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Das nekrobiotische Xanthogranulom ist eine seltene Non-Langerhans-Zell-Histiozytose. Vorgestellt wird eine 62-jahrige Patientin mit gelblich-rotlichen Plaques, Knoten und Papeln periorbital und an den Extremitaten. Zusatzlich bestand ein knotiger Tumor des linken Oberlides, der klinisch als Keratoakanthom eingeordnet wurde, histologisch jedoch dem Xanthogranulom zuzuordnen war. Es lag eine monoklonale Gammopathie unklarer Signifikanz von κ‑Typ vor. Zur Therapie wurde eine Kombination aus Dapson/Prednisolon und topischen Kortikosteroiden verwendet.

Mixed tumor of skin (chondroid syringoma) is a rare skin tumor. We report on the rare occasion of an atypical mixed tumor of skin (chondroid syringoma) in a 71-year old Caucasian male patient. The tumor presented as a firm subcutaneous nodule on his flank. Histopathologic investigations revealed a focally increased proliferative activity and satellites in the adipose tissue. This is in accordance with the diagnosis of an atypical chondroid syringoma. The differential diagnosis to malignant chondroid syringoma is discussed. The tumor was removed by delayed Mohs surgery. Atypical chondroid syringoma is a very rare subtype of mixed tumor of skin. It is an important differential diagnosis to malignant chondroid syringoma of skin.

Background Atypical fibroxanthoma is a rare mesenchymal tumor of the head and neck region. Methods We analyzed the files of 3 large dermatology hospitals from the years 2001 to 2013 in southeast Germany. Results We identified 53 patients (56 tumors) with a male predominance. The mean age was 78.0 years ± 8.3 years. Mohs surgery was performed in all cases resulting in complete remission in 45 patients. Five patients had a relapse within 2 years, and 4 developed metastases. None of the tumors with a safety margin of ≥2 cm relapsed. The majority of cases were found in the Dresden region. Conclusion Atypical fibroxanthoma demonstrates an uneven geographic distribution in southeast Germany that demands further investigations. Mohs surgery with ≥2 cm safety margins is the treatment of choice. A regular follow-up is recommended. © 2014 Wiley Periodicals, Inc. Head Neck 37: 829–834, 2015

The intention of this review on nevus comedonicus (NC) is to update on clinical features, pathogenesis, and therapy. NC is a rare epidermal nevus type. It is part of the nevus comedonicus syndrome, a neurocutaneous disorder with ocular, skeletal, and central nervous symptoms. Recently, acne-related signaling pathways and somatic mutations of tyrosine kinase receptors have been identified and may play a role in NC pathogenesis. On preexistent NC secondary skin tumors can develop, which are often benign. Treatment options of NC include topical therapy, laser, and surgery.

Background Sentinel lymph node biopsy (SLNB) is an important tool for accurate staging of patients with melanoma. There is an ongoing debate whether the procedure provides therapeutic benefits or not. Objective We wanted to analyze 10-year data from an academic teaching hospital. Patients and methods During 1999–2009, 977 patients with early cutaneous melanoma have been treated. Of these, 419 patients had tumors ≥1 mm thickness. Patients with head and neck tumors were excluded, leaving 364 patients (202 men and 162 women). SLNB was not performed in 163 patients but was performed in 201 patients. For correction of bias, tumors >4 mm thickness were excluded from further statistical analysis. Results The detection rate of SLN was 94.4%. False negative SLN were observed in 8.9%. Adverse effects occurred in 5.5%. The rate of positive SLNB was 16.4% and lymph node involvement 20%. Patients undergoing SLNB had a lower relapse rate (10.6% vs. 33.3%; P

Background Cutaneous angiosarcoma (AS) is a rare aggressive malignant vascular tumour of the skin. Methods We analysed the cases of cutaneous AS in two large dermatological departments of Saxony during the last 10 years. Results A total of eight AS cases were identified, mostly with head and neck involvement. Because of age and late diagnosis, only a minor part was completely resectable. As a palliative therapy pegylated liposomal doxorubicin was used. The drug was well tolerated. Our cases and those published in the literature suggest a high rate of responses and a possible sensitisation to subsequent radiation therapy. Conclusions Although complete surgical resection followed by wide-field radiation therapy has been established as a therapeutic standard for AS, delay in diagnosis and older age with comorbidities warrant for a safe and effective adjuvant treatment. Pegylated liposomal doxorubicin combined with radiotherapy seems to be a useful and safe alternative.

