Rohit Singla, Pihu Sethi, TG Savitha, Amrinder Jit Kanwar
Indian Journal of Paediatric Dermatology 2018 19(3):261-262
Pachyonychia congenita is an autosomal dominantly inherited disease that primarily presents as nail dystrophy and dyskeratosis of skin and mucous membranes. A 32-year-old female presented with thickening of the nails and thickening of both heels. Her father showed pincer nails with periungual thickening of skin and fissures with focal plantar keratoderma. Her two sons had similar nail changes.
Πέμπτη 28 Ιουνίου 2018
Monogenic autoinflammatory syndromes in children: Through the dermatologist's lens
Vishal Gupta, M Ramam
Indian Journal of Paediatric Dermatology 2018 19(3):194-201
Autoinflammatory syndromes are a group of rare disorders of innate immunity characterized by repeated episodes of inflammation without an obvious cause. Many of these disorders have a childhood onset, and present as recurrent fevers, skin lesions, joint pains and other systemic features. Newer autoinflammatory syndromes with previously undescribed clinical phenotypes are being increasingly recognized. Several of these have distinctive cutaneous manifestations, and dermatologists have an important role to play in the diagnosis of these conditions. Recently, molecular basis for many of these diseases has been identified, thus paving the way for novel targeted therapies. Interleukin-1 blockers have been found to be more effective than the conventional immunosuppressants in their treatment. In this article, we focus on the cutaneous features of well-recognized as well as some of the recently described monogenic autoinflammatory syndromes in children.
Indian Journal of Paediatric Dermatology 2018 19(3):194-201
Autoinflammatory syndromes are a group of rare disorders of innate immunity characterized by repeated episodes of inflammation without an obvious cause. Many of these disorders have a childhood onset, and present as recurrent fevers, skin lesions, joint pains and other systemic features. Newer autoinflammatory syndromes with previously undescribed clinical phenotypes are being increasingly recognized. Several of these have distinctive cutaneous manifestations, and dermatologists have an important role to play in the diagnosis of these conditions. Recently, molecular basis for many of these diseases has been identified, thus paving the way for novel targeted therapies. Interleukin-1 blockers have been found to be more effective than the conventional immunosuppressants in their treatment. In this article, we focus on the cutaneous features of well-recognized as well as some of the recently described monogenic autoinflammatory syndromes in children.
Severe acute malnutrition with hyperpigmentation: An uncommon association
Ibrahim Aliyu, Zainab F Ibrahim
Indian Journal of Paediatric Dermatology 2018 19(3):285-286
Indian Journal of Paediatric Dermatology 2018 19(3):285-286
Pediatric cutaneous tuberculosis: Indian scenario
Bhushan Kumar, Sheetanshu Kumar
Indian Journal of Paediatric Dermatology 2018 19(3):202-211
Burden of tuberculosis still persists in developing countries despite major advances in its treatment strategies. Cutaneous tuberculosis which is a form of extra-pulmonary tuberculosis is seen in a small but significant subset of patients visiting dermatology outpatient services. Cutaneous tuberculosis is characterized by a spectrum of multiple distinct clinical and histopathology presentations. A significant proportion of patients with cutaneous tuberculosis are seen in paediatric age group. Clinical features in children remain mostly the same as that in adults with cutaneous tuberculosis. However, systemic and lymph node involvement and incidence of disseminated disease is observed more commonly in paediatric age group . Awareness among clinicians of the clinical manifestations of cutaneous tuberculosis is of paramount importance for early diagnosis and management of cases with paediatric cutaneous tuberculosis. This would significantly prevent morbidity and complications of the disease . This review aims to discuss the epidemiology, clinical and histopathological features, diagnosis, differential diagnosis and treatment options in children with tuberculosis, especially in the Indian context.
Indian Journal of Paediatric Dermatology 2018 19(3):202-211
Burden of tuberculosis still persists in developing countries despite major advances in its treatment strategies. Cutaneous tuberculosis which is a form of extra-pulmonary tuberculosis is seen in a small but significant subset of patients visiting dermatology outpatient services. Cutaneous tuberculosis is characterized by a spectrum of multiple distinct clinical and histopathology presentations. A significant proportion of patients with cutaneous tuberculosis are seen in paediatric age group. Clinical features in children remain mostly the same as that in adults with cutaneous tuberculosis. However, systemic and lymph node involvement and incidence of disseminated disease is observed more commonly in paediatric age group . Awareness among clinicians of the clinical manifestations of cutaneous tuberculosis is of paramount importance for early diagnosis and management of cases with paediatric cutaneous tuberculosis. This would significantly prevent morbidity and complications of the disease . This review aims to discuss the epidemiology, clinical and histopathological features, diagnosis, differential diagnosis and treatment options in children with tuberculosis, especially in the Indian context.
