Hemodialysis-related pruritus and associated cutaneous manifestations

BACKGROUND: Uremic pruritus is one of the most common disabling problems in patients with chronic renal failure. Few studies have evaluated itching and cutaneous manifestations in hemodialysis-dependent patients. OBJECTIVES: The aim of this prospective study was to identify the prevalence of pruritus and cutaneous changes affecting patients undergoing hemodialysis. METHODS: The degree of itching in 70 patients treated at the Haemek Medical Center Hemodialysis Unit, in northern Israel, was scored according to presence and severity. We examined the relationship between the quality of dialysis and the frequency of pruritus, and identified concurrent cutaneous disorders. RESULTS: Pruritus was a common problem in the study cohort and affected 74.3% of hemodialysis patients at some point. The main characteristics of pruritus were a general pattern in 65.7% and mild intensity in 78.3% of observed patients. Duration of hemodialysis varied between 3 months and 13 years. There was no correlation between occurrence of pruritus and demographic or medical parameters (sex, type of kidney disease, regular medications or duration of hemodialysis) of the patients. Higher dialysis efficacy, as expressed by dialyser clearance, volume distribution of area, dialysis duration (Kt/v), may reduce the prevalence of pruritus (P < 0.02). None of the blood and chemical values considered (hemoglobin, creatinine, urea, phosphorus, calcium, albumin, parathormone and alkaline phosphatase) revealed any statistically relevant differences between pruritus groups. The appearance of foot ulcers was different between diabetic and nondiabetic individuals undergoing hemodialysis (P < 0.001). CONCLUSIONS: Pruritus is still a common problem in hemodialysis-dependent patients. The prevalence of xerosis and excoriations was high in patients undergoing replacement therapy. Efficient replacement hemodialysis may provide a clinical benefit.     

Icodextrin cutaneous hypersensitivity: report of 3 psoriasiform cases

BACKGROUND: Icodextrin is proposed as a new osmotic agent for use in peritoneal dialysis. Because of its recent use, adverse reactions are not well known. Cutaneous adverse effects have been described. We report 3 cases of cutaneous hypersensitivity to icodextrin and discuss the pathogenesis of this reaction. OBSERVATIONS: The cutaneous adverse reaction was psoriasiform in our 3 cases. The eruption was generalized with acute generalized exanthematous pustulosis in 1 case, and limited to the palms and soles in 1 case. It occurred 10 to 15 days after icodextrin therapy was initiated. In patient 1, the results of a rechallenge with icodextrin were positive. Icodextrin therapy was discontinued in all patients. CONCLUSIONS: Some cases of cutaneous reactions to icodextrin have been reported in the literature, but they are rare. As in our cases, most eruptions are psoriasiform, limited to the palms and soles, or extensive. Although the etiology is unclear, a hypersensitivity reaction, with the formation of immunocomplexes, is probable.     

Cutaneous manifestations in patients with chronic renal failure on hemodialysis

BACKGROUND: Chronic renal failure (CRF) presents with an array of cutaneous manifestations. Newer changes are being described since the advent of hemodialysis, which prolongs the life expectancy, giving time for these changes to manifest. AIM: The aim of this study was to evaluate the prevalence of dermatologic problems among patients with chronic renal failure (CRF) undergoing hemodialysis. METHODS: One hundred patients with CRF on hemodialysis were examined for cutaneous changes. RESULTS: Eighty-two per cent patients complained of some skin problem. However, on examination, all patients had at least one skin lesion attributable to CRF. The most prevalent finding was xerosis (79%), followed by pallor (60%), pruritus (53%) and cutaneous pigmentation (43%). Other cutaneous manifestations included Kyrle's disease (21%); fungal (30%), bacterial (13%) and viral (12%) infections; uremic frost (3%); purpura (9%); gynecomastia (1%); and dermatitis (2%). The nail changes included half and half nail (21%), koilonychia (18%), onychomycosis (19%), subungual hyperkeratosis (12%), onycholysis (10%), splinter hemorrhages (5%), Mees' lines (7%), Muehrcke's lines (5%) and Beau's lines (2%). Hair changes included sparse body hair (30%), sparse scalp hair (11%) and brittle and lusterless hair (16%). Oral changes included macroglossia with teeth markings (35%), xerostomia (31%), ulcerative stomatitis (29%), angular cheilitis (12%) and uremic breath (8%). Some rare manifestations of CRF like uremic frost, gynecomastia and pseudo-Kaposi's sarcoma were also observed. CONCLUSIONS: CRF is associated with a complex array of cutaneous manifestations caused either by the disease or by treatment. The commonest are xerosis and pruritus and the early recognition of cutaneous signs can relieve suffering and decrease morbidity.     