Summary Background: Pityriasis rubra pilaris (PRP) is a rare papulosquamous dermatosis. We evaluated evaluate co-morbidities, complications, and outcome of treatment regimens. Patients and Methods: This is a retrospective study at an academic teaching hospital. We analyzed all patients with the definite diagnosis of PRP seen since 2001. Epidemiologic data, co-morbidities, response to and course during treatment were investigated. Results: We identified 10 PRP-patients (6 men, 4 women), mean age 56.4 years, with type I (n = 9) and type IV (n = 1). Three patients had internal co-morbidities (atrial fibrillation with cardiac insufficiency, dilated cardiomyopathy, diabetes mellitus). Two patients needed psychiatric treatment because of depression. PRP caused ectropium (2 ×), diffuse effluvium (1 ×), and stenosis of the outer ear canal (1 ×). We did not observe a spontaneous remission. Among 9 patients with PRP type I, five were treated with acitretin (two of them as Re-PUVA), and two with methotrexate (in one patient combined with fumaric acids). Systemic corticosteroids were not effective. One patient was treated with infliximab i.v., 5 mg/kg body weight. Starting with the first application, inflammatory activity decreased and erythema got paler. The treatment was well tolerated. Conclusions: PRP type I is a severe, chronic inflammatory dermatosis responding hesitantly to classic systemic therapies. Tumor necrosis factor-α antagonists are an effective treatment option for difficult cases.

Objective Atypical fibroxanthoma is a rare mesenchymal tumour of skin that develops on skin of elderly patients. We analysed our patients with atypical fibroxanthoma over the last 8 years. Patients and methods We analysed the pathology files of our hospital for the period 2001–2009. In all cases, histology and immunohistology were performed. Data on co-morbidities, treatment and outcome were obtained. Results We identified 25 patients (except two female patients, the rest were men) aged 52–95 years (mean: 79.5 years; standard deviation ± 9.06 years). All tumours were localized in the head and neck region, except a single tumour on the shoulder. Fourteen patients had a cancer history, six had actinic keratoses or Bowen’s disease (n = 1). Five patients had cardiac surgery or pacemaker, one each had a renal transplant, systemic sarcoidosis or non-Hodgkin’s lymphoma. Medical history was positive for radiotherapy or chemotherapy in four patients. Histology showed a spindle-shaped dermal and subcutaneous tumour growth intermingled with multinucleated giant cells. The phenotype of tumour cells was vimentin-positive, but S100- and keratin-negative. Some tumours showed a focal expression of CD68. Complete microhistographic controlled surgery (‘Mohs like’) was possible in all cases followed by mesh-graft transplantation in three patients. Eighteen patients showed a complete remission. Four patients had a relapse within 2 years of follow-up treated by surgery. One patient is still under radiotherapy. All patients with a safety margin of 2 cm had no recurrence or relapse during follow-up. Conclusions Micrographic controlled surgery with wide 2 cm safety margins is the treatment of choice. A regular follow-up for the next 5 years is recommended.

The authors present a rare case of panniculitis ossificans (PO) in a 30-year-old woman. Differential diagnoses and treatment are discussed. PO is an important differential diagnosis to sarcomas.

Calciphylaxis is a cause of painful deep ulcers. There is controversy about best wound management in this disease. A retrospective study of inpatients during the 3 years was made. Seven calciphylaxis patients were identified. All patients suffered from various associated pathologies including diabetes mellitus type II and chronic renal insufficiency. Ulcers were treated by aggressive and deep shaving combined with autologous split-skin grafting in the same session. A 30% to 90% take rate of the grafts eventually with a complete ulcer healing in 6 of 7 patients was achieved. No patient developed a deep cutaneous infection or sepsis. All patients are still alive except one. The single death was related to cardiovascular complications. In distal calciphylaxis, aggressive ulcer surgery with defect closure offers a marked improvement in quality of life and prevents early deep skin infections and sepsis as major causes of mortality.