Focal actinic porokeratosis: A new variant?
Pihu Sethi, Neeti Kumari, Bhuvan Adhlakha, Amrinder Jit Kanwar
Indian Journal of Paediatric Dermatology 2018 19(3):272-273
Porokeratosis is an autosomal dominantly transmitted disorder of keratinization predominantly affecting trunk and extremities. Many clinical variants have been described having a common histopathological feature of coronoid lamella-porokeratosis of Mibelli, superficial disseminate, disseminated superficial actinic porokeratosis, linear porokeratosis and punctuate palmoplantar porokeratosis. We present a case of 16-year-old female who had focal lesions of porokeratosis aggravated by sun exposure, over alae of nose confirmed by histopathology. The previous reports show quite a number of cases of isolated lesions of the disease on nose and perinasal area. This article highlights the actinic nature of focal facial porokeratosis.
Indian Journal of Paediatric Dermatology 2018 19(3):272-273
Porokeratosis is an autosomal dominantly transmitted disorder of keratinization predominantly affecting trunk and extremities. Many clinical variants have been described having a common histopathological feature of coronoid lamella-porokeratosis of Mibelli, superficial disseminate, disseminated superficial actinic porokeratosis, linear porokeratosis and punctuate palmoplantar porokeratosis. We present a case of 16-year-old female who had focal lesions of porokeratosis aggravated by sun exposure, over alae of nose confirmed by histopathology. The previous reports show quite a number of cases of isolated lesions of the disease on nose and perinasal area. This article highlights the actinic nature of focal facial porokeratosis.
Childhood vitiligo: A clinicoepidemiological study
Mrinal Gupta
Indian Journal of Paediatric Dermatology 2018 19(3):212-214
Background: Vitiligo is a chronic disorder of pigmentation which has serious psychosocial implications. Early onset or childhood vitiligo, although clinically similar to adult-onset vitiligo, has been found to have a distinct epidemiological, clinical, therapeutic, and prognostic profile. Aims and Objectives: This study was carried out to study the clinicoepidemiological characteristics of vitiligo in patients aged <18 years. Materials and Methods: It was a prospective, observational study carried out over a period of 1 year in which fifty children having vitiligo were examined. After taking an informed consent from the attendants/parents of the patients, a complete history including age, sex, duration of the disease, family history, history of Koebner's phenomenon, and history of associated diseases was noted. Results: The study group comprised of 29 females and 21 males (male:female 1:1.38), aged between 6 months and 17 years with a mean age of 8.45 ± 2.34 years and the mean age of onset was 5.6 years. The most common clinical subtypes observed in childhood vitiligo were vitiligo vulgaris in 42% (n = 21) followed by focal in 24% (n = 12), segmental in 12% (n = 6), and acrofacial in 10% patients (n = 5). A family history of vitiligo was seen in 16% patients (n = 8). Leukotrichia was observed in four patients, Koebner phenomenon in 5, and halo nevus in one patient. Conclusions: Childhood vitiligo is a common entity with vitiligo vulgaris being the most common subtype and lower limbs being the most common site of involvement. Children with positive family history had an earlier age of onset, and thyroid abnormalities were more common in nonsegmental variant.
Indian Journal of Paediatric Dermatology 2018 19(3):212-214
Background: Vitiligo is a chronic disorder of pigmentation which has serious psychosocial implications. Early onset or childhood vitiligo, although clinically similar to adult-onset vitiligo, has been found to have a distinct epidemiological, clinical, therapeutic, and prognostic profile. Aims and Objectives: This study was carried out to study the clinicoepidemiological characteristics of vitiligo in patients aged <18 years. Materials and Methods: It was a prospective, observational study carried out over a period of 1 year in which fifty children having vitiligo were examined. After taking an informed consent from the attendants/parents of the patients, a complete history including age, sex, duration of the disease, family history, history of Koebner's phenomenon, and history of associated diseases was noted. Results: The study group comprised of 29 females and 21 males (male:female 1:1.38), aged between 6 months and 17 years with a mean age of 8.45 ± 2.34 years and the mean age of onset was 5.6 years. The most common clinical subtypes observed in childhood vitiligo were vitiligo vulgaris in 42% (n = 21) followed by focal in 24% (n = 12), segmental in 12% (n = 6), and acrofacial in 10% patients (n = 5). A family history of vitiligo was seen in 16% patients (n = 8). Leukotrichia was observed in four patients, Koebner phenomenon in 5, and halo nevus in one patient. Conclusions: Childhood vitiligo is a common entity with vitiligo vulgaris being the most common subtype and lower limbs being the most common site of involvement. Children with positive family history had an earlier age of onset, and thyroid abnormalities were more common in nonsegmental variant.
