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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.

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.

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.

Primary immunodeficiency diseases (PIDs) are rare but important conditions found predominantly in children. We studied PIDs in a large pediatric hospital, their association with cutaneous alterations, and the importance of cutaneous alterations as diagnostic markers. Among 382,383 pediatric patients, 130 (0.0003%) had a PID: humoral in 27, cellular and combined in 18, phagocytic in 37, and associated with major defects in 45. An average of two cutaneous alterations were present in 90 (69%) patients: infections in 80, eczema-dermatitis in 38, and miscellaneous in 57. In 71 (79%) patients the cutaneous alterations preceded and were the basis for the clinical immunologic diagnosis. Only two PIDs were not associated with cutaneous lesions.

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.

To give a child the ominous diagnosis of malignant melanoma is a very difficult decision for both clinician and pathologist. Difficulties in histologic diagnosis combined with a reluctance on the part of the clinician may lead to delays in diagnosis and curative surgical intervention. In fact, some melanomas in children are misdiagnosed clinically as benign lesions and recognized as melanomas only after excision. It is imperative to recognize that malignant melanoma occurs in children and to appreciate its clinical characteristics and predisposing factors. Incidence and Prognosis Approximately 2 percent of melanomas occur in patients under the age of 20 years, . . .

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.

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.

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.

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.

The purpose of this investigation was to find out how often pathogenic dermatophytes are found in apparently normal versus scaly feet of children. In this prospective protocol, we studied 100 patients, 2 to 12 years of age, consulting for plantar and/or interdigital scaling with or without erythema, maceration, and pruritus, and 100 controls with apparently healthy feet. Direct microscopic examination (KOH) and mycologic culture (Mycosel) of skin scrapings were performed from the lesions of patients and from the soles and interdigital folds of controls. Mean age of patients and controls was 7 years 7 months (SD = 3 years 2 months). Scaling was present in 100% of patients with a mean time of evolution of 8.9 months (SD = 16.3 months). Shoes fashioned from man-made material were significantly more frequently used by patients than by controls (chi 2(df = 1) = 9.4; p = 0.002). Pathogenic dermatophytes were present in the soles and/or interdigital webs of 21 patients and 7 controls. Not all foot scaling in children, pruritic or not, is associated with dermatophytes. Dermatophytes may be present on the apparently healthy feet of children.

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.

Characteristic features of acute annular urticaria in 34 infants and small children were large, erythematous annular and polycyclic lesions with violaceous centers, eyelid, hand, and foot edema, absence of angioedema of the airway, absence of pruritus, spontaneous resolution in 8 to 10 days, and frequent history of furazolidone medication for diarrhea. Nondermatologists often misdiagnose acute annular urticaria as erythema multiforme and unnecessarily overtreat patients. The differential diagnosis with other conditions presenting with annular lesions in children is discussed.

Twenty-six children with scabies and 32 contacts were treated with 10% sulfur in cold cream. A 100% clinical cure rate was observed, although 56.8% of patients experienced some kind of mild, transient cutaneous reaction. An additional 25 children with scabies and 28 contacts were treated with 10% sulfur and 1% salicylic acid in pork fat. Of these, 88% were clinically cured and 73.5% had some cutaneous adverse effects. In both groups, most adverse effects were related to skin dryness or postscabetic reaction. The cold cream base was more acceptable to patients than the pork fat base. However, the pork fat base was significantly cheaper and easier to obtain than the cold cream base, and 238 times less expensive than the cheapest commercial scabicidal medication available in the United States.

An 11-year-old boy with typical lesions of pachyonychia congenita (Jadassohn-Lewandowsky) type II and with keratotic lesions histologically characteristic of Kyrle's Disease is described.

We examined four patients who had a combination of extensive nevus flammeus, significant oculocutaneous pigmentation, and severe neurologic alterations. All cases were sporadic. The vascular and neurologic alterations were clinicaily similar to those observed in the Sturge-Weber syndrome. The capillary vessels are ultrastructurally different in phacomatosis pigmentovascularis from those of a nevus flammeus in the Sturge-Weber syndrome; however, tnelanocytes of normal aspect were present in the middle and deep der-mis. The characteristic oculocutaneous pigmentation probably represented a noncoincidental association. The term phacomatosis pigtnentovascuiaris seems appropriate for this apparently new neurocutaneous syndrome.

Rud's syndrome is a neuroichthyosis with hypogonadism, associated with mental deficiency and epilepsy. Short stature is a frequent component of the syndrome. The primary genetic defect and the pattern of inheritance have not yet been determined. A typical patient is presented, with mental deficiency, short stature, pypoacusia, muscular atrophy, tylosis, pseudoacanthosis nigricans and endocrine disturbances. The neuroichthyosis with hypogonadism must be considered Rud's syndrome. A classification of neuroichthyosis is proposed. In a first group is neuroichthyosis with hypogonadism, in the second group is neuroichthyosis with spasticity and in the third group, neuroichthyosis without hypogonadism or spasticity.

Publisher Summary This chapter describes neuroichthyosis, which refers to those conditions associating ichthyosis or ichthyosiform dermatoses to central or peripheral nervous system involvement. Ichthyosis is a group of genetically determined alterations of keratinization clinically characterized by persistent, clinically evident scales on the skin surface. Ichthyosiform dermatosis is a heterogeneous group of skin disorders characterized by different degrees of hyperkeratosis, scaling, and erythema. Lesions with capricious designs are often found in ichthyosiform dermatoses. Several well-recognized diseases and a few infrequently reported syndromes strongly support the concept that the association among ichthyosis, ichthyosiform dermatoses, and nervous system pathology is not coincidental. Ruds syndrome is inherited as an autosomal recessive trait. Tay syndrome is unusual ichthyotic syndrome of autosomal recessive inheritance is characterized by pili torti and a tricorrhexis nodosa-like alteration whose substrate is trichothiodystrophy. A syndrome of migratory ichthyosiform dermatosis with neurologic and ocular abnormalities, present since birth, has recently been described. The association of ichthyosis, thrombocytopenia, myasthenia, miosis, asplenia, migraine, and dyslexia has been recently reported.

Progressive Cardiomyopathic lentiginosis (PCL) is a syndrome with progressive multisystem abnormalities. Lentigines, the cutaneous hallmark, are usually present from early age and are of utmost diagnostic value. Six children with PCL arc reported. The most constant diagnostic features in this age group were multiple cutaneous lentigines, structural cardiac anomalies, and electrocardiographic alterations. Less constant findings were neurosensory deafness, short stature, and osseous, ocular, and genitourinary abnormalities.

We cared for 124 pediatric patients With a histologic diagnosis of Langerhans' cell histiocytosis (histiocytosis X) over a period of 14 years. Clinical, laboratory. and radiographic findings were analyzed. The most frequent manifestations were bone lesions, lymph node involvement, and skin infiltralion. Liver disease was noted in 50% of patients and lung disease in 23% he-matologic changes were also frequent- Dysfunction and involvement of these three organ systems, plus age of onset, distinguished the group of patients with the highest mortality. All palienis with generalized disease or organ dysfunclion were Lrealed with systemie chemotherapy. The actuarial survival curve at 10 Years was 63%