Letters to the Editor Table 1: Recording of patient's vital parameters around episode of generalized hyperhidrosis Time (am) Clinical situation
Pulse (per minute) Blood pressure (mmHg) Temperature (ºF) Pupil
9.04
Aura (epigastric discomfort)
68
9.10
Onset of hyperhidrosis
68
110/80
98.6
Normal size, reacting to light
9.16
Episode subsides
69
112/80
98.0
Normal size, reacting to light
9.20
Patient normal
68
114/82
96.2
Normal size, reacting to light
9.30
Patient normal
68
112/80
98.0
Normal size, reacting to light
10.05
Aura (epigastric discomfort)
67
110/80
98.4
Normal size, reacting to light
Spontaneous endogenous hypermelatoninemia has been postulated as a cause in a patient who responded to propranolol.[6] But, in our case, no response was noted with propranolol. Some investigators have described this condition as a manifestation of “diencephalic epilepsy” and reported responses to antiepileptic medications.[7] However, direct evidence for the same has not been found in any case till date to the best of our knowledge. The aura of epigastric discomfort, fixed interval between episodes and electroencephalogram (EEG) findings seen in our patient support an epileptiform origin of this condition. However, the unresponsiveness to antiepileptic medications and response with cyproheptadine and clonidine indicates the possibility of some other associated pathomechanisms.
112/80
2. 3.
4.
5.
6. 7.
98.6
Shapiro WR, Williams GH, Plum F. Spontaneous recurrent hypothermia accompanying agenesis of the corpus callosum. Brain 1969;92:423-36. Hirayama K, Hoshino Y, Kumashiro H, Yamamoto T. Reverse Shapiro’s syndrome. A case of agenesis of corpus callosum associated with periodic hyperthermia. Arch Neurol 1994;51:494-6. Rodrigues MM, Lin J, Arita JH, De Castro Neto EF, Scerni DA, Cavalheiro EA, et al. Spontaneous periodic hypothermia and hyperhidrosis: A possibly novel cerebral neurotransmitter disorder. Dev Med Child Neurol 2011;53:378-80. Tambasco N, Belcastro V, Prontera P, Nigro P, Donti E, Rossi A, et al. Shapiro’s syndrome: Defining the clinical spectrum of the spontaneous paroxysmal hypothermia syndrome. Eur J Paediatr Neurol 2014;18:453-7. Duman O, Durmaz E, Akcurin S, Serteser M, Haspolat S. Spontaneous endogenous hypermelatoninemia: A new disease. Horm Res Paediatr 2010;74:444-8. DePlaen JL, Sepulchre D, Bidingija M. Paroxysmal hypertension and spontaneous periodic hypothermia. Acta Clin Belg 1992;47:401-7. Access this article online Quick Response Code:
The episodes of hypothermia in our patient occurred hourly, 24 times a day, with no diurnal or seasonal variation, for the past 8 years. Most previously reported cases suffered from episodes once in a few days. This case is being reported because of its peculiar severely disabling manifestation and evidence of epileptiform activity, which may be helpful in understanding the pathophysiology of the condition.
Sharad Mehta, Tarun Ralot, Vaishali Masatkar, Nidheesh Agarwal, Atul Rana Department of Dermatology, Venereology and Leprosy and Department of Neurology, R. N. T. Medical College, Udaipur, Rajasthan, India Address for correspondence: Asst. Prof. Sharad Mehta, Department of DVL, R.N.T. Medical College, Udaipur, 8A, Tulsinagar, Near Sai Baba Temple, Hiranmagri, Sector 5, Udaipur, Rajasthan, India. E-mail: [email protected]
REFERENCES 1.
186
EA Hines, EG Bannick. Intermittent hypothermia with disabling hyperhidrosis report of a case with successful treatment. Mayo Clin Proc 1934;9:705.
