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Davener’s dermatosis is a variant of frictional hypermelanosis characterized by midline hyperpigmented patches with indistinct borders, typically distributed along the skin overlying the bony protuberances of the lower thoracolumbar spine. Hereby, we report two children aged 10 and 12 years who presented with hyperpigmented skin lesions on the lower back. Both children had a history of exposure to prolonged friction at the affected site. The clinical diagnosis of Davener’s dermatosis was made, supported by dermoscopic findings. The parents were educated regarding the benign nature of the condition and the required changes to be made in postural habits and avoidance of friction.

Universal acquired melanosis, also referred to as Carbon baby syndrome, is a rare cause of progressive generalized hyperpigmentation of skin and mucosa. We describe a case of a 2-year-old boy with diffuse darkening of skin and mucosa for 3 months of age. Histopathology revealed increased melanin in the basal and suprabasal layers of the epidermis.

Piebaldism is a rare genetic disorder of congenital leukoderma caused by mutation in KIT proto-oncogene receptor tyrosine kinase. We present a 10-year-old boy with congenital depigmented macules suggestive of piebaldism associated with café au lait macules and skin fold freckling complicating the diagnosis. A diagnosis of piebaldism was made via exome sequencing that showed a pathogenic variant of KIT gene with no pathogenic variants of NF1 or SPRED1 gene. Our current understanding of the KIT tyrosine kinase function may provide a better explanation into this phenotypic coexistence and does not necessarily represent an overlap with Neurofibromatosis type 1.

Purpose: An algorithm for automated segmentation of meibomian glands from infrared images obtained using a novel prototype infrared hand-held imager has been proposed in this study. Meibomian gland dysfunction (MGD) is quantified in terms of five clinically relevant metrics. A comparison of these metrics in patients with MGD has been presented against a sample of the normative healthy population. Methods: This is a prospective cross-sectional observational study. Patients presenting to the clinics were enrolled after written informed consent. The everted eyelids of 200 eyes of patients (of which 100 were healthy and 100 were diagnosed with MGD) were imaged using a prototype hand-held camera. The proposed algorithm was used to process the images using enhancement techniques and the glands were automatically segmented. A comparison of glands of normal eyes versus MGD-affected eyes is performed using five metrics presented in this study: (i) drop-out, (ii) length, (iii) width, (iv) the number of glands, and (v) the number of tortuous glands. Results: The 95% confidence interval for the metrics did not show any overlap between the two groups. In MGD patients, the drop-out ratio was higher than normal. The length and number of glands were significantly lesser than normal. A number of tortuous glands were more in the MGD group. The metrics for MGD versus healthy and cut-off ranges were computed in the results. Conclusion: The prototype infrared hand-held meibographer and the proposed automatic algorithm for gland segmentation and quantification are effective aids in MGD diagnosis. We present a set of five metrics, which are clinically relevant for guiding clinicians in the diagnosis of MGD.

Hypereosinophilic syndrome is a myeloproliferative disorder characterized by abnormal accumulation of eosinophils in the blood or peripheral tissues. It is uncommonly seen in children. We describe a 14-year-old girl diagnosed with idiopathic hypereosinophilic syndrome presenting with recurrent, painful oral and genital ulcers, hepatosplenomegaly along with consistently high eosinophil count and leucocytosis. Genetic studies showed negative for FIPIL-PDGFRA fusion gene. Mucosal ulcers were recalcitrant to conventional therapy and responded well to thalidomide.

Insulin is the cornerstone of type 1 diabetes mellitus (T1DM) treatment. Insulin allergy in patients with T1DM on insulin is an uncommon problem that might manifest itself as immediate or delayed symptoms after injections. We present 2 cases: the first is a 17-year-old girl who was diagnosed with T1DM at the age of 14 and has had several skin lesions at injection sites over the past 2 months that have not responded to antibiotics. The second case involves a 4-year-old boy who was diagnosed with T1DM at the age of 15 months and had non-tender, erythematous, and indurated lesions. Insulin hypersensitivity was detected in both cases, thus newer analogues were administered instead of regular and isophane insulin, and the lesions dramatically reduced.

