Xeroderma pigmentosum (XP), is an autosomal recessive genetic disorder of enzymes in which the ability to repair damage caused to DNA by UV light is deficient. As this disorder is commonly due to exposure to sunlight and requires protection from sunlight such patients with the disease are often commonly referred to as “children of the night”. In this disorder, the patients suffer from various cutaneous malignancies such as basal cell carcinomas (BCC), malignant melanoma (MM), and squamous cell carcinoma (SCC). It is a rare disorder with an incidence of 1:250,000 in the United States. This disease involves both sexes and all races, with a common occurrence in Japanese people. The disease is characterized by a mutation in nucleotide excision repair (NER) enzymes resulting in a reduction or elimination of NER which is required for repair of UV light induced deoxyribonucleic acid damage thus increasing susceptibility to cutaneous malignancies. These patients rarely survive beyond 20 years of age. This case reports a rare occurrence of three malignancies in a single tumor BCC, SCC, and the rarely occurring sebaceous carcinoma, which is not known to be associated with UV radiation exposure.

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Chronic mucocutaneous candidiasis is a group of diseases in which patients have recurrent or persistent infections of the skin, nails or mucosae, caused by Candida, most commonly by Candida albicans. These patients may also develop noncandidal infections, mostly dermatophytoses and viral infections of the skin. Treatment of this condition is a dilemma as there is no sustained improvement on antifungals alone. This is attributable to the basic cause of the syndrome, which is a primary defect in the cellular immunity of skin. Three siblings, born out of a nonconsanguineous marriage, presented with persistent candidal infections since early childhood and had never been completely lesion-free. Two of them also had lesions of molluscum contagiosum and scarring reminiscent of herpes zoster infection. They were started on azole antifungal therapy, but they continued to develop recurrent skin and oral lesions over the next 3 months. Levamisole was then added as an immunomodulator and a sustained remission was observed over a follow-up period of 6 months. The addition of levamisole to the antifungal drugs led to a sustained clinical improvement which was maintained over the next 6 months. This suggests immunomodulators like levamisole could be a useful adjunct in such patients and their role needs to be investigated in larger trials.

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The hereditary sensory and autonomic neuropathies encompass a number of inherited disorders that are associated with sensory dysfunction and varying degree of autonomic dysfunction. Hereditary sensory and autonomic neuropathy (HSAN) with anhidrosis type IV is the second most common form of hereditary sensory neuropathy. We report here the youngest case diagnosed with HSAN type IV having all features of congenital insensitivity to pain and anhidrosis.

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Introduction: Cutaneous infection forms an important component of all dermatological practice including children. The infection will reflect the status of health, hygiene, personal cleanliness of the community, improved health awareness and with proper cleanliness the chance of getting infection is less. Aim: This study was undertaken to know, which type of infection is common, particularly in pediatric age group and factors contributing the changing trends. Materials and Methods: After obtaining ethical committee clearance and informed consent from the patients, 100 cases below the age of 18 years, attending the outpatient Department of Dermatology were taken for the study. Results: In this study, out of 100 patients, the most common infection was fungal (57%), followed by viral (23%) and bacterial (20%) infection. Conclusion: The study will provide data for future clinical research and helps in knowing changing trend of cutaneous infections in children.

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Pigmented xerodermoid is a variant of xeroderma pigmentosum (XP-V). It occurs at a later age with the onset in the third or fourth decade of life in comparison to XP which mostly becomes manifest within 1–2 years after birth. It is more common in regions where consanguinity and marriages between close relatives are practiced and also in the tropics where there is greater exposure to sunlight and ultraviolet radiation. XP has seven complementation groups namely A-G and an XP-V. Recently, the XP-V is considered to be the same as pigmented xerodermoid.

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Erythromelanosis follicularis faciei et colli (EFFC), is a very uncommon disease characterized by a triad of hyperpigmentation, erythema and follicular papules on face and neck. There are reports of its association with keratosis pilaris (KP), but coexistent EFFC, and generalized KP has rarely been reported. In this article, the author reports a 12-year-old boy with concomitant EFFC and KP in a generalized distribution. It is the rarity of generalized KP in association with EFFC which prompted the author to report this case.

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Cutaneous polyarteritis nodosa (CPN) is a benign form of vasculitis of small and medium sized arteries without systemic involvement. Moreover, this entity is very rare in children. It usually manifest, as subcutaneous tender nodules, livedo reticularis, and ulcerations as predominant features while myalgia, arthralgia, peripheral neuropathy, and general symptoms such as malaise and fever may also be present in many cases. Here we report a case of a 12-year-old boy with erythema of distal phalanges and gangrene of one fingertip on the left hand as unusual initial manifestation of CPN.

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Kawasaki disease (KD), also known as the mucocutaneous lymph node syndrome is a diffuse vasculitis of unknown etiology affecting children with a characteristic clinical presentation. It is a disease with a unique clinical presentation. Knowledge about this established disease is required as it can involve the coronary and other medium and small-sized arteries causing vasculitis in about 20% of cases, which can be the cause of ischemic heart disease and sudden deaths in third and fourth decade of life. It is a condition diagnosed entirely on a clinical basis and hence the need of awareness particularly in pediatricians and dermatologists is the need of time, as no of cases are on the rise. We hereby report two cases of KD diagnosed on the basis of history and clinical findings.

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Severe purpuric rash due to photosensitivity is rare and is reported to occur in cases of porphyria or transiently raised porphyrin levels. We are presenting a case of severe purpuric rash in a preterm neonate, which developed after intensive phototherapy and exchange transfusion, and improved gradually by itself, possible cause being transient porphyrinemia.

