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  Citation statistics : Table of Contents
   2015| April-June  | Volume 16 | Issue 2  
    Online since April 9, 2015

 
 
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CASE REPORTS
A case of silvery hair syndrome: Griscelli syndrome
Priti Khemka, Ritabrata Kundu, Prabal Niyogi, Joydev Tudu
April-June 2015, 16(2):72-74
DOI:10.4103/2319-7250.152126  
Chediak-Higashi syndrome, Griscelli syndrome (GS) and Elejalde disease are rare autosomal recessive disorders known as "silvery hair syndromes." Here, we describe a case of a 7-year-old girl with bronze skin and silvery hair, diagnosed as GS. Three genetic types of GS are known. Pigmentary dilution is common to all three and is the only finding in Griscelli Type 3. Type 1 is associated with neurological problems and Type 2 with immunodeficiency.
  2 3,390 344
The H syndrome
Sharad Mehta, Vaishali Masatkar, Asit Mittal, Ashok K Khare, Lalit K Gupta
April-June 2015, 16(2):102-104
DOI:10.4103/2319-7250.152135  
The H syndrome is a rare autosomal recessive genodermatosis caused by mutations in the nucleoside transporter hENT3. It is characterized by progressive skin sclerosis, hyperpigmentation, and hypertrichosis, along with multiple systemic manifestations including insulin-dependent diabetes mellitus. We report this case for its rarity.
  1 4,100 580
Papular xanthoma in a child: Report of a rare entity
Shruti Jadhav, Nidhi Choudhary, Rahul Ahar, Arghyaprasun Ghosh, Joydeep Singha, Archana Saha
April-June 2015, 16(2):78-80
DOI:10.4103/2319-7250.152139  
Papular xanthoma (PX) is a form of Non-Langerhan's cell histiocytosis, which is rare and primarily affects adults. Rarely cases have been reported in children as well though the presentation in both the age groups vary considerably. We present a case of PX in a 4½-year-old male child who presented with multiple asymptomatic skin-colored papular lesions on the body.
  1 2,539 246
ORIGINAL ARTICLE
Pediatric dermatoses in Benghazi, Libya
Safa Suleman Elfaituri
April-June 2015, 16(2):64-71
DOI:10.4103/2319-7250.152125  
Introduction: Skin diseases in the pediatric population are common all over the world. They are important health problem affecting children in both developing and industrialized countries with variable prevalence and pattern. Materials and Methods: Over 1-year, 954 pediatric patients attending Dermatology Department Outpatient Clinic in Jumhoria Hospital in Benghazi, Libya were studied to determine the spectrum and frequency of pediatric dermatoses in Libyan pediatric patients. Results: Infections were the most frequent dermatoses (42%) followed by eczemas (32.4%), insect bite (9.6%), pilosebaceous disorder (4.5%), hair diseases (4%) papulosquamous diseases (2.3%) and genodermatoses (1%). Conclusions: Dermatologic diseases in Libyan pediatric patients showed various features of pediatric dermatology in developing countries, such as high frequency of infections and infestations, and environmentally associated disorders; and to the best of our knowledge, this is the first study carried out on pediatric dermatoses in Libya.
  1 3,114 360
CASE REPORTS
Early congenital syphilis
Gnaneshwar Rao Angoori
April-June 2015, 16(2):75-77
DOI:10.4103/2319-7250.152127  
Syphilis is a well-known cause of morbidity and mortality since ages. Significant increase in the incidence of congenital syphilis (CS) has been observed in developing countries. Herein, we report a case of CS in 1-month-old baby, who was referred for erythematous maculo-papular rash with desquamation. Serology of baby and mother for venereal disease research laboratory and treponema pallidum hamagglutination assay confirmed the diagnosis of CS.
  - 1,450 293
Menke's kinky hair disease with visual pathway defect: An association or coincidence?
