Christoph Trautmann

Severe Calciphylaxis in a Renal Patient on Long-Term Oral Anticoagulant Therapy

The pathogenesis of calciphylaxis, a potentially life-threatening condition, is not well understood. Several factors such as end-stage renal disease (azotemia), hyperparathyroidism, hyperphosphatemia, hypercalcemia, a high calcium-phosphate product, and the use of steroids and cytotoxic drugs after kidney transplantation are believed to interact in calciphylaxis. Recently, hypercoagulability due to functional protein C deficiency has been suggested to play a pathogenic role in this condition. Here, we present a renal transplant patient, with secondary hyperparathyroidism and on long-term oral anticoagulant therapy, who developed calciphylaxis with severe skin necrosis of her legs. The patients condition improved dramatically after total parathyroidectomy. Hypercoagulability, therefore, does not appear to have played a significant role in this case of calciphylaxis.

Multiple cutaneous B-cell pseudolymphomas after allergen injections

Cutaneous pseudolymphomas are benign lymphoid hyperplasias that clinically and histologically simulate malignant cutaneous B- or T-cell lymphomas. Cutaneous T-cell pseudolymphomas are extremely rare and, in most cases, are caused by systemic treatment with antiepileptic drugs such as phenytoin or carbamazepine. 1 Cutaneous B-cell pseudolymphomas are somewhat more frequent. The single most frequent cause of cutaneous B-cell pseudolymphomas is infection with Borrelia burgdorferi, resulting in lymphocytoma cutis. Because B. burgdorferi induced cutaneous B-cell pseudolymphomas are reactive in nature in most cases, appropriate antibiotic treatment regularly results in complete remission. In contrast, rare cases of B. burgdorferi associated malignant B-cell lymphoma have been reported. 2 Other causes of cutaneous B-cell pseudolymphomas include trauma, insect bites, tattoos, gold hypersensitivity, 3 and cowpox vaccination. Only two cases of B-cell pseudolymphomas resulting from antigen injections for allergy hyposensitization have been reported. 4,5 We describe multiple cutaneous B-cell pseudolymphomas in a 24-year-old woman at sites of antigen injections during allergy hyposensitization for pollinosis.

Seltene Varianten kutaner T-Zell-Lymphome

ZusammenfassungNeben den klassischen kutanen T-Zell-Lymphomen (C-CTCL), wie der Mykosis fungoides und dem Sézary-Syndrom, kommen ungewöhnliche Varianten kutaner T-Zell-Lymphome vor, die als „seltene“ kutane T-Zell-Lymphome bezeichnet werden können. Die Einordnung dieser seltenen kutanen T-Zell-Lymphome ist problematisch und umstritten. Neuere Klassifikationen der Lymphome allgemein wie die Revised European-American Lymphoma (R.E.A.L.) Classification stellen wohldefinierte klinisch-morphologische Entitäten in den Vordergrund. In Anlehnung an den Grundgedanken dieser Klassifikation ist auch größere Klarheit bei den seltenen kuktanen T-Zell-Lymphomen zu erzielen. In dieser Übersicht wird der Versuch gemacht, die seltenen kutanen T-Zell-Lymphome aufzulisten und zu charakterisieren. Wir stellen dabei die folgende Einteilung vor, wobei insbesondere alle pleomorphen, immunoblastischen und großzellig-anaplastischen CD30−kutanen T-Zell-Lymphome entsprechend ihrem Verlauf als progressive T-Zell-Lymphome zusammengefaßt werden: 1) Klinische Sonderformen und Varianten der Mykosis fungoides 2) progressive kutane T-Zell-Lymphome (P-CTCL) einschließlich der transformierten klassischen kutanen T-Zell-Lymphome (TC-CTCL) und der primär-progressiven kutanen T-Zell-Lymphome (PP-CTCL); 3) angiozentrisches und angioimmunoblastisches kutanes T-Zell-Lymphom; 4) großzellig-anaplastisches, CD30+kutanes T-Zell-Lymphom; 5) HTLV-I-assoziierte adulte T-Zell-Leukämie/Lymphom (ATLL); 6) kutane Manifestationen primär extrakutaner T-Zell-Neoplasien; 7) andere, schwer klassifizierbare kutane T-Zell-Lymphome.SummaryBesides the classical forms of cutaneous T-cell lymphoma (C-CTCL), such as mycosis fungoides and Sézarys syndrome, unique variants may be encountered. The classification of these rare cutaneous T cell lymphomas is problematic and controversial. Newer classifications of lymphoma in general, such as the Revised European-American Lymphoma (REAL) Classification, emphasize well-established clinico-pathological entities. It seems appropriate to attempt to bring greater clarity to the classification of cutaneous T-cell lymphomas using the same principles. In this review, we list and characterize the rare variants of cutaneous T-cell lymphoma, such as (1) clinical, histological and immunological variants of mycosis fungoides; (2) progressive cutaneous T-cell lymphoma (P-CTCL) including transformed classical cutaneous T-cell lymphoma (TC-CTCL) and primary progressive cutaneous T-cell lymphoma (PP-CTCL); (3) angiocentric and angioimmunoblastic cutaneous T-cell lymphomas; (4) large cell anaplastic, CD30+ cutaneous T-cell lymphoma; (5) HTLV-Iassociated adult T-cell leukemia/lymphoma (ATLL); (6) cutaneous manifestations of primary extracutaneous T-cell neoplasias; (7) unclassifiable cutaneous T-cell lymphoma.Key wordsRare cutaneous T-cell lymphomas – Classical cutaneous T-cell lymphomas – Primary-progressive cutaneous T-cell lymphomas

