[Photodynamic therapy of cutaneous T-cell lymphoma at special sites]. / Photodynamische Therapie kutaner T-Zell-Lymphome in besonderer Lokalisation.

Therapy of the heterogenous group of primary cutaneous B- and T-cell lymphomas is based upon type of lymphoma and its staging. Treatment, especially for rare forms, has not yet been standardised. Two patients with uncommon primary cutaneous T-cell lymphomas received both traditional therapy and topical photodynamic therapy (PDT) in areas that are difficult to reach with classic methods (ear, eyebrow, side of foot). The first patient had a rare type of medium/large cell pleomorphic, CD8-positive, CD30-negative, primary cutaneous T-cell lymphoma with primary involvement of the ear. As the tumor progressed during PUVA, interferon alpha and retinoid therapy, topical PDT was employed. A histologically confirmed partial remission was obtained. As the disease further evolved the patient received radiotherapy and has now been in complete remission for 10 months. The other patient preserted with the classical picture of mycosis fungoides in initial tumour stage. This patient received in addition to PUVA therapy and interferon alpha, topical PDT to the eyebrow and side of the foot. A complete remission was achieved and histologically confirmed. In the palliative treatment of cutaneous T-cell lymphoma, PDT is a new experimental method, especially for problem locations. It is tissue sparing, has few side effects, can be repeated as often as necessary and achieves good cosmetic results.

[Post-traumatic pyoderma gangrenosum: combination therapy with intravenous immunoglobulins and systemic corticosteroids]. / Posttraumatisches Pyoderma gangraenosum: Kombinationstherapie mit intravenösen Immunglobulinen und systemischen Kortikosteroiden.

Pyoderma gangraenosum is a rare, chronic type of skin ulceration of unknown aetiology. It occasionally appears following trauma. There are no set standards for the treatment of pyoderma gangraenosum. It is essential to consider acuteness of progression as well as underlying systemic diseases and possible drug side effects before initiating a therapy. Immunosuppressive as well as immunomodulating agents are most often used with varying degrees of success. We report a 45-year-old patient who suffered from an extensive posttraumatic pyoderma gangraenosum after cardiac bypass operation. With short-term combination therapy consisting of high dose intravenous immunoglobulins and systemic corticosteroids, the progression of the disease could be stopped within a few days.

Prospective randomized multicenter clinical trial on the use of interferon -2a plus acitretin versus interferon -2a plus PUVA in patients with cutaneous T-cell lymphoma stages I and II.

Cutaneous T-cell lymphoma (CTCL) constitutes a malignant proliferative disease involving mostly CD4(+) T cells arising in the skin. Because of the lack of curative treatment options, interferons (IFN) have been introduced into the therapy of CTCL. Although effective even in advanced disease, response rates were about 50% and the duration of response was short. To improve the results of interferon monotherapy, combinations of IFN with oral photochemotherapy (PUVA) or retinoids were investigated in nonrandomized trials showing higher response rates. We have therefore conducted this prospective randomized multicenter trial to compare these two combination therapies, ie, IFN plus PUVA and IFN plus acitretin. IFN -2a was administered at 9 MU three times weekly subcutaneously in both groups, with lower increasing doses during the first week. Photochemotherapy was applied after oral intake of 8-methoxypsoralen (0.6 mg/kg body weight) 5x weekly during the first 4 weeks, 3x weekly from weeks 5 through 23, and 2x weekly from weeks 24 through 48, with escalating doses beginning with 0.25 J/cm2. Twenty-five milligrams of acitretin was administered daily during the first week, and 50 mg was administered from weeks 2 through 48. Of 98 patients randomized in this study, 82 stage I and II patients were evaluable: 40 in the IFN+PUVA group and 42 in the IFN+acitretin group. With 70% complete remissions in the IFN+PUVA group, this treatment was significantly superior to the IFN+acitretin group with only 38.1% complete remissions. Time to response was significantly shorter in the IFN+PUVA group, with 18.6 weeks compared with 21.8 weeks in the IFN+acitretin group. Side effects were mostly mild to moderate and did not differ significantly in both treatment groups. However, there were more adverse events leading to study discontinuation in the IFN+acitretin group. Based on these findings, we conclude that IFN plus oral photochemotherapy is superior to IFN plus acitretin, inducing more complete remissions in patients with CTCL stages I and II.

[Congenital poikiloderma with verruciform hyperkeratoses (Dowling type)]. / Kongenitale Poikilodermie mit verruziformen Hyperkeratosen (Typ Dowling).

A male patient with congenital poikiloderma (type Dowling) developed Bowen's disease and initial squamous cell carcinoma. As the patient suffered from hypertriglyceridaemia (Frederickson type IV) and a long term tumor prophylaxis with retinoids was not appropriate, we attempted to treat the hyperkeratotic plaques with dermabrasion. After a period of six month we found complete healing of several plaques and only slight keratoses left in a few others. Long term follow up results are not yet available. Through this case report, the heterogenous clinical picture of congenital poikiloderma with warty hyperkeratoses and its high risk of malignancies is discussed.