Normal size, reacting to light
Website: www.ijdvl.com DOI: 10.4103/0378-6323.152292 PMID: *****
Unilateral pruritus following stroke Sir, Post-stroke pruritus is a rare type of central itch resulting from lesions within the brain that affect afferent neural pathways, controlling centres or modulating regions of itch. However, its exact pathophysiology is unclear. A 52-year-old male presented to the dermatology outpatient department with severe and continuous itching on the left side of his body of 10 days duration. He was on treatment for systemic hypertension for the past 4 years. Three weeks before presentation to
Indian Journal of Dermatology, Venereology, and Leprology | March-April 2015 | Vol 81 | Issue 2
Letters to the Editor
us, he had a cerebrovascular accident with left-sided hemiparesis from which he recovered. Cutaneous examination revealed numerous excoriations over the left arm, left side of the trunk, and left thigh with complete sparing of the right side of the body [Figure 1]. Haematological and biochemical investigations were normal except for dyslipidaemia. A computed tomography scan done 3 weeks prior to this, showed multiple infarcts involving the left cerebellar hemisphere. Magnetic resonance imaging with magnetic resonance angiography, also performed 3 weeks ago, showed acute infarcts in various areas of the brain [Figure 2] but mainly involving the distribution of right anterior and middle cerebral arteries. Of note, lacunar infarcts were seen involving the right internal capsule and parts of right thalamus. In addition, the left lobe of cerebellum showed chronic infarcts with surrounding gliosis. Based on this background of unilateral pruritus after a recent stroke and normal renal, liver, and endocrinological function, a diagnosis of post-stroke pruritus was made and the patient started on tablet amitriptyline 25 mg once daily. Follow-up after 15 days revealed marked improvement of pruritus. Post-stroke pruritus was first described due to infarction of the internal capsule.[1] It has also been reported after contralateral thalamic stroke and cerebral infarction in the middle cerebral artery distribution.[2] In a case series of nine patients, five had involvement of the thalamus and subcortical regions.[3] Localized pruritus has also been described in many neurological disorders, such as multiple sclerosis, brain tumours, and brain abscess.[2,3] Post-stroke pain is well known and more frequently documented compared to post-stroke pruritus,
Figure 1: Numerous excoriations over the left arm, left side of the trunk with complete sparing of the right side
although both are mediated by anatomically identical, but functionally distinct C-fibres.[2] Moreover, it has also been found that the brain areas activated during itch and pain have some degree of overlap. Further, pruritus remits with painful stimuli.[4] Another possible evidence in support of the relation between the two entities is the responsiveness of post-stroke pruritus to treatment with amitriptyline, a drug used as a first-line treatment option for central post-stroke pain. The sensation of itch is multidimensional and involves different areas of the brain, such as anterior cingulate cortex, premotor area, primary somatosensory cortex, insular cortex, supplementary motor area, cerebellum and thalamus. A centre in the medulla is also believed to control scratching. Ischemic strokes involving the lateral medulla preferentially produce facial neuropathic itch that can lead to trigeminal trophic syndrome. The cerebellum might also act as a coordinating centre for the itch–scratch cycle.[5] Our patient had acute infarcts affecting the right frontal lobe, right centrum semiovale, genu and splenium of corpus callosum on the right side. He also had lacunar infarcts in the genu, posterior limb of right internal capsule and right thalamus. This explains the left-sided unilateral pruritus. However, bilateral central pruritus due to a unilateral brain lesion has also been described, though with a unilateral onset or predominance.[4] Onset of this condition is always delayed, but may be episodic lasting from several seconds to minutes, unlike the continuous pruritus in our case.[4] This report highlights the need for having a high index of suspicion for post-stroke pruritus in patients presenting with unilateral itch in the aftermath
Figure 2: Acute infarcts were seen involving the right frontal lobe, genu and splenium of corpus callosum
Indian Journal of Dermatology, Venereology, and Leprology | March-April 2015 | Vol 81 | Issue 2
187
Letters to the Editor
of a stroke. A detailed history, complete clinical examination and relevant imaging studies of brain are necessary to establish a diagnosis. Further, accurate analysis of central nervous system findings in patients with unilateral pruritus after stroke could reveal fascinating insights into the ill-defined pathomechanisms of central itch.