Nail disorders in patients with chronic renal failure and undergoing haemodialysis treatment: a case–control study

BACKGROUND: Few controlled studies have compared nail disorders in patients with chronic renal failure (CRF) and haemodialysis (HD)-dependent individuals with a healthy population. OBJECTIVE: The aim of this study was to compare the prevalence of nail disorders in patients with CRF and patients undergoing HD treatment with a healthy population, and evaluate the relationship between nail changes and various demographic, medical and laboratory parameters in these groups. METHODS: In this case-control study we recruited 73 patients affected with CRF, 77 patients undergoing regular HD and 77 healthy individuals. All patients were examined for the presence of nail disorders. Various parameters [age, gender, type of kidney disease, regular medications, duration of renal failure and HD, dialysis efficacy (Kt/v), haemoglobin, neutrophil count, calcium, phosphorus, albumin, creatinine, urea, alkaline phosphatase and parathyroid hormone (PTH) levels] of the patients were determined by multivariate analysis and compared. RESULTS: Forty-four patients (60.3%) with CRF and 48 patients (62.3%) undergoing HD treatment had at least one type of nail pathology. The most common nail alterations found in patients with CRF and those undergoing HD were absence of lunula (AL) and half-and-half nails (HHN), respectively. Prevalence of nail disorders among patients with CRF was influenced significantly by PTH level (P = 0.03). In the HD group, male sex, age above 65 years and comorbidities (diabetes mellitus, hypertension and heart failure) were significantly associated with nail pathologies. CONCLUSION: Patients with CRF and those undergoing HD therapy have higher rates of nail disorders when compared to a healthy population. Efficient HD does not improve nail changes.     

维持性血液透析终末期肾病患者皮肤病变调查分析

目的　调查进行维持性血液透析（maintenance hemodialysis, MHD）的终末期肾脏病患者皮肤病变的发病情况和病变特点，探讨其危险因素。方法　对181例MHD的终末期肾病患者进行皮肤体检；收集其临床和生化资料，分析皮肤病变的患病率，病变类型及其危险因素。结果　 ①181例患者中161例（88.95％）存在不同种类的皮肤病变，其中38例（23.60％）存在1种皮肤病变，52例（32.30％）有2种，40例（24.84％）有3种、26例（16.15％）有4种、5例（3.11％）有5种。②最常见是皮肤干燥（128/181， 77.72％），其次是瘙痒（101/181，55.80％）、脱屑（73/181，40.33％）和色素沉着（70/181，38.67％）。其他有皮肤苍白（6/181，3.31％）、瘀斑（4/181，2.21％）、指（趾）干性坏疽（3/181，1.66％）、足部溃疡（2/181，1.10％）、甲纵裂（1/181，0.55％）。③皮肤病变组患者的透析龄、超滤量、血磷、钙磷乘积、甲状旁腺激素、超敏C-反应蛋白（Hs-CRP）水平，乙肝、丙肝病毒标志物阳性率明显高于无皮肤病变组；而血红蛋白、血清尿素清除指数（Kt/V）明显低于无皮肤病变组。④Logistic回归分析结果显示，透析龄、KT/V、血钙磷乘积、血甲状旁腺激素、高敏C反应蛋白（Hs-CRP）和丙肝病毒标志物阳性是MHD患者发生皮肤病变的独立危险因素。结论MHD的终末期肾病患者皮肤病变的患病率高，以皮肤干燥症和皮肤瘙痒最常见。透析龄、KT/V、血钙磷乘积、血甲状旁腺激素、Hs-CRP和丙肝病毒标志物阳性是MHD终末期肾病患者发生皮肤病变的独立危险因素。     