Psoriasis is a common, chronic, immune-mediated, multisystem, inflammatory disorder. It affects all age groups, including infancy. In one-third of the cases, the onset of the disease is in the first and second decades of life. Childhood psoriasis significantly affects the quality of life of the child as well as that of the entire family. Pediatric psoriasis has distinct clinical presentations and evolves with time. Like in adults, chronic plaque psoriasis has been found to be the most common type of childhood psoriasis. Psoriatic plaques in children are less pruritic, smaller and thinner with less prominent scaling. In pigmented skin, the erythema is less prominent and plaques appear violaceous or hyperpigmented. Pediatric psoriasis can be associated with arthritis, metabolic syndrome, depression and anxiety. Hence all children should be screened routinely for associated comorbidities. Management of pediatric psoriasis is challenging owing to the limitation of approved therapies. 'Proactive therapy' is a recent approach in childhood-onset psoriasis that would help to prevent the severity of flare-ups, thus improving the quality of life.

Objective: Short intracortical inhibition (SICI) is a GABAA-mediated phenomenon, argued to mediate selective muscle activation during coordinated motor activity. Markedly reduced SICI has been observed in the acute period following stroke and, based on findings in animal models, it has been posited this disinhibitory phenomenon may facilitate neural plasticity and contribute to early motor recovery. However, it remains unresolved whether SICI normalizes over time, as part of the natural course of stroke recovery. Whether intracortical inhibition contributes to motor recovery in chronic stroke also remains unclear. Notably, SICI is typically measured at rest, which may not fully reveal its role in motor control. Here we investigated SICI at rest and during voluntary motor activity to determine: (1) whether GABAA-mediated inhibition recovers, and (2) how GABAA-mediated inhibition is related to motor function, in the chronic phase post-stroke. Methods: We studied 16 chronic stroke survivors (age: 64.6 ± 9.3 years; chronicity: 74.3 ± 52.9 months) and 12 age-matched healthy controls. We used paired-pulse transcranial magnetic stimulation (TMS) to induce SICI during three conditions: rest, submaximal grip, and performance of box-and-blocks. Upper-extremity Fugl-Meyer Assessment and Box-and-Blocks tests were used to evaluate motor impairment in stroke survivors and manual dexterity in all participants, respectively. Results: At rest, SICI revealed no differences between ipsilesional and contralesional hemispheres of either cortical or subcortical stroke survivors, or healthy controls (P's > 0.05). During box-and-blocks, however, ipsilesional hemisphere SICI was significantly reduced (P = 0.025), especially following cortical stroke (P < 0.001). SICI in the ipsilesional hemisphere during box-and-blocks task was significantly related to paretic hand dexterity (r = 0.56, P = 0.039) and motor impairment (r = 0.56, P = 0.037). Conclusions: SICI during motor activity, but not rest, reveals persistent impairment in chronic stroke survivors indicating that inhibitory brain circuits responsible for motor coordination do not fully normalize as part of the natural history of stroke recovery. Observation that reduced SICI (i.e., disinhibition) is associated with greater motor impairment and worse dexterity in chronic hemiparetic individuals suggests the response considered to promote neuroplasticity and recovery in the acute phase could be maladaptive in the chronic phase post-stroke.

Pachydermoperiostosis is an inherited osseocutaneous disorder. The unusual increased levels of prostaglandin E2 due to mutations in either HPGD gene or SLCO2A1 gene are regarded as the causative factor. Our case report is about a 16-year-old boy who presented with cutis verticis gyrata, clubbing of digits, arthralgia, seborrhea, acne vulgaris, blepharoptosis, macrocheilia, column-like legs, and periosteal thickening in all long bones of the extremities characteristic of complete form of pachydermoperiostosis. No effective treatment has been proposed for this condition. Hence, counseling the child and parents about the disease progression, detecting the complications, and efficaciously managing them will be the mainstay of the treatment.

Entomophthoromycosis is a rare yet potentially life-threatening, chronic deep fungal infection affecting immunocompetent host, primarily children, and young adults. The infection commonly affects trauma-prone sites mainly extremities and trunk with regional lymph node involvement. We report a 1.5-year-old child with postcleft lip surgery presenting with painless swelling on the right side of the face for the past 4 months. Although culture showed no growth in our case, histopathological examination was diagnostic of entomophthoromycosis which aided the treatment. There was partial improvement in the size of the swelling with amphotericin B and a complete resolution with oral potassium iodide.

Infantile systemic hyalinosis (ISH; MIM #236490) and juvenile hyaline fibromatosis (MIM #228600) represent two spectrums of the rare autosomal recessive disorder, the hyaline fibromatosis syndrome caused by mutations in ANTXR2/CMG2 encoding capillary morphogenesis protein-2. Herein, we report two cases of ISH with different clinical presentations confirmed by CMG2 gene mutations. Homogenous 79 bp deletion of the entire exon 11 reported in one of the cases has not been reported previously.

