodegeneration. MPS-IIB is the mild form, which results in skeletal abnormalities with normal intelligence and survival into adulthood.1
(b)
(a)
(c)
(d)
Figure 1 (a) Firm, ivory-white papules were observed on both scapulae before enzyme replacement therapy. (b) The number and size of the papules on both scapulae decreased after 17 weeks of therapy. (c) Papules on the lateral aspect of the right upper arm before enzyme replacement therapy. (d) The papules almost completely disappeared after 17 weeks of therapy International Journal of Dermatology 2014, 53, e578–e596
ª 2014 The International Society of Dermatology
This document is a scanned copy of a printed document. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material.