Skin lesions simulating child abuse
Sahana M Srinivas, Celia Moss
Indian Journal of Paediatric Dermatology 2018 19(3):187-193
Child abuse is a broad term which describes various forms of maltreatment and neglect in children and is recognized increasingly. Physical abuse presents to dermatologists as well as pediatricians because the skin is so readily accessible for harm. Doctors unfamiliar with the wide range of skin disorders that simulate child abuse may mistakenly diagnose child abuse or "fabricated or induced illness by carers" (Munchausen by proxy), with traumatic consequences for the family. Mimics of child abuse include various cultural practices, birthmarks, bleeding disorders, bacterial infections, bullous diseases, and hereditary conditions. Dermatitis artefacta and self-harm must also be considered. Observation of the skin lesions and their evolution during hospitalization may provide the correct answer, but knowledge of the morphology and presentation of various skin disorders is crucial to avoid incorrect diagnosis of child abuse. This article describes some of the less well-known mimics of child abuse. It is essential that dermatologists support pediatricians in managing conditions that appear unusual and possibly artifactual.
Indian Journal of Paediatric Dermatology 2018 19(3):187-193
Child abuse is a broad term which describes various forms of maltreatment and neglect in children and is recognized increasingly. Physical abuse presents to dermatologists as well as pediatricians because the skin is so readily accessible for harm. Doctors unfamiliar with the wide range of skin disorders that simulate child abuse may mistakenly diagnose child abuse or "fabricated or induced illness by carers" (Munchausen by proxy), with traumatic consequences for the family. Mimics of child abuse include various cultural practices, birthmarks, bleeding disorders, bacterial infections, bullous diseases, and hereditary conditions. Dermatitis artefacta and self-harm must also be considered. Observation of the skin lesions and their evolution during hospitalization may provide the correct answer, but knowledge of the morphology and presentation of various skin disorders is crucial to avoid incorrect diagnosis of child abuse. This article describes some of the less well-known mimics of child abuse. It is essential that dermatologists support pediatricians in managing conditions that appear unusual and possibly artifactual.
Cutaneous markers of spinal dysraphism: A cross sectional study
Farhana Tahseen Taj
Indian Journal of Paediatric Dermatology 2018 19(3):215-219
Background: Spinal dysraphism is a rare condition where there are many cutaneous markers which will help a dermatologist to arrive at early diagnosis and prevent complications. Aims and Objectives: The aim and objectives of this are to study the various cutaneous marker of spinal dysraphism in neonatal period and its importance of recognizing early to plan for further management. Materials and Methods: Neonates were examined for cutaneous signs of spinal dysraphism in the first 4 weeks of life. Both occult and open spinal dysraphism clinical features were recorded. Detail history was recorded with respect to age of the mother, parity, consanguinity, and folic acid supplementation. Diagnosis was made clinically. Biopsy is not done as it may introduce infection into spinal canal. Results: A total of 1000 neonates were examined, out of which cutaneous signs of spinal dysraphism were seen in 135 (13.5%) newborns. Sacral dimple was most common seen in 128 (12.8%) neonates, meningomyelocele in 5 (0.5%), dermoid cyst in 1 (0.1%) and acrochordons in 1 (0.1%) neonate. Conclusion: Dermatologist should be aware of the cutaneous marker of spinal dysraphism. Although dermatologist has a less role in the management of spinal dysraphism, their early diagnosis will help in the further management and prevent orthopedic, vertebral, and urological complications.
