The problem of "Duhring-Brocq's" disease in children (author's transl)]

Inquiry in the french dermatological main centers and a survey of the literature suggest that the once described as Duhring-Brocq disease in children, may refer to three distinct bullous diseases. Taking in account clinical features, photon and electron-microscopical data, investigation for basement membrane antibodies, search for intestinal villous atrophy, effectiveness of dapsone or sulfapyridine, it is indeed possible to distinguish:1) bullous pemphigoid (very rarely); 2) dermatitis herpetiformis (not frequently), those two affections being similar to adult forms; 3) benign chronic bullous dermatitis of childhood. This last child specific disease, is the most frequently observed. Its clinical features are comparable to pemphigoids, but histopathology and immunology data are different. Its response to dapsone of sulfapyridine and to free gluten diet is note perfect and not constant, but its evolution is spontaneously favorable after a few years.     

CRUSTED PILOLEIOMYOMA WITH MENTAL RETARDATION: A RARE ASSOCIATION

Piloleiomyoma is an uncommon benign smooth muscle neoplasm arising from arrector pili muscle. It is clinically defined by the presence of solitary or multiple reddish brown, dome-shaped, smooth papules or nodules, ranging in size from a few millimeters to a centimeter. The patients are otherwise healthy; but mental retardation developing in some patients with multiple Piloleiomyomas has been emerging as an intriguing matter for analysis by the scientists. In this case report, a mentally retarded patient with Piloleiomyoma is described, who, besides the characteristic smooth and dome-shaped lesions on the anterolateral aspect of the dorsum of the right foot, had developed crusting on one of the largest lesions. The histopathological features were consistent with Piloleiomyoma. The occurrence of Piloleiomyoma in a mentally retarded child and its unusual crusted nature has been rarely reported. The association between Piloleiomyoma and mental retardation is further stressed in this case report.     

Progeria in siblings: a rare case report

Progeria, also known as Hutchinson-Gilford syndrome, is an extremely rare, severe genetic condition wherein symptoms resembling aspects of aging are manifested at an early age. It is an autosomal dominant disorder. It is not seen in siblings of affected children although there are very few case reports of progeria affecting more than one child in a family. Here we are presenting two siblings, a 14-year-old male and a 13-year-old female with features of progeria, suggesting a possible autosomal recessive inheritance.     

Fetal alcohol syndrome (FAS) in dermatology: an overview and an evaluation.

Certain skin disorders are affected by alcohol abuse. Maternal alcohol abuse during pregnancy also gives rise to a typical malformation pattern in the child with a varying degree of severity (grade I-III). The diagnosis of fetal alcohol syndrome (FAS) is based on the detailed history of the maternal alcohol consumption in combination with the clinical, morphological, intellectual and psychosocial development of the child. The behavior of the child is also of diagnostic value. The grade of severity of the FAS can be determined during the first three years of life on the basis of a scoring system. In addition to medical care and surgical treatment of associated disorders, early support for the mental development of the child is of immense importance. Furthermore, the medical, social and psychological care of the mother must be considered. In the field of dermatology, knowledge about the pathomechanism of FAS is also important for its prevention in unborn children. Women of childbearing age presenting with dermatological signs of possible alcohol abuse, should alert the dermatologist to the possible risk to the child of FAS. Familiarity with FAS and the ability to recognise its clinical features are important for the adequate treatment and support of the affected child and for the mother suffering from alcohol disease, in cooperation with other treating doctors.     

Radiologic Improvement After Early Medical Intervention in Localised Facial Morphea

We report the case of a young girl who presented with hemiparesis, seizures, and subtle features consistent with a linear form of facial morphea (en coup de sabre). She was treated with pulsed parenteral steroids and oral steroids and started on methotrexate. Magnetic resonance imaging results and neurologic problems improved after 6 months. Switching off inflammation early in the course of disease seemed to reverse some of the central nervous system changes. Assessment of children with unexplained hemiparesis and seizures should include careful examination of the face and scalp, looking for subtle signs of skin change and asymmetry. This is one of the few reported cases of neuroradiologic improvement after immunosuppressive treatment in a child with en coup de sabre.     

