Clinical spectrum of skin manifestations of Lyme borreliosis in 204 children in Austria.

The spectrum of skin manifestations of Lyme borreliosis in children is not well characterized. We conducted a retrospective study to analyze the clinical characteristics, seroreactivity to Borrelia burgdorferi sensu lato, and outcome after treatment in 204 children with skin manifestations of Lyme borreliosis seen in 1996-2011. Solitary erythema migrans was the most common manifestation (44.6%), followed by erythema migrans with multiple lesions (27%), borrelial lymphocytoma (21.6%), and acrodermatitis chronica atrophicans (0.9%). A collision lesion of a primary borrelial lymphocytoma and a surrounding secondary erythema migrans was diagnosed in 5.9% of children. Rate of seroreactivity to B. burgdorferi s.l. was lower in solitary erythema migrans compared to other diagnosis groups. Amoxicillin or phenoxymethylpenicillin led to complete resolution of erythema migrans within a median of 6 (solitary) and 14 days (multiple lesions), respectively, and of borrelia lymphocytoma within a median of 56 days. In conclusion, erythema migrans with multiple lesions and borrelial lymphocytoma appear to be more frequent in children than in adults, whereas acrodermatitis chronica atrophicans is a rarity in childhood. The outcome after antibiotic therapy was excellent in children, and appears to be better than in adults.

Zinc deficiency and its management in the pediatric population: a literature review and proposed etiologic classification.

Zinc is a trace element essential to the gastrointestinal, immune, integumentary, reproductive, and central nervous systems. Zinc deficiency is prevalent in many areas of the world and is a diagnostically challenging condition. Cutaneous manifestations typically occur in moderate to severe zinc deficiency and present as alopecia and dermatitis in the perioral, acral, and perineal regions. Zinc deficiency is a potentially fatal disease process. The aim of this review is to focus on the cutaneous manifestations, diagnosis, and treatment of zinc deficiency in children, and to propose an etiologic classification system.

Gianotti-Crosti syndrome following childhood vaccinations.

A 19-month-old boy was evaluated for a skin eruption after recent vaccinations. Clinical and histopathologic findings supported a diagnosis of Gianotti-Crosti syndrome (GCS). This case report examines the link between GCS and vaccinations, particularly the diphtheria, tetanus, and pertussis vaccine and the varicella virus live vaccine.

Acroangiodermatitis of Mali (Pseudo-Kaposi Sarcoma) Associated with Chronic Venous Insufficiency and Obesity: A Case Report.

INTRODUCTION: Acroangiodermatitis, also known as psesudo-Kapsoi sarcoma, is a rare and benign angioproliferative malady. It presents clinically as purple-colored plaques, patches, or nodules resembling Kaposi sarcoma and is often related to chronic venous insufficiency, arteriovenous malformation of the legs, paralyzed limbs, and residual limbs. Obesity is a risk factor for venous insufficiency and could be related to acroangiodermatitis. CASE REPORT: The authors report a case of acroangiodermatitis of the bilateral legs that was misdiagnosed for 3 years. The diagnosis was confirmed using histopathological and immunohistochemical examinations such as hematoxylin and eosin staining and CD34 immunostaining. Multiple modalities were utilized to treat the wounds, including serial debridement, compression dressings, and silver antimicrobial dressings. CONCLUSIONS: It should be stressed that chronic, nonhealing wounds should be biopsied to obtain a proper diagnosis.

[Acrodermatitis enteropathica]. / Acrodermatitis enteropática.

INTRODUCTION: Acrodermatitis enteropathica is a low-incidence disease due to inherited or acquired zinc deficiency. It is characterized by acral dermatitis, alopecia, diarrhea and growth retardation. The dermatological condition may mimic a cutaneous fungal infection or other pathogen-related skin diseases. CASE REPORT: We report the case of a female patient of 7 months of age, who was sent to Centro Médico Nacional 20 de Noviembre for suspicion of immunodeficiency and cutaneous mycosis. Her condition began with dermatosis disseminated to the head, trunk and genital region; initial treatment with antifungal and broad spectrum antibiotics was given, without improvement. Upon admission, immunodeficiency and fungal infection were discarded. Acrodermatitis enteropathica was suspected, and corroborated later by low serum zinc levels. Immediately after the start of oral treatment with zinc, the patient showed improvement. CONCLUSIONS: There are multiple differential diagnoses of acrodermatitis enteropathica, which includes cutaneous infections. Therefore, the early recognition of the characteristic lesions favors suspicion, diagnosis and appropriate treatment.

Metabolic diseases and pregnancy.

The skin mirrors the hormonal metabolic and physiologic changes that occur during pregnancy. The metabolic effects are manifested primarily in accentuation of two disorders, porphyria cutanea tarda and acrodermatitis enteropathica. The former may be exacerbated, not only during pregnancy, but also during the post-partum period and with the intake of estrogen-containing birth control pills. Exacerbations of acrodermatitis enteropathica characteristically appear during pregnancy.

Current aspects of Lyme disease and other Borrelia burgdorferi infections.