Abhijit Saha, Samipa Mukherjee, Joly Seth, Suprit Basu
April-June 2015, 16(2):81-83
DOI:10.4103/2319-7250.152128  
Menke's kinky hair disease (MKHD) is a rare X-linked recessive lethal disease of multisystem involvement. The predominant clinical findings are twisted, kinky, short, sparse, coarse, whitish, silver, or gray hair, eyebrows, and eyelashes. It is a disorder of defective copper metabolism leading to progressive neurodegeneration. The prognosis is poor with most of the patients dying at the age of 3-4 years due to respiratory failure secondary to infections. It generally always affects males with no racial predilection. We hereby report a classic case of Menke's disease with visual pathway defect but normal brain imaging. To the best of our knowledge, this type of presentation has not been reported before in the literature. Awareness amongst physicians about this rare entity is important to prevent misdiagnosis and institution of necessary supportive care. Our observation further reinforces the importance of systemic evaluation in a suspected case of MKHD.
  - 1,594 194
Faun tail nevus with neurofibroma: An uncommon cocktail…
Indrashis Podder, Anupam Das, Shamick Biswas, Nilay Kanti Das
April-June 2015, 16(2):84-86
DOI:10.4103/2319-7250.152129  
Faun tail nevus is a congenital, posterior midline cutaneous defect, characterized by a circumscribed, hypertrichotic area on the lumbosacral region. It may be a cutaneous marker of underlying spine and spinal cord anomaly. Although, patients with this condition present to us mostly for cosmetic disfigurement, we should be vigilant, and look beyond cosmetic disfigurement, to unearth any underlying spinal anomaly. Here, we present a case of faun tail nevus in a 6-year-old girl, who consulted us merely for cosmetic disability, which was found to be associated with underlying spinal anomalies affecting the lower part of spinal cord and solitary neurofibroma. The clinical importance of such a finding has prompted this report.
  - 2,063 177
Aplasia cutis congenita: Involvement of lower limbs and the feet: A rare presentation
Pradeep Kumar, Rohit Gorakh
April-June 2015, 16(2):87-89
DOI:10.4103/2319-7250.152130  
Aplasia cutis congenita is a rare heterogeneous group of congenital developmental disorder of the skin. ACC usually involves scalp. We report an interesting presentation of ACC in a neonate with extensive lesions over bilateral thighs as well as feet. Such symmetrical distributions being rarely reported.
  - 2,206 254
Well's syndrome in a child: A rare case report
Jayakar Thomas, C Zohra Begum, S Tamilarasi
April-June 2015, 16(2):90-92
DOI:10.4103/2319-7250.152131  
Well's syndrome is an uncommon condition of unknown etiology, clinically characterized by pruritic or tender cellulitis like eruption and histologically by flame figures in the dermis. We report a case of Well's syndrome in an 18-month-old boy who presented with urticaria like eruptions.
  - 1,630 206
Cooks syndrome
BM Shashikumar, MR Harish, Shweta P Bhadbhade, K Deepadarshan
April-June 2015, 16(2):93-95
DOI:10.4103/2319-7250.152132  
Cooks syndrome is an autosomal dominant ectodermal dysplasia. It is characterized by congenital onychodystrophy and anonychia along with fifth finger brachydactyly, digitalization of the thumb, absent or hypoplastic distal phalanges of hands and feet with normal hair, teeth and sweat glands. Anonychia involving all the 20 digits is a rare occurrence. Here we report a 4 day old male baby with congenital anonychia of all 20 digits along with digitalization of the thumb.
  - 2,148 207
Job's syndrome in a pediatric patient: Case report and the clinical review
Ajit Singh, Kanav Khera, Ashwin Karanam, Sheetal Chauhan
April-June 2015, 16(2):96-98
DOI:10.4103/2319-7250.152133  
Job's syndrome or hyper-immunoglobulin E syndrome (HIES) is a heterogeneous complex of immunodeficiencies distinguishes by skin abscesses, recurrent pneumatoceles or staphylococcal infections, high serum immunoglobulin E levels and eczematous dermatitis. It has two types depends on type of gene involved; autosomal-dominant HIES (AD-HIES), which develops due to signal transducer and activator of transcription 3 gene and autosomal recessive HIES caused by DOCK8 gene mutation. Hereby we present a case of 13-month-old male child who admitted to our hospital with pustular lesions on the neck, face and upper chest associated with itching; some of these were hemorrhaged and crusted. The complete clinical presentation and lab investigations have confirmed AD-HIES syndrome. The therapeutic strategy was directed mainly toward the prevention and management of infections and symptoms. This case presents the clinical features, investigational procedures and management strategy for that particular condition in pediatrics.