Ulceration of the palms and soles. An unusual feature of cutaneous T-cell lymphoma

Two patients, one with Sézary syndrome and one with mycosis fungoides are described, in whom lesions on the palms and soles were associated with extensive ulceration and gave rise to diagnostic difficulty. Extensive ulceration of the palms and soles is uncommon; its presence should alert clinicians to the possibility of cutaneous T-cell lymphoma.     

Acquired perforating folliculitis in chronic renal failure: report of two cases

Background Patients with chronic renal failure, regardless of its cause, with/without hemodialysis/peritoneal dialysis, showed cutaneous alterations. Two patients had a follicular hyperkeratotic papular eruption, with clinical and histological features of perforating folliculitis. Methods A dermatological examination and multiple skin biopsies were done. Results We diagnosed acquired perforating folliculitis in two chronic renal failure patients on hemodialysis. The lesions were located predominantly on the lower extremities with hyperkeratotic papules and small nodules. Microscopically, the papule always affected the follicular structure. The follicle was widely dilated and packed with keratotic material, necrotic debris and degenerated basophilic coarse connective fibers passing which passed through follicular perforations. Conclusion Acquired perforating folliculitis is a cutaneous manifestation in chronic renal failure, with/without hemodialysis treatment, that can mimic essential primary perforating folliculitis. Transepidermal elimination mechanisms and the distinction between primary and acquired perforating dermatoses arc discussed.     

Palmoplantar lesions in leprosy.

Palms and soles are considered immune to leprosy. A study was carried out to assess the frequency of lesions over palms and soles and to correlate their occurrence with various parameters. Two hundred eighty leprosy patients were screened for lesions over palms and soles. Palmo-plantar lesions were observed in 10% of the patients screened. Slit-skin smears and biopsies were done from routine sites and palmo-plantar lesions. Histopathology and slit-skin smear confirmed the presence of disease. Eight were in type I reaction, and 50% of patients with type I reaction screened showed lesions over palms and/or soles. The reason for this is not known; probably inapparent lesions become apparent during reactions. Lesions of various morphology were observed. Silky hand was observed in one case.     

PIGMENTED MACULES ON PALMS AND SOLES IN PUERTO RICANS

Background. One-third of melanomas in Puerto Ricans occur on the skin of palms and soles. This is a study to define the characteristics of melanocytic nevi in those locations. Background. One-third of melanomas in Puerto Ricans occur on the skin of palms and soles. This is a study to define the characteristics of melanocytic nevi in those locations. Methods. A sample of 1,039 patients were randomly examined for pigmented macules on the palms and soles. After informed consent, biopsies of these lesions were done on 67 patients. Results. Among the patients, 13% were found to have pigmented macules on the palms and soles. Volar melanocytic macule was the most frequent lesion. Conclusions. Melanocytic nevi occur frequently on volar skin. The criteria for removal of these lesions should not be different from those lesions at other locations.     

Cardiac Myxoma Diagnosed by Signs of Purpuric Macules on Both Palms and Soles

Cardiac myxoma, the most prevalent primary cardiac tumor, is rare. The clinical features of this tumor are principally intracardiac obstruction, extracardiac embolism, and general symptoms including fever, myalgia, arthralgia. Although cutaneous manifestations in patients with cardiac myxoma are frequent, in rare cases, cutaneous signs have been clues to the correct diagnosis. We report a 42-year-old male who presented with recurrent multiple purpuric patches on both palms and soles for 4 months. Histopathological finding showed a myxomatous embolus in the arteriole in the lower dermis. Echocardiogram demonstrated the presence of a left atrial myxoma with a provisional diagnosis of left atrial myxoma. In our patient, skin examinations and histopathological finding led us to the diagnosis of cardiac myxoma.     

