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Vitiligo is a chronic pigmentary condition and severely impacts patient quality of life (QoL). It is an underrecognized burden for patients, healthcare systems, and society in Latin America (LA). This paper examines the journey of a vitiligo patient in LA and assesses the disease landscape. Americas Health Foundation (AHF) assembled a panel of six Argentine, Brazilian, Colombian, and Mexican vitiligo experts. On 10-12 May 2022, they met in a virtual meeting. Each panelist wrote a short paper on barriers to vitiligo diagnosis and treatment in LA before the meeting. AHF staff moderated as the panel reviewed and modified each paper over three days. The panel approved the recommendations based on research, professional opinion, and personal experience. The panel agreed that lack of disease awareness and research, social ostracization, and limited therapeutic options hinder patients in their quest for diagnosis and treatment. In addition to the medical and psychological difficulties associated with vitiligo, problems connected to the Latin American healthcare system may negatively impact diagnosis, prognosis, and treatment. Access to timely diagnosis and treatment is crucial for improving outcomes. Governments, medical societies, academics, patient organizations, industry, and the public must unite to eliminate these challenges.

Background Kawasaki disease (KD) is an acute systemic vasculitis that predominantly affects patients younger than 5 years. In the absence of an available, affordable diagnostic test, detailed clinical history and physical examination are still fundamental to make a diagnosis. Methods We present five representative cases with KD-like presentations: systemic onset juvenile idiopathic arthritis, mycoplasma-induced rash and mucositis, staphylococcal scalded skin syndrome, BCGosis, and the recently described multisystemic inflammatory syndrome in children (MIS-C) associated with the severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) virus. Results Rash, fever, and laboratory markers of inflammation can be present in several childhood diseases that may mimic KD. Conclusion The term 'Kawasaki syndrome' instead of 'Kawasaki disease' may be more appropriate. Physicians should consider an alternative diagnosis that may mimic KD, particularly considering MIS-C during the present pandemic, as an aggressive diagnostic and therapeutic approach is needed.

Introduction Hypohidrotic ectodermal dysplasia (HED) is a genetic condition typified by alterations in skin structures including sweat glands, hair, nails, and teeth. Hair findings in HED have been poorly characterized in larger series. Objective To characterize scalp and hair findings of patients with HED clinically and with trichoscopy and light microscopy. Methods A cross-sectional study in 21 pediatric HED patients was performed using available clinical and scalp dermatoscopic images, as well as pulled-hair samples for clinical evaluation, trichoscopic, and light microscopic analyses. Results Seventeen out of 21 patients (81%) were men. Twenty patients had straight hair. Sixteen patients had decreased hair density, 6 of whom had hair loss mainly in the temporal and occipital regions. Fourteen patients had hair whorls. On trichoscopy, we observed: single-hair follicular units (n = 19, 90%), scalp hyperpigmentation (n = 13, 62%), variable diameter of the hair shafts (n = 12, 57%), perifollicular scales (n = 8, 38%), scalp erythema (n = 8, 38%), and short curly pigtail hairs (n = 6, 29%). On light microscopy, findings included: hair shafts with irregular diameter (n = 7, 33%), heterogeneous hair color (n = 6, 29%), trichoptilosis (n = 2, 10%), and pili torti (n = 1, 5%). Conclusions In this series, hair findings in HED were similar to those described in previous studies. However, we describe two new clinical and two trichoscopic findings: decreased hair density mainly in the temporal and occipital regions, oblique upwards occipital hair follicles orientation, angled hairs, and short curly pigtail hairs. These heterogeneous findings may reflect the multiple factors and signaling pathways that can be affected in these syndromes.

Griscelli syndrome type 2 (GS2) features silvery-grey hair, bronzed skin and immunodeficiency.1 The only curative treatment for GS2 is haematopoietic stem cell transplantation (HSCT) which corrects the immune and haematologic defects with persistence of oculocutaneous abnormalities.