Chronic ergotism is a rare cause of limb ischemia. In this case report, the authors present a 62-year-old woman with history of long-term use of ergotamine alkaloids for the treatment of menstrual pain, who developed a severe painful disease initially misdiagnosed as systemic sclerosis (scleroderma) for 3 decades. She presented with a combination of acral gangrene, foot ulcer, renal obstruction, mild pulmonary fibrosis, and reduced esophageal motility. Right-sided renal obstruction was evident. The condition was extremely painful and had led to muscular contractions and immobility, drug abuse, and anemia. After establishing the diagnosis of chronic gangrenous ergotism, changing drug therapy, mobilization, and treatment of chronic wounds, she showed a remarkable recovery. Eventually the foot ulcer was closed successfully using a mesh graft transplantation, and the patient was able to walk alone. Chronic ergotism is rare but has to be taken into account when presented with painful chronic digital and foot ulcers.

Leprosy is very rare in Germany. In 2003, only three cases were reported. In the Department of Dermatology, Dresden-Friedrichstadt, two cases from Asia had been diagnosed during the last 5 years, a paucibacillary tuberculoid leprosy and a multibacillary lepromatous leprosy. In both, there was a diagnostic delay of several months (3-7 months). The need for early detection of this mycobacteriosis also in nonepidemic areas of the world and the importance of a correct diagnosis by the dermatologist is hereby emphasized.

Objective: Elephantiasis nostras is a rare complication in advanced lipo-lymphedema. While lipedema can be treated by liposuction and lymphedema by decongestive lymphatic therapy, elephantiasis nostras may need debulking surgery. Methods: We present 2 cases of advanced lipo-lymphedema complicated by elephantiasis nostras. After tumescent microcannular laser-assisted liposuction both patients underwent a debulking surgery with a modification of Auchincloss-Kim's technique. Histologic examination of the tissue specimen was performed. Results: The surgical treatment was well tolerated and primary healing was uneventful. After primary wound healing and ambulation of the patients, a delayed ulceration with lymphorrhea developed. It was treated by surgical necrectomy and vacuum-assisted closure leading to complete healing. Mobility of the leg was much improved. Histologic examination revealed massive ectatic lymphatic vessels nonreactive for podoplanin. Conclusions: Debulking surgery can be an adjuvant technique for elephantiasis nostras in advanced lipo-lymphedema. Although delayed postoperative wound healing problems were observed, necrectomy and vacuum-assisted closure achieved a complete healing. Histologic data suggest that the ectatic lymphatic vessels in these patients resemble finding in podoplanin knockout mice. The findings would explain the limitations of decongestive lymphatic therapy and tumescent liposuction in such patients and their predisposition to relapsing erysipelas.

A 48-year-old female with an atypical plaque-like lesion of the lower leg is presented in this article. Histologic investigation revealed a rare low-grade fibromyxoid sarcoma (pT1a cN0 cM0; stage Ia) of suprafascial localization. Staging of the patient did not reveal metastatic spread. The tumor was surgically removed with wide safety margins. The defect was closed using a mesh graft transplant and vacuum-assisted closure. Healing was complete. Regular follow-up for at least 5 years is recommended. Besides the rareness of this tumor, this case is also remarkable because of the localization on the lower leg and the suprafascial soft tissue.

A 79-year-old female patient presented with a slowly developing crusting, itching verrucous lesion of the forehead of 3 months' duration. She had no personal or family history of skin disease. On examination she presented with a hyperpigmented plaque of the glabellar region that resembled dyskeratosis follicularis Darier. A detailed medical history was taken and a skin biopsy was performed. Epidermal acanthosis and enlargement of follicle ostia with foreign material were found. The patient reported use of an ointment twice daily. She had a single cosmetic treatment where powder of unspecified composition had been used. The diagnosis of pomade crust was confirmed. Follicular material was enucleated mechanically and the area was treated with a metronidazole cream. The lesions completely disappeared.

