Palmar Erythema Research Articles

Frequency of Cutaneous Manifestations in Patients of Hepatitis C Virus Infection

Objective: To determine the frequency of cutaneous manifestations of hepatitis C infection in patients pre-senting in a tertiary care hospital. Study Design: Descriptive study. Place and Duration of Study: Department of Derma-tology, King Edward Medical University / Mayo Hospital, Lahore, from 1 st January, 2009 to 30 th June, 2009. Methodology: A descriptive (observational) study was conducted in the hepatitis C virus (HCV) infected patients, attending the Skin Department during a period of six months. The patients were diagnosed as HCV positive by enzyme-linked immunosorbent assay (ELISA) and / or polymerase chain reaction (PCR). A thorough medical history was taken and a detailed cutaneous examination was carried out in every pat-ient. Results: A total of 180 patients were enrolled. Out of them, 95 (52.8%) were males and 85 (47.2%) females. Most of the patients were in the age group of 21 – 30 years. Thirty five (19.4%) patients were taking treat-ment of hepatitis C. Pruritus was the most common dermatological feature seen in 33.8%, followed by lichen planus in 27.2%. Less common manifestations noted were utricaria (7.8%), vitiligo (5.6%), mixed cryoglobulinemia (4.4%), erythema nodosum (2.8%), erythema multiforme (2.2%), porphyria cutanea tarda (1.1%) and necrolytic acral erythema (1.1%). The signs of chronic liver disease (palmar erythema, jaun-dice, spider naevi, telangiectasia, leukonychia) were found in 13.8% of patients. Conclusion: Hepatitis C virus infection is associated with a number of extra-hepatic cutaneous manifesta-tions which may help to identify the silent cases of this grave disease. Key words: Hepatitis C Virus, Cutaneous manifesta-tions, Pruritus, Lichen planus

Different patterns of dermatological presentations in patients co-infected with human immunodeficiency virus and hepatitis C virus (HCV), and those infected with HCV alone

Hepatitis C virus (HCV) and human immunodeficiency virus (HIV) share the same transmission routes. About 30% of HIV-positive patients are co-infected with HCV. Of the various HCV-related extrahepatic events, those involving the skin may be the first sign of infection. To specify the skin presentations in patients co-infected with HIV and HCV (co-infected patients; CP) and compare them with those found in patients with HCV mono-infection (mono-infected patients; MP). This was a cross-sectional study, in which the studied population consisted of MP and CP from a tertiary hospital in the South of Brazil, who underwent complete skin examination and laboratory tests. In total, 201 patients were assessed, of whom 108 were CP, and 93 were MP. Pruritus tended to be more common in MP. MP also had significantly more dermatological conditions (mean of 5.2) than CP (mean of 4.5). In total, 104 different skin diseases were identified. There was a higher prevalence of infectious diseases and pigmentation disorders, such as verruca vulgaris and facial melasma, in CP, whereas trunk and facial telangiectasias, palmar erythema, and varicose veins were more common in MP. We found a high prevalence of skin conditions both in MP and in CP; however, the patterns of the dermatological conditions were different. CP were found to have significantly fewer skin lesions than MP, but had a higher prevalence of infectious and pigmentation disorders. By contrast, vascular conditions were more common in MP.

Recent developments in the specific dermatoses of pregnancy

During pregnancy, the mother undergoes changes to sustain and enable normal growth and development of the fetus. Common physiological changes include linea nigra, fibroepithelial polyps, striae, spider angioma, palmar erythema and pruritis gravidarum. However, there are some changes that are purely pathological, and these are termed the pregnancy-specific dermatoses (PSDs). The PSDs occur during pregnancy or in the immediate postpartum period. They do not include the various benign conditions or pre-existing dermatoses and tumours that may present or worsen with pregnancy. They do include a number of distinct and identifiable conditions: atopic eruption of pregnancy (AEP), polymorphic eruption of pregnancy (PEP), intrahepatic cholestasis of pregnancy (ICP) and pemphigoid gestationis (PG). These are a heterogeneous group of skin conditions characterized by pruritis and inflammatory changes. In addition, pruritis gravidarum is sometimes considered pathophysiological and thus part of this group, rather than a physiological process. Each of these conditions has a distinct, but not fully understood, pathogenesis. The mechanisms leading to PSD may be a reflection on the hormonal and immunological changes associated with pregnancy. AEP and PEP are benign conditions, and although they can cause distress to the mother, they are otherwise minor. However, ICP and PG are more serious conditions, and both carry the potential for serious risks to both the mother and the fetus. Thus, the pathophysiology of these latter two conditions is considered in more detail in the following article.