There are no pathognomonic histopathological features to distinguish acute graft-vs-host disease (aGVHD) from skin drug reactions (SDRs) in pediatric patients with multiple drug regimens that have received blood transfusions and/or transplants. We aimed to determine if the addition of apoptosis markers is helpful to distinguish aGVHD from SDRs in these patients.Skin biopsy specimens from patients with a clinical diagnosis of aGVHD or SDRs were evaluated for the presence of apoptotic bodies, satellitosis, interface damage, vasculitis, and inflammatory infiltrate on HE stain. Information was completed with apoptotic markers (transferase-mediated dUTP nick end-labeling [TUNEL], bcl-2, and caspase-3).The skin biopsy specimens of 32 patients with aGVHD and 11 with SDRs were included for study. Only the number of apoptotic keratinocytes per 10 high-power fields (hpf) showed a significant difference between both groups (P = 0.02); the presence of ≥4 apoptotic keratinocytes per 10 hpf was identified as the optimal cut-off point to discriminate aGVHD from SDRs. No SDRs cases had follicular apoptotic cells. TUNEL, bcl-2, and caspase-3 determination showed no difference between both groups.The presence of ≥4 apoptotic keratinocytes per 10 hpf (in aGVHD) and the absence of follicular apoptotic cells (in SDRs) might be a useful marker to distinguish between them.

Superficial granulomatous pyoderma gangrenosum, a rare variant of pyoderma gangrenosum, has been considered to be the most benign form of the disease. We present the case of a 15-year-old boy with pulmonary involvement and nodular scleritis associated with this unusual type of pyoderma gangrenosum and discuss its differential diagnosis.

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.

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.

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.

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.

Acquired diffuse hyperpigmentation in children secondary to metabolic disorders can be divided into nutritional diseases, endocrinological disorders, and metabolic causes. Although pigmentation is categorized as diffuse, it is often accentuated in some areas. Adequate approach of diffuse hyperpigmentation may lead to the early diagnosis of a specific disease. The initial and most prominent sign can be in the skin and thus the dermatologist is the first physician consulted.

The aim was to be able to evaluate the diagnosis of two diseases by a consensus of clinical opinion used in the Department of Dermatology of the National Institute of Paediatrics in Mexico City. To differentiate between scleroderma 'en coup de sabre' (SCS) and progressive facial hemiatrophy (PFH), colour slides of 13 patients diagnosed as SCS and nine as PFH were examined by two dermatologists and microscopic slides by two pathologists. In both cases, the slides were randomly presented and no clinical information was given. The clinical and histopathological findings were statistically compared with two-tailed tests and alpha = 0.05. Kappa coefficients were obtained to evaluate the concordance between dermatologists, pathologists, and in terms of the consensus diagnosis. The usefulness of photographic assessment is limited by the inability to palpate the consistency of lesions. The most important clinical feature that differentiated both conditions was cutaneous sclerosis present in eight of 13 patients with SCS and in none of the PFH patients (P < 0.005). Other clinical features more frequently found in SCS were cutaneous hyperpigmentation and alopecia. The more frequent clinical features in PFH were total hemifacial involvement and ocular changes. Statistically significant histopathological features were: connective tissue fibrosis present in all cases with SCS and two of nine patients with PFH (P < 0.0002); adnexal atrophy present in 11 of 13 patients with SCS, and in three of nine with PFH (P < 0.02), and mononuclear cell infiltrates in all patients with SCS cf. six with PFH (P < 0.05). Our results suggest that in most cases it is possible to differentiate SCS from PFH based on clinicopathological findings.

In addition to onchocerciasis and other filarial diseases, ivermectin has been used for the treatment of scabies, head lice, larva migrans and gnathostomiasis. However, there is concern regarding the safety of its use in children under 5 years of age or weighing less than 15 kg. We present our experience in 18 children (aged 14 months to 17 years), with scabies or cutaneous larva migrans successfully treated with ivermectin. They included four cases of crusted scabies associated with immunosuppression and seven cases of common scabies four of whom had associated clinical mental retardation, immunosuppression or hypomobility. A further seven patients had cutaneous larva migrans. Fifteen patients were cured with a single dose of ivermectin, and three patients with crusted scabies required a second dose. None of our patients suffered significant adverse effects. We believe that ivermectin is a safe and effective alternative treatment of cutaneous parasitosis in children.

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.