Indian Journal of Paediatric Dermatology 2018 19(3):215-219
Background: Spinal dysraphism is a rare condition where there are many cutaneous markers which will help a dermatologist to arrive at early diagnosis and prevent complications. Aims and Objectives: The aim and objectives of this are to study the various cutaneous marker of spinal dysraphism in neonatal period and its importance of recognizing early to plan for further management. Materials and Methods: Neonates were examined for cutaneous signs of spinal dysraphism in the first 4 weeks of life. Both occult and open spinal dysraphism clinical features were recorded. Detail history was recorded with respect to age of the mother, parity, consanguinity, and folic acid supplementation. Diagnosis was made clinically. Biopsy is not done as it may introduce infection into spinal canal. Results: A total of 1000 neonates were examined, out of which cutaneous signs of spinal dysraphism were seen in 135 (13.5%) newborns. Sacral dimple was most common seen in 128 (12.8%) neonates, meningomyelocele in 5 (0.5%), dermoid cyst in 1 (0.1%) and acrochordons in 1 (0.1%) neonate. Conclusion: Dermatologist should be aware of the cutaneous marker of spinal dysraphism. Although dermatologist has a less role in the management of spinal dysraphism, their early diagnosis will help in the further management and prevent orthopedic, vertebral, and urological complications.
Systematized inflammatory linear verrucous epidermal nevus moderately responsive to systemic acitretin and topical calcipotriol
Sanjay Singh, Mamta Rai, Neetu Bhari, Savita Yadav
Indian Journal of Paediatric Dermatology 2018 19(3):266-268
Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare form of epidermal nevus which usually presents in childhood, mostly on the lower half of the body in a blaschkoid distribution. A wide variety of topical, systemic, and surgical modalities have been tried for its treatment, but it is known to be refractory to treatment. We hereby are reporting a rare case of extensive systematized ILVEN which showed moderate response to treatment with acitretin and topical calcipotriol.
Indian Journal of Paediatric Dermatology 2018 19(3):266-268
Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare form of epidermal nevus which usually presents in childhood, mostly on the lower half of the body in a blaschkoid distribution. A wide variety of topical, systemic, and surgical modalities have been tried for its treatment, but it is known to be refractory to treatment. We hereby are reporting a rare case of extensive systematized ILVEN which showed moderate response to treatment with acitretin and topical calcipotriol.
A retrospective study of the pattern of sexually transmitted diseases in teenagers attending sexually transmitted disease clinic during a 7-year period at a tertiary care centre
Jigna P Barot, Avanita D Solanki, Neela M Patel, Khushboo R Modi, Miral B Bodar
Indian Journal of Paediatric Dermatology 2018 19(3):220-223
Background: Adolescent period corresponds to the age group of 10-15 years. While teenage period, which corresponds to 13-19 year of age group, is the stage of psychosocial development. More and more young people are becoming sexually active in their mid-teens making them vulnerable to contracting the STDs. Adolescents especially in urban areas have favorable attitudes toward premarital and extramarital sex. Material and Methods: This is a retrospective study conducted at tertiary care center. Data regarding STD in teenagers (13-19 year) and their sexual behavior from January 2009 to December 2015 was collected from STI clinic. Result: Total number of adolescent attended STI clinic was 381,out of which 200 were male and 181 were female. Most common STD in female was VVC and in male was nodular scabies. out of 381 patients 155 male and 93 female had confessed about indulging in sexual activity.10 patients were tested positive for HIV and 11 patients were tested positive for syphilis. Conclusion: There is increasing incidence & prevalence of STDs in adolescents due to risky sexual behavior. It is essential to include sex education in teaching methods.
Indian Journal of Paediatric Dermatology 2018 19(3):220-223
Background: Adolescent period corresponds to the age group of 10-15 years. While teenage period, which corresponds to 13-19 year of age group, is the stage of psychosocial development. More and more young people are becoming sexually active in their mid-teens making them vulnerable to contracting the STDs. Adolescents especially in urban areas have favorable attitudes toward premarital and extramarital sex. Material and Methods: This is a retrospective study conducted at tertiary care center. Data regarding STD in teenagers (13-19 year) and their sexual behavior from January 2009 to December 2015 was collected from STI clinic. Result: Total number of adolescent attended STI clinic was 381,out of which 200 were male and 181 were female. Most common STD in female was VVC and in male was nodular scabies. out of 381 patients 155 male and 93 female had confessed about indulging in sexual activity.10 patients were tested positive for HIV and 11 patients were tested positive for syphilis. Conclusion: There is increasing incidence & prevalence of STDs in adolescents due to risky sexual behavior. It is essential to include sex education in teaching methods.