Punctate keratoses of the palms and soles and keratotic pits of the palmar creases

Punctate keratoses of the palms and soles and keratotic pits of the palmar creases are frequently confused. A prospective study of 283 patients revealed a prevalence of 11% and 3%, respectively, in a metropolitan county hospital dermatology clinic. Punctate keratoses of the palms and soles and keratotic pits of the palmar creases are similar in size, number of lesions per palm, aggravation by trauma, and predominance in blacks and in men. These entities are different in appearance, distribution, age at onset, prevalence, symptoms, and prognosis. Punctate keratosis of the palms and soles and keratotic pits of the palmar creases should be considered independent entities. To help eliminate confusion, we propose that punctate keratoses refer only to the hyperkeratotic papules scattered diffusely in the palms and occasionally in the soles and that keratotic pits of the palmar creases refer only the hyperkeratotic, conical depressions confined to the palmar creases.     

Superficial hyperkeratotic candidiasis on the palms and soles

This case report described a 59-year-old man with superficial hyperkeratotic candidiasis on the palms and soles. This rare form of cutaneous candidiasis is regarded as a complication of chronic mucocutaneous candidiasis or other immunodeficient disorders. However, the patient had no underlying disease and his cellular immunity was normal. The main abnormal findings in laboratory studies were markedly high IgE values and low vitamin A values. It was assumed that these played some role in the pathogenesis of the disease. The patient was cured through the oral administration of ketoconazole. Nine cases of superficial hyperkeratotic candidiasis on the palms have been reported in Japan, but the case given here is the first reported instance of this disease on the soles.     

PUVA-related punctate keratoses of the hands and feet

BACKGROUND: We have observed, in patients undergoing high-dose PUVA treatment, a type of keratosis not previously described. The lesions usually occur on the sides of the palms or soles and are clinically distinct. They are generally painless and often go unnoticed by patients. OBJECTIVE: We sought to further characterize these lesions both clinically and histologically. METHODS: Patients attending a PUVA clinic were screened for these keratoses. Other PUVA-related complications were recorded. Representative lesions were photographed, and biopsy specimens were taken. RESULTS: Biopsy specimens were taken from lesions in 10 patients. All had plaque psoriasis and had received high UVA doses (>1000 J/cm(2)) through PUVA therapy. All patients had PUVA-induced keratoses elsewhere, but the number varied greatly between patients. The hand and foot keratoses were well defined and circular and had a characteristic histologic appearance, with a sharp demarcation between normal and abnormal markedly pale-staining epidermis. CONCLUSION: These lesions are a further cutaneous manifestation of prolonged PUVA therapy.     

"I forgot to shave my hands": A case of spiny keratoderma

A 57-year-old Caucasian man presented with multiple asymptomatic spiny papules on the palms and soles that he had been shaving off with a razor for many years. He was otherwise healthy with no personal or family history of skin disease or malignancy. A diagnosis of spiny keratoderma of the palms and soles or "music box spine dermatosis" was made. The clinical, histologic, and electron-microscopic features of spiny keratoderma are distinct. This entity previously had multiple classifications and it is important to distinguish it from other keratodermas as some keratodermas can be linked to cutaneous and internal malignancies and conditions: polycystic kidney disease, liver cysts, Darier's disease, and hyperlipoproteinemia among others. Spiny keratodermas can have systemic associations and do not resolve spontaneously. Treatment is generally ineffective.     

Olmsted syndrome: report of a case with study of the cellular proliferation in keratoderma.

