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Objective To characterize the clinical features, response to therapy, evolution and prognosis of cutaneous mastocytosis in children. Background Mastocytosis in children, instead of being induced by a potentially oncogenic c-kit mutation is probably a clonal disease with benign prognosis. Methods The clinicopathological features, evolution and response to treatment were analysed in 71 children with mastocytosis. Results There were 53 (75%) cases of urticaria pigmentosa, 12 (17%) cases of mastocytoma, and six (8%) cases of diffuse cutaneous mastocytosis. In 92% of cases disease onset was in the first year of life. There was a male predominance 1.8:1. Treatment did not modify the disease evolution. Eighty per cent of patients improved or had spontaneous resolution of the disease. Conclusion The most frequent clinical form of mastocytosis was urticaria pigmentosa followed by mastocytoma and diffuse cutaneous mastocytosis. Darier's sign was present in 94% of cases. A negative Darier's sign does not rule out mastocytosis. In contrast to adults, mastocytosis in children usually has a benign course making sophisticated or invasive diagnostic tests unnecessary. A classification of paediatric cutaneous mastocytosis is proposed. [ABSTRACT FROM AUTHOR]

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Abstract A case of circle hairs is reported and the literature on the subject reviewed. Differential diagnoses are discussed as well as some theories on the nature of circle hairs. [ABSTRACT FROM AUTHOR]

Copyright of International Journal of Dermatology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

We describe a patient with universal alopecia of 8 years duration, and an alopecia-resistant hairy pigmented congenital naevocellular naevus on the scalp. [ABSTRACT FROM AUTHOR]

Background/objectives: Silvery hair syndrome is a rare, autosomal-recessive entity characterized by silvery gray hair, eyebrows, and eyelashes and may be associated or not with immunologic or neurologic alterations. Two main types have been recognized: Chediak-Higashi syndrome and Griscelli syndrome. Hair shaft examination under light microscopy has been a useful tool to differentiate Chediak-Higashi syndrome from Griscelli syndrome, although distribution of melanin varies according to hair color related to ethnicity. The objective was to compare the pattern of melanin in the skin and with the pattern of melanin distribution in the hair shaft., Methods: Sixteen patients with silvery hair syndrome were selected (Chediak-Higashi syndrome 5, Griscelli syndrome 11). The distribution of melanin granules in skin and hair shafts was compared and correlated with clinical diagnoses., Results: Chediak-Higashi syndrome was characterized by small granules of melanin uniformly distributed throughout the thickness of the epidermis. Griscelli syndrome was characterized by an irregular pigment distribution in the epidermal basal layer with large and dense granules alternating with areas lacking melanin pigment. In two cases, study of the hair was not conclusive, but the skin showed the characteristic pattern of Griscelli syndrome., Conclusion: Skin biopsy is a useful tool in differentiating Chediak-Higashi syndrome from Griscelli syndrome and as a complementary study in cases in which hair shaft pigment distribution does not support the diagnosis, especially in patients with fair hair. The distribution of melanin granules in the skin correlates with that observed in the hair shaft, allowing Chediak-Higashi syndrome to be differentiated from Griscelli syndrome, at any age., (© 2018 Wiley Periodicals, Inc.)

Cutaneous rhabdomyomatous mesenchymal hamartoma (RMH) is a rare benign tumor composed of two or more types of mesenchymal-derived cells. RMHs are generally sporadic and independent, but they can be associated with congenital abnormalities. We report a subcutaneous case of RMH in the sternoclavicular area with two recurrences after complete surgical excision. The course is variable and can range from spontaneous resolution to repeated recurrences., (© 2016 Wiley Periodicals, Inc.)

Dermoid cysts (DCs) are benign cutaneous tumors that tend to persist and grow. The aim of this study was to examine the clinicopathologic features of congenital DCs. We present a case series of 75 children with a clinicopathologic diagnosis of DC. Seventy-two cysts were located on the head, one on the neck, and two on the trunk. Six cysts were located along the midline. Eight patients had symptoms other than changes in cyst size. Imaging studies were performed on 15 patients. Surgical excision was the primary treatment in all 75 cases. Neurosurgery and ophthalmology services were involved in the care of some patients. Histopathologic studies reported a foreign body giant cell reaction in 17 of the cysts. No recurrence was documented. DCs can remain stable for years, but they can become symptomatic as a result of enlargement and rupture or, more rarely, as a result of extension into surrounding tissues. Physicians should be aware that certain locations have a higher risk of DC extension, and adequate diagnostic investigations should be performed before their complete resection., (© 2013 Wiley Periodicals, Inc.)