Darier's sign
Tarang Goyal, Sakshi Kohli
Indian Journal of Paediatric Dermatology 2018 19(3):277-279
Indian Journal of Paediatric Dermatology 2018 19(3):277-279
Efficacy and safety of propranolol on the proliferative phase of infantile hemangioma: A hospital-based prospective study
Jignaben Krunal Padhiyar, Nayan H Patel, Trusha P Gajjar, Mansi D Buch, Yogesh B Shah, Rekha Solanki
Indian Journal of Paediatric Dermatology 2018 19(3):224-229
Background: Propranolol may be more effective and safer than previously established therapies, and it may be used as a first-line therapy for infantile hemangioma (IH). Propranolol is thought to inhibit the growth of blood vessels by decreasing vascular endothelial growth factor. Aims and Objectives: The aim of this study is to study the efficacy and safety of propranolol in IH and for standardization of dose in tablet form. Materials and Methods: A total of 23 patients with 30 IH s were recruited in the study prospectively, after ruling out any contraindications for oral propranolol and obtaining consent from parents. Patients with <7 kg weight were given oral propranolol 5 mg twice daily and >7 kg weight were given 5 mg thrice daily. Patients were evaluated according to visual analog scale and ultrasonographically on day 0, day 30, day 60, and day 90. Results: Out of a total of 30, 25 (83.33%) were superficial, 3 (10%) were deep, and 2 (6.66%) were mixed hemangioma. All patients with superficial hemangiomas showed a change in the color of the lesion and arrest of growth within the 1st month of therapy and a gradual decrease in size was noticed in 23 (92%, n = 25) patients during the study period except in 2 (8%). Complete clearance was noticed in 68% of patients of superficial hemangioma at the end of the study period. Out of 25 patients, a total of 5 (20%) patients had ulceration at the time of presentation which started to heal within 15 days of therapy. Five (16.66%, n = 30) patients with deep and mixed variety showed arrest of growth but no decrease in size. No side effects were seen except temporary coldness of extremity in 1 (3.33%) patient. Conclusion: Propranolol is safe and effective for treatment of proliferative phase of superficial hemangiomas with very less side effects compared to oral steroids and other recommended therapies, and unavailability of syrup can be overcome by giving tablet in fixed dosage.
Indian Journal of Paediatric Dermatology 2018 19(3):224-229
Background: Propranolol may be more effective and safer than previously established therapies, and it may be used as a first-line therapy for infantile hemangioma (IH). Propranolol is thought to inhibit the growth of blood vessels by decreasing vascular endothelial growth factor. Aims and Objectives: The aim of this study is to study the efficacy and safety of propranolol in IH and for standardization of dose in tablet form. Materials and Methods: A total of 23 patients with 30 IH s were recruited in the study prospectively, after ruling out any contraindications for oral propranolol and obtaining consent from parents. Patients with <7 kg weight were given oral propranolol 5 mg twice daily and >7 kg weight were given 5 mg thrice daily. Patients were evaluated according to visual analog scale and ultrasonographically on day 0, day 30, day 60, and day 90. Results: Out of a total of 30, 25 (83.33%) were superficial, 3 (10%) were deep, and 2 (6.66%) were mixed hemangioma. All patients with superficial hemangiomas showed a change in the color of the lesion and arrest of growth within the 1st month of therapy and a gradual decrease in size was noticed in 23 (92%, n = 25) patients during the study period except in 2 (8%). Complete clearance was noticed in 68% of patients of superficial hemangioma at the end of the study period. Out of 25 patients, a total of 5 (20%) patients had ulceration at the time of presentation which started to heal within 15 days of therapy. Five (16.66%, n = 30) patients with deep and mixed variety showed arrest of growth but no decrease in size. No side effects were seen except temporary coldness of extremity in 1 (3.33%) patient. Conclusion: Propranolol is safe and effective for treatment of proliferative phase of superficial hemangiomas with very less side effects compared to oral steroids and other recommended therapies, and unavailability of syrup can be overcome by giving tablet in fixed dosage.