Mysterious Foot Lesions in a Healthy Eight Year Old

Mysterious Foot Lesions in a Healthy Eight Year Old

Caput medusae in alcoholic liver disease

Caput medusae and palmar erythema are cardinal signs in cirrhosis of liver with portal hypertension. Palmar erythema is described more often as a marker for alcoholic etiology of chronic liver disease. The peripheral stigmata of chronic liver disease are not routinely seen now a days due to early diagnosis and better therapy. We recently encountered an interesting patient of alcoholic liver disease with two classical signs of the disease and report the same for this unusual presentation.

小児日本紅斑熱9 例の臨床的検討

To clarify the clinical manifestations of pediatric Japanese spotted fever (JSF), which remain unclear, we retrospectively reviewed the records of 9 consecutive hospitalized children 5 boys and 4 girls aged 0-15 years (median: 2.3) whose diagnosis was patients with JSF who were serologically confirmed from April 2008 to October 2009. We initially studied the polymerase chain reaction (PCR) assay validity for specific Rickettsia japonica DNA in the blood. We also studied febrile duration, the history of contact with tick-infested areas, body temperature, eschars at tick bite sites, skin rash, treatment drugs, and laboratory data. Five of the 9 (56%) had positive PCR tests. Prehospitalization febrile duration was 1-5 days. Five had had contact with tick-infested areas and 4 had not despite living near such areas. Body temperature was 40 degrees C in 7. Only 4 had eschars at bite sites. Characteristic spotted palmar and/or plantar erythema seen in 8 was useful in diagnosis. Laboratory studies showed typical hyponatremia of < 135mEq/L in 6 JSF was diagnosed easily at hospitalization in 7. Diagnosis in a 2-month-old infant proved difficults, however, worsening the child's condition and causing hepatosplenomegaly, thrombocytopenia, anemia, and hyperferritinema. The infant was treated with high-dose gamma-globulin and azithromycin (AZM) followed by minocycline (MINO). Another case was difficult to diagnose due to clinical manifestations consistent with Kawasaki disease. The child was treated with high-dose gamma-globulin and AZM. Three of the 9 were treated with MINO alone and 4 with combined MINO and new quinolones. Fever was resolved within 2 days of treatment in all cases. Our findings show that children with high fever and spotted palmar and/or plantar erythema should be treated immediately for JSF in prevalent areas, even in the absence of eschars.

Case 39-2010

A 19-year-old woman was transferred to this hospital because of nausea, jaundice, and pruritus. She was 3 months post partum. Examination revealed no spider angiomas, palmar erythema, telangiectasias, abdominal tenderness, or organomegaly. Bilirubin and aminotransferase levels were elevated.

Assessment of Safety and Efficacy of a New Intravenous Immunoglobulin (IGIV3I 10% Grifols) In Subjects with Chronic Immune Thrombocytopenia

