Acrodermatitis continua of Hallopeau treated successfully with ustekinumab and acitretin after failure of tumour necrosis factor blockade and anakinra.

Acrodermatitis continua of Hallopeau (ACH) is a rare form of chronic acral pustular eruption. Considered to be a variant of pustular psoriasis, it is a refractory condition that may not respond to conventional treatments. We report herein the case of a 53-year-old patient whose ACH was refractory to all conventional systemic treatment modalities and to anti-tumour necrosis factor. Because he had increased plasma levels of interleukin (IL)-1ß, he received anakinra for 7 weeks, without further improvement however. Achievement of complete response was obtained with ustekinumab 90 mg s.c. every 12 weeks combined with acitretin; the plasma level of IL-1ß concomitantly returned to normal. This case report is associated with a review on recent data on ACH treatment with biological agents, including anakinra and ustekinumab.

Development of a plasma zinc concentration cutoff to identify individuals with severe zinc deficiency based on results from adults undergoing experimental severe dietary zinc restriction and individuals with acrodermatitis enteropathica.

Plasma zinc concentration (PZC) is a recommended biomarker to assess zinc status and the risk of zinc deficiency in populations. However, the relation between PZC and clinical signs of zinc deficiency remains uncertain. These analyses were conducted to evaluate the relation between PZC and clinical signs of zinc deficiency and to determine a cutoff for PZC below which individuals would have an increased likelihood of having clinical signs associated with zinc deficiency. Electronic bibliographic searches were conducted of literature indexed in PubMed, Embase, CINAHL Plus, and EBSCO and related to experimental zinc depletion studies in adults and case reports in children and adults (ages <1 mo-43 y) with acrodermatitis enteropathica (AE). Data extracted included demographic characteristics, PZCs, and the presence or absence of clinical signs likely associated with zinc deficiency (e.g., dermatitis, diarrhea). Mean PZC was significantly lower among adults consuming severely zinc-restricted diets (<1 mg Zn/d) who developed clinical signs compared with those who remained asymptomatic (36.0 ± 16.8 vs. 67.9 ± 13.3 µg/dL, P < 0.034). Likewise, patients with AE had a lower mean PZC when symptomatic compared with post-treatment PZC when they were asymptomatic (38.2 ± 20.7 vs. 102 ± 34.7 µg/dL, P < 0.01). Among individuals with restricted dietary zinc intake, PZC predicted clinical signs with 82% sensitivity and 92% specificity when using a cutoff of 50 µg/dL. Among individuals with AE, PZC predicted clinical signs with 80% sensitivity and 89% specificity when applying a cutoff of 50 µg/dL. These analyses demonstrate a clear relation between PZC and the presence of clinical signs associated with zinc deficiency among presumably healthy individuals undergoing periods of dietary zinc restriction, as well as in individuals with AE, further validating the usefulness of PZC as a biomarker of severe zinc deficiency.

A diagnostic challenge: a case of acrodermatitis enteropathica without hypozincemia and with maternal milk of low zinc level.

Acrodermatitis enteropathica is a rare and distinct form of zinc deficiency with a requirement of life-long zinc supplementation and inherited in a recessive manner. Transient nutritional zinc deficiency is also a well known condition mimicking acrodermatitis enteropathica like skin changes in preterm and term infants who are generally breastfed with a low level of zinc containing milk. Here, a 4-month-old male, term and fully breastfed acrodermatitis enteropathica case without hypozincemia and with maternal milk of low zinc level is presented.

Acrodermatitis enteropathica from zinc-deficient total parenteral nutrition.

Acrodermatitis enteropathica (AE) is a rare autosomal-recessive disorder of zinc malabsorption, characterized by acral and periorificial dermatitis, alopecia, and diarrhea. Acquired AE is the result of decreased zinc intake, excessive zinc loss, or other malabsorptive processes. We present a case of a 54-year-old woman who developed characteristic skin lesions of acquired AE after zinc supplementation was removed from her total parenteral nutrition (TPN) solution. She was found to have hypozincemia and eventually exhibited prompt resolution of skin lesions after zinc was added to TPN. This case provides a unique opportunity to illustrate the direct correlation between decreased zinc intake and development of acquired AE.

Acrodermatitis enteropathica-like skin eruption in a case of short bowel syndrome following jejuno-transverse colon anastomosis.

Acrodermatitis enteropathica is a rare autosomal recessive disorder of zinc deficiency. Zinc is an essential trace element in human metabolism and acquired zinc deficiency may manifest with skin eruptions simulating acrodermatitis enteropathica. We report an unusual case of acrodermatitis enteropathica-like skin eruption due to deficiency of zinc and other nutritional factors in a patient who has undergone extensive small bowel resection and jejuno-transverse colon anastomosis for mesenteric ischemia.

Acrodermatitis Enteropathica: A Case Report.

Acrodermatitis enteropathica is a rare genetic autosomal recessive disorder, characterized by periorificial dermatitis, alopecia, and diarrhea. It is caused by mutations in the gene that encodes a membrane protein that binds zinc. We report a 14-month-old boy, admitted for erythematous, scaly and pustular lesions, initially located in the inguinal and perianal regions and on thighs, and very few erythematous lesions on the face. Due to the numerous bacterial skin superinfections with Staphylococcus aureus, including abscesses that required surgical incision, the clinical picture was modified, leading to a delayed establishment of the diagnosis. Later, the symptoms became suggestive for this disease, the diagnostic having been confirmed by low plasma zinc values. Under zinc therapy, skin lesions improved significantly in a few days, with favorable outcome. Two months later, the skin lesions almost disappeared.Abscesses due to bacterial skin superinfections may lead to initially misdiagnosed acrodermatitis enteropathica.