LEOPARD syndrome with late onset lentigines: A rarity
Sunder Nagakeerthana, Sankar Madhavi, Kaliaperumal Karthikeyan
Indian Journal of Paediatric Dermatology 2018 19(3):287-290
Indian Journal of Paediatric Dermatology 2018 19(3):287-290
The efficacy of a combination of oral low-dose prednisolone with propranolol for the treatment of infantile hemangioma
Pradyumna Pan
Indian Journal of Paediatric Dermatology 2018 19(3):230-235
Background: Infantile hemangiomas (IHs) are very common vascular tumors. Corticosteroid and propranolol are drugs for the treatment of hemangioma. High dose given for longer duration causes higher side effects. Aim and Objective: The aim and objective of this study was to determine the outcome of a combination of low-dose oral prednisolone with oral propranolol for the treatment of IH. Methods: The study comprised 42 consecutive patients with IH managed with low-dose oral prednisolone and oral propranolol between 2013 and 2016. Patients fulfilling the inclusion criteria were registered through the outpatient department. Diagnosis was confirmed clinically and on Color Doppler. All the patients were given oral prednisolone at a dose of 1 mg/kg/day and propranolol at a dose of 1.5 mg/kg/day. Treatment was given for 4 months and then titrated down for 2 months before the cessation of treatment. Results: Distribution was more on head, face, and neck. The median age at the start of treatment was 4.7 months. Out of the total 42 patients, forty patients responded to therapy (95.2%). Results were found to be excellent in 57.14% of infants and good in 30.95%. Treatment failure was seen in 4.7%. Side effects were not serious and resolved when treatment was discontinued. Rebound growth occurred in two infants (4.7%). No patients suffered drug morbidity. Conclusion: The frequency of acceptable outcome of a combination of low-dose oral prednisolone with oral propranolol for the treatment of IH shows high efficacy, low severe complication rate, and rapid clinical improvement.
Indian Journal of Paediatric Dermatology 2018 19(3):230-235
Background: Infantile hemangiomas (IHs) are very common vascular tumors. Corticosteroid and propranolol are drugs for the treatment of hemangioma. High dose given for longer duration causes higher side effects. Aim and Objective: The aim and objective of this study was to determine the outcome of a combination of low-dose oral prednisolone with oral propranolol for the treatment of IH. Methods: The study comprised 42 consecutive patients with IH managed with low-dose oral prednisolone and oral propranolol between 2013 and 2016. Patients fulfilling the inclusion criteria were registered through the outpatient department. Diagnosis was confirmed clinically and on Color Doppler. All the patients were given oral prednisolone at a dose of 1 mg/kg/day and propranolol at a dose of 1.5 mg/kg/day. Treatment was given for 4 months and then titrated down for 2 months before the cessation of treatment. Results: Distribution was more on head, face, and neck. The median age at the start of treatment was 4.7 months. Out of the total 42 patients, forty patients responded to therapy (95.2%). Results were found to be excellent in 57.14% of infants and good in 30.95%. Treatment failure was seen in 4.7%. Side effects were not serious and resolved when treatment was discontinued. Rebound growth occurred in two infants (4.7%). No patients suffered drug morbidity. Conclusion: The frequency of acceptable outcome of a combination of low-dose oral prednisolone with oral propranolol for the treatment of IH shows high efficacy, low severe complication rate, and rapid clinical improvement.
Solitary mastocytoma arising from a tight infant identification bracelet
Wei-Liang Koh, Yong-Kwang Tay, Chee-Seng Sim
Indian Journal of Paediatric Dermatology 2018 19(3):258-260
Mastocytosis is characterized by a proliferation of mast cells in the skin and extracutaneous organs. Cutaneous mastocytoma is the second most common presentation of mastocytosis in children and has been reported to arise post-trauma. We report herein the second case of solitary mastocytoma resulting from application of an excessively tight infant identification bracelet and the first in the English literature.
Indian Journal of Paediatric Dermatology 2018 19(3):258-260
Mastocytosis is characterized by a proliferation of mast cells in the skin and extracutaneous organs. Cutaneous mastocytoma is the second most common presentation of mastocytosis in children and has been reported to arise post-trauma. We report herein the second case of solitary mastocytoma resulting from application of an excessively tight infant identification bracelet and the first in the English literature.
Cutaneous manifestations of juvenile onset lupus erythematosus: A clinical study
Vinitha Panicker, Anil Mathew, Gopikrishnan Anjaneyan, Soumya Jagadeesan, S. Lekshmi, Jacob Thomas
Indian Journal of Paediatric Dermatology 2018 19(3):236-240
Introduction: Juvenile-onset systemic lupus erythematosus (SLE) is one of the most common systemic autoimmune connective tissue disorders in children. Studies focusing on the mucocutaneous manifestations of childhood lupus are scanty in literature. Objectives: This study was an attempt to describe the cutaneous changes seen in Pediatric Lupus and to correlate with the histopathological and immunofluoresecence findings. Methods: This was a retrospective study. All children under the age of 18 years who were diagnosed to have SLE according to the ACR criteria and presented to the dermatology outpatient department during a period of 1 year were included in the study. Details including clinical features, investigation findings including immunological tests and skin biopsy findings were noted. The analysis was performed using descriptive statistical tools such as percentage and frequency. Results: A total of 14 cases, average age at presentation was 10.14 years. Among the cutaneous manifestations, the most common presenting feature was urticarial vasculitis. Malar rash was seen in 57% of cases. Other cutaneous features seen were diffuse alopecia, oral ulcers. Antinuclear antibody was positive in all cases. Conclusion: Pediatric lupus tends to have a more aggressive course than adult lupus. Early diagnosis and treatment is necessary to prevent progression and development of complications.
