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.

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.

Acrodermatitis enteropática en paciente con ileostomía de alto débito / Enteropathic acrodermatitis in a patient with high-out-put ileostomy

La deficiencia de zinc es relativamente frecuente en ciertas patologías, pero su manifestación clínica es rara. Se expone el caso de un paciente que ingresó con cuadro de deshidratación hiponatrémica e insuficiencia prerrenal secundaria por ileostomía de alto débito e intolerancia oral. Aunque se normalizó el débito de la ilesotomía al tercer día del ingreso y los niveles plasmáticos de zinc iniciales fueron normales, el paciente después de estar con NPT suplementada con 13 mg/día de zinc, sufrió una deficiencia severa de zinc con acrodermatitis enteropática que se resolvió completamente después de una semana con 26,5mg/día. Sin embargo, los niveles plasmáticos no se normalizaron hasta pasado un mes de estar en tratamiento con estas macrodosis de zinc. El zinc plasmático no es un buen indicador de los depósitos de zinc en el organismo, y muchas veces, su alteración es un indicador tardío de deficiencia. Tampoco se observa una correlación entre la mejoría clínica y la normalización de sus niveles, siendo frecuente la resolución clínica a los pocos días de la suplementación sin detectarse aumento de zinc en plasma (AU)

Zinc deficiency is relatively common in certain pathologies, although its clinical manifestation is uncommon. We present the case of a patient who was admitted pre -senting hyponatremic dehydration and pre-renal failure secondary to high-output ileostomy and oral intolerance. Although the ileostomy output was normalized within 3days of admission and initial zinc plasma levels were normal, after being fed with TPN supplemented with 13mg/day of zinc, he suffered a severe zinc deficit with enteropathicacrodermatitis that was completely resolved after one week with 26.5 mg/day. However, plasma levels did not return to normal until one month on therapy with high doses of zinc. Zinc plasma levels are not a good indicator of zinc stores in the body and many times their changes are a late indicator of zinc deficit. There is not a correlation between the clinical improvement and normalization of zinc levels, being common the clinical resolution within few days of the supplementation without observing an increase of zinc plasma levels (AU)

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.

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.

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.

Deficiencia de zinc sintomática en prematuros alimentados con lactancia materna / Symptomatic zinc deficiency in breast-fed infants

La deficiencia sintomática de zinc en prematuros alimentados con lactancia materna exclusiva es un trastorno adquirido cuyas manifestaciones clínicas pueden ser similares a las de la acrodermatitis enteropática (AE) hereditaria. Los síntomas remiten tras la administración de suplementos orales de zinc. Caso clínico: lactante, varón, de 4 meses de edad, con antecedentes de prematuridad, que fue alimentado durante 8 días con nutrición parenteral en el periodo neonatal y posteriormente con lactancia materna exclusiva. A los 2 meses de vida presentó diarrea persistente, vómitos y lesiones cutáneas eczematosas, de predominio acro y periorificial. Los exámenes complementarios revelaron bajos niveles de zinc en el suero del lactante (3µmol/l) y en leche materna (8µmol/l), con niveles normales de zinc en suero materno (17µmol/l). Se efectuó tratamiento con sulfato de zinc oral (2mg/Kg), remitiendo los síntomas sin recidivas posteriores. Conclusión: El reconocimiento de la deficiencia sintomática de zinc en prematuros hijos de madre con leche deficitaria en zinc es importante, ya que el tratamiento es eficaz y definitivo. La nutrición parenteral sin suplementos de zinc puede agravar el déficit. En los lactantes de riesgo es recomendable determinar niveles séricos de zinc

Symptomatic zinc deficiency in breast-fed infants is a severe disorder, caused by a low level of zinc in mother’s milk. Premature infants are more vulnerable to develop zinc deficiency than full-term infants. The clinical symptoms resemble those of hereditary acrodermatitis enterophatica (AE). In acquired zinc deficiencies the disorder is transient and patients respond well to oral zinc supplements. Clinical case: A 4 month-old exclusively breast-fed premature infant presented with persistent diarrhea, vomiting and eczematoid skin lesions, with acral and peri-orificial dermatitis resembling AE. The symptoms began at 2 months of age. She received parenteral nutrition for the first eight days followed by mother’s milk exclusively. Laboratory investigations revealed lowered zinc levels in the infant’s serum (3µmol/l) and in her mother’s milk (8µmol/l), with normal level of zinc in the mother’s serum (17µmol/l). Healing was rapid after oral zinc supplementation (zinc sulphate 2mg/Kg), with complete remission of the symptoms. Conclusions: Recognition of zinc deficiency in premature newborns to mothers who seem to secrete milk that is zinc- deficiency is essential because its treatment is effective. This may lead to severe symptomatic zinc deficiency in premature infants, requiring treatment with oral zinc supplement. Parenteral nutrition without zinc supplements may be a contributory factor to zinc deficiency in preterm infants. Serum zinc levels should be checked in at-risk babies

Peripheral neuropathy in acrodermatitis chronica atrophicans - effect of treatment.

Forty-seven patients with the late borrelial manifestation acrodermatitis chronica atrophicans (ACA) and with objective neurological and/or neurophysiological findings were followed up after antibiotic treatment with dermatological, serological, neurological and neurophysiological controls. Despite a good therapeutic effect on ACA lesions, specific antibody values and symptoms of irritative nerve lesions, the objective neurological and neurophysiological findings of nerve deficit remained unchanged. There was no progress of neuropathy findings during the follow-up time. Our interpretation of the results is that the remaining neuropathy signs after treatment of ACA are neurological sequelae and not manifestations of persisting Borrelia infection.

Polyneuropathy in late Lyme borreliosis - a clinical, neurophysiological and morphological description.

In a prospective study, detailed clinical and neurophysiological examinations were performed in 17 patients with polyneuropathy associated with the late borrelial manifestation acrodermatitis chronica atrophicans (ACA). Similar clinical and neurophysiological signs were found in most of the patients. The findings were those of a sensory polyneuropathy, mainly affecting large nerve fibres. Marked abnormality of vibration threshold was a common finding and in 4 patients this raised a suspicion of spinal cord engagement, in addition to a polyneuropathy. Sural nerve biopsy, performed in 3 of the patients, showed a mainly axonal neuropathy. Biopsy findings did not confirm earlier reports of vasculitis of epineural vessels in ACA-associated polyneuropathy.

Acrodermatitis chronica atrophicans: histopathologic findings and clinical correlations in 111 cases.

We studied 111 consecutive, untreated and serologically confirmed patients with acrodermatitis chronica atrophicans. Emphasis was on the histopathologic patterns of erythematous and fibrous lesions, and on an assay used to correlate histopathologic findings with such clinical features as fibrous nodules, ulnar bands and the pain reaction allodynia. There was a significant correlation between allodynia and signs of marked inflammation, but not between allodynia and neural and perineural cell infiltrates or fibrosis. Moreover, there was no significant correlation between serum IgG titers to Borrelia and the density of inflammatory cell infiltrates or the proportion of plasma cells in tissue. Histopathologic examination did not reveal any important differences between fibrous nodules, ulnar bands and sclerodermatous lesions. The histopathologic pattern is not diagnostic per se, but characteristic enough to alert the experienced pathologist.

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.