Satyaki Ganguly, C. Vijay Krishna, Nisha V. Parmar, Sheela Kuruvila, Dilip S. Phansalkar1 Departments of Dermatology, Venereology and Leprosy and 1 Radiodiagnosis, Pondicherry Institute of Medical Sciences, Puducherry, India Address for correspondence: Dr. Satyaki Ganguly, Department of Dermatology, Venereology and Leprosy, Pondicherry Institute of Medical Sciences, Pondicherry - 605 014, Puducherry, India. E-mail: [email protected]
REFERENCES 1. 2. 3. 4. 5.
King CA, Huff FJ, Jorizzo JL. Unilateral neurogenic pruritus: Paroxysmal itching associated with central nervous system lesions. Ann Intern Med 1982;97:222-3. Kimyai-Asadi A, Nousari HC, Kimyai-Asadi T, Milani F. Poststroke pruritus. Stroke 1999;30:692-3. Massey EW. Unilateral neurogenic pruritus following stroke. Stroke 1984;15:901-3. Canavero S, Bonicalzi V, Massa-Micon B. Central neurogenic pruritus: A literature review.Acta Neurol Belg 1997;97:244-7. Hassan I, Haji ML. Understanding itch: An update on mediators and mechanisms of pruritus. Indian J Dermatol Venereol Leprol 2014;80:106-14. Access this article online Quick Response Code:
Website: www.ijdvl.com
in widespread dysplasia of ectoderm and mesoderm.[1,2] The most distinctive change is skin atrophy that appears as streaky atrophic plaques, unilateral or bilateral, along the Blaschko’s lines. Atrophic plaques contain dermal fat dysplasia and may develop nodular outpouching. Other classical changes are papillomatous growths around lips, anus, and eyes, patchy alopecia, aplasia cutis, palmoplantar hyperkeratosis and hyperhidrosis, abnormalities in the bones and eyes, presence of hernias, and a typical facial appearance. Herein, we report two cases of focal dermal hypoplasia with large and extreme abdominal wall dehiscence. The first case was a 2-month-old male child who was delivered vaginally and born at term with low birth weight to non-consanguineous parents. He presented with respiratory distress and an extremely thin abdominal wall with bulged out intra-abdominal organs. There were multiple, cribriform, depigmented, atrophic, small plaques over the trunk, limbs, and face along the Blaschko’s lines. In addition, sternal cleft, cleft palate and lip, syndactyly of multiple toes of both feet, and lobster claw deformity of the right foot were present [Figure 1]. There were no signs of neurological abnormality or any malignancy. Histological examination of a skin biopsy from the atrophic plaque on abdomen revealed thinned out dermis with separated sparse collagen with herniation of the subcutaneous fat extending into the dermis and reaching up to the subepidermal level [Figure 2]. The second case was a 4-month-old female child (born to non-consanguineous parents) who presented with an extremely thin membranous lower abdominal
DOI: 10.4103/0378-6323.152294 PMID: *****
Severe abdominal wall defect leading to dehiscence in focal dermal hypoplasia (Goltz syndrome) Sir, Focal dermal hypoplasia (FDH), Goltz syndrome, or Goltz–Gorlin syndrome (OMIM #305600) is a rare X-linked dominant disease. Mutation in the PORCN gene (Xp11.23) causes defective Wnt signaling and results 188
D
A
B
C
Figure 1: Images of the first patient showing: (A) large area of abdominal wall dehiscence that has been operated; (B) many hypopigmented atrophic plaques along Blaschko’s lines on the lateral abdominal wall; (C) lobster claw deformity in the right foot; (D) syndactyly in the right foot
Indian Journal of Dermatology, Venereology, and Leprology | March-April 2015 | Vol 81 | Issue 2