Indian Journal of Paediatric Dermatology 2018 19(3):236-240
Introduction: Juvenile-onset systemic lupus erythematosus (SLE) is one of the most common systemic autoimmune connective tissue disorders in children. Studies focusing on the mucocutaneous manifestations of childhood lupus are scanty in literature. Objectives: This study was an attempt to describe the cutaneous changes seen in Pediatric Lupus and to correlate with the histopathological and immunofluoresecence findings. Methods: This was a retrospective study. All children under the age of 18 years who were diagnosed to have SLE according to the ACR criteria and presented to the dermatology outpatient department during a period of 1 year were included in the study. Details including clinical features, investigation findings including immunological tests and skin biopsy findings were noted. The analysis was performed using descriptive statistical tools such as percentage and frequency. Results: A total of 14 cases, average age at presentation was 10.14 years. Among the cutaneous manifestations, the most common presenting feature was urticarial vasculitis. Malar rash was seen in 57% of cases. Other cutaneous features seen were diffuse alopecia, oral ulcers. Antinuclear antibody was positive in all cases. Conclusion: Pediatric lupus tends to have a more aggressive course than adult lupus. Early diagnosis and treatment is necessary to prevent progression and development of complications.
A child with multiple café au lait macules: Rare presentation of plexiform neurofibromatosis with facial dysmorphism, alopecia, proptosis, stridor, and limb length discrepancy
Aniruddha Ghosh, Payel Kundu, Sandipan Dhar, Arunaloke Bhattacharya
Indian Journal of Paediatric Dermatology 2018 19(3):263-265
Multiple café au lait macules are one of the cornerstones in diagnosing neurofibromatosis Type 1 (NF 1). NF 1 is often a multisystem neurocutaneous disorder, plexiform NF being one of the most important variants of this phacomatosis. Here, a rare case of a 2.5-year-old male child without positive family history has been described who presented with multiple large café au lait spots, alopecia, dysmorphic facies, proptosis, protrusion and deviation of tongue, biphasic stridor, bilateral neck swelling, and abnormal gait due to foreshortened left leg. Magnetic resonance imaging revealed abnormal soft-tissue infiltration of cavernous sinus through sphenoid bone, retro-orbital, retropharyngeal, parapharyngeal spaces, floor of mouth, and encasement of unilateral neck vessels and compression over trachea. Indirect laryngoscopy revealed unilateral vocal cord palsy most likely due to recurrent laryngeal nerve involvement. Biopsy confirmed the diagnosis of NF. Pediatricians and dermatologists should be aware of syndromic causes and their varied presentations while encountering a child with multiple large café au lait spots.
Indian Journal of Paediatric Dermatology 2018 19(3):263-265
Multiple café au lait macules are one of the cornerstones in diagnosing neurofibromatosis Type 1 (NF 1). NF 1 is often a multisystem neurocutaneous disorder, plexiform NF being one of the most important variants of this phacomatosis. Here, a rare case of a 2.5-year-old male child without positive family history has been described who presented with multiple large café au lait spots, alopecia, dysmorphic facies, proptosis, protrusion and deviation of tongue, biphasic stridor, bilateral neck swelling, and abnormal gait due to foreshortened left leg. Magnetic resonance imaging revealed abnormal soft-tissue infiltration of cavernous sinus through sphenoid bone, retro-orbital, retropharyngeal, parapharyngeal spaces, floor of mouth, and encasement of unilateral neck vessels and compression over trachea. Indirect laryngoscopy revealed unilateral vocal cord palsy most likely due to recurrent laryngeal nerve involvement. Biopsy confirmed the diagnosis of NF. Pediatricians and dermatologists should be aware of syndromic causes and their varied presentations while encountering a child with multiple large café au lait spots.
