Red/orange–brown transverse nail bed lines in the acute phase of Kawasaki disease (KD) was a new clinical finding reported initially in 2010. Since then, it has been universally and consistently described in the acute phase of KD and may act as an accessory clinical clue in diagnosis. This article tries to review the clinical implication and the various reports citing this finding over the last 10 years.

Background: Lipoid proteinosis (LP) is a rare autosomal recessive disorder characterized by deposition of hyaline material in the skin, mucous membrane, and multiple organs resulting in varied manifestations such as scarring and thickening of the skin, beaded papules, and hoarseness of voice. It results from mutations in the gene encoding extracellular matrix protein 1 (ECM1). The objectives of this study are to find the mutation spectrum of the ECM1 gene in the Indian children and study the genotype-phenotype correlation. Materials and Methods: This pilot study included three unrelated Indian families with the clinical diagnosis of LP. Next-generation sequencing was done to look for ECM1 gene mutations in the proband and was confirmed by Sanger sequencing in their siblings and parents. Chromosomal microarray was done wherever necessary. Results: All six children from three unrelated consanguineous families had characteristic clinical features of LP. Two children in family 1 exhibited systemic features like temporal lobe epilepsy in one and central precocious puberty in the other. One child from family 1 and one child from family 3 had short stature. Genetic analysis revealed novel homozygous missense variation in exon 7 (c.856T>C) in family 1 and homozygous nonsense variation in exon 8 (c.1327C>T) in family 2 of the ECM1 gene. Family 3 revealed a large homozygous deletion of the ECM1 gene which has not been previously described. Conclusion: All the three families had clinical heterogeneity. This study documented two novel mutations in the ECM1 gene. Skin severity and systemic involvement were associated with missense mutation. Confirmation of ECM1 gene mutation is necessary for proper genetic counseling.

Background: Human skin color is the result of a complex interplay of various intrinsic and extrinsic factors and has great heterogeneity across the globe. It also carries an esthetic value with it, and any deviation from the normal may bear immense psychological and social implications, especially among the pediatric population where it can even be a sign of systemic diseases. Aim: The aim was to study clinical and epidemiological patterns of hyper and hypo/depigmentary disorders among children. Materials and Methods: This was a prospective study where 250 individuals ≤18 years were taken up for the study. A detailed history and clinical examination was done, and relevant investigations were carried out. The data were statistically analyzed and inferences were drawn. Results: The females outnumbered the males with 136 females and 114 males. The youngest age noted was 3 months and the oldest was 17 years. There were 118 cases of hyperpigmentary disorders, and the rest 132 patients presented with hypopigmentary and depigmented disorders. The most common hyperpigmentary disorders seen were postinflammatory hyperpigmentation, periorbital melanoses, pigmentary mosaicism, and café au lait macules. Among the hypo/depigmentary disorders, most common were pityriasis alba (39, 29.5%), followed by vitiligo seen in (35, 26.5%) and postinflammatory hypopigmentation (30, 22.7%). Conclusion: This study helped us in giving a detailed insight regarding the pigmentary alterations among the pediatric population in this area, thus aiding in prompt diagnosis among various differentials. Various aggravating and predisposing factors were also noted down which could be helpful in the prevention of some of these conditions.

Background: Pityriasis alba (PA) is a common disease of children and seen in most of the developing countries. The exact pathogenesis of PA is still not well established. Some cases of PA have been associated with atopic dermatitis, while most cases have no association with it. Objectives: The objective was to evaluate the role played by zinc (Zn) in the pathogenesis of PA. Patients and Methods: Sixty patients suffering from PA were included in the study, and 30 apparently healthy individuals of matching age and sex were recruited as a control group. Serum Zn, hemoglobin, and albumin were measured in both groups. In addition, skin phototype and body mass index were recorded. The American Academy Criteria of atopic dermatitis diagnosis were applied to the participants in this study. PA patients were examined dermatologically with a recording of the site, size, and number of lesions. Results: There was no difference in the presence of atopic dermatitis between the patient and control groups (P = 0.672). PA lesions were detected in the faces of all patients. There were no statistically significant differences between patients and controls regarding serum hemoglobin concentration (P = 0.763) or serum albumin (P = 0.487). The serum level of Zn was lower in patients with PA than controls (P < 0.001) with statistically significant positive correlations with both numbers and size of PA lesions (P < 0.001 and P = 0.011, respectively). Conclusions: The present study showed that Zn may have a role in PA, and oral supplementation with Zn may be considered as a therapeutic approach in this disease.