Atypical fibroxanthoma is a rare mesenchymal tumor of the head and neck region.We analyzed the files of 3 large dermatology hospitals from the years 2001 to 2013 in southeast Germany.We identified 53 patients (56 tumors) with a male predominance. The mean age was 78.0 years ± 8.3 years. Mohs surgery was performed in all cases resulting in complete remission in 45 patients. Five patients had a relapse within 2 years, and 4 developed metastases. None of the tumors with a safety margin of ≥2 cm relapsed. The majority of cases were found in the Dresden region.Atypical fibroxanthoma demonstrates an uneven geographic distribution in southeast Germany that demands further investigations. Mohs surgery with ≥2 cm safety margins is the treatment of choice. A regular follow-up is recommended.

Mesenchymal neoplasms (sarcomas) of skin are rare. Patients with sarcomas were analyzed over the last decade.Over a 10-year period, we conducted a retrospective analysis of patients diagnosed and treated in an urban academic teaching hospital in Saxony, Germany. Clinical and pathologic files were used.We identified 65 adult patients with 67 primary cutaneous sarcomas. The mean age was 73.1 (± 15.5) years with a male predominance (78.5%). None of the sarcomas was detected by a skin cancer screening program. The diagnosis was atypical fibroxanthoma (n = 41 patients with 43 tumors), cutaneous angiosarcoma (eight), dermatofibrosarcoma protuberans (two), nodular epithelioid cell sarcoma (one), Kaposi sarcoma (three), leiomyosarcoma (five), malignant fibrous histiocytoma (two), fibromyxoid sarcoma (one), and cutaneous angiomyxoma (two). The preferred tumor localization was the head and neck area (44 patients). Follow-up was 0.5-5.5 years (mean 18 ± 12 months). We observed metastatic spread of atypical fibroxanthoma in 12.5%, demonstrating that this type of sarcoma can run an aggressive course. Mohs surgery is still the cornerstone of treatment, although new options in palliative or adjuvant treatment are available.Mesenchymal neoplasms (sarcomas) are an important group of cutaneous malignancies. Awareness needs to be improved.

Interstitial granulomatous dermatitis and arthritis (IGDA) is a rare disease entity with female predominance. The case of a 53-year-old woman with erythemas, plaques and nodules associated with polyarthritis is presented. She was treated with cyclosporin A, with improvement of the joint affliction and complete clearance of skin lesions. The differential diagnosis of IGDA is discussed briefly.

We report a 30-year-old-patient with penile sclerosing lipogranuloma following injections of Vaseline. He was successfully treated by complete surgical removal of the subcutaneous material and excision of atrophic skin, followed by penoplasty. Temporary impairment of wound healing was overcome by application of topical carbon dioxide gas. A brief review on international literature is given.

Malignant nail tumours are rare. We report on a 52-year-old patient with chronic verrucous lesions of the nail bed of fingers and toes. Nail biopsy revealed polydactylous Bowen's disease. This is the first case reported with hand and foot lesions.

Background Severe anogenital acne inversa (AI) is a debilitating chronic inflammatory disease with a major negative effect on quality of life. Objectives To evaluate the role of surgery in the treatment of severe anogenital AI. Methods We analyzed the records of patients with anogenital AI from 2000 to 2010. Assessment was done using the Hidradenitis Suppurativa Lesion, Area, and Severity Index (HS-LASI), pain visual analogue scale, physician global assessment (PhGA), and patient global assessment (PaGA). Comorbidities and adverse events were analyzed. Results Sixty-seven patients with a Hurley score of 3 were identified. Mean follow-up was 56.9 ± 41.3 months. A number of comorbidities were observed. After surgery, mean pain scores decreased from 6.3 ± 1.5 to 0.8 ± 0.7, PhGA improved from 6.8 ± 1.2 to 0.9 ± 0.6, PaGA improved from 7.3 ± 1.2 to 1.1 ± 0.5, and HS-LASI decreased from 41.8 ± 21.3 to 2.4 ± 2.8. Adverse reactions were seen in 10.4%. The total relapse rate was 6% (5 patients) with only one case with healing by secondary intention (2% of 49 patients). Conclusions Surgery is the cornerstone of treatment for advanced AI. Healing by secondary intention results in significant reduction of complaints and achieves satisfying body contouring.