Interstitial granulomatous lesions as part of the spectrum of presenting cutaneous signs in pediatric sarcoidosis

Abstract:  Skin findings in childhood sarcoidosis vary greatly, but only a few occurrences have been published in which the histopathology has been characterized well. We describe a child with sarcoidosis in whom the cutaneous findings were atypical, resembling granuloma annulare. Histologic examination of these cutaneous lesions, however, revealed areas of sarcoid-like epithelioid cell granulomas, a palisading granulomatous process with features of granuloma annulare, as well as palisading neutrophilic and granulomatous dermatitis and interstitial granulomatous dermatitis. This underscores the variability of skin findings in childhood sarcoidosis—even within the same patient—and suggests that sarcoidosis should be considered in the differential diagnosis of children initially diagnosed with granulomatous skin lesions, such as granuloma annulare, palisading neutrophilic, and granulomatous dermatitis or interstitial granulomatous dermatitis, who demonstrate associated signs of systemic disease.     

Vesiculobullous lesions in lipoid proteinosis: a case report

Vesiculobullous disorders in a child can be a diagnostic challenge. Common causes of blisters in early childhood include genodermatoses like epidermolysis bullosa and infections like herpes simplex. Lipoid proteinosis may rarely present with vesiculobullous lesions in childhood. We report a child, who presented in early childhood with blistering dermatosis. On long term follow-up, typical features of lipoid proteinosis developed. A high index of suspicion is required when one deals with blistering dermatosis in a child.     

Clinical aspects of atopic dermatitis

Atopic dermatitis (AD), or atopic eczema, is the most common, chronic inflammatory disease among children in industrialized countries. We still lack knowledge of its pathophysiology and in particular the role of allergy as both an eliciting factor for disease expression and for disease activity. This article describes the clinical symptoms of the disease and its qualitatively different aspects. AD cannot be understood as being induced by one factor only, e.g. allergy, and this is important when planning treatment strategies. It is also important to realize the very wide range of disease intensity: from subclinical, or latent AD, in which only a few symptoms are present and thus which prevents a clear diagnosis of AD, to its most severe forms including erythroderma. It's unknown aetiology, the wide range in symptomatology, and the fluctuating course (including the many eliciting factors) form the background for our diagnostic and therapeutic difficulties of atopic eczema. AD is prevalent in childhood, but the atopic trait continues, not only for later respiratory allergies, but also for skin diseases in adulthood (such as AD itself or the frequent irritant contact dermatitis of the hands). A child with an acute and first attack of AD is therefore a challenge to the child, its parents and - certainly - to the doctor. However, after stressing the chronicity of the disease, it is equally important to assure the parents that this disease is, in most cases, controllable through correct treatment and that it has a good prognosis: It is not a 'life sentence', but a controllable disease in an otherwise healthy child.     

Postinflammatory elastolysis and cutis laxa. A case report

One of the rarest forms of cutis laxa is postinflammatory elastolysis and cutis laxa, a disease previously reported only in children in Africa and South America. This disease is characterized by an urticarial or papular eruption followed by acute destruction of elastic tissue that results in atrophy and severe disfigurement. It is distinguished from anetoderma and acquired cutis laxa by its clinical features, its occurrence in young children, and its relatively benign course. This article describes the first case of postinflammatory elastolysis and cutis laxa reported in a white child from North America.     

