Acquired Acrodermatitis Enteropathica Syndrome in a Kidney Transplant Receipt: A Case Report.

Acrodermatitis enteropathica syndrome (AE) is a clinical entity that results in severe zinc deficiency. It can be genetic or acquired. Acquired AE has been reported in patients with chronic liver disease, malabsorption syndrome, sickle cell anemia, and chronic renal failure. We present a kidney transplant recipient with skin rash and watery diarrhea. The patient had low serum zinc levels, which quickly resolved after zinc supplementation. Skin biopsy showed cytoplasmic pallor and vacuolization and ballooning degeneration of keratinocytes within the superficial epidermis, which may have led to confluent necrosis of keratinocytes. Large amounts of keratinosome-derived lamellae were found in the intercellular spaces in the keratinized area, probably related to disturbance of keratinosome metabolism due to zinc deficiency.

The dermatology hospitalist: creating value by rapid clinical pathologic correlation in a patient-centered care model.

BACKGROUND: The active and continuous presence of dermatologists in hospitals has undergone continued involution over the past two decades. Our patient-centered, value-based dermatology hospitalist model describes an efficient system for the integration of the dermatologist in the hospital treatment team. METHODS: We describe five difficult inpatient cases to illustrate the value of dermatology intervention and clinical pathologic correlation in facilitating timely diagnosis and treatment. RESULTS: Prompt specialty evaluation and clinicopathologic correlation by hospital dermatologists led to decreased morbidity and the avoidance of delay in initiating definitive treatment. CONCLUSIONS: Efficient evaluation and clinicopathologic correlation by dermatology hospitalists are essential to hospitals that provide comprehensive care. This value-based model has the potential to produce better patient outcomes and greater satisfaction in both patients and other health care providers.

Disseminated hypertrophic lichen planus: relevant response to acitretin.

Hyperthrofic lichen planus is considered a variant of lichen planus with marked epidermal hyperplasia in response to persistent itch. It is clinically, characterized by symmetric hyperkeratotic plaques, of purplish-grey color, often located in the pretibial region. Intense pruritus, refractoriness to conventional treatments and the possibility of association of the long-term injuries with squamous cell carcinoma requires an effective treatment. The first-line treatment is corticosteroids which can be applied either topically or systemically. Other therapeutic modalities proposed are: NB-UVB phototherapy or PUVA, immunosuppressive drugs and systemic retinoids, notably acitretin. We report a case with exuberant clinical presentation of hyperthrofic lichen planus with excellent response to acitretin after nine months of treatment.

Líquen plano hipertrófico disseminado: relevante resposta à acitretina / Disseminated hypertrophic lichen planus: relevant response to acitretin

O líquen plano hipertrófico é uma variante do líquen plano, com pronunciada hiperplasia epidérmica em resposta à coçadura persistente. Clinicamente, caracterizam-se por placas hiperceratósicas, simétricas, de coloração cinza-violácea, com predileção pela região pré-tibial. O prurido intenso, a refratariedade aos tratamentos convencionais e a possibilidade de associação de um carcinoma epidermoide às lesões de longa duração impõem um tratamento eficaz. Os corticoides são considerados o tratamento de primeira linha e podem ser aplicados topicamente ou empregados de forma sistêmica. Outras modalidades terapêuticas propostas são a fototerapia com UVB-NB ou PUVA, imunossupressores e retinoides sistêmicos, com destaque para a acitretina. Relatamos um caso com apresentação clínica exuberante e excelente resposta à acitretin, totalizando um seguimento de nove meses.

Hyperthrofic lichen planus is considered a variant of lichen planus with marked epidermal hyperplasia in response to persistent itch. It is clinically, characterized by symmetric hyperkeratotic plaques, of purplish-grey color, often located in the pretibial region. Intense pruritus, refractoriness to conventional treatments and the possibility of association of the long-term injuries with squamous cell carcinoma requires an effective treatment. The first-line treatment is corticosteroids which can be applied either topically or systemically. Other therapeutic modalities proposed are: NB-UVB phototherapy or PUVA, immunosuppressive drugs and systemic retinoids, notably acitretin. We report a case with exuberant clinical presentation of hyperthrofic lichen planus with excellent response to acitretin after nine months of treatment.