Hydroa vacciniforme-like lymphoma (HVLL) is an Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disorder of childhood that occurs mainly in Central and South America and Asia. We present the clinicopathological features of 20 Mexican children with HVLL with a median age of 8 years at diagnosis (range, 1-15). All patients presented with skin lesions involving sun-exposed areas, but not exclusively. Fever, lymphadenopathy, and hepatosplenomegaly were often observed. Most patients were treated with immunomodulators and/or immunosuppressive agents, resulting in temporary remission. For 13 patients follow-up was available for a median of 3 years (range, 1 month-13 years). Three patients with long follow-up (9-13 years) are alive with disease. Four patients died, 2 after developing systemic lymphoma. Histologically, the skin showed a predominantly angiocentric and periadnexal Epstein-Barr early RNA+ lymphoid infiltrate with variable atypia and subcutaneous involvement. Fifteen patients showed a T-cell phenotype (12, αβ; 2, γδ; 1, silent phenotype) and monoclonal T-cell receptor-γ rearrangements, whereas 6 exhibited a natural killer (NK)-cell phenotype. Four patients had hypersensitivity to mosquito bites. One patient showed both phenotypes. HVLL is an EBV-associated lymphoproliferative disorder of αβ-, γδ-, or NK-cell phenotype with a broad clinical spectrum, usually prolonged clinical course, and risk for progression to systemic disease.

The common manifestations of atopic dermatitis (AD) appear sequentially with involvement of the cheeks in infancy, flexural extremities in childhood, and hands in adulthood. Although less common clinical manifestations are well described, they have not been the subject of epidemiologic studies to describe their prevalence in specific age groups. This observational, cross-sectional, comparative study included 131 children younger than 18 of both sexes with AD who attended the clinics of the Dermatology Department of the National Institute of Pediatrics in Mexico City. Patients were examined to determine the presence of infrequent clinical manifestations of AD during infancy, preschool and school age, and adolescence and stratified according to sex, age, and number of clinical signs. A chi-square test was used to detect differences according to age and sex. Logistic regression analysis was also performed. The main findings according to age were genital dermatitis and papular-lichenoid dermatitis variant in infants; atopic feet, prurigo-like, nummular pattern, and erythroderma in preschool and school-aged children; and eyelid eczema and nipple dermatitis in adolescents. The risk of development of nipple dermatitis and eyelid eczema increased with age, and the development of genital dermatitis decreased with age. The knowledge of the prevalence of less common clinical manifestations of AD according to age in different populations might be helpful in diagnosing incipient cases of AD., (© 2012 Wiley Periodicals, Inc.)

Background. Skin diseases among pediatric patients differ from those in adults. Epidemiological studies are scarce, and those performed in Mexican population date back thirty years. It is likely that these diseases might have changed their frequency. Material and Methods. Retrospective study in first-time patients referred to a pediatric dermatology service between January 1994 and December 2003. Demographics and diagnosis were recorded and compared with the results of a previous study performed in the same institution. Results. We included 5250 patients (52.55% female, 47.47% male) with 6029 diagnoses. The most frequent dermatoses found were atopic dermatitis (14.59%), viral warts (6.62%), acne (5.53%), pityriasis alba (3.98%), melanocytic nevi (3.85%), xerosis (3.57%), keratosis pilaris (3.19%), seborrheic dermatitis (2.37%), hemangioma (2.26%), and papular urticaria (2.24%). Most dermatoses increased their frequency when compared to the previous study. Conclusion. The frequency of pediatric dermatoses in our institution has changed in the last two decades. Environmental and sociocultural factors and institutional policies might account for these results.

Congenital cutaneous angioleiomyoma is an extremely rare benign smooth muscle tumor. We present a case of a firm, painful subcutaneous mass noticed at birth on the left leg that on surgical excision proved to be an angioleiomyoma. Prognosis is good, and recurrences are uncommon. To our knowledge, this is the second report of a congenital angioleiomyoma., (© 2011 Wiley Periodicals, Inc.)