Lyme disease, acrodermatitis chronica atrophicans, and borrelial lymphocytoma are caused by species of the spirochete Borrelia burgdorferi. Lyme disease has emerged as the leading vector-borne infectious disease in the United States. This article presents a current review of these entities.

Transient lymphoblastosis and thrombocytopenia in Gianotti-Crosti syndrome.

Gianotti-Crosti Syndrome, or papular acrodermatitis of childhood, represents a characteristic rash that is irregularly associated with hepatitis B infection. The authors report papular acrodermatitis in a 10-month-old child with leukopenia, thrombocytopenia, circulating lymphoblasts, and acute anicteric hepatitis B. Physical examination revealed a densely distributed papular rash on the patient's extremities and face and neck, but not on his trunk, buttocks, palms, or soles. Laboratory investigation revealed a normal bone marrow and positive hepatitis B serology. This case reinforces the fact that hematologic findings should not dissuade the work-up of papular acrodermatitis for hepatitis B or other less commonly associated viruses.

[Zinc]. / Le zinc.

Zinc is indispensable for life from bacteria to man. As a trace element it is included in numerous enzymes or serves as their activator (more than 80 zinc metallo-enzymes). It is necessary for nucleic acid and protein synthesis, the formation of sulphated molecules (insulin, growth hormone, keratin, immunoglobulins), and the functioning of carbonic anhydrase, aldolases, many dehydrogenases (including alcohol-dehydrogenase, retinal reductase indispensable for retinal rod function), alkaline phosphatase, T cells and superoxide dismutase. Its lack provokes distinctive signs: anorexia, diarrhea, taste, smell and vision disorders, skin lesions, delayed healing, growth retardation, delayed appearance of sexual characteristics, diminished resistance to infection, and it may be the cause of congenital malformations. Assay is now simplified by atomic absorption spectrophotometry in blood or hair. There is a latent lack prior to any disease because of the vices of modern eating habits, and this increases during stress, infections or tissue healing processes. Its lack is accentuated during long-term parenteral feeding or chronic gastrointestinal affections. Correction is as simple as it is innocuous, and zinc supplements should be given more routinely during surgical procedures.

[Paraneoplastic acrokeratosis (Bazex' syndrome) associated with metastatic adenopathy of the neck from a differentiated carcinoma simultaneous with a well differentiated carcinoma of the oral cavity]. / Acroqueratosis paraneoplásica (síndrome de Bazex) asociada a metástasis ganglionar cervical de carcinoma indiferenciado simultáneo con carcinoma bien diferenciado de cavidad oral.

The Acrokeratosis paraneoplastica is a skin pathology sometimes linked with malignancies of the upper respiratory or digestive ways. We report a case of a 71-year-old man enduring and dedifferenciated carcinoma of the parotid gland and metastatic cervical lymph nodes. Perusal of the literature about Bazex's syndrome.

Severe dermatitis with loss of epidermal Langerhans cells in human and mouse zinc deficiency.

Zinc deficiency can be an inherited disorder, in which case it is known as acrodermatitis enteropathica (AE), or an acquired disorder caused by low dietary intake of zinc. Even though zinc deficiency diminishes cellular and humoral immunity, patients develop immunostimulating skin inflammation. Here, we have demonstrated that despite diminished allergic contact dermatitis in mice fed a zinc-deficient (ZD) diet, irritant contact dermatitis (ICD) in these mice was more severe and prolonged than that in controls. Further, histological examination of ICD lesions in ZD mice revealed subcorneal vacuolization and epidermal pallor, histological features of AE. Consistent with the fact that ATP release from chemically injured keratinocytes serves as a causative mediator of ICD, we found that the severe ICD response in ZD mice was attenuated by local injection of soluble nucleoside triphosphate diphosphohydrolase. In addition, skin tissue from ZD mice with ICD showed increased levels of ATP, as did cultured wild-type keratinocytes treated with chemical irritants and the zinc-chelating reagent TPEN. Interestingly, numbers of epidermal Langerhans cells (LCs), which play a protective role against ATP-mediated inflammatory signals, were decreased in ZD mice as well as samples from ZD patients. These findings suggest that upon exposure to irritants, aberrant ATP release from keratinocytes and impaired LC-dependent hydrolysis of nucleotides may be important in the pathogenesis of AE.

One novel homozygous mutation of SLC39A4 gene in a Chinese patient with acrodermatitis enteropathica.

Acrodermatitis enteropathica, a rare autosomal recessive disease, manifests as periorificial and symmetrical acral dermatitis, alopecia, and diarrhea due to insufficient zinc uptake by the intestine. Recent research revealed that mutations in the SLC39A4 gene are responsible for acrodermatitis enteropathica. This gene encodes one member of a human zinc transporter-like protein, also known as ZIP4. We detected one novel homozygous mutation c.1115T > G in the human SLC39A4 gene in one Chinese patient, which leading to p.L372R of the ZIP4. Homology analysis shows Leu372 in ZIP4 is conserved in Eutheria.