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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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