Depigmented nevi, pityriasis alba, and postinflammatory hypopigmentation are the most frequent hypomelanotic conditions in newborns and infants. These, and examples of less frequent hypopigmentations are briefly discussed in this article. A new classification for depigmented nevi is also proposed.

Immunohistochemical staining is useful in the diagnosis of bone marrow infiltration in systemic mastocytosis. However, it is not clear if antibody staining may be helpful in the diagnosis of cutaneous mastocytosis (CM). We studied the histological appearance of CM in 35 pediatric patients. Cases were assigned to three basic clinical groups: I--Urticaria pigmentosa (UP, n=29); II--Mastocytomas (n=4); and III--Diffuse Cutaneous Mastocytosis (DCM, n=2). The analysis of clinical information revealed an association between the presence of diarrhea and a higher number of cells/field. Nine doubtful cases, all of them macules, were selected based on the scarcity of mast cells (MC) and the absence or rarity of other inflammatory cells. We compared the number of cells identified in Giemsa and immunohistochemical stains in definite and doubtful cases. The intraclass correlation statistic tested the concordance between each staining method. All 9 dubious cases according to the Giemsa stain had their CM diagnosis confirmed by the immunohistochemistry analysis. The intraclass correlation between Giemsa and c-kit was good (0.7) when the number of MC was high. However, there was no correlation between the mast cells counts in the two different stains in the dubious cases. The immunohistochemistry with c-kit might make CM diagnosis easier, especially in the macular cases, when there is a lower number of MC.

Keratosis lichenoides chronica (KLC) is a rare acquired disease of adulthood, of unknown etiology, characterized by keratotic parallel linear lesions, retiform plaques, and keratotic, often follicular papules, chronicity and lichenoid histopathologic features. KLC of pediatric onset is considered extremely rare. Its features and relationship to adult onset KLC are unknown. We studied 8 cases of pediatric-onset KLC in the literature and 6 personal cases and compared them with 40 reported adult-onset KLC patients. The following features characterize pediatric-onset KLC: familial occurrence; probable autosomal recessive inheritance; early or congenital onset with facial erythemato-purpuric macules; forehead, eyebrow, and eyelash alopecia; pruritus; and a low frequency of other cutaneous and systemic abnormalities. Pediatric-onset KLC may represent a different disease or a subset of adult-onset KLC, with special genetic and clinical characteristics. Determining its precise nosology will have prognostic and therapeutic implications.

Dermal dendrocyte hamartomas are extremely rare; only two examples have been described with clinical features different from our cases and with incomplete immunohistochemical characterization. We report three female patients presenting a medallion-shaped, well-defined, slightly atrophic and asymptomatic congenital lesion. All 3 patients showed a fusiform-cell proliferation. Immunohistochemistry was positive for CD34, factor XIIIa, and fascin. Electron microscopy showed typical features of dermal dendrocytes. We believe that the lesions described represent a new, clinically and histopathologically distinct lesion originating in dermal dendrocytes. We propose to name it medallion-like dermal dendrocyte hamartoma.

Of the patients with epidermal nevi, 10-18% may have disorders of the eye, nervous, and musculoskeletal systems. A predisposition to malignant neoplasms in ectodermal and mesodermal structures may also be found. There are six different epidermal nevus syndromes described so far: Proteus, congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome, phakomatosis pigmentokeratotica, sebaceous nevus, Becker nevus, and nevus comedonicus. Thirty-five patients with epidermal nevus syndrome seen at the National Institute of Pediatrics in Mexico City during a 31-year period are described. This syndrome represented 7.9% of 443 patients with epidermal nevi; its relative frequency was 1 case per 11,928 pediatric patients and 1 case per 1080 dermatologic patients. Nine epidermal nevus syndrome patients (26%) had Proteus syndrome. Sebaceous nevus syndrome was found in six patients (17%), while the nevus comedonicus syndrome was found in three (8%). Two patients were diagnosed with phakomatosis pigmentokeratotica and one patient with congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome. This is the first report of phakomatosis pigmentokeratotica and congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome in Mexican patients. One patient had an inflammatory linear verrucous epidermal nevus with systemic involvement. Thirteen patients (37%) had keratinocytic nevi with systemic involvement. We propose the keratinocytic nevus syndrome to be defined as the association of a keratinocytic nevus with neuronal migration and/or musculoskeletal disorders in addition to a higher risk for mesodermal neoplasms.