The purpose of this study is to compare the esthetics of scars resulting from small surgical facial wounds sutured either with simple interrupted percutaneous suture (SIPS) or with running intradermal suture (RIS). 93 small (0.6-1.9 cm) benign, facial new growths, mostly (86%) intradermal nevi were surgically removed. Forty-seven surgical wounds were sutured with SIPS and 46 with RIS. All surgical procedures were performed by the same dermatologic surgeon. For comparison, lesions were grouped in five facial areas. Other variables such as the age of the patient and type and size of lesions were similar in both groups. Evaluation of each scar was made blindly by two independent observers and by the patients. Judged by independent observers 90 days after surgery, excellent (45 %) to good (45%) results were obtained in similar proportion with either SIPS or RIS suturing in 90% of the patients. In the patients' self evaluation, excellent (85%) to good (14%) results were obtained in 99% of the cases. Suture marks and tracks present 30 days after surgery in 28% of scars sutured with SIPS disappeared 90 days after surgery. The esthetic results obtained by suturing small surgical facial wounds with SIPS or RIS were similarly good to excellent when observed 90 days after surgery. There was no advantage in using RIS over SIPS in the type of wounds described.

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.

Oral retinoids are molecules derived from vitamin A that represent one of the most important steps forward in dermatologic therapeutics in the present century. The treatment of acne, severe psoriasis, and severe disorders of keratinization, prevalent diseases in children and adolescents, have radically changed since the advent of oral retinoids. Like most highly-effective medications, oral retinoids also have important untoward effects. Specialists, and in particular, dermatologists and pediatricians should be prepared to maneuver the delicate balance between therapeutic efficacy and side effects in order to give the pediatric patient the maximum benefit with the lowest possible risk.

Background: Extensive epidermal necrosis in newborn infants is an unusual event of heterogeneous cause. Objective: The objective of this article is to describe what seems to be a previously unrecognized lethal disease. Methods: The clinical and histopathologic features of three premature infants, two of them nonidentical twins, and the autopsy findings of one of them were analyzed. Results: Intrauterine lethal epidermal necrosis with hair follicle calcification, except for the face, hands, feet, elbows, and knees, was present in all three patients. Some histopathologic features were suggestive of epidermal apoptosis. Conclusion: We propose that the clinicopathologic alterations in our patients represent a new condition that may be caused by massive epidermal apoptosis.(J Am Acad Dermatol 1998;38:712-5.)

Background Transient myeloproliferative disorder (TMD) affects up to 10% of patients with Down syndrome (DS). A small proportion of newborns are asymptomatic and only manifest circulating blast cells, with or without leukocytosis, while others present with hepatomegaly, splenomegaly, serous effusions, and liver fibrosis. Few cases in the literature also have skin manifestations, described as crusted, erythematous, vesiculopustular eruptions occurring mainly on the face, with spreading to the trunk and extremities. Materials and methods Four patients with DS and TMD were studied due to the presence of cutaneous eruptions. Systemic involvement, work-up, and follow-up were documented for each patient. Our results were compared with the previously reported cases. Results All patients were males, with ages ranging from 1 to 20 days at the time of diagnosis. In three patients, the eruption was papulopustular, and two of them also had vesicles. In one patient, lesions resembled bullous impetigo. In all, the lesions involved the face, followed by the extremities in three and the trunk in two patients. Pathergy phenomena was present in one patient. Hepatomegaly and a leukemoid reaction were present in all patients. Bone marrow showed an M7 immunophenotype in three patients and normal cellularity in one. Follow-up ranged from 2 to 11 months, during which the patients were healthy. Conclusions Recognition of the cutaneous eruptions associated with TMD in neonate patients with DS may lead to early diagnosis and avoidance of unnecessary chemotherapy. However, because leukemia may develop later, careful follow-up is mandatory in all cases.