Giant cutaneous horns are suspicious of malignancy, in particular squamous cell carcinoma. We report on a 67-year-old man that developed a giant horn on his upper leg that resembled a keratoacanthoma. The lesion was surgically removed. Histologic investigation revealed a giant verruca vulgaris. No risk factors such as immunosuppression were evident. Giant verruca vulgaris is a very rare cause of giant cutaneous horns. Complete excision is recommended in order not to overlook a malignant skin tumor in such cases.

Pityriasis rubra pilaris (PRP) is a rare papulosquamous dermatosis. We evaluated evaluate co-morbidities, complications, and outcome of treatment regimens.This is a retrospective study at an academic teaching hospital. We analyzed all patients with the definite diagnosis of PRP seen since 2001. Epidemiologic data, co-morbidities, response to and course during treatment were investigated.We identified 10 PRP-patients (6 men, 4 women), mean age 56.4 years, with type I (n = 9) and type IV (n = 1). Three patients had internal co-morbidities (atrial fibrillation with cardiac insufficiency, dilated cardiomyopathy, diabetes mellitus). Two patients needed psychiatric treatment because of depression. PRP caused ectropium (2 x), diffuse effluvium (1 x), and stenosis of the outer ear canal (1 x). We did not observe a spontaneous remission. Among 9 patients with PRP type I, five were treated with acitretin (two of them as Re-PUVA), and two with methotrexate (in one patient combined with fumaric acids). Systemic corticosteroids were not effective. One patient was treated with infliximab i.v., 5 mg/kg body weight. Starting with the first application, inflammatory activity decreased and erythema got paler. The treatment was well tolerated.PRP type I is a severe, chronic inflammatory dermatosis responding hesitantly to classic systemic therapies. Tumor necrosis factor-alpha antagonists are an effective treatment option for difficult cases.

Myxoid neurothekeoma is a rare benign asymptomatic tumor of the skin. Most tumors are located on the head, shoulders and arms. We present a 19-year-old female patient with a relapsing tumor of this type in a less common localization, i.e., lower trunk, and with painful sensations under mechanical pressure. Wide excision surgery led to complete remission.

BACKGROUND Dystrophic calcification (DC) is a risk factor for conservative treatment failure in chronic leg ulcers of various pathologies. PATIENTS AND METHODS We performed a retrospective noncontrolled trial of 212 patients with 362 chronic leg ulcers who underwent ulcer shave excision with subsequent skin grafting. The ulcers existed for at least 3 months, and no healing was achieved with good ulcer care. Tissue was subjected to histopathology (hematoxylin-eosin and van Kossa stains). RESULTS DC was evident in 39 patients (18%). Metaplastic subcutaneous bone formation was observed in 15 patients (7%). Clinical symptoms associated with DC were resistance to good ulcer care, pain, and ineffective effects of compression therapy (in venous ulcers). Ulcers were treated with deep dermatome shaving of the ulcer bed and surgical removal of DC. In the same setting, defects were closed using mesh graft transplantation. The procedure achieved a complete take rate in 80% and a significant decrease of pain in 95% of cases. When comparing the take rates in patients with and without DC, DC had a negative effect on outcome (take rate: 91% without DC vs 80% with DC, p

Background The plexiform schwannoma, a peripheral nerve sheath tumor, is a very rare entity. But dermatologists should be familiar with since they may be the first who make diagnosis possible by taking a deep biopsy.