HTLV-I-associated granulomatous T-cell lymphoma in a child

Adult T-cell leukemia/lymphoma (ATLL) is a T-cell malignancy closely associated with human T-cell lymphotropic virus-1 (HTLV-I). Because of its long latency period, ATLL occurs almost exclusively in adults. We report a case of a 13-year-old boy with an 8-year history of skin eruptions. After complete evaluation, a diagnosis of HTLV-I-associated lymphoma/leukemia was made. The T-cell lymphoma exhibited a granulomatous histomorphology. There have been very few reports of ATLL presenting in childhood and none, to our knowledge, demonstrating granulomatous histology. We conclude that ATLL may rarely present as a chronic granulomatous eruption in a child.     

A novel mutation in the connexin 26 gene (GJB2) in a child with clinical and histological features of keratitis–ichthyosis–deafness (KID) syndrome

Background. Keratitis–ichthyosis–deafness (KID) syndrome is a rare congenital ectodermal disorder, caused by heterozygous missense mutation in GJB2, encoding the gap junction protein connexin 26. The commonest mutation is the p.Asp50Asn mutation, and only a few other mutations have been described to date. Aim. To report the fatal clinical course and characterize the genetic background of a premature male neonate with the clinical and histological features of KID syndrome. Methods. Genomic DNA was extracted from peripheral blood and used for PCR amplification of the GJB2 gene. Direct sequencing was used for mutation analysis. Results. The clinical features included hearing impairment, ichthyosiform erythroderma with hyperkeratotic plaques, palmoplantar keratoderma, alopecia of the scalp and eyelashes, and a thick vernix caseosa‐like covering of the scalp. On histological analysis, features characteristic of KID syndrome, such as acanthosis and papillomatosis of the epidermis with basket‐weave hyperkeratosis, were seen. The skin symptoms were treated successfully with acitretin 0.5 mg/kg. The boy developed intraventricular and intracerebral haemorrhage, leading to hydrocephalus. His condition was further complicated by septicaemia and meningitis caused by infection with extended‐spectrum beta‐lactamase‐producing Klebsiella pneumoniae. Severe respiratory failure followed, and the child died at 46 weeks of gestational age (13 weeks postnatally). Sequencing of the GJB2 gene showed that the child was heterozygous for a novel nucleotide change, c.263C>T, in exon 2, leading to a substitution of alanine for valine at position 88 (p.Ala88Val). Conclusions. This study has identified a new heterozygous de novo mutation in the Cx26 gene (c.263C>T; p.Ala88Val) leading to KID syndrome.     

Epidermal nevus misinterpreted as child abuse

Determining what constitutes true child abuse is not always easy. A child with epidermal nevus and multiple café-au-lait spots was erroneously thought to be a victim of abuse. This report details the features of epidermal nevi. We present these findings with the hope that such misdiagnoses can be avoided in the future.     

AN INTERROGATIVE STUDY OF 142 PSORIASIS PATIENTS

The incidence of psoriasis was determined in 21,441 patients of dermatologic population, of which 142 were psoriasis, giving its incidence as 0.66 percent. The mean age in the males and females was computed as 33.1 and 33.7 years respectively, while the age at onset of the disease was almost similar in males and females. The emotional undertones were the major provocating factors, although trauma, parturition and infection were incriminated in a few cases. In 4.9 percent of psoriatics, the family history was suggestive. The duration of the disease was varying from a few days to a few years, more than 50 percent however reported before a years. Remissions and exacerbations were noticed in many patients with or without treatment, but seasonal variation had no significant influence on the course of the disease. Classical papulosquamous cutaneous rash and associated itching were the salient clinical features, although joint pains were featured in a few. Pitting, thickening and subungual hypertrophy were the main salient features in the nails. Very few patients had manifested clinical joint involvement, swelling of the distal interphalangeal joints being the commonest. Other associated diseases were rarely seen in these patients.     

Systemic mastocytosis associated with Hodgkin’s lymphoma in a 4-year-old child

Systemic mastocytosis with associated clonal hematologic non-mast cell lineage disease (SM-AHN) represents a specific subtype of mastocytosis and is extremely rare in children. We describe a 4-year-old child with systemic mastocytosis associated with Hodgkin's lymphoma. The child had cutaneous mastocytosis and lymphadenopathy without other clinical features of SM, which was diagnosed only by bone marrow examination.     