AbstractAbstract 4669Two similarly designed prospective, uncontrolled, open-label clinical trials were planned to assess the safety and efficacy of a new intravenous immunoglobulin (IVIG), IGIV3I 10% Grifols, in subjects with chronic immune thrombocytopenia (ITP) in the USA, Canada and Europe (Spain, Russia and the United Kingdom). Subjects were candidates to be enrolled if they had a diagnosis of chronic ITP and a baseline platelet count ≤ 20 × 109/l. Eligible subjects received treatment with IGIV3I 10% Grifols 1g/kg for 2 consecutive days or 0.4 g/kg for 5 consecutive days. The primary efficacy endpoint was the response rate, defined as the proportion of subjects reaching or exceeding the threshold value of 50 × 109/l on or before day 8 (American study) or on day 30 (European study) where day 1 is the day of the first infusion. Secondary efficacy endpoints were the time to response, defined as the number of days elapsed from day 1 to achievement of the threshold value; duration of response, defined as the number of consecutive days with a platelet count known to be ≥50 × 109/l; the maximum (peak) platelet count and regression of haemorrhagic diathesis for those subjects presenting with bleeding signs at baseline. Safety endpoints included adverse events (AEs), physical examinations, vital signs and clinical laboratory parameters monitoring. A total of 27 subjects (18 adults and 9 pediatrics) have been enrolled, i.e. administered with at least one infusion of the product at any dose, in the study. Twenty-four (24) subjects (89%) were considered responders. This proportion was higher in pediatric subjects (9/9, 100%) than in adult subjects (15/18, 83%). The mean time to response was 1.6 days (Standard Deviation (SD) 0.9); the mean duration of response was 14.0 days (SD 12.1) and the mean maximum platelet count was 263 × 109/l (SD 195.6). Twenty-three (23) subjects (all the pediatric subjects 9/9 and 14/18 adults) presented with some sign of bleeding at baseline. All subjects (23/23, 100%) experienced an improvement in the haemorrhagic diathesis, regardless of some of them being considered non-responders according to the platelet count criterion. A total of 92 AEs potentially related to study drug were reported. The most common of these were headache (25 events), nausea (8 events), pyrexia (7 events) and chills (6 events). Only 1 AE was reported as definitely related to study drug, an event of palmar erythema. Two serious AEs (SAE) potentially related to the study drug were reported in 2 subjects. One of them was an unexpected laboratory alteration which consisted in leukopenia and decreased hemoglobin and advised to maintain the subject hospitalized for further observation during the weekend. The reaction was transient, without complications or other clinical symptoms and total recovery of the normal laboratory values. The second SAE was an event of thrombosis in the right humeral vein, in a patient with a number of predisposing medical conditions. At the time of discharge the patient's overall condition was satisfactory. Analysis of AEs, clinical laboratory values, physical assessments and vital signs did not indicate any safety concerns for subjects receiving the study drug and are in line with those expected for the ITP population treated with IVIG. Both primary and secondary efficacy endpoints show a good response to the product in terms of rapid elevation of platelet counts to an hemostatic level in line with what is expected for an IVIG product. Moreover, the improvement of the baseline bleeding diathesis even in patients considered as non-responders according to the platelet count criterion is highly suggestive of the clinical efficacy of the product. Overall, these results indicate that treatment with the new IGIV3I 10% Grifols is safe and efficacious for rapidly increasing platelet counts in chronic ITP subjects and improving their bleeding diathesis. Disclosures:Khojasteh:Grifols S.A.: Research Funding. Kato:Grifols S.A.: Research Funding. Parikh:Grifols S.A.: Research Funding. Singleton:Grifols S.A.: Research Funding. Tebbi:Grifols S.A.: Research Funding. Cromwell:Grifols S.A.: Research Funding. Fu:Grifols S.A.: Research Funding. Kessler:Grifols S.A.: Research Funding. Letzer:Grifols S.A.: Research Funding. Ritchie:Grifols S.A.: Research Funding. Sexauer:Grifols S.A.: Research Funding. Saxena:Grifols S.A.: Research Funding. Sirpal:Grifols S.A.: Research Funding. Torres:Grifols S.A.: Research Funding. Loriya:Grifols S.A.: Research Funding. Kovaleva:Grifols S.A.: Research Funding. Sandoval:Grifols S.A.: Research Funding. Sanz:Grifols S.A.: Research Funding. Julià:Grifols S.A.: Research Funding. Montañés:Grifols S.A.: Employment. Navarro:Grifols S.A.: Employment.