Mollusca contagiosa generalisata bei einem afrikanischen Kind mit Aids

ZusammenfassungBei einem Kind aus Zaire mit fortgeschrittener Aids-bedingter Immundefizienz (CDC: P2D1) traten generalisierte Mollusca contagiosa von ungewöhnlicher Größe auf. Während die Molluscum-contagiosum-Infektion bei Immunkompetenten meist selbstlimitiert ist, treten bei fortgeschrittener Immundefizienz disseminierte und persistierende Infektionen auf. Histologisch und immunhistochemisch zeigte sich eine starke Verminderung der Langerhans- und T-Zellpopulationen, die der Disseminierungstendenz zugrunde liegen können. Wegen ihrer klinischen Ähnlichkeit mit Mollusca contagiosa und, aufgrund einer vorausgegangenen systemischen Kryptokokkose im Laufe der Grunderkrankung der Patientin, mußte eine kutane Kryptokokkose differentialdiagnostisch berücksichtigt werden. Als weitere Differentialdiagnosen waren kutane Manifestationen der amerikanischen und afrikanischen Histoplasmose, kutane Toxoplasmose und Pneumocystis-carinii-Infektion sowie kutane Mykobakteriosen in Betracht zu ziehen.SummaryA 12-year-old girl from Zaire with Aids (CDC: P2 D1) presented with a generalized molluscum contagiosum infection. She had suffered from systemic cryptococcosis and from cryptosporidiosis several months before admission. While molluscum contagiosum infection is usually a self-limiting disease in immunocompetent persons, a fulminant appearance and persistence of giant mollusca occurs with advanced immunodeficiency. Histological and immunohistological examinations showed a severe diminution of Langerhans and T cell populations that might enhance the dissemination of the infection. Molluscum-like lesions of cryptococci have been described, and cutaneous cryptococcosis is the main condition to be considered in the differential diagnosis. Further differential diagnoses should include American and African histoplasmosis, and the cutaneous manifestations of mycobacterial infections, of toxoplasmosis and of Pneumocystis carinii infection.