Summary Tattooing has become quite popular in Western countries. With the increasing prevalence, there is also an increased risk of adverse effects. We describe a 17-year-old female patient with a black and red–colored tattoo, who developed immediately after red tattooing general malaise with fever, nausea, and vomiting. A bullous reaction was temporarily seen within the red part of her tattoo. The reaction later shifted to a subacute dermatitis with bacterial superinfection. Two months later, she felt ill again. She developed painful tender nodules on the anterior aspect of both lower legs identified as erythema nodosum without sarcoidosis. Is this is a unique case of adverse reaction to tattoo pigments with a type I and a type IV reaction, or is this a coincidence? The treatment was initiated with systemic and topical corticosteroids and topical antibiotics combined with compression bandages for the legs. After 3 weeks of treatment, the erythema nodosum completely resolved and did not reappear during a 1-year follow-up. The treatment of the local reactions, however, was unsatisfactory without complete response. There is an indispensable need for regulation of tattoo pigments and tattooing to improve consumer safety.

Tumor necrosis factor-alpha (TNFalpha) inhibition is effective in the treatment of moderate-to-severe psoriasis. We report on 120 patients from the literature including six new patients (three women and three men) who developed pustular lesions during treatment with TNFalpha inhibitors. We identified 72 women and 36 men (several papers did not specify the gender of patients) with an age range of 13-78 years (mean 42.3 years). The primary diagnoses were rheumatoid arthritis (n = 61), ankylosing spondylitis (n = 21), psoriasis (n = 10), Crohn disease (n = 8), SAPHO (synovitis acne pustulosis hyperostosis osteitis) syndrome (n = 3), psoriatic arthritis (n = 2), and other diagnoses (n = 15). Psoriasis (except palmoplantar pustular type) was the most common adverse effect during anti-TNFalpha treatment (n = 73), followed by palmoplantar pustular psoriasis (n = 37) and psoriasis of the nail (n = 6), sometimes combined in the same patient. Palmoplantar pustulosis and psoriasiform exanthema was the diagnosis in ten patients each. A positive personal history of psoriasis was recorded in 25 patients. A positive family history was noted in eight patients. No data about personal (n = 7) or family history (n = 46) were available in a number of patients. Newly induced psoriasis was diagnosed in 74 patients whereas an exacerbation or aggravation of a pre-existing psoriasis was noted in another 25 patients. All three TNFalpha inhibitors available on the market were involved: infliximab (63 patients), etanercept (37 patients), and adalimumab (26 patients). Several patients were treated with more than a single TFNalpha inhibitor. The timing of cutaneous adverse effects (psoriasis and psoriasiform rash) varied considerably among patients, ranging from after a single application to a delayed response of up to 63 months after initiation of treatment. The mean time to appearance of the cutaneous adverse effect for all TNFalpha inhibitors was 9.5 months. Cessation of the responsible TNFalpha inhibitor was carried out in 47 patients either alone or in association with adjuvant anti-psoriatic therapy (mostly topical). This resulted in complete remission in 21 patients, partial remission in 20 patients, and stable disease in another three patients; in the other three patients, the outcome was not reported. TNFalpha inhibition was continued in 47 patients but anti-psoriatic adjuvant therapy was introduced. The outcome in this group was complete remission in 22 patients, partial remission in 25 patients, and stable disease in 2 patients. The response rate (complete remission plus partial remission) was 93.2% and 95.9%, respectively, in each group. In six patients, switching from one TNFalpha inhibitor to another one immediately after cutaneous adverse effects occurred resulted in an improvement in five patients. In nine patients, a second TNFalpha inhibitor was initiated after a break in TNFalpha inhibition. The response to a second or third drug in these patients was mixed. The underlying pathomechanisms of induction of psoriasis or psoriasiform exanthemata by TNFalpha inhibitors remain elusive but there is reason to assume that induction of such adverse events has more than one pathophysiology.

Disabling pansclerotic morphea of childhood (DPMC) is a rare and severe variant of scleroderma. This report presents 3 cases that presented to the authors and studies 25 patients from the literature (English language only) for the presence of chronic nonhealing ulcers of skin and skin cancer. The authors identified a total of 30 patients (9 male and 21 female) aged between 1 and 37 years at time of presentation. All cases were less than 14 years old when the disease started. The majority of patients had an aggressive course with deep sclerotic lesions leading to joint contractures and immobility. Five patients suffered from chronic nonhealing leg ulcers (17%), but ulcers were present on other parts of the body (upper limbs, trunk, head) as well (n = 6). Four patients died because of complications of the disease such as sepsis or gangrene. Two patients developed a squamous cell carcinoma at the age of 16 years and 19 years, respectively (6.7%). The available treatment of DMPC-associated ulcers is unsatisfying. Only temporary improvements have been seen in a minority of patients. We report on marked improvement of chronic leg ulcers by a combination of sildenafil 3 × 20 mg/day and repeated application of a porcine small intestinal submucosal acellular matrix.