Dermatitis artefacta is a factitious disorder in which there is deliberate conscious production of skin lesions. There are only a few reports that evaluate instances of dermatitis artefacta in the pediatric population. The aim of this retrospective study was to assess the characteristics of patients with this disorder who were seen at the National Institute of Pediatrics in Mexico City. The records of all patients diagnosed with dermatitis artefacta from January 1980 to December 1999 were analyzed. There were 29 patients (25 females, 4 males). The upper limbs and the face were the most commonly involved areas. Superficial erosions were the most frequent initial event, and residual lesions consisted of scars and crusts. Time taken to diagnosis was on average 10 months. Half of the patients were lost to follow-up. No correlation was found between the length of time from the disease onset to diagnosis, the type of lesions, and the clinical outcome. Twelve patients had an associated systemic disorder. The possible association with chronic disease has not been sufficiently stressed and demonstrates the importance of providing psychological support for these patients. Psychiatric diagnoses were anxiety, depression, and personality disorder. No correlation was found between the psychiatric diagnosis and the outcome of dermatitis artefacta. A young age at presentation, which has been considered important as a favorable prognostic sign, could not be demonstrated in our patients.

Nearly 200 cases of phakomatosis pigmentovascularis (PPV) have been reported worldwide, most of them of Japanese origin. There are 5 types and 10 subtypes of PPV. Its etiology might be explained by the twin spotting phenomenon. The relative frequency of PPV at the National Institute of Pediatrics was 5.8 per 100,000 pediatric patients and 0.634 per 100,000 dermatological patients. We report 24 cases of PPV with an average follow up of 5 years and the following findings: PPV type II A in 4 male and 2 female patients with melanosis bulbi in 3 and glaucoma in 1. PPV type II B in 7 male and 11 female patients, with melanosis bulbi in 9, glaucoma in 9, iris mammillations in 2, Sturge Weber syndrome in 6 female patients, and Klippel-Trenaunay syndrome in 2 males, hemifacial, hemicorporal, or limb hypertrophy without venous insufficiency in 6 female and 4 male patients. During the follow-up time of 60 months, progressive fading of melanotic and vascular macules were observed in 7 patients. No other types of PPV were found. Systemic involvement in PPV was related to the body surface area affected by the vascular macules. Ectodermal and mesodermal migration disorders might be involved in the pathogenesis of PPV.

Among 27 pediatric patients with a clinicopathologic diagnosis of discoid lupus erythematosus (DLE), 15 had localized cutaneous lesions and 12 had disseminated lesions. During a mean follow-up period of 36 months, seven patients (26%) developed systemic lupus erythematosus (SLE). Four of these patients were less than 10 years of age. No correlation was found between localized and disseminated lesions and evolution to SLE. Three of four patients with a positive family history for rheumatoid disease developed SLE (p < 0.05). Hyperpigmentation was significantly more frequent (p < 0.04) in children less than 10 years of age. There was a female predominance of 5:1 among patients less than 10 years of age. Our findings suggest that onset of DLE prior to 10 years of age does not indicate a greater risk of developing SLE. The occurrence of localized or disseminated lesions does not seem to influence the outcome.

An infant with self-healing, multifocal cutaneous infantile myofibromatosis with leg-length discrepancy as a sequela is reported. This condition should be suspected in infants with one or more firm or hard nodules in the skin, subcutaneous tissue, bone, muscle, or viscera. The histopathologic picture is diagnostic. Treatment and prognosis depend on the extension and location of the tumors.

Due to the presence of two different clones of cells in early embryogenesis, numerous congenital and acquired dermatoses have a linear distribution following the lines of Blaschko. Acquired inflammatory skin diseases are rarely observed in linear patterns. Our patient was born with macrocephaly, left eye glaucoma, and a left facial and contralateral corporal hemihypertrophy, cerebral dysgenesis, and skeletal abnormalities. Hypopigmented S-shaped linear macules on the trunk and linear streaks on the arms and legs were compatible with hypomelanosis of Ito. At 5 years of age the patient presented with an erythematous follicular exanthem compatible with scarlet fever exclusively in the lines of Blaschko. This fact suggests a genetic mosaicism.