  - 2,386 320
Epidermolytic hyperkeratosis with Vitamin D deficiency rickets in a 5-year-old Indian girl
Amrita Roy, Debalina Sarkar, Pranab Kumar Dey, Sumita Pal
April-June 2015, 16(2):99-101
DOI:10.4103/2319-7250.152134  
We report a 5-year-old child with hyperkeratotic lesions over upper and lower limbs, with bowing of legs and limping. It was proved to be epidermolytic hyperkeratosis (EHK) with nutritional rickets on the basis of clinical, biochemical, radiological and histopathological findings. Although many cases of lamellar ichthyosis and X-linked ichthyosis with rickets have been reported, the association of EHK with rickets is rare (the first such association being reported in 2004). Such association being rare demands careful follow-up for signs and symptoms of rickets so that treatment can be started early.
  - 1,289 168
Familial trichoepithelioma
Ramesh Kumar, Prafull Mehta, Manali Jain, Devendra Yadav, Suresh Kumar Jain
April-June 2015, 16(2):105-107
DOI:10.4103/2319-7250.152136  
A 36-year-old mother and her 9-year-old daughter presented with multiple facial papules. In both cases, the papules had started to develop at about the age of 8-9 years. Biopsy of one of the mother's papules revealed a trichoepithelioma. Both mother and daughter were otherwise well. However, there have been rare reports of multiple trichoepitheliomas being associated with systemic conditions. Multiple trichoepitheliomas are inherited in an autosomal dominant manner and have been mapped to chromosome 9p21.
  - 1,576 239
Keratoderma hereditarium mutilans (Vohwinkel's syndrome)
Sweta Hasmukh Rambhia, Niranjana Raj, Madhu Suguna Madan, Bhanu Prakash
April-June 2015, 16(2):108-109
DOI:10.4103/2319-7250.152137  
Vohwinkel's syndrome is an autosomal dominant type of palmoplantar keratoderma characterized by honeycomb appearance, pseudoainhum leading to autoamputation, stellate keratosis on knuckles, and associated with sensorineural deafness.An Ichthyotic variant is recently described .We report a rare case of Vohwinkels syndrome with ichthyosis.
  - 1,564 191
LETTERS TO EDITOR
Verrucous epidermal nevus on female genitalia: A rare presentation
Tejinder Kaur, Amarjit Singh Kataria, Anisha Sethi
April-June 2015, 16(2):110-111
DOI:10.4103/2319-7250.152122  
  - 2,019 214
Universal acquired melanosis
Anirban Das
April-June 2015, 16(2):111-112
DOI:10.4103/2319-7250.152138  
  - 2,404 241
Generalized morphea in an infant with favorable outcome to topical steroid
Pranab Kumar Dey, Joyashree Banerjee, Kalpana Datta, Debabrata Bandyopadhyay
April-June 2015, 16(2):113-114
DOI:10.4103/2319-7250.152123  
  - 1,166 143
Beare-Stevenson syndrome: Rare case report
Mallesh Kariyappa, Prabjyoth Kaur Sekhon, Prakash Javarappa, S Shilpa
April-June 2015, 16(2):114-115
DOI:10.4103/2319-7250.152124  
  - 2,437 147
REVIEW ARTICLE
Mastocytosis in children
Sahana M Srinivas, Sandipan Dhar, Deepak Parikh
April-June 2015, 16(2):57-63
DOI:10.4103/2319-7250.154801  
Mastocytosis is a heterogeneous group of disorders with increase in mast cells in the skin and other extracutaneous organs. Mastocytosis is seen in children and adults. Cutaneous mastocytosis is more common in children than systemic forms. Among the cutaneous form urticaria pigmentosa is the most common type of mastocytosis seen in children. There is a tendency of regression with age. Childhood onset mastocytosis has a good prognosis. Counseling forms a major role in the management of pediatric-onset mastocytosis.
  - 6,423 705
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