A novel case of chronic mucocutaneous candidiasis: Overlap between autoimmune polyendocrinopathy, candidiasis, ectodermal dystrophy syndrome and hyper IgE syndrome
Bauyelal Mahto, Payel Kundu, Aniruddha Ghosh, Sandipan Dhar
Indian Journal of Paediatric Dermatology 2018 19(3):241-244
Chronic mucocutaneous candidiasis (CMC) is a clinical entity where extensive fungal infection of skin, hair, nail, and mucosa with Candida sp. is seen. It has got association with several immunological and endocrinal dysfunctions. We report a case of a 14-year-old boy who presented with peculiar facies, gross failure to thrive, fungal granulomatous lesions with scaring alopecia, oropharyngeal candidiasis, ectodermal dystrophy, grade 3 clubbing of all four limbs, interstitial keratitis with leukoma due to recurrent corneal ulcerations, and persistent deciduous teeth. A diagnosis of CMC was made and evaluation of immunological pathways revealed a high titer of immunoglobulin E. CMC with overlapping features of autoimmune polyendocrinopathy candidiasis ectodermal dystrophy syndrome and hyper IgE syndrome is extremely rare in literature. Timely diagnosis of an underlying etiology and proper treatment of a case of CMC may provide a better quality of life of the patient.
Indian Journal of Paediatric Dermatology 2018 19(3):241-244
Chronic mucocutaneous candidiasis (CMC) is a clinical entity where extensive fungal infection of skin, hair, nail, and mucosa with Candida sp. is seen. It has got association with several immunological and endocrinal dysfunctions. We report a case of a 14-year-old boy who presented with peculiar facies, gross failure to thrive, fungal granulomatous lesions with scaring alopecia, oropharyngeal candidiasis, ectodermal dystrophy, grade 3 clubbing of all four limbs, interstitial keratitis with leukoma due to recurrent corneal ulcerations, and persistent deciduous teeth. A diagnosis of CMC was made and evaluation of immunological pathways revealed a high titer of immunoglobulin E. CMC with overlapping features of autoimmune polyendocrinopathy candidiasis ectodermal dystrophy syndrome and hyper IgE syndrome is extremely rare in literature. Timely diagnosis of an underlying etiology and proper treatment of a case of CMC may provide a better quality of life of the patient.
Extensive childhood alopecia areata responding to combination of oral cyclosporine and corticosteroid therapy – clinical experience in four patients
Nibedita Patro, Maitreyee Panda, Sibasish Patro, Madhuchhanda Mohapatra
Indian Journal of Paediatric Dermatology 2018 19(3):269-271
Alopecia areata (AA) is a disease of unpredictable treatment outcome. Due to a great psychosocial impact associated with the disease, multitudes of therapy have been tried. We recommend safe consideration of oral cyclosporine in cases of extensive nonresponsive childhood AA.
Indian Journal of Paediatric Dermatology 2018 19(3):269-271
Alopecia areata (AA) is a disease of unpredictable treatment outcome. Due to a great psychosocial impact associated with the disease, multitudes of therapy have been tried. We recommend safe consideration of oral cyclosporine in cases of extensive nonresponsive childhood AA.
Homozygous familial hypercholesterolemia: A rare case report
Shashikant Malkud, Sirisha Varala, Geeta Kiran Arakkal, Purnachandra Badabagni
Indian Journal of Paediatric Dermatology 2018 19(3):245-247
A 9-year-old male child presented with asymptomatic skin-colored-to-yellowish nodules over both the buttocks, feet, and over the right Achilles' tendon for 4 years. There was a history of similar complaints in elder sibling and also a history of premature sudden cardiac death in father. Biopsy from the lesions was suggestive of xanthoma. Fasting lipid profile was consistent with the pattern of Type IIa hyperlipoproteinemia. The child was further worked up for systemic involvement which was normal. He was started on rosuvastatin and fenofibrate and is under regular follow-up. The timely diagnosis of familial hypercholesterolemia, especially the homozygous variant, is important for early intervention and prevention of complications.
Indian Journal of Paediatric Dermatology 2018 19(3):245-247
A 9-year-old male child presented with asymptomatic skin-colored-to-yellowish nodules over both the buttocks, feet, and over the right Achilles' tendon for 4 years. There was a history of similar complaints in elder sibling and also a history of premature sudden cardiac death in father. Biopsy from the lesions was suggestive of xanthoma. Fasting lipid profile was consistent with the pattern of Type IIa hyperlipoproteinemia. The child was further worked up for systemic involvement which was normal. He was started on rosuvastatin and fenofibrate and is under regular follow-up. The timely diagnosis of familial hypercholesterolemia, especially the homozygous variant, is important for early intervention and prevention of complications.
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