Cutaneous large cell Ki-1-positive lymphoma

Primary cutaneous large cell anaplastic non-Hodgkin lymphomas positive for Ki-1-antigen are rarely described. There are 100 published cases worldwide. Typically large cell anaplastic lymphomas have an inflammatory appearance, which often leads to false diagnosis and unsuccessful treatment with antibiotics. Histological examination reveals a highly malignant non-Hodgkin lymphoma. The tumour is composed of large pleomorphic lymphoid cells composed of T-cells in 80% of the cases and of B-cells in 10%. The immunological phenotype in the remaining 10% remains unclear. Crucial for the diagnosis is the expression of CD30 antigen in >70% of the tumour cells. This article presents 5 cases of cutaneous Ki-1-positive lymphoma seen in our Berlin department during the last 10 years. In 4 patients the diagnosis was established in clinical stage I of cutaneous lymphoma without further manifestation; 1 patient had lymph node involvement and was in stage II. Total excision of the primary tumour in stage I with adjuvant polychemotherapy in stages II – IV led to complete remission in all cases. Long-term remissions were seen in case 1 (2 years) and in case 5 (1 year), whilst 2 patients showed local relapse, and 1 patient showed generalized lymphogenic and haematogenic metastasis. After repeated surgical removal or irradiation of the tumour and adjuvant polychemotherapy, further complete remission was achieved in 2 patients (up to now lasting 1 and 4 years). Another patient has been in partial remission for the last 2 years. Our observations underline the high relapse rate of large cell anaplastic lymphoma. There was no difference in the incidence of relapse whether the primary tumour was surgically excised or treated with total excision plus adjuvant polychemotherapy. In anaplastic tumours of the skin the diagnosis of Ki-1-lymphoma should be considered.Zusammenfassung. Das großzellig-anaplastische Ki-1-positive Non-Hodgkin-Lymphom der Haut ist eine seltene Entität. Bisher wurden weltweit etwa 100 Fälle beschrieben. Typisch für das großzellig-anaplastische Lymphom ist der entzündliche Aspekt des Tumors, der oft zu klinischen Fehldiagnosen führt. Histologisch handelt es sich um ein hochmalignes Non-Hodgkin-Lymphom, bestehend aus großen pleomorphen Blasten, die in 80% der Fälle der T-Zell-Reihe und in 10% der B-Zell-Reihe angehören. 10% der großzellig-anaplastischen Lymphome exprimieren weder T- noch B-Zell-Marker. Das immunhistochemische Charakteristikum ist die Expression des CD30-Antigens in mehr als 70% der Blasten. Wir stellen hier 5 in unserer Klinik in den letzten 10 Jahren beobachtete Patienten vor. 4 Patienten befanden sich bei der ersten Diagnosestellung im Stadium I eines kutanen Lymphoms ohne Nachweis weiterer Organbeteiligung, ein Patient befand sich im Stadium II. Im Stadium I führten wir die Totalexzision des Primärtumors, im Stadium II – IV zusätzlich eine adjuvante Polychemotherapie durch, in allen Fällen mit dem Ergebnis der kompletten Remission. Bei 2 Patienten hält die Remission an (Beobachtungszeitraum 1 bzw. 2 Jahre), 2 Patienten zeigten Rezidive, ein Patient wies eine lymphogene und hämatogene Generalisation auf. Erneute Exzision bzw. Röntgenbestrahlung und adjuvante Polychemotherapie führte in 1 Fall zur partiellen, in 2 Fällen zur kompletten Remission (Beobachtungszeiträume 1 bzw. 4 Jahre). Unsere Beobachtungen unterstreichen die Tatsache, daß das Ki-1-Lymphom häufig in loco rezidiviert und sich auch viszeral manifestieren kann. Bei unseren Fällen fand sich kein Unterschied, ob lediglich eine Exzision des Primärtumors durchgeführt wurde oder ob zusätzlich eine adjuvante Polychemotherapie erfolgte. Bei anaplastischen Tumoren unklarer Herkunft muß auch an das großzellig-anaplastische Lymphom gedacht werden.Abstract. Primary cutaneous large cell anaplastic non-Hodgkin lymphomas positive for Ki-1-antigen are rarely described. There are 100 published cases worldwide. Typically large cell anaplastic lymphomas have an inflammatory appearance, which often leads to false diagnosis and unsuccessful treatment with antibiotics. Histological examination reveals a highly malignant non-Hodgkin lymphoma. The tumour is composed of large pleomorphic lymphoid cells composed of T-cells in 80% of the cases and of B-cells in 10%. The immunological phenotype in the remaining 10% remains unclear. Crucial for the diagnosis is the expression of CD30 antigen in >70% of the tumour cells. This article presents 5 cases of cutaneous Ki-1-positive lymphoma seen in our Berlin department during the last 10 years. In 4 patients the diagnosis was established in clinical stage I of cutaneous lymphoma without further manifestation; 1 patient had lymph node involvement and was in stage II. Total excision of the primary tumour in stage I with adjuvant polychemotherapy in stages II – IV led to complete remission in all cases. Long-term remissions were seen in case 1 (2 years) and in case 5 (1 year), whilst 2 patients showed local relapse, and 1 patient showed generalized lymphogenic and haematogenic metastasis. After repeated surgical removal or irradiation of the tumour and adjuvant polychemotherapy, further complete remission was achieved in 2 patients (up to now lasting 1 and 4 years). Another patient has been in partial remission for the last 2 years. Our observations underline the high relapse rate of large cell anaplastic lymphoma. There was no difference in the incidence of relapse whether the primary tumour was surgically excised or treated with total excision plus adjuvant polychemotherapy. In anaplastic tumours of the skin the diagnosis of Ki-1-lymphoma should be considered.