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Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder characterized by capillary malformation, vascular anomalies, and bony and soft tissue hypertrophy. Angiokeratomas are of several types. Angiokeratoma circumscriptum (AC) is a nevoid form which presents as dark red papules and plaques mostly with a verrucous surface. AC is itself very rare. KTS along with AC is even rarer. Here, we present a rare case of KTS with AC on the background of port-wine stain.

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Iso-Kikuchi syndrome (IKS) or congenital onychodysplasia of the index fingers (COIF) is a rare clinical entity characterized by nail dysplasia’s involving the index fingers. Nail changes may include micronychia, polyonychia, anonychia, malalignment or irregular lunula. We report a newborn of IKS associated with blue (azure) lunula of the finger nails and brachydactyly of all the toes of the right foot.

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Goltz syndrome is a rare genetic X-linked dominant condition in which ectodermal and mesodermal structures – primarily skin, bones, teeth and eyes – are affected in a mosaic pattern. The skin, being the most accessible organ, allows better visualization of this mosaicism. Skin lesions follow the Blaschko’s line and consist of dermal atrophy, telangiectasia, hypopigmentation or hyperpigmentation. Mutation in the PORCN gene is said to cause these defects. A 12-year-old female born with hyperpigmented plaques with areas of atrophy and deformities of the digits (syndactyly and claw-like deformities) consulted due to masses in pharyngeal wall. She also had particular facies, dental anomalies, short stature and umbilical hernia. Skin biopsy revealed hypoplastic dermis with loose collagen bundles and lipocytes in nests insinuating in between the dermis.

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Waardenburg syndrome is a rare disorder characterized by sensorineural deafness with pigmentary anomalies and defects of the neural crest cells transmitted in an autosomal dominant fashion. Depending on the deformities caused by the disease some patients may need a multi-disciplinary approach while some may not need any treatment. Based on associated characteristics found and variable mutations there are four types of the syndrome. Here, we report a case of an 11-year-old male child who was found to have this disorder when a school survey was conducted at the school for deaf and dumb. It was also noted that there was generalized follicular hyperkeratosis, which could be a co-incidental finding.

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Delleman–Oorthuys syndrome or oculocerebrocutaneous syndrome (OCCS) is a rare genetic disorder characterized by eye abnormalities, skin abnormalities, areas of alopecia in combination with hydrocephalus. Until date, only 28 patients with equivocal diagnosis of OCCS have been reported. All of which are sporadic, with no risk of recurrence in the siblings. No etiological cause has been found as yet. It may also show overlapping clinical features with other syndromes like Goldenhar syndrome, encephalo-cranio-cutaneous lipomatosis and Goltz syndrome. We present a case of a 9-month-old male child who presented with unilateral temporo parietal aloepecia, multiple cystic lesions on the upper eyelid and adjacent periorbital areas, as well as an upper eyelid coloboma and epibulbar dermoid, all of which were present since birth.

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Sweet’s syndrome (SS) is characterized by a constellation of clinical symptoms and physical findings, which include fever, blood, and tissue neutrophilia, leading to the development of tender, erythematous plaques, histopathologically characterized by the presence of abundant mature neutrophils. Here, we present two cases of SS. First, a 4-year-old girl, who presented with fever, features of upper respiratory infection, abdominal pain, and multiple skin lesions of 10 days duration. She had similar illness 6 months ago. Second, an 8-month-old male baby who presented with fever and multiple skin lesions of 20 days duration. On examination, both children had papules and plaques with elevated borders all over the body. Laboratory investigations revealed raised inflammatory markers with neutrophilic predominance. Skin biopsy of the lesions showed dense neutrophilic infiltration of the dermis. In view of recurrent SS of the first case, underlying malignancy was ruled out. All these fit into the diagnostic criteria of SS classical type probably para-infectious etiology. These case reports bring to light that SS should be borne in mind when dealing with children with fever of unknown etiology with tender plaques, blood, and tissue neutrophilia.

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Nicolaides–Baraitser is a very rare clinical condition characterized by short stature, mental retardation, absent or limited speech, seizures, sparse hair, peculiar facial appearance, short metacarpals, and interphalangeal swelling. In this paper, we report a case of a 5-year-old female child belonging to the ethnic Kashmiri population with clinical features suggestive of Nicolaides–Baraitser syndrome. Clinical rarity of this condition prompted this communication.

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Effective treatment and management of any dermatoses is dependent not just on simple prescription of medicines to a patient. Adequate education and counseling are equally important when it comes to the holistic management of a disease. It improves patient compliance as well as satisfaction with any treatment. It also improves the doctor-patient (parent) relationship. Patient (parent) education and counseling is even more important when it comes to pediatric dermatology because here patients are young children who can neither verbalize their discomfort nor adhere to the treatment prescribed and are often completely dependent on their parents (caregivers). Thus, adequate counseling and education becomes imperative for the successful outcome of any management protocol.

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The rapidly adapting neonatal skin, being anatomically and physiologically distinct from that of an adult, may exhibit a variety of entities ranging from mild and/or self-limiting ones to the severe and/or life-threatening. However, their occurrence makes the parents/caregivers disconsolate. We carried out an exhaustive literature search using Medline, Embase, EBSCO and Cochrane library in order to review and incorporate recent knowledge in the salient features of these entities, so as to facilitate their rapid screening by the busy clinicians. However, neither a comprehensive classification nor adequate information at a single source was available but was scattered sketchily among the description of adult disorders in the common dermatology textbooks.

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