Objective To report clinical experience with the rare neuroendocrine Merkel cell carcinoma of the skin. Subjects and setting Seventeen patients with Merkel cell carcinoma of the skin treated at the Departments of Dermatology and ENT, Krankenhaus Dresden-Friedrichstadt, Dresden, Germany, during the years 1984–2000 were evaluated. Methods A retrospective analysis was performed. Age and sex distribution, clinical data and therapy were collected. Outcome measures including overall survival, tumour-free survival and relapse-free survival were determined. Results Six male and 11 female patients with an age range of 68–90 years (mean age 73.3 years) were identified. The primary tumour localization was head and neck region (n = 8), upper limbs (8), lower limbs (1). Twelve patients presented in tumour stage I, three in stage II and one in stage III. First line therapy was complete surgical excision with wide margins in 16 patients followed by loco-regional radiation in 12 of 16 cases. In 16 patients follow up data were available. After primary treatment complete response was achieved in 14 of 16 patients (87.5%), two patients had a partial response. The median of relapse-free survival was 44 weeks [mean ± standard deviation: (44 ± 118) weeks]. The median of overall survival was 102 weeks [mean ± standard deviation: (137 ± 94) weeks]. Three patients with a PR after primary treatment had a median overall survival of only 48 weeks [mean ± standard deviation: (51 ± 20) weeks]. Conclusions Primary surgical treatment with wide excision combined with radiotherapy seems to be a reasonable first-line treatment but prospective controlled multicentre trials are necessary for validation.

Advanced cutaneous T-cell lymphoma (CTCL) is a hard-to-treat condition. Complete response is rare even with polychemotherapy. The use of liposomal formulation anti-cancer drugs can improve the efficacy and the risk-benefit ratio. Liposomal doxorubicin was shown to be effective as a second-line treatment in CTCL. There is no data available on another classical anthracycline, daunorubicin, when given in liposomal formulation as a monotherapy.Monotherapy with liposomal-encapsulated daunorubicin (DNX) was given as a monotherapy once a month at 20 mg/m(2) three times to achieve a clinical response. In the case of limited response the drug was given once every 3 weeks and a dose increase was performed. Three patients were treated.A complete response was achieved in one patient (dosage 20 mg/m(2 )once per month). Two other patients achieved a partial response. The final outcome was disease-free survival of more than 10 months in the patient with a complete response and survival of8 months and 6 months in those with a partial response. Adverse effects were grade 4 anemia in one patient, lymphopenia grade 2 with grade 1 anemia, and grade 1 lymphopenia in the other patients.This is the first report on DNX monotherapy in CTCL. In a small group of three patients a response rate of 100% was achieved with one complete response. DNX seems to be another option in advanced cases of CTCL.

A papulose linfomatóide (PL) constitui uma variante rara dos linfomas cutâneos de células T com a presença de amplo infiltrado intracutâneo de células T positivas para CD30. O exame histológico sugestivo de doença altamente maligna e agressiva opõe-se ao curso crônico-recidivante, muitas vezes auto-limitado, presente na maioria dos casos. São apresentados os relatos de dois pacientes. Uma mulher com 64 anos de idade e história de 10 anos de PL recebeu terapia Puva em creme, sendo a doença controlada. No segundo caso, uma mulher de 42 anos apresentava história de 18 anos de PL tratada com Puva e, posteriormente, com fotoquimioterapia extracorpórea (FEC). Durante a FEC, observou-se rápida disseminação metastática que não pôde ser controlada por poliquimioterapia. A paciente foi a óbito após um ano, em razão de metástase no sistema nervoso central.