Comparison of clobetasol propionate cream plus coal tar vs. topical psoralen and solar ultraviolet A therapy in palmoplantar psoriasis.

AIM: Palmoplantar psoriasis (PPP) produces significant morbidity and requires prompt treatment. Topical agents form the mainstay of therapy. We compared the efficacy and side-effect profile of a steroid/coal-tar combination with topical psoralen and solar ultraviolet A (PUVAsol) in PPP. METHODS: In total, 52 patients with PPP were randomized to receive either a combination of clobetasol propionate cream and coal tar daily (group 1) or topical PUVAsol on alternate days (group 2) for 16 weeks. Response was assessed as change in Psoriasis Activity and Severity Index (PASI) and Patient Global Assessment (PGA). RESULTS: Of the 52 patients, 43 completed the treatment phase. There was a reduction in PASI for the palms and soles in both treatment groups throughout the treatment period until week 16. There was a greater reduction in PASI in palmar psoriasis with topical PUVAsol, and a greater reduction in psoriasis of the soles with the steroid/coal-tar combination. In both groups, patients perceived 'good improvement'. Improvement or cure in palmar lesions was observed in 90% of cases in the topical steroid/coal-tar group and in 75% of cases in the topical PUVAsol group; for the soles, these figures were 76% and 79%, respectively. No adverse effects were experienced with the steroid/coal-tar combination, whereas for the topical PUVAsol, phototoxicity occurred in 22% of cases. CONCLUSION: Both treatments had comparable efficacy. In both groups, patients experienced 'good improvement' after 16 weeks of therapy.

Newer trends in the management of psoriasis at difficult to treat locations: scalp, palmoplantar disease and nails.

Psoriasis is a common, chronic, inflammatory disease with a wide range of clinical presentations. The disease severity ranges from mild to severe. Plaque type of psoriasis is the most common. A number of factors like previous treatment history and comorbid conditions influence the treatment of psoriasis in an individual patient. Location of the lesions is also an important consideration. Psoriasis localized to certain areas of the body like scalp, nails, palms and soles remains difficult to treat. These sites have been referred to as the difficult locations in literature. This article covers the management of psoriasis limited to these special areas.

Hypertension and hand-foot skin reactions related to VEGFR2 genotype and improved clinical outcome following bevacizumab and sorafenib.

BACKGROUND: Hypertension (HT) and hand-foot skin reactions (HFSR) may be related to the activity of bevacizumab and sorafenib. We hypothesized that these toxicities would correspond to favorable outcome in these drugs, that HT and HFSR would coincide, and that VEGFR2 genotypic variation would be related to toxicity and clinical outcomes. METHODS: Toxicities (> or = grade 2 HT or HFSR), progression-free survival (PFS), and overall survival (OS) following treatment initiation were evaluated. Toxicity incidence and VEGFR2 H472Q and V297I status were compared to clinical outcomes. RESULTS: Individuals experiencing HT had longer PFS following bevacizumab therapy than those without this toxicity in trials utilizing bevacizumab in patients with prostate cancer (31.5 vs 14.9 months, n = 60, P = 0.0009), and bevacizumab and sorafenib in patients with solid tumors (11.9 vs. 3.7 months, n = 27, P = 0.052). HT was also linked to a > 5-fold OS benefit after sorafenib and bevacizumab cotherapy (5.7 versus 29.0 months, P = 0.0068). HFSR was a marker for prolonged PFS during sorafenib therapy (6.1 versus 3.7 months respectively, n = 113, P = 0.0003). HT was a risk factor for HFSR in patients treated with bevacizumab and/or sorafenib (OR(95%CI) = 3.2(1.5-6.8), P = 0.0024). Carriers of variant alleles at VEGFR2 H472Q experienced greater risk of developing HT (OR(95%CI) = 2.3(1.2 - 4.6), n = 170, P = 0.0154) and HFSR (OR(95%CI) = 2.7(1.3 - 5.6), n = 170, P = 0.0136). CONCLUSIONS: This study suggests that HT and HFSR may be markers for favorable clinical outcome, HT development may be a marker for HFSR, and VEGFR2 alleles may be related to the development of toxicities during therapy with bevacizumab and/or sorafenib.