Palmar Fasciitis and Polyarthritis Syndrome in Patients with Ovarian Cancer—A Case Report and Review of the Literature

Palmar Fasciitis and Polyarthritis Syndrome in Patients with Ovarian Cancer—A Case Report and Review of the Literature

Autoimmune Hemolytic Anemia in Treatment-Naïve Chronic Hepatitis C Infection

Purpose: Hepatitis C Virus (HCV) is a blood borne pathogen that infects the liver and is associated with great morbidity. Chronic HCV infection can lead to cirrhosis, hepatocellular carcinoma, and ultimately liver failure. Less commonly known extrahepatic manifestations of HCV exist as numerous studies have been published demonstrating Autoimmune Hemolytic Anemia (AIHA) in chronic Hepatitis C patients receiving Ribavarin or Interferon. On the contrary, there is minimal literature demonstrating AIHA in treatment-naive chronic hepatitis. Case Presentation: A 53-year-old man with chronic Hepatitis C infection presented with 7 days of sharp right upper quadrant abdominal pain complicated by rapidly increasing abdominal girth, nausea, vomiting, weakness, fever and jaundice. His HCV course was complicated by cirrhosis, esophageal varices, portal hypertensive gastropathy and spontaneous bacterial peritonitis. Physical examination: Jaundiced sleepy male with normal vital signs. Scleral icterus, distended abdomen tender to palpation in the right upper quadrant, gynecomastia, diffuse spider angiomata on anterior chest, and +2 bilateral pitting edema up to the knees. No asterixis, palmar erythema, or caput medusae. Laboratory data: WBC 5700/UL, Hemoglobin 8.3 g/dl, MCV 112.4, platelets 47,000/UL. Indirect bilirubin of 14.3 mg/dl was worrisome for hemolytic anemia. Reticulocyte count 9.6% and direct Coomb's test antibody positive. Peripheral smear showed increased spherocytes, polychromasia, and thrombocytopenia consistent with AIHA. Workup: Etiologies of AIHA include malignancy, SLE, Ribavarin and Interferon. MRI and CT abdomen with contrast illustrated cirrhosis, but did not depict neoplastic lesions or lymphadenopathy. Chest CT and x-ray demonstrated no neoplasms, mediastinal or axillary lymphadenopathy, and no changes of the bony thorax consistent with a cancerous process. Bone marrow biopsy demonstrated erythroid hyperplasia with cell lines. No tumor or lymphoma was noted. Thus, malignancy was ruled out as an etiology of AIHA. SLE was subsequently ruled out with a negative ANA panel, and iatrogenic etiology was non-contributory as the patient had never received Ribavarin or Interferon. Conclusion: We present a rare case of AIHA infection where conceivably all etiologies of AIHA were thoroughly ruled out - demonstrating HCV as a unique and novel cause of AIHA. This is of great interest in idiopathic AIHA, as physicians we can look to HCV as an underlying source of AIHA. More recently, HCV is becoming an epidemic in the United States. Thus, unprecedented medical conditions associated with HCV are of great interest.

Hepatic Arteriovenous Malformations from Hereditary Hemorrhagic Telangiectasia: Treatment with Liver Transplantation