Copyright of Indian Journal of Dermatology, Venereology & Leprology is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Pulse (per minute) Blood pressure (mmHg) Temperature (ºF) Pupil
9.04
Aura (epigastric discomfort)
68
9.10
Onset of hyperhidrosis
68
110/80
98.6
Normal size, reacting to light
9.16
Episode subsides
69
112/80
98.0
Normal size, reacting to light
9.20
Patient normal
68
114/82
96.2
Normal size, reacting to light
9.30
Patient normal
68
112/80
98.0
Normal size, reacting to light
10.05
Aura (epigastric discomfort)
67
110/80
98.4
Normal size, reacting to light
Spontaneous endogenous hypermelatoninemia has been postulated as a cause in a patient who responded to propranolol.[6] But, in our case, no response was noted with propranolol. Some investigators have described this condition as a manifestation of “diencephalic epilepsy” and reported responses to antiepileptic medications.[7] However, direct evidence for the same has not been found in any case till date to the best of our knowledge. The aura of epigastric discomfort, fixed interval between episodes and electroencephalogram (EEG) findings seen in our patient support an epileptiform origin of this condition. However, the unresponsiveness to antiepileptic medications and response with cyproheptadine and clonidine indicates the possibility of some other associated pathomechanisms.
112/80
2. 3.
4.
5.
6. 7.
98.6
Shapiro WR, Williams GH, Plum F. Spontaneous recurrent hypothermia accompanying agenesis of the corpus callosum. Brain 1969;92:423-36. Hirayama K, Hoshino Y, Kumashiro H, Yamamoto T. Reverse Shapiro’s syndrome. A case of agenesis of corpus callosum associated with periodic hyperthermia. Arch Neurol 1994;51:494-6. Rodrigues MM, Lin J, Arita JH, De Castro Neto EF, Scerni DA, Cavalheiro EA, et al. Spontaneous periodic hypothermia and hyperhidrosis: A possibly novel cerebral neurotransmitter disorder. Dev Med Child Neurol 2011;53:378-80. Tambasco N, Belcastro V, Prontera P, Nigro P, Donti E, Rossi A, et al. Shapiro’s syndrome: Defining the clinical spectrum of the spontaneous paroxysmal hypothermia syndrome. Eur J Paediatr Neurol 2014;18:453-7. Duman O, Durmaz E, Akcurin S, Serteser M, Haspolat S. Spontaneous endogenous hypermelatoninemia: A new disease. Horm Res Paediatr 2010;74:444-8. DePlaen JL, Sepulchre D, Bidingija M. Paroxysmal hypertension and spontaneous periodic hypothermia. Acta Clin Belg 1992;47:401-7. Access this article online Quick Response Code:
The episodes of hypothermia in our patient occurred hourly, 24 times a day, with no diurnal or seasonal variation, for the past 8 years. Most previously reported cases suffered from episodes once in a few days. This case is being reported because of its peculiar severely disabling manifestation and evidence of epileptiform activity, which may be helpful in understanding the pathophysiology of the condition.
Sharad Mehta, Tarun Ralot, Vaishali Masatkar, Nidheesh Agarwal, Atul Rana Department of Dermatology, Venereology and Leprosy and Department of Neurology, R. N. T. Medical College, Udaipur, Rajasthan, India Address for correspondence: Asst. Prof. Sharad Mehta, Department of DVL, R.N.T. Medical College, Udaipur, 8A, Tulsinagar, Near Sai Baba Temple, Hiranmagri, Sector 5, Udaipur, Rajasthan, India. E-mail: [email protected]
REFERENCES 1.
186
EA Hines, EG Bannick. Intermittent hypothermia with disabling hyperhidrosis report of a case with successful treatment. Mayo Clin Proc 1934;9:705.