Asymptomatic phimosis is normal in young children which self-corrects itself but leads to parental anxiety. Topical steroids lead to correction of this physiologic phimosis avoiding the need of circumcision. Six male children aged between 3 and 14 years presented with asymptomatic tight phimosis present since birth. All the children were advised application of small amount of clobetasol propionate cream at night along with manual retraction during bath. At the end of 2 months, all the children had fully retractable prepuce. By the first visit (2 weeks), all the patients showed at least partial retractability of the prepuce. Till the follow-up period of 6 months, there was no recurrence in any patient. No side effects were observed.

We describe two siblings with diffuse, mutilating palmo-plantar keratoderma suggestive of Olmsted syndrome, with significantly compromised quality of life in the form of inability to walk and grasp objects. This was accompanied by severe pruritus and erythromelalgia-like symptoms. The keratoderma responded to acitretin therapy but there was paradoxical worsening of burning and pruritus which were further disabling. The poor response to medical and phototherapy in relieving these symptoms is described.

Erythrokeratodermas are disorders of keratinization with two major types, namely erythrokeratodermia variabilis and symmetrical progressive erythrokeratoderma. Apart from the two main types, rare variants include erythrokeratoderma en cocardes (EEC), erythokeratoderma with erythema gyratum repens-like lesions, erythema annulare centrifugum-like lesions, and reticulate erythrokeratoderma. Herein, we report a rare case of EEC with associated palmoplantar keratoderma, the association which has not been described in previous reports.

Eruptive vellus hair cyst (EVHC) is a rare developmental abnormality of the vellus hair follicles presenting in children and adolescents as multiple, asymptomatic papules over the chest, axillae, and buttocks. Clinically, it can easily be misdiagnosed as comedonal acne, keratosis pilaris, steatocystoma multiplex, syringomas, milia, lichen nitidus, perforating folliculitis, and molluscum contagiosum. Here, we report a 4-year-old girl who was being treated as a case of steroid-induced folliculitis and was later diagnosed with EVHC based on dermoscopic features and confirmed by histopathological examination. Dermoscopy of EVHC shows the presence of homogenous blue areas, round or oval yellowish structures, with occasional erythematous halos and cystic openings in the epidermis. We report this case to emphasize the role of dermoscopy in early diagnosis of eruptive vellus hair cyst which is a rare condition and can be missed easily. Dermoscopy, being a non-invasive and painless diagnostic tool, can prove to be a useful, particularly in children for differentiating EVHC from its mimickers, thus avoiding the need for biopsy.

Giant congenital melanocytic nevus (GCMN) is a deeply pigmented lesion, often associated with hypertrichosis and multiple scattered satellite lesions. During infancy apart from textural changes, rapidly growing “proliferative nodules” mimicking melanoma can occur in the nevus. Areas of the nevus may be infiltrated by an overgrowth of fat or nerve tissue, so-called lipomatous or neurotized areas, respectively, common in larger nevi. We report a case of GCMN associated with multiple neurotized areas mimicking large neurofibromas, a rare phenomenon.

Laryngo-onycho-cutaneous (LOC) first described by Shabbir in 1986 in Muslim families from Punjab is a rare autosomal recessive disorder characterized by increased skin and mucous membrane fragility associated with exuberant granulation. The 4^th International Consensus Meeting on “Diagnosis and Classification of Epidermolysis Bullosa” classifies it as a localized form of Junctional Epidermolysis Bullosa (JEB-LOC Syndrome) with mutations in α3 chain of Laminin 332. While laryngeal, ocular, and cutaneous involvement in these cases has been widely reported, urinary tract involvement is relatively rare. We report a case of JEB-LOC syndrome in a child from South India with extensive urethral involvement.

Granuloma annulare (GA) is a benign granulomatous dermatosis. Clinically, it has been classified into localized, generalized, subcutaneous, macular, erythematous, and perforating types. Generalized GA (GGA) is an unusual pattern of GA reported mostly in adults aged 50 years or over and children aged about 10 years. Occurrence in infants is very rare and few cases of papular GGA have been reported in infants. We report a case of annular pattern of GGA in a 3-month-old infant. This is the first case report of annular pattern GGA in an infant.

Primary lymphangioma circumscriptum (LC) is a superficial lymphatic malformation, presenting as multiple fluid-filled grouped vesicular lesions which may be associated with slight hemorrhages. We report a rare case of primary LC with exclusive involvement of area around the external urethral meatus in a 10-year-old male child. We treated the patient with radiofrequency ablation under topical anesthesia with eutectic mixture of local anesthetics (EMLA). No recurrence has been observed in the past 1 year. LC must be included in differentials when presented with vesicular lesions near the external urethral meatus in male pediatric patients.

