Hereditary sensory autonomic neuropathy (HSAN) is the term used to describe a group of inherited disorders that are associated with sensory dysfunction (reduced reflexes, altered pain and temperature perception) and varying degrees of autonomic dysfunction (gastro-esophageal reflux, postural hypotension, excessive sweating). Five different entities have been described under HSAN (types I-V) we report a case of HSAN IV in a one and a half year old female child, with the classical features of this disorder - insensitivity to pain, self mutilating behaviour, recurrent episodic fevers and anhidrosis.

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Urticaria is a common condition in children for which physicians are consulted. The management of childhood urticaria is similar as in adults; however, there are pediatric-specific features that must be taken into account for better management of childhood urticaria.

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Neonatal lupus erythematosus (NLE) is lupus erythematosus occurring in infants due to transplacental transmission of autoantibodies usually of anti SSA/RO and SSB/La antibodies. Like lupus erythematosus it presents with varied skin manifestations. Three cases of NLE with different presentations are presented. In all the three children, skin lesions were present since birth. All patients and mothers were positive for anti-SSA/Ro and anti-SSB/La antibodies. First case presented with few annular lesions second case with thrombocytopenia and purpura. Third case presented with hypopigmented atrophic patches on the face and upper trunk. Mothers of Cases 1 and 3 were asymptomatic, whereas the mother of Case 2 presented with purpuric lesions with mild arthritis during pregnancy, but was not suspected as connective tissue disorder. Echocardiography was normal in all the three cases indicating no cardiac involvement. Case 1 was lost to follow-up. Lesions resolved by 6-8 months in Cases 2 and 3 requiring only topical steroids. Case 3 received platelet transfusions and intravenous IgG for persisting thrombocytopenia.

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Child abuse is a major public health crisis and is on the rise. Dermatologists are frequently involved in its evaluation in differentiating abusive injuries from accidental injuries and in excluding pathological conditions that may mimic abuse. Munchausen syndrome by proxy (MSBP) is a rare form of child abuse where the parents or caretaker, intentionally causes an illness in the child for her own self-serving psychological needs. A 6-year-old orphan girl presented with spontaneous ulcers over bilateral lower limbs since last 6 months. The girl was investigated thoroughly in detail for possible causes of ulcers but nothing came out. Diagnosis of MSBP was firmly established in the case.

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Becker's nevus is an acquired and persistent asymmetrical area of skin pigmentation sometimes showing evidence of increased androgen sensitivity which typically occurs, postpuberty. Though the usual site is described as shoulder, anterior chest, or scapular region, it can occur over other unusual sites as well. We report such a rarer presentation of Becker's nevus on leg in a young female.

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Tay syndrome is a rare autosomal recessive disorder characterized by brittle hair and congenital ichthyosis. It is one of the syndromes of trichothiodystrophy - a group of DNA repair disorders with wide range of phenotypic expressions unified by the presence of sulfur-deficient brittle hair. Currently, prevalence of Tay syndrome is unknown. Approximately, only 75 reported cases are reported worldwide and 15 case reports published on this condition; there are no cases reported in the Philippines. Because the hair and skin are involved, dermatologists play an important role in both diagnosis and the multidisciplinary management of patients.

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Acro-dermato-ungual-lacrimal-tooth syndrome is characterized by ectrodactyly, syndactyly, nail dysplasia, hypoplasia of the breast and nipple, excessive freckling, lacrimal duct atresia, alopecia, primary hypodontia, and/or early loss of permanent teeth. It is a rare autosomal dominant disorder, which has been linked to mutation in the p63 gene. However unfortunately, the facilities to detect this mutation are not present in many centers. Five overlapping limb malformation syndromes have been described, including the ectrodactyly-ectodermal dysplasia-cleft syndrome .

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We report a case of a 6-year-old girl with typical anomalies of Goltz syndrome characterized by linear and reticulate atrophy of the skin, significant defects of the skeleton, dental structures, eyes, and soft tissues. No family member had such features. A brief overview of the syndrome is presented in an attempt to incorporate associated anomalies reported so far.

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Pachyonychia congenita (PC) is a rare genodermatosis characterized by hypertrophic nail changes and nail dystrophy. A 17-year-old male presented with thickened, yellowish brown discoloration of all the nails and multiple hyperkeratotic papules over his chest. Patient's younger sister also had similar nail and skin lesions since childhood. Presence of characteristic clinical features led to a diagnosis of a rare condition of PC type-1.

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Idiopathic eruptive macular pigmentation (IEMP) is an uncommon, rather under-reported disease entity of the pediatric age characterized by asymptomatic, brownish hyperpigmented macules involving the neck and trunk with no preceding inflammation or exposure to drug. Here we report this case to increase awareness of this entity among dermatologists, dermatopathologists and pediatricians. A 11-year-old healthy boy presented with brownish-gray to dark, discrete, asymptomatic macules on the face, trunk, neck and limbs of insidious onset. Histopathologic examination showed melanophages, mild perivascular lymphohistiocytic infiltrate in the papillary dermis and papillomatosis. The natural course of the disease is spontaneous remission without treatment, which was so even in our patient. IEMP is a benign entity with an excellent prognosis as it exhibits spontaneous resolution. It falls into the differential diagnoses of asymptomatic hyperpigmentatory disorders in pediatric population. Awareness of the entity leads to avoidance of unnecessary aggressive damaging treatment.

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Erythema multiforme is an acute, self-limited skin disorder that is considered to be a hypersensitivity reaction associated with certain infections, medications, and many other reasons. Parainfluenza virus infection may be an etiologic factor for this uncommon entity. Here we report a newborn with EM associated with parainfluenza virus (PIV) infection and a review of the literature on neonatal EM.

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Netherton syndrome (NS) is a rare disorder consisting of ichthyosis, hair shaft abnormalities and atopic dermatitis. It is inherited in an autosomal recessive pattern. Patients with NS have been found to have a mutation in a gene named serine protease inhibitor, Kazal type-5. Here, we report a female child with NS.

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Autoimmune lymphoproliferative syndrome (ALPS) is an inherited disorder characterized by chronic splenomegaly, lymphadenopaphy hypergammaglobulinemia and autoimmune phenomena especially immune-mediated cytopenias. The disease is characterized by presence of "double-negative (CD3 ⁺ CD4⁻ CD8⁻ )" T-cells in the peripheral blood and lymphoid tissue. Rarely it can also present as urticaria. We report a child of ALPS who presented to us with fever, generalized lymphadenopathy, chronic urticaria and hepatosplenomegaly.

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Annular erythemas in infancy are uncommon to rare in occurreance. Erythema migrans is the most common variety to be reported amidst the younger age group. This group of conditions poses a diagnostic challenge to the physician as the differentials can range from a benign annular erythema of infancy with good prognosis and no long-term defects to neonatal lupus erythematosus (NLE) having a bad prognosis with increased morbidity and mortality. We report a case of annular erythema of infancy in a 5 day old male infant to emphasize that in spite of the history of spontaneous abortions in the mother, which pointed towards a more grave diagnosis of NLE, benign conditions like annular erythema of infancy needs to be kept in mind.

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