Overview of Inherited Zinc Deficiency in Infants and Children.

Zinc nutrition is of special practical importance in infants and children. Poor zinc absorption causes zinc deficiency, which leads to a broad range of consequences such as alopecia, diarrhea, skin lesions, taste disorders, loss of appetite, impaired immune function and neuropsychiatric changes and growth retardation, thus potentially threatening life in infants and children. In addition to dietary zinc deficiency, inherited zinc deficiency, which rarely occurs, is found during the infant stage and early childhood. Recent molecular genetic studies have identified responsible genes for two inherited zinc deficiency disorders, acrodermatitis enteropathica (AE) and transient neonatal zinc deficiency (TNZD), clarifying the pathological mechanisms. Both of these zinc deficiencies are caused by mutations of zinc transporters, although the mechanisms are completely different. AE is an autosomal recessive disorder caused by mutations of the ZIP4 gene, consequently resulting in defective absorption of zinc in the small intestine. In contrast, TNZD is a disorder caused by mutations of the ZnT2 gene, which results in low zinc breast milk in the mother, consequently causing zinc deficiency in the breast-fed infant. In both cases, zinc deficiency symptoms are ameliorated by a daily oral zinc supplementation for the patients. Zinc is definitely one of the key factors for the healthy growth of infants and children, and thus zinc nutrition should receive much attention.

[Skin disorder as an indication of viral infection]. / Huidafwijking als aanwijzing voor virusinfectie.

BACKGROUND: Gianotti-Crosti syndrome (GCS) is a dermatosis primarily affecting children between 2 and 6 years of age. It is characterized by multiple, itching, monomorphous, pink to red-brown papules or papulovesicles between 1 and 5 mm in size. GCS is associated with viral infections such as hepatitis B virus and Epstein-Barr virus (EBV). It is often accompanied by fever, hepatosplenomegaly and lymphadenopathy. The syndrome is self-limiting and disappears within 2 months. No treatment is therefore required. CASE DESCRIPTION: We report a case of a 16-year-old female who presented with itching papulovesicles on the extremities and on the buttocks. She was diagnosed with GCS because of the clinical findings, histopathological tests and positive EBV serology. CONCLUSION: GCS can also affect patients older than 6 years of age. Moreover, this syndrome may be a first sign of infection with Epstein-Barr virus, hepatitis B virus, HIV or other viral infections.

Normal proliferative responses of peripheral blood mononuclear cells to streptococcal preparation OK-432 in patients with pustulosis palmaris et plantaris constitute a distinct feature from the reduced responses observed in those with psoriasis vulgaris, pustular psoriasis, and acrodermatitis continua of Hallopeau.

It was previously reported that peripheral blood mononuclear cells (PBMC) from the patients with psoriasis vulgaris (PV) showed a reduced proliferative response in vitro to the stimulation of a lyophilized preparation of penicillin-treated low virulence Su-strain of Streptococcus pyogenes group 3, OK-432. In this study, at first it was examined whether OK-432 acts as a superantigen. By analyzing the usage of Vbeta T-cell receptor (TCR) of proliferating T cells stimulated with OK-432, it was found that OK-432 stimulated preferentially Vbeta2 TCR-bearing T cells. Next, to find differences in in vitro responses of PBMC among various types of sterile pustular dermatoses such as pustulosis palmaris et plantaris (PPP), acrodermatitis continua of Hallopeau (AC), and generalized pustular psoriasis (GPP), the proliferative responses of PBMC obtained from these patients under the stimulation of OK-432 were compared. When the PBMC was stimulated with interleukin (IL)-2, no significant difference was found in their proliferative responses among those obtained from the patients with these sterile pustular dermatoses, PV or healthy controls. However, like those from PV patients, PBMC from AC and GPP patients showed significantly smaller responses to OK-432 than those from the healthy controls. In contrast, the proliferative responses of PBMC from the patients with PPP to OK-432 was comparable to those from healthy controls. These results, in addition to its unique clinical and histopathological characteristics, suggest that PPP has a different pathogenetic background from that underlying PV, AC, or GPP.

Gluconate zinc in acrodermatitis enteropathica.

Two children with characteristic findings of acrodermatitis enteropathica were found to have low serum zinc levels prior to therapy. Treatment with zinc gluconate in low dosages resulted in an increase in serum zinc levels and clinical improvement. All family members studied had low serum zinc levels. In addition, we noted a change of hair color to red and excessive drooling just prior to exacerbations in these two patients. The hair color returned to normal with remission.

Methylmalonic acidemia, cobalamin C type, presenting with cutaneous manifestations.

BACKGROUND: Erosive dermatitis resembling the skin lesions of acrodermatitis enteropathica has been described in a number of aminoacidopathies and organic acidemias. In some, the dermatitis is a manifestation of untreated disease, while in others, including methylmalonic acidemia, skin lesions have been ascribed to nutritional deficiency due to therapeutic amino acid restrictions. OBSERVATIONS: We report 2 cases of methylmalonic acidemia presenting with cutaneous manifestations in the perinatal period before restrictive nutritional interventions. The cutaneous involvement consisted of cheilitis and diffuse erythema with erosions and desquamation. Methylmalonic acidemia, cobalamin C type, was subsequently diagnosed in both cases. CONCLUSIONS: An erosive, desquamating dermatitis with histopathologic characteristics resembling acrodermatitis enteropathica may be a presenting sign in cobalamin C methylmalonic acidemia, even in the absence of long-standing nutritional restrictions or deficiency.