Olmsted syndrome is a rare disorder that consists of sharply marginated keratoderma of the palms and soles, constriction of digits and toes that may result in spontaneous amputation of the distal phalanges, hyperkeratotic plaques around the body orifices, onychodystrophy, and other less common cutaneous and extracutaneous anomalies. Although some patients had other affected family members, most cases of Olmsted syndrome seem to be of sporadic occurrence. We describe a patient with the characteristic features of Olmsted syndrome. The symptoms consisted of diffuse transgrediens palmoplantar keratoderma and keratotic plaques around the mouth and nose. Our patient also had the associated anomalies of hyperhidrosis of the palms and soles and congenital deaf-mutism. Histopathologic study of the keratoderma demonstrated epidermal hyperplasia with acanthosis, papillomatosis, and orthokeratotic hyperkeratosis.Immunohistochemical study showed more basal and suprabasal keratinocytes of the epidermis with immunoreactivity for Ki-67 marker when compared with the keratinocytes of the epidermis of the adjacent non-involved skin. These results support the notion that Olmsted syndrome is a hyperproliferative disorder of the epidermis.     

HL-A antigens in pustular psoriasis.

HL-A typing was performed on 97 patients with pustular psoriasis. HLA-B27 was found increased for the combined three subgroups: localized psoriasis of palms and soles, acrodermatitis continua and generalized pustular psoriasis, who were associated with a high incidence of arthritis. These subgroups have this in common with Reiter's disease indicating a link between the entities. In persistent palmo-plantar pustulosis an increased incidence of HLA-Bw35 was found. HLA-B13, HLA-B17 and HLA-Bw37 which are found markedly increased in psoriasis vulgaris were in acrodermatitis continua, generalized pustular psoriasis and persistent palmo-plantar pustulosis either absent or not increased as compared with the control population. 7 of 30 patients with localized psoriasis of palms and soles had one of these antigens. Our findings confirm that psoriasis vulgaris and pustular psoriasis as such, seem to be different aetiological entities. Some patients with localized psoriasis of palms and soles may be true psoriatics which besides their psoriasis have a tendency to develop a pustular reaction in their palms and soles similar to persistent palmo-plantar pustulosis.     

Palmoplantar keratoderma (PPK): acquired and genetic causes of a not so rare disease

Palmoplantar keratodermas (PPK) comprise a heterogeneous group of keratinization disorders with hyperkeratotic thickening of palms and soles. Sporadic or acquired forms of PPKs and genetic or hereditary forms exist. Differentiation between acquired and hereditary forms is essential for adequate treatment and patient counseling. Acquired forms of PPK have many causes. A plethora of mutations in many genes can cause hereditary PPK. In recent years several new causative genes have been identified. Individual PPK may be quite heterogeneous with respect to presentation and associated symptoms. Since the various hereditary PPK – like many other monogenic diseases – exhibit a very low prevalence, making of the correct diagnosis is challenging and often requires a molecular genetic analysis. Knowledge about the large but quite heterogeneous group of hereditary PPK is also important to dissect the molecular mechanisms of epidermal differentiation on palms and soles, ultimately leading to targeted corrective therapies in the future.     

Tripe Palms: A Cutaneous Marker of Internal Malignancy

Tripe palms is a distinctive paraneoplastic cutaneous sign which is frequently associated with internal malignancy, specially carcinomas of the lung and stomach. We describe a patient with ovary carcinoma who showed a curious rugose thickening of the palms with accentuation of the normal dermatoglyphic ridges. The lesions were a specially prominent on the fingertips. The patient also showed classical acanthosis nigricans in the axillae and groin. The soles were spared. Histopathologic findings in palmar lesions consisted of an undulant epidermis, with hyperkeratosis, acanthosis, and papillomatosis. Excision of the ovary carcinoma was followed by complete regression of the cutaneous lesions. We review the literature about tripe palms and discuss the relationship between this striking cutaneous manifestation and internal malignancy.     

Richner-Hanhart syndrome and tyrosinemia type II

A patient already published a case of Richner-Hanhart syndrome (RHS) (stabilized corneal lesions and hyperkeratotic lesions on the palms and soles) proved to be associated with tyrosinemia type II. 2 other cases (sister and brother) with only typical dermatologic features of RHS and tyrosinemia type II are described. The treatment with a low phenylalanine and tyrosine diet improves the cutaneous lesions in our 3 cases.