A 47-year-old handyman presented to the emergency department with worsening exertional dyspnea and persistent lassitude at rest for 2 weeks. He had experienced moderate unremitting epigastric pain over the preceding months requiring narcotics, as well as intermittent jaundice and low-grade fever. His epigastric pain frequently worsened after meals, resulting in anorexia and weight loss, but also was present at rest and radiated through to his back. He denied epistaxis, hemoptysis, and gingival or gastrointestinal bleeding. Although he admitted binge drinking of alcohol in the past, without obvious social or medical complications, he had been sober for several years. He continued to chew tobacco daily. His mother reportedly had alcoholic liver disease, and his two children were healthy. Physical examination revealed a slender man with a fever of 102 F but in no obvious distress. His conjunctivae were anicteric, and his oropharynx did not reveal any lesions or bleeding. His breath sounds were normal. Cardiac examination revealed tachycardia, a 3/6 systolic ejection murmur best heard over the apex, and jugular venous distention. His abdomen was soft and not distended, but tender over the epigastrium, where a loud bruit was also present. The liver and spleen were both enlarged. No jaundice, palmar erythema, or rashes were present. The patient was fully alert and oriented, without asterixis. Comprehensive laboratory studies were significant for a normocytic anemia with a hematocrit of 35%, mild thrombocytopenia with a platelet count of 142,000/mm, and abnormal liver tests with a total bilirubin of 3.7 mg/dl, aspartate aminotransferase (AST) 43 U/l, alanine aminotransferase (ALT) 55 U/l, and alkaline phosphatase of 579 U/l. His international normalized ratio for prothrombin time (INR) was normal at 1.1. B-type natriuretic peptide was elevated at 1,067 pg/ml. Blood cultures grew Streptococcus anginosus (a member of the Streptococcus milleri family). Echocardiogram revealed moderate left ventricular enlargement with normal left ventricular systolic function, and mild right ventricular enlargement with mildly decreased right ventricular systolic function. There was trace aortic regurgitation and mild mitral regurgitation, with increased aortic flow velocity with a mean gradient of 19 mmHg. There was also mild tricuspid regurgitation with an estimated right ventricular systolic pressure of 51 mmHg. No other valvular lesions or vegetations were found. Abdominal computed tomography (CT) revealed diffuse angiomatous tissue within the liver, early opacification of all hepatic veins, and low-attenuation tissue in segment 7 of the liver likely representing biliary necrosis (Fig. 1). Abdominal magnetic resonance imaging (MRI) confirmed the diffuse heterogeneous enhancement of the liver and presence of multiple arteriovenous malformations (Fig. 2). Angiography was performed, and the initial aortogram revealed a hugely dilated celiac artery and replaced left hepatic and common hepatic arteries (Fig. 3). The proper M. Lee (&) T. J. Daugherty Division of Gastroenterology and Hepatology, Department of Medicine, Stanford University Medical Center, 300 Pasteur Drive, Alway Building, M211, Stanford, CA 94305-5187, USA e-mail: maxlee@stanford.edu

Advanced aggressive fibromatosis: Effective palliation with chemotherapy

Background. Aggressive fibromatosis (AF) is a locally invasive proliferative disease. The mainstay of treatment is surgery. Chemotherapy may be considered in inoperable AF following failure of hormonal therapy and/or NSAIDs. Material and methods. We conducted a retrospective search of the prospectively maintained Royal Marsden Hospital Sarcoma Unit database to identify patients with AF treated with chemotherapy between 1987 and 2009. Results. Thirty-nine patients, thirty one females and eight males, received one or more lines of chemotherapy. The most frequently employed chemotherapy regimens were methotrexate/vinblastine [MTX/VBL] (18) and pegylated liposomal doxorubicin [PLD] (14). MTX/VBL was administered weekly or every two weeks at MTX 50 mg and VBL 10 mg. Treatment duration ranged from three weeks to one year with a median of 4.5 months. Partial response (PR) was observed in 11% of cases, disease stabilisation (SD) in 60% and progressive disease (PD) in 22%. Time to progression ranged from one month to sixteen years. The main toxicities reported were mucositis (4), peripheral neuropathy (3), vomiting (3), and neutropenia (3). PLD was administered at 40–50 mg/m2 every four weeks, for up to six cycles. PR was achieved in 33% and in the remainder the disease was stable with no progression during treatment. Three (25%) patients have so far progressed after treatment. Symptomatic benefit, especially pain relief, was reported in 86% (12/14) of cases. Main toxicities included palmar plantar erythema (5) and mucositis (4). Discussion. MTX/VBL remains a useful combination but PLD is emerging as a well tolerated and effective systemic therapy in advanced AF.

The effects of alcohol and drug abuse on the skin

Skin changes associated with alcohol and drug abuse can be the earliest clinical manifestation of these disorders. The signs associated with these conditions may be distinctive and easily recognizable. Alcohol abuse can present with jaundice, pruritus, hyperpigmentation, and urticaria. Commonly associated vascular changes include spider telangiectasias, angiomas, caput medusas, flushing, and palmar erythema. Disease states related to alcohol abuse include psoriasis, porphyria cutanea tarda, and nutritional deficiencies. Alcohol abuse may predispose to the development of carcinomas of the skin, oropharynx, liver, pancreas, and breast. Cutaneous signs of drug abuse include skin granulomas, ulcerations, and recurrent infections. Specifically, oral disease and tooth decay are examples of stigmata often associated with methamphetamine abuse, a popular and inexpensive drug now on the scene. By being cognizant of these cutaneous markers of alcohol and drug abuse, dermatologists are often in the unique position of being able to recognize these changes, prompting early diagnosis and intervention, hopefully resulting in a better clinical outcome for these troubled patients and their families.