Paring and intense pulsed light versus paring alone for recalcitrant hand and foot warts: a randomized clinical trial with blinded outcome evaluation.

BACKGROUND AND OBJECTIVES: Treatment of recalcitrant viral warts remains a therapeutic challenge. Intense pulsed light (IPL) has been suggested effective to clear wart tissue. The objective was in a randomized controlled trial to assess the efficacy of paring followed by IPL versus paring alone for recalcitrant hand and foot warts. MATERIALS AND METHODS: Eighty-nine patients with recalcitrant hand and foot warts were included and randomized (1:1) to three treatments at 3-week intervals with either paring of warts followed by IPL or paring of warts alone. IPL was given with the Ellipse Flex IPL system (Danish Dermatologic Development A/S, Hørsholm, Denmark, 400-950 nm, 5.5 millisecond pulse duration in double pulses with a 2 millisecond interval, 26.0-32.5 J/cm(2) repetitive passes). The primary outcome was complete and partial clearance of warts evaluated by blinded photo assessment at 6 weeks after final treatment. Secondary outcomes were treatment related pain and adverse reactions. RESULTS: We found no significant difference in clearance of warts between the two intervention groups (OR 1.64, 95% confidence interval 0.62-4.38). Paring followed by IPL resulted in complete or partial clearance of wart tissue in nine (22%) and five patients (12.2%) versus five (13.5%) and four patients (10.8%) from paring alone. Mostly plantar warts were treated (92.1%). The pain intensity after paring and IPL was moderate and significantly higher than the pain intensity after paring alone (P<0.0005). No adverse reactions were observed from the two interventions. CONCLUSION: Paring followed by IPL did not differ significantly from paring alone in clearance of recalcitrant hand and foot warts but caused significantly more pain.

Efficacy of topical PUVA soaks for palmoplantar dermatoses: an audit.

BACKGROUND: With a lack of evidence base for individual topical PUVA protocols, treatment is presently based on the consensus of current practice. This audit was designed to investigate the effectiveness of topical PUVA for palmoplantar dermatoses. METHODS: Phototherapy notes were reviewed on all patients who received hand and/or foot PUVA 2002-2007 in the Northern Health and Social Care Trust (NHSCT), Northern Ireland. RESULTS: Thirty patients met the inclusion criteria for the study. The mean number of treatments, maximum single UVA dose, and cumulative dose, were 18.4, 4.2 J/cm2, and 48.3 J/cm2, respectively. A positive response to treatment occurred in 51.3% of patients, which fell short of the 70% standard set. In a multivariate logistic regression analysis, number of treatments (P=0.04) and maximum single UVA dose (P=0.03) were the only variables associated with positive treatment outcome. The response was not influenced significantly by skin type, concurrent topical treatments, or cumulative UVA dose. Limitations to the study: Small patient numbers may have prevented the statistical significance of individual variables. CONCLUSIONS: UV dose increments should be clearly defined to avoid excess caution at the expense of an adequate patient response, and a minimum of 20 treatments administered to all patients, if tolerated.

Hand-foot skin reaction in patients treated with sorafenib: a clinicopathological study of cutaneous manifestations due to multitargeted kinase inhibitor therapy.

BACKGROUND: Hand-foot skin reaction is a distinctive cutaneous side-effect of antineoplastic kinase inhibitor-targeted therapy. Severe hand-foot skin reaction requires postponement of treatment or dose reduction. Histopathological studies of skin toxicity associated with kinase inhibitors are currently unavailable. OBJECTIVES: To report the clinical and histopathological findings of hand-foot skin reaction produced by the multikinase inhibitor sorafenib. METHODS: Nine patients with metastatic carcinoma-seven with renal cell carcinoma (RCC), one with melanoma and one with hepatocellular carcinoma (HCC)-received continuous, oral sorafenib 400 mg twice daily. Hand-foot skin reaction was defined and graded according to National Cancer Institute Common Toxicity Criteria 3.0. Biopsies from lesions of erythematous scaly or blistering skin were obtained from five cases (four RCC and one HCC). RESULTS: Seven of the nine (78%) patients developed hand-foot skin reaction characterized by well-demarcated, tender, erythematous papules and plaques with greyish blisters or hyperkeratotic, callus-like formations on palmoplantar surfaces and distal phalanges. Skin biopsy of hand-foot skin reaction lesions revealed epidermal acanthosis, papillomatosis, parakeratosis, dispersed dyskeratotic cells and keratinocyte vacuolar degeneration. Other skin toxicities included angular cheilitis, seborrhoeic dermatitis and perianal dermatitis. CONCLUSIONS: The clinical manifestations and histopathological features of sorafenib-induced skin reactions are unique. The most relevant histopathological findings of hand-foot skin reaction include keratinocyte vacuolar degeneration, the presence of intracytoplasmic eosinophilic bodies, and intraepidermal blisters in the stratum malpighii. Further studies are warranted to elucidate the mechanisms of this novel multitargeted kinase inhibitor-associated skin reaction.