Leukemia Cutis in Three Children: Clinical and Immunohistochemical Studies

Abstract: We report 3 children with leukemia cutis observed at the initial diagnosis of systemic leukemia. Leukemia subtypes in the three children were congenital monocytic, acute undifferentiated, and acute monocytic, respectively. The patients were girls age 10 days, 14 years, and 11 months, respectively, at diagnosis. We describe the clinical features of the cases and the results of immunohistochemical studies on paraffin-embedded skin biopsy specimens. The skin lesions were tumors and areas of reddish purple erythema in the first child, pigmented erythema in the second, and bright red erythema in the third. In the first two patients skin lesion biopsy specimens had dense leukemic infiltrates in the dermis with reactive T lymphocytes scattered among them. In the third patient, the infiltrating cells were almost all reactive T lymphocytes, with a few leukemic cells. A relationship between the leukemic-reactive cell ratio and the prognosis was suggested; dense leukemic cell infiltrates may be associated with a poor prognosis.     

Childhood psoriasis

Psoriasis is a common chronic inflammatory skin disease. Recently, few data have been published on epidemiology, comorbidity, or therapy in children with psoriasis. Psoriasis affects up to 2% of children in Europe, even during the first months of life. The link between psoriasis and metabolic comorbidities has been highlighted, notably in relation to excessive weight and obesity. The clinical picture of psoriasis in childhood resembles adult disease, however, some clinical features are noteworthy: neonatal diaper rash is relatively specific, face involvement and guttate psoriasis are more common, plaques are often smaller, and scales are finer and softer than in adults. Napkin, guttate and palmoplantar psoriasis appear to have specific features in childhood and prevalence depends on the age of the child. Although benign, the effect of psoriasis on social interaction can be major, especially in children. Topical therapies are the first line of treatment for skin-limited disease. For chronic cases and more severe cases, phototherapy or traditional biologic systemic treatments must be discussed. The great challenge will be to propose international guidelines to manage these children.     

Cardiofaciocutaneous syndrome: a rare entity

The cardiofaciocutaneous (CFC) syndrome is a condition of sporadic occurrence, with patients showing multiple congenital anomalies and mental retardation and characteristic dysmorphic features. We, thus, report a rare case of this syndrome in a 1-year-old child who presented with typical features of CFC syndrome.     

嗜酸粒细胞增多性血管淋巴样增生1例

患者,女,38岁。右耳廓暗红色丘疹2年。组织病理检查示:真皮中上部血管增生,血管周围淋巴细胞、嗜酸粒细胞浸润。根据患者临床及镜下特点诊断为嗜酸粒细胞增多性血管淋巴样增生。二氧化碳激光治疗后消退,未见复发。     

Childhood granulomatous periorificial dermatitis in children with extra-facial involvement

Childhood granulomatous periorificial dermatitis (CGPD) is a self-limiting and well-recognized entity. A six-year-old male child, a known case of juvenile rheumatoid arthritis (JRA) presented with multiple red raised and yellowish lesions over the face, neck, trunk and upper extremities since one month with occasional itching. Cutaneous examination revealed multiple erythematous scaly papules of size up to 5 mm around the mouth, nose and periorbital areas, neck, trunk and upper extremities with few excoriations. Lesional skin biopsy was pathognomic of CGPD. We report a six-year-old Indian male child with extra-facial involvement and healing with small atrophic pigmented scars in a known case of JRA.     

Erosive pustular dermatosis of the scalp after aplasia cutis congenita in a 9‐year‐old patient: A 5‐year follow‐up

Erosive pustular dermatosis of the scalp (EPDS) is a rare inflammatory skin disease that occurs mainly in elderly adults with a history of trauma to the scalp, but a few cases of EPDS in children have been reported. We report a rare case of EPDS after aplasia cutis congenita in a child.