Juvenile xanthogranuloma (JXG) is a rare non-Langerhans cell histiocytosis that usually occurs during infancy and early childhood. It is seen in combination with juvenile chronic myelomonocytic leukemia and/or neurofibromatosis type 1. The association with acute lymphoblastic leukemia (ALL) is rarely seen. We present a 2-year-old boy who had this rare combination and presented with multiple tan-orange-colored macules, papules, and nodules over the upper body and distal aspect of lower limbs for 4 months of age. The histologic evaluation and immunohistochemistry analysis resulted in the diagnosis of JXG. Bone marrow biopsy showed lymphoblastosis with marked suppression of erythroid and myeloid cells, suggesting ALL.

Cutis marmorata telangiectatica congenita (CMTC) is an uncommon, congenital, vascular malformation first described by van Lohuizen in 1922. CMTC is an infrequently reported congenital cutaneous disorder and is usually present at birth but can appear up to 3 months to 2 years of age. Hereby, we report a case of a 2-day-old infant with CMTC involving trunk with rarely reported congenital anomaly involving multiple system, i.e., musculoskeletal, cardiovascular, pulmonary, ophthalmic, excretory, oropharyngeal, and reproductive system.

Acute hemorrhagic edema of infancy is a disease marked by its dramatic presentation of purpuric plaques in a cockade or targetoid pattern. A 1-year-old boy was brought by his mother to our outpatient department with large purpuric plaques in annular and cockade pattern over the cheeks and bilateral lower limbs. Both the pinna showed purpura in a peculiar reticulate pattern, with vesiculation seen at the margin of the lesions along with mild edema. The skin biopsy showed cutaneous small-vessel vasculitis; direct immunofluorescence showed fibrinogen deposits around the small cutaneous vessels. The unusual features of our case were the reticulate pattern of purpura seen over the ears.

In this case series, five girls and two boys presented with purpuric-ecchymotic lesions that appeared around their lips. All patients were under the age of 15. While patients did not reveal any history of trauma at the beginning, they later accepted a history of sucking their lips inside their mouths or suctioning cups. In addition to negative routine laboratory tests including parvovirus in these patients, the lesions disappeared in several days. To the best of our knowledge, childhood suction purpura was examined for the first time in this case series.

Epidermolytic ichthyosis (EI) previously named bullous congenital ichthyosiform erythroderma of Brocq or epidermolytic hyperkeratosis (mostly considered as a histological term now) is rare with a variable defect of cornification, clinically characterized by generalized erythema, erosions, scaling, and easily breaking blisters that become less frequent later in life while hyperkeratosis increases. It is usually inherited in an autosomal-dominant fashion and is caused by the mutations in the genes KRT1 and KRT10, coding for the proteins keratin 1 and keratin 10, respectively. To date, only six recessive KRT10 mutations have been shown to cause autosomal-recessive EI, all of which have been found to lead to the complete absence of the keratin 10 protein. To the best of our knowledge, this is the first such case report from India.

Ectodermal dysplasia (ED) is used to define a group of inherited disorders characterized by the presence of abnormalities in two or more ectodermal derived structures, including hair, teeth, nails, or sweat glands. A 2-month-old boy presented with complaints of reduced scalp hair growth and loss of eyebrows since birth. He had a history of recurrent high fever and had been admitted twice for the same. Clinical examination of the patient revealed a fine, sparse, light-colored, short and dry hair over the scalp, with the absence of eyebrows, and eyelashes. The skin was smooth, dry with a prominent forehead and protuberant lips. Growth and developmental history were not significant. Trichoscopy showed absence or markedly reduced number of white dots. It corresponded to a finding of hypoplastic follicles with the absence of eccrine and sebaceous glands; these findings were consistent with histopathology. Dermoscopy is a noninvasive technique which aids in the clinical diagnosis of Christ-Siemens-Touraine syndrome by correlating clinical features histopathologically.

Subcutaneous phycomycosis is a chronic deep fungal infection caused by Basidiobolus ranarum, which usually occurs in children, predominantly affecting males. Herein, we report subcutaneous phycomycosis in a 3-year-old boy who presented with a gradually progressive painless swelling over the anterior abdomen of 20-day duration. Lesional biopsy confirmed the diagnosis of subcutaneous phycomycosis which revealed multiple well-defined granulomas composed of histiocytes, epithelioid cells, and multinucleate giant cells with broad nonseptate hyphae stained with Gomori methenamine silver stain. The child was managed with a saturated solution of potassium iodide which completely melted the swelling.

Nevus comedonicus (NC) is an uncommon condition which belongs to the spectrum of the epidermal nevi syndrome. Dermoscopy is a non-invasive tool which can help to rule out other differential diagnoses, making it especially valuable in pediatric cases. We present three children with NC and describe their dermoscopic findings.

There has been a rise in the incidence of dermatophytic infections in children in the last decade. Additionally, there has been a sea change in the clinical presentation, treatment practices and course of the condition seen in practice. This article summarises information on the available topical and oral antifungal agents in question and asnwer format for better understanding.