Nine cases of Japan spotted fever diagnosed at our hospital in 2008

The diagnosis of Japan spotted fever (JSF) is very difficult in some cases. The initial diagnosis of JSF is very important to treat. We report nine cases of Japan spotted fever (JSF) with variable clinical features diagnosed at our hospital in 2008. Concerning clinical symptom, the most frequent symptoms were fever (8/9) and erythema of the whole body (8/9), followed by eschar (4/9). Palmar erythema, vomiting, and headache were observed in two cases. Purpura and lymph node swelling were observed in one case. Complication with Disseminated intravascular coagulation (DIC) was observed in one case. Laboratory findings revealed elevated plasma level of C-reactive protein (CRP) and liver dysfunction in all cases, and decreased platelet (7/9). Interestingly, all patients had a history of presumed infection in the Southern area of Miya River, where wild Japanese deer with ticks (vector of Rickettsia japonica) may reside. Different procedures are performed to make a diagnosis of JSF. For an initial definite diagnosis and adequate treatment of JSF, PCR of samples taken from blood, and skin biopsy from erythema and eschar lesions are necessary. Paired serum to measure the titers of antibody against R. japonica is also important.

En mann med feber og magesmerter

A 34-year-old man with no previous medical history was referred to our surgical department with abdominal pain and fever. Over the next days he developed a cluster of symptoms, which finally led us in the right diagnostical direction. On admission he had clinical findings and biochemistry supportive of cholecystitis. However, he also had a non-symptomatic rash on his chest. Intravenous antibiotic treatment was instigated. Radiological findings did not show intraabdominal pathology, except for mesenterial lymphadenopathy. After three days on antibiotics the patient still had fever. The biochemical work-up revealed persistent cholestasis, lymphopenia and increasing infectious stigmata. A more extensive laboratory work-up was performed, including bacterial, viral, and immunological tests. During the next few days the patient developed palmar erythema, polymorphic exanthema, conjunctival bleeding and a strawberry tongue. The patient gradually improved, but developed skin desquamation, thrombocytosis and reactive arthralgia. This is consistent with the clinical picture of Kawasaki disease. As all supplemental tests were negative, we could therefore conclude (based on clinical, radiological and biochemical results) that this was a rare case of adult Kawasaki disease. A review of literature describing adult Kawasaki disease is also presented.

Indurated reticulate palmar erythema as a sign of paraneoplastic palmar fasciitis and polyarthritis syndrome

A 62-year-old woman presented with a 6-month history of polyarthritis. She had also noted a 2-month history of indurated palmar erythema and increasing bilateral hand swelling and stiffness. A biopsy from the area of palmar erythema showed interstitial fibroplasia within the dermis and subcutis representing a palmar fibromatosis. This presentation appears to belong to the spectrum of palmar fasciitis and polyarthritis syndrome. Rheumatologists have recognised this syndrome as a paraneoplastic disorder and subsequent investigations in our patient revealed an elevated cancer antigen 125 and an inoperable ovarian carcinoma. Indurated palmar erythema is a sign that is not widely recognised by dermatologists as a clue for this paraneoplastic syndrome, and skin biopsy demonstrating dermal and subcutaneous fibroplasia may help in diagnosis in the absence of advanced signs of palmar fasciitis.

Phase I feasibility study of carboplatin plus capecitabine followed by maintenance capecitabine in patients (pts) with recurrent platinum-sensitive epithelial ovarian cancer (EOC)