Normal size, reacting to light
Website: www.ijdvl.com DOI: 10.4103/0378-6323.152292 PMID: *****
Unilateral pruritus following stroke Sir, Post-stroke pruritus is a rare type of central itch resulting from lesions within the brain that affect afferent neural pathways, controlling centres or modulating regions of itch. However, its exact pathophysiology is unclear. A 52-year-old male presented to the dermatology outpatient department with severe and continuous itching on the left side of his body of 10 days duration. He was on treatment for systemic hypertension for the past 4 years. Three weeks before presentation to
Indian Journal of Dermatology, Venereology, and Leprology | March-April 2015 | Vol 81 | Issue 2
Letters to the Editor
us, he had a cerebrovascular accident with left-sided hemiparesis from which he recovered. Cutaneous examination revealed numerous excoriations over the left arm, left side of the trunk, and left thigh with complete sparing of the right side of the body [Figure 1]. Haematological and biochemical investigations were normal except for dyslipidaemia. A computed tomography scan done 3 weeks prior to this, showed multiple infarcts involving the left cerebellar hemisphere. Magnetic resonance imaging with magnetic resonance angiography, also performed 3 weeks ago, showed acute infarcts in various areas of the brain [Figure 2] but mainly involving the distribution of right anterior and middle cerebral arteries. Of note, lacunar infarcts were seen involving the right internal capsule and parts of right thalamus. In addition, the left lobe of cerebellum showed chronic infarcts with surrounding gliosis. Based on this background of unilateral pruritus after a recent stroke and normal renal, liver, and endocrinological function, a diagnosis of post-stroke pruritus was made and the patient started on tablet amitriptyline 25 mg once daily. Follow-up after 15 days revealed marked improvement of pruritus. Post-stroke pruritus was first described due to infarction of the internal capsule.[1] It has also been reported after contralateral thalamic stroke and cerebral infarction in the middle cerebral artery distribution.[2] In a case series of nine patients, five had involvement of the thalamus and subcortical regions.[3] Localized pruritus has also been described in many neurological disorders, such as multiple sclerosis, brain tumours, and brain abscess.[2,3] Post-stroke pain is well known and more frequently documented compared to post-stroke pruritus,
Figure 1: Numerous excoriations over the left arm, left side of the trunk with complete sparing of the right side
although both are mediated by anatomically identical, but functionally distinct C-fibres.[2] Moreover, it has also been found that the brain areas activated during itch and pain have some degree of overlap. Further, pruritus remits with painful stimuli.[4] Another possible evidence in support of the relation between the two entities is the responsiveness of post-stroke pruritus to treatment with amitriptyline, a drug used as a first-line treatment option for central post-stroke pain. The sensation of itch is multidimensional and involves different areas of the brain, such as anterior cingulate cortex, premotor area, primary somatosensory cortex, insular cortex, supplementary motor area, cerebellum and thalamus. A centre in the medulla is also believed to control scratching. Ischemic strokes involving the lateral medulla preferentially produce facial neuropathic itch that can lead to trigeminal trophic syndrome. The cerebellum might also act as a coordinating centre for the itch–scratch cycle.[5] Our patient had acute infarcts affecting the right frontal lobe, right centrum semiovale, genu and splenium of corpus callosum on the right side. He also had lacunar infarcts in the genu, posterior limb of right internal capsule and right thalamus. This explains the left-sided unilateral pruritus. However, bilateral central pruritus due to a unilateral brain lesion has also been described, though with a unilateral onset or predominance.[4] Onset of this condition is always delayed, but may be episodic lasting from several seconds to minutes, unlike the continuous pruritus in our case.[4] This report highlights the need for having a high index of suspicion for post-stroke pruritus in patients presenting with unilateral itch in the aftermath
Figure 2: Acute infarcts were seen involving the right frontal lobe, genu and splenium of corpus callosum
Indian Journal of Dermatology, Venereology, and Leprology | March-April 2015 | Vol 81 | Issue 2
187
Letters to the Editor
of a stroke. A detailed history, complete clinical examination and relevant imaging studies of brain are necessary to establish a diagnosis. Further, accurate analysis of central nervous system findings in patients with unilateral pruritus after stroke could reveal fascinating insights into the ill-defined pathomechanisms of central itch.