Background: Necrotizing fasciitis (NF) is a severe, life-threatening soft tissue infection. General features and risk factors for fatal outcome in children are not well known., Objective: To characterize the features of NF in children and the risk factors for fatal outcome., Design: Retrospective, comparative, observational, and longitudinal trial., Setting: Dermatology department of a tertiary care pediatric hospital., Patients: All patients with clinical and/or histopathological diagnosis of NF seen from January 1, 1971, through December 31, 2000., Main Outcome Variables: Incidence, age, sex, number and location of lesions, preexisting conditions, initiating factors, clinical and laboratory features, diagnosis at admission, treatment, evolution, sequelae, and risk factors for fatal outcome., Results: We examined 39 patients with NF (0.018% of all hospitalized patients). Twenty-one patients (54%) were boys. Mean age was 4.4 years. Single lesions were seen in 30 (77%) of patients, with 21(54%) in extremities. The most frequent preexisting condition was malnutrition in 14 patients (36%). The most frequent initiating factor was varicella in 13 patients (33%). Diagnosis of NF at admission was made in 11 patients (28%). Bacterial isolations in 24 patients (62%) were polymicrobial in 17 (71%). Pseudomonas aeruginosa was the most frequently isolated bacteria; gram-negative isolates, the most frequently associated bacteria. Complications were present in 33 patients (85%), mortality in 7 (18%), and sequelae in 29 (91%) of 32 surviving patients. The significant risk factor related to a fatal outcome was immunosuppression., Conclusions: Necrotizing fasciitis in children is frequently misdiagnosed, and several features differ from those of NF in adults. Immunosuppression was the main factor related to death. Early surgical debridement and antibiotics were the most important therapeutic measures.

Papular urticaria (PU) is among the commonest skin ailments in children. Induced specific desensitization to insect bites is theoretically an effective means of prevention of PU. In this double blind placebo controlled study, an oral vaccine prepared from insect saliva was compared with placebo (stable vaccine solvent). Vaccine and placebo effectiveness were tested by counting active PU lesions, serum eosinophils, and IgE, before and after 4 months of treatment. Statistically significant differences between oral vaccine and placebo were not found in the clinical or the immunological variables tested. We conclude that, although a lack of oral vaccine efficacy was suspected, larger study samples are needed to strengthen our conclusion.

Background: The relationship between infantile seborrheic dermatitis (ISD) and infantile atopic dermatitis (IAD) is controversial., Methods: Ninety-six children aged 2-12 months diagnosed with atopic dermatitis and a comparable control group of healthy children were evaluated. Demographic data, personal history of ISD and personal or family history of atopy was considered in both groups., Results: A personal history of ISD was found in 49% of IAD cases and in 17% of controls (P < 0.05)., Conclusions: Our result and those of the literature do not demonstrate a relationship between ISD and IAD. However, a number of cases of AD have an ISD-like clinical picture. It is probable that ISD is a syndrome and not a disease.

Background: Exanthems in children often represent a diagnostic challenge., Purpose: To determine the concordance between pediatric and dermatological diagnoses of exanthems., Design: Prospective study., Procedure: Exanthems that appeared in hospitalized pediatric patients were diagnosed by pediatricians. Pediatric dermatologists, by consensus, either confirmed or modified the diagnoses. Whenever possible, laboratory tests were used to confirm the final clinical diagnoses. Age and evolution were compared with the Kruskal-Wallis test; the kappa coefficient was used to determine concordance., Setting: Institutional tertiary referral pediatric care center., Results: Concordance between pediatric and dermatological diagnoses was found in only 44 patients. When pediatric and dermatological diagnoses of exanthems were classified, the concordance between both diagnoses was very low (kappa = 0.165)., Conclusions: Pediatric specialists, other than dermatologists, failed to diagnose common skin diseases. This may be a consequence of insufficient training in dermatology by medical schools and pediatric residency., (Copyright 2002 S. Karger AG, Basel)

A pediatric case of relapsing polychondritis is reported. Diagnostic features were recurrent episodes of painful inflammation of ear cartilage, episcleritis, and atopic disease. A good therapeutic response was obtained with systemic corticosteroids, dapsone, and methotrexate.