The term woolly hair nevus was first described and coined in 1927 by Fred Wise. It is a nevoid condition characterized by a circumscribed patch of unruly and curled hair with an altered texture and may be associated with other nevoid conditions. This paper reports a case of a 7-year-old girl with woolly hair nevus overlying nevus sebaceous associated with linear pigmentation.

Atopic dermatitis (AD), the most burdensome skin condition worldwide, is influenced by climatic factors and air pollution; however, the impact of increasing climatic hazards on AD remains poorly characterized. Leveraging an existing framework for 10 climatic hazards related to greenhouse gas emissions, we identified 18 studies with evidence for an impact on AD through a systematic search. Most climatic hazards had evidence for aggravation of AD the impact ranged from direct effects like particulate matter‐induced AD exacerbations from wildfires to the potential for indirect effects like drought‐induced food insecurity and migration. We then created maps comparing the past, present, and future projected burden of climatic hazards to global AD prevalence data. Data are lacking, especially from those regions most likely to experience more climatic hazards. We highlight gaps important for future research: understanding the synergistic impacts of climatic hazards on AD, long‐term disease activity, the differential impact on vulnerable populations, and how basic mechanisms explain population‐level trends. [ABSTRACT FROM AUTHOR]

Introduction: Treatment of moderate to severe atopic dermatitis (AD) is a real challenge for the dermatologists. Dupilumab is the first targeted biologic therapy approved for the treatment of children and adults with moderate-to-severe AD. The efficacy and safety of dupilumab in Indian patients is limited to date, it is necessary to assess the performance of this treatment in real clinical practice in the Indian context. Methodology: Patients from three centers of India, two from Kolkata and one from Bangalore were included in the study for retrospective chart analysis. Efficacy was assessed by comparing the SCORAD and EASI and impact on quality of life was assessed by DLQI scores. All patients received standard doses of Dupilumab. Any side effect of the treatment was noted in the bi-weekly follow-up visit. Results: Twenty-five patients who were treated with dupilumab for at least 6 months were retrospectively included to study. The mean EASI score improved from 19.48 at baseline to 4.84 at six months. Seventeen patients (68%) achieved EASI 75 (≥75% improvement from baseline) at the end of 6 months of treatment. All these patients were earlier treated with at least one systemic immunomodulator without any significant improvement. The mean SCORAD score also improved with dupilumab treatment from 37.32 at baseline to 8.04 at six months. The improvement were found to be statistically significant (P < 0.001). The quality of life also improved significantly (P < 0.001) from a baseline mean of 17.08 at baseline to 6.52 at 6 months. Conclusions: We observed significant efficacy, tolerability, and safety of dupilumab in Indian patients with AD in a real-world setting, which was similar to that shown in clinical trials in the western populations.

Relation between dietary habits and pediatric dermatoses is well established in literature. Every dermatologist needs to have a thorough evidence-based knowledge of all the skin conditions that are affected by food either directly or indirectly, especially in today's world, where patients are looking for holistic solutions for all their ailments. Nutritional deficiencies, metabolic conditions, dermatitis herpetiformis, and urticaria are well-known entities related to food. Apart from these, a few skin conditions such as atopy, acne, and psoriasis have some correlation to food intake. A thorough knowledge of diet and its effect on dermatological conditions is important where alternative systems lay a lot of stress on the kinds of food that may be consumed in certain skin conditions. Parents should be counseled about the myths related to dietary modifications as this may lead to nutritional deficiencies in children.

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.

Tuberous sclerosis complex is a rare autosomal dominant neurocutaneous disorder characterized by the presence of multiple tumors influencing diverse body frameworks including the central nervous system, skin, eyes, heart, lungs, kidney, and bones. It is described by cutaneous changes, neurologic conditions, and the presence of hamartomas in numerous organs. The treatment of these patients requires a multidisciplinary approach. A port-wine stain is characterized as a telangiectatic macule that manifests during childbirth and stays all throughout life. They are associated with many syndromes. This article reports a rare case of co-occurrence of tuberous sclerosis with port-wine stain in a 2-year-old baby.

Acute febrile illness caused by multiple infectious etiologies is not uncommon, especially in endemic countries like India. Recently, there has been increased mortality with chikungunya infection in children. There are several studies of coinfection of chikungunya with dengue, but coinfection with rickettsial infection is rare. We describe a 14-year-old boy presenting with chikungunya with probable rickettsial coinfection with a fatal outcome.