Satyaki Ganguly, C. Vijay Krishna, Nisha V. Parmar, Sheela Kuruvila, Dilip S. Phansalkar1 Departments of Dermatology, Venereology and Leprosy and 1 Radiodiagnosis, Pondicherry Institute of Medical Sciences, Puducherry, India Address for correspondence: Dr. Satyaki Ganguly, Department of Dermatology, Venereology and Leprosy, Pondicherry Institute of Medical Sciences, Pondicherry - 605 014, Puducherry, India. E-mail: [email protected]
REFERENCES 1. 2. 3. 4. 5.
King CA, Huff FJ, Jorizzo JL. Unilateral neurogenic pruritus: Paroxysmal itching associated with central nervous system lesions. Ann Intern Med 1982;97:222-3. Kimyai-Asadi A, Nousari HC, Kimyai-Asadi T, Milani F. Poststroke pruritus. Stroke 1999;30:692-3. Massey EW. Unilateral neurogenic pruritus following stroke. Stroke 1984;15:901-3. Canavero S, Bonicalzi V, Massa-Micon B. Central neurogenic pruritus: A literature review.Acta Neurol Belg 1997;97:244-7. Hassan I, Haji ML. Understanding itch: An update on mediators and mechanisms of pruritus. Indian J Dermatol Venereol Leprol 2014;80:106-14. Access this article online Quick Response Code:
Website: www.ijdvl.com
in widespread dysplasia of ectoderm and mesoderm.[1,2] The most distinctive change is skin atrophy that appears as streaky atrophic plaques, unilateral or bilateral, along the Blaschko’s lines. Atrophic plaques contain dermal fat dysplasia and may develop nodular outpouching. Other classical changes are papillomatous growths around lips, anus, and eyes, patchy alopecia, aplasia cutis, palmoplantar hyperkeratosis and hyperhidrosis, abnormalities in the bones and eyes, presence of hernias, and a typical facial appearance. Herein, we report two cases of focal dermal hypoplasia with large and extreme abdominal wall dehiscence. The first case was a 2-month-old male child who was delivered vaginally and born at term with low birth weight to non-consanguineous parents. He presented with respiratory distress and an extremely thin abdominal wall with bulged out intra-abdominal organs. There were multiple, cribriform, depigmented, atrophic, small plaques over the trunk, limbs, and face along the Blaschko’s lines. In addition, sternal cleft, cleft palate and lip, syndactyly of multiple toes of both feet, and lobster claw deformity of the right foot were present [Figure 1]. There were no signs of neurological abnormality or any malignancy. Histological examination of a skin biopsy from the atrophic plaque on abdomen revealed thinned out dermis with separated sparse collagen with herniation of the subcutaneous fat extending into the dermis and reaching up to the subepidermal level [Figure 2]. The second case was a 4-month-old female child (born to non-consanguineous parents) who presented with an extremely thin membranous lower abdominal
DOI: 10.4103/0378-6323.152294 PMID: *****
Severe abdominal wall defect leading to dehiscence in focal dermal hypoplasia (Goltz syndrome) Sir, Focal dermal hypoplasia (FDH), Goltz syndrome, or Goltz–Gorlin syndrome (OMIM #305600) is a rare X-linked dominant disease. Mutation in the PORCN gene (Xp11.23) causes defective Wnt signaling and results 188
D
A
B
C
Figure 1: Images of the first patient showing: (A) large area of abdominal wall dehiscence that has been operated; (B) many hypopigmented atrophic plaques along Blaschko’s lines on the lateral abdominal wall; (C) lobster claw deformity in the right foot; (D) syndactyly in the right foot
Indian Journal of Dermatology, Venereology, and Leprology | March-April 2015 | Vol 81 | Issue 2
Copyright of Indian Journal of Dermatology, Venereology & Leprology is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