5564 Background: In a previous study, we noted a response rate (RR) of 61% for the 3 drug combination of carboplatin, epirubicin and capecitabine in platinum-sensitive recurrent EOC. This combination however resulted in excessive grade (G) 3–4 haematological toxicity (55%) (BJC 2006; 94:74). The current trial therefore assessed the feasibility and efficacy of the 2 drugs, carboplatin and capecitabine as second- or third-line treatment. Methods: Pts were administered carboplatin (AUC5) day 1 and capecitabine at a starting dose of 750 mg/m2 bd, days 1–21, q21 (dose level 1). The capecitabine dose was deescalated to 625 mg/m2 (dose level -1) and 500 mg/m2 (dose level -2) according to toxicity. Pts with an objective response or stable disease received maintenance capecitabine (at the same dose level) for up to 12 months or until progression. Responses were assessed with RECIST criteria and CA-125. Results: 19 of the 20 pts enrolled were evaluable for toxicity and response. Dose-limiting toxicity was observed at dose level 1 (G3 fatigue, G3 diarrhoea, G3 neutropenia of &gt; 14 days; n = 3/5), dose level -1 (G3 angina (n = 2), G3 vomiting, G3 palmar plantar erythema; n = 4/7) and dose level-2 (diarrhoea / fatigue; n = 1/7). One patient had a G3 carboplatin hypersensivity reaction. 8 pts received maintenance capecitabine which was well tolerated. The overall RR was 53% with 10 partial responses and 5 stable diseases. The median progression free survival (PFS) was 6.5 months (m) and the 6mPFS was 63% with 2 pts currently ongoing treatment. The median PFS on maintenance was 3.2 m. Conclusions: The combination was well tolerated at the recommended phase II dose of carboplatin (AUC 5) and capecitabine (500 mg/m2 bd) with partial responses in over half of the cases. [Table: see text]

Sequential Combination Therapy Leading to Sustained Remission in a Patient with SAPHO Syndrome

The SAPHO syndrome represents a variety of clinically similar disorders with the key features of hyperostotic bone lesions in combination with chronic pustular skin disease. The respective pathophysiology of bone and joint manifestations in SAPHO syndrome is still a matter of discussion. For example it does not appear to represent reactive arthritis and HLA B27 antigen, with the latter being typically present in patients with spondyloarthopathies. Treatment of SAPHO syndrome is also not well established and consists of various antiinflammatory and antirheumatic drugs. Here, we report a female patient with active SAPHO syndrome suffering from sternal swelling of unknown origin that had been known for 10 years and a 4-year-history of severe lower back pain. Remarkable were also a typical pustulous palmar erythema associated with swelling and decreased motility of both MCP-I joints. Inflammation parameters were high with an ESR 68 mm/1st hour and a CRP of 19.6 mg/l. She was initially treated with rofecoxib and doxycycline, followed by sulfasalazine with only partial clinical response. Thereafter, both articular symptoms as well as cutaneous lesions responded well to a combination therapy with methotrexate and sulfasalazine. Thus, the case illustrates nicely that methotrexate in combination with another DMARD can be successfully applied to patients with long-term active SAPHO syndrome.

Acute Calcific Tendinitis

A 56-year-old woman presented to the emergency department with a 3-day history of severe pain in and loss of movement of her right index finger. She reported that for the previous 2 months, she had noted a small, tender lump, with no progression in size, near the finger joint. Marked palmar erythema and a tender, firm swelling overlying the index metacarpophalangeal joint were noted (Panel A). Initially, a flexor-sheath infection was suspected, and antibiotics were administered. However, radiography showed a calcified mass within the flexor digitorum tendon (Panel B, arrow), and calcific tendinitis was diagnosed. Calcific tendinitis may be an . . .

Syndrome mains-pieds secondaire à la prise de 6-mercaptopurine chez un enfant de quatre ans

Hand-foot syndrome (HFS) is a dose-dependent cutaneous side effect of cytostatic chemotherapy. It has also been described under the names of acral erythema and palmoplantar erythrodysesthesia. We report a case of HFS during treatment of acute lymphoblastic leukemia with 6-mercaptopurine (6-MP) (Purinethol) in a four-year-old child. A four-year-old boy treated for acute lymphoblastic leukemia developed dry and painful palmar and plantar erythema with fissures. The rash began three weeks after up-titration of 6-MP. There was no past history of cutaneous disease and no other potential trigger factors. The rash ceased after 6-MP withdrawal. To our knowledge, this is the first case of HFS due to 6-MP therapy in a child. 6-MP is a major reference drug for the management of acute lymphoblastic leukemia. Numerous cytostatic drugs have been involved in such eruptions.