The frequency of different malignant cutaneous tumors (MCTs), primary and metastatic, in children is not known. We reviewed all MCTs, primary and metastatic, seen during a 20-year period in a large general pediatric hospital. Fifty-three MCTs, 36 primary and 17 metastatic, were diagnosed in 36,207 pediatric dermatology patients. The incidence was 1.4 per 1000 patients. The relative frequency of occurrence of the different tumors was as follows: rhabdomyosarcoma, 25%; lymphomas, 19%; basal cell carcinoma, 13%; leukemia, 13%; neuroblastoma, 10%; malignant melanoma, 6%; squamous cell carcinoma, 6%; unclassified sarcomas, 4%; epithelioid schwannoma, 2%; ependymoma, 2%. The mean follow-up was 3 years; 48% died, 27% were lost to follow-up, and 25% are under control. We conclude that primary and metastatic MCTs in children are rare. Their types differ from MCTs in an older age population. MCTs in children are associated with a high mortality rate, often related to late recognition.

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.

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.

Darter’s disease is an autosomal dominant acantholytic disorder (OMIM # 124200) characterized by a peculiar keratinization of the epidermis, nails, and mucous membranes, resulting in a persistent eruption of keratotic papules predominantly in seborrheic areas (upper and central trunk, flexures, scalp and forehead), palmar pits and nail dystrophy. Involvement may be severe, with widespread itchy malodorous crusted plaques, painful erosions, blistering, and mucosal lesions. Secondary infection is common. Sun, heat, and sweating exacerbate the disease, which never remits (even though oral retinoids may reduce hyperkeratosis). Neuropsychiatric abnormalities including mild mental retardation, epilepsy, and schizophrenia have been reported in some families (Burge and Wilkinson 1992, Jacobsen et al. 1999): whether this association is due to pleiotropism of the mutated gene or reflects coincidence is not clear (OMIM 2006). Several variants have been described (see below).

Keratitis-ichthyosis-deafness (KID) syndrome is a genetically determined disorder of keratinization, a congenital ichthyosiform dermatosis with additional extracutaneous features (keratitis and deafness). Because of the association of ichthyosis or ichthyosiform dermatosis with central and/or peripheral nervous system involvement, KID syndrome has been (and is) traditionally defined as a neuroichthyosis or neurocutaneous disorder.

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.

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.

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.

Desmoplasia has been described in melanoma and Spitz naevus but not in giant congenital melanocytic naevus (GCMN). In melanoma desmoplasia is associated with a better survival. Four paediatric patients with hard, ligneous, progressively hypopigmented and alopecic GCMN were seen among 143 cases of GCMN at the Department of Dermatology of the National Institute of Paediatrics, Mexico City. Clinically, induration was progressive in three patients and regressive in one. Pigmentation was regressive in all. Histopathologically, all four patients showed intense dermal fibrosis, scarce naevus cells, and hypotrophic or absent hair follicles. Follow-up and serial biopsies in three patients documented the progressive nature of fibrosis and naevus cell depletion. No evidence of malignant transformation was found. Naevus cell depletion resulted in pigment loss and may have reduced the risk of malignant transformation. Although the cause of fibrosis is unknown, the possibility of an immune reaction to naevus cells is postulated.

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.

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.

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.

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 ĸ 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 (ĸ = 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.

Antecedentes: la pitiriasis rosada es un exantema agudo diseminado de etiología desconocida y duración prolongada. Se caracteriza por placas eritematosas con un collarete de escama periférica. Aunque el cuadro clínico es aparatoso para el paciente y el familiar, la pitiriasis rosada es involutiva y generalmente remite sin secuelas. Frecuentemente es confundida con micosis y se prescriben tratamientos innecesarios. Objetivo: describir las características clínicas y demográficas de la pitiriasis rosada en un grupo de pacientes pediátricos mexicanos. Pacientes y métodos: estudio retrospectivo y descriptivo en el que se analizaron las características clínicas y demográficas de los pacientes evaluados en el Servicio de Dermatología del Instituto Nacional de Pediatría con diagnóstico de pitiriasis rosada en un periodo de 10 años. Resultados: se encontraron treinta pacientes con pitiriasis rosada para una frecuencia de 3.6 por cada 1 000 pacientes dermatológicos. Pre- dominio en mujeres con una relación de 1.5:1 y edad promedio de 10 años. En más de la mitad de los casos (56%) se observaron formas atípicas que hicieron necesaria la toma de biopsia en 7 pacientes para establecer el diagnóstico definitivo. Conclusiones: el conocimiento de las características clínicas de la pitiriasis rosada, por médicos de primer contacto, evitará tratamientos y exámenes innecesarios.