Onychomycosis by Scytalidium dimidiatum in green tea leaf pluckers: report of two cases.

Two cases of onychomycosis in green tea leaf pluckers caused by Scytalidium dimidiatum, a dematiaceous non-dermatophytic mold has been described. Onychomycosis caused by Scytalidium dimidiatum is clinically indistinguishable from that caused by dermatophytes and responds poorly to antifungals. The clinical presentations in the cases were of distal subungual type with associated melanonychia involving thumbnails. These cases have unique importance, because besides disfigurement, this has adversely affected the livelihood of the patient and poor response to antifungal dissuaded them from treatment compliance. To the best of our knowledge, this is the first report of onychomycosis caused by Scytalidium dimidiatum in green tea leaf pluckers.

Comparison of the efficacy of local narrowband ultraviolet B (NB-UVB) phototherapy versus psoralen plus ultraviolet A (PUVA) paint for palmoplantar psoriasis.

Palmoplantar psoriasis is an idiopathic disabling condition, often resistant to conventional therapies. The purpose of this study was to evaluate the efficacy and safety of local narrowband ultraviolet B (NB-UVB) phototherapy and to compare it with local psoralen plus ultraviolet A (PUVA) paint in patients with palmoplantar psoriasis unresponsive to conventional therapies other than phototherapy. A cohort of 25 patients with palmoplantar psoriasis were included in this study, which was based on a left-to-right comparison pattern. The treatments were administered with local narrowband UVB irradiation on one side and local PUVA on the other side three times a week over 9 weeks. Clinical assessments were performed at baseline and every 3 weeks during the 9-week treatment. There was a statistically significant decrease in the mean clinical scores at the third, sixth and ninth week with both treatments. The difference in clinical response between the two treatment modalities was statistically significant at the end of the treatment period, with the percentage reduction in severity index scores with the PUVA-paint-treated side being 85.45% compared with 61.08% for the NB-UVB treated side (t = 5.379, P = 0.0001, Student's t-test for unpaired samples). Our results show that, although some clinical improvement was achieved with local NB-UVB phototherapy, the results were better with local PUVA, and such a treatment option may be reserved for patients with palmoplantar psoriasis who experience phototoxic reaction to psoralens.

Pápulas queratósicas acrales / Acral keratotic papules

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UVA1 phototherapy for cutaneous diseases: an experience of 92 cases in the United States.

BACKGROUND: The efficacy and safety of UVA1 (340-400 nm) phototherapy were established by studies from European countries. PURPOSE: Evaluate experience with UVA1 phototherapy for patients with cutaneous diseases in the United States. METHODS: A retrospective analysis of 92 cases of UVA1-treated cutaneous conditions from four medical centers in the United States was performed. RESULTS: Two-third of the patients showed a fair to good response (26-100% improvement) and one-third of the patients showed a poor response (0-25% improvement). Diseases with a moderate to good response (51-100% improvement) included scleredema adultorum, hand or foot dermatitis, atopic dermatitis, morphea (medium or medium- to high-dose UVA1), systemic sclerosis, and urticaria pigmentosa. Besides tanning, other adverse effects were found in 15% of patients, which include pruritus, erythema, tenderness, and burning sensation. Patients with skin types I-III responded better that those with a darker skin type. CONCLUSION: UVA1 phototherapy is a useful and well-tolerated treatment option for a variety of skin conditions.