Erythema multiforme is an inflammatory disorder of the skin that usually fades without sequelae. It is well known that after inflammatory events, hyper- or hypochromic spots can remain, especially in skin types III-VI, but achromia is very rare. We report a case of residual leukoderma after erythema multiforme.

The purpose of this investigation was to explore the frequency and nature of ungual alterations in patients of a pediatric dermatology department at a third-level pediatric hospital. The first 20 patients with nail alterations seen each year during a 5-year period from 1992 through 1996 were included, totaling 100 patients. The rate of nail alterations was 11% (1/9) in pediatric dermatology patients. There were 5 infants, 19 preschoolers (2- to 5-year-olds), 38 school children (6- to 11-year-olds), and 38 adolescents (12- to 17-year-olds). The most frequent diagnoses were onychomycosis (23), nail alterations in a genodermatosis (23), nail alterations associated with dermatoses (16), onychocryptosis (11), and paronychia (10). Toenails were involved in 54 patients, fingernails in 25, and both in 21 patients. Twenty nails were involved in 21 patients. A high prevalence of nail alterations was found in pediatric dermatology patients, some of which were nonspecific, while others provided important diagnostic clues.

Three cutaneous manifestations are characteristic of Bart syndrome: congenital localized absence of skin (CLAS), mucocutaneous blistering, and nail abnormalities. Six cases of Bart syndrome are herein reported. Localized absence of skin is present at birth, particularly on the anterior aspects of the lower extremities and dorsa of the feet. Physical trauma in utero has been proposed as a mechanism to explain the denuded areas on the limbs. The recurrent, highly similar pattern of the congenital defect in regard to location and clinical appearance in our patients and in most of the reported cases strongly suggests that trauma is too simplistic an explanation. Because of the observed bilateral and symmetric distribution of denuded areas in an S-shaped broad band, their sharply demarcated borders, the involvement of the toe webs, and the frequent similar involvement of the soles, we suggest that congenital localized absence of skin in Bart syndrome may follow the lines of Blaschko.

Background Silvery hair and severe dysfunction of the central nervous system (neuroectodermal melanolysosomal disease or Elejalde syndrome) characterize this rare autosomal recessive disease. Main clinical features include silver-leaden hair, bronze skin after sun exposure, and neurologic involvement (seizures, severe hypotonia, and mental retardation). Large granules of melanin unevenly distributed in the hair shaft are observed. Abnormal melanocytes and melanosomes and abnormal inclusion bodies in fibroblasts may be present. Differential diagnosis with Chediak-Higashi syndrome and Griscelli syndrome must be done. Observations We studied pediatric patients with silvery hair and profound neurologic dysfunction. Immune impairment was absent. Age of onset of neurologic signs ranged from 1 month to 11 years; the signs included severe muscular hypotonia, ocular alterations, and seizures. Mental retardation since the first months of life was noted in 4 cases. Psychomotor development was normal in 3 cases, but suddenly the patients presented with a regressive neurologic process. Four patients died between 6 months and 3 years after the onset of neurologic dysfunction. One patient showed characteristic ultrastructural findings of Elejalde syndrome. Conclusions Elejalde syndrome is different from Chediak-Higashi and Griscelli syndrome and is characterized by silvery hair and frequent occurrence of fatal neurologic alterations. Psychomotor impairment may have 2 forms of presentation: congenital or infantile. Although Elejalde syndrome and Griscelli syndrome are similar, the possibility that they are 2 different diseases, although probably allelic related, is suggested.

The use of cosmetics (cleansers, moisturizers, and talcs) was investigated in 200 infants and children of low socioeconomic level with and without skin disease. For children with atopic dermatitis, mothers preferred to use unscented soap. Lubricating or hydrating creams were widely used in children without skin disease. Talc and mineral oil were significantly more frequently used in infants than in children. Both of these products are potentially harmful and represent an unnecessary expense for families with a survival-level budget. Dermatologists should discourage their use.