[Sarcoidosis in interferon-alpha therapy]. / Sarkoidose bei Interferon-alpha-Therapie.

Sarcoidosis is characterised by the formation of sarcoidal granulomas in all affected organs. Despite intensive research, the cause of the disease in unknown. There are only a few reports suggesting an induction of sarcoidosis by interferons. Three patients are presented in whom sarcoidosis developed during or after interferon alpha therapy. The probability of interferon therapy as cause of sarcoidosis is discussed.

[Association of chronic urticaria with Helicobacter pylori-induced antrum gastritis]. / Assoziation der chronischen Urtikaria mit Helicobacter pylori-induzierter Antrum-Gastritis.

In spite of its high incidence, not much is known about the etiology of chronic urticaria. We performed gastroscopic evaluation of 10 patients in whom no cause for chronic urticaria had been found. In 8 of these 10 patients, Helicobacter pylori was identified in the gastric mucosa. The chronic urticarial lesions disappeared within a few days after starting therapy with amoxicillin and omeprazol.

[Therapy of Sézary syndrome by extracorporeal photochemotherapy]. / Therapie des Sézary-Syndroms durch extrakorporale Photochemotherapie.

Sézary syndrome is the leukemic and erythrodemic form of cutaneous T-cell lymphoma (CTCL). Since 1987 extracorporeal photochemotherapy (ECP) has been available for treatment of erythrodermic CTCL in the USA. Through a group of case reports, we want to emphasize ECP as the treatment of choice for erythrodermic CTCL.

Combination therapy of cutaneous T cell lymphoma with interferon alpha-2a and photochemotherapy.

The efficacy of a combination treatment with interferon alpha-2a and photochemotherapy was investigated in 16 patients with cutaneous T cell lymphoma. During the initial treatment, interferon alpha-2a was given subcutaneously at maximum dose of 9 million IU. Simultaneously, photo-chemotherapy with a maximum single dose of 3.0 J/cm2 was applied. After a complete or partial remission had been achieved, the dose of interferon was continued as permanent treatment at 3-9 million IU a week. Photochemotherapy was maintained twice a week for a minimum of 2 months and then stopped depending on the course of the disease. The combination treatment was well tolerated and all patients responded to the initial therapy. Three patients were withdrawn at an early stage of therapy, as they developed erythrodermia after photochemotherapy. The permanent treatment led to a complete remission in ten out of 13 patients and to a partial remission in three patients. During the follow-up period (now 10-40 months), therapy was stopped in four patients because of progression and in one patient because of newly diagnosed Hodgkin's disease. In another three patients, who developed local recurrences, a repetition of the initial treatment schedule was necessary to keep them in remission.

[Combination therapy with interferon alfa 2a and PUVA in cutaneous T-cell lymphoma]. / Kombinationstherapie mit Interferon alfa 2a und PUVA bei kutanen T-Zell-Lymphomen.

Eleven patients with cutaneous T-cell lymphoma stages Ib-IV a were treated with a combination of interferon alfa-2a and photochemotherapy. The diagnosis was confirmed by histological and immunohistological investigations. During the initial treatment, interferon alfa-2a was given at a maximum dose of 9 million IU 3 times a week. Few patients received for a short time 9 million IU daily s.c. The total dose amounted to an average of 125 million IU. Simultaneously, photochemotherapy with a maximum single dose of 2 J/cm2 was applied. The total dose ranged between 7 and 62.9 J/cm2, which corresponds to an average dose of 22.6 J/cm2. After achieving a complete or partial remission the dose of interferon was reduced to 1.5 or 3 million IU, which was then maintained for long-term treatment 2 or 3 times a week. Photochemotherapy was given twice weekly for a minimum of 2 months and then stopped, depending on the course of the disease. The success of therapy was judged from the clinical and histological picture. The combination treatment was well tolerated and led to complete remission in 5 patients (45%) and a partial remission in 6 patients (55%) after the initial therapy which lasted for an average of 48 days. During the follow-up period, which now amounts to 2-13 months with under 1.5 or 3 million IU interferon, three patients have shown disease progression. One patient responded well to newly applied photochemotherapy and in two patients local tumors regressed after radiation.

[Multiple woolly hair nevi with linear epidermal nevus and persistent pupillary membrane]. / Multiple Wollhaarnävi mit linearem epidermalen Nävus und persistierender Pupillarmembran.

The woolly hair nevus is a very rare disease that only occurs sporadically. So far, approximately 48 patients with woolly hair nevus have been described in the world literature. Both sexes are equally affected. Mostly, the dermatosis manifests itself from birth or within the first 2 years of life. Up to 5 circumscribed areas with clearly lighter curly hairs can be observed. About 50% of the patients present a linear epidermal nevus at the same time. Microscopically, the hair shaft diameter of the woolly hair is significantly reduced. Next to oval structures the cross-sections of the hair often show triangular and irregular forms. Furthermore, pili canaliculi and pili torti as well as circumscribed cuticula defects can be observed. We present a 5-year-old boy, who manifested 4 woolly hair nevi on the capillitium in the 9th month of life. From the 2nd year of life onwards he developed a linear epidermal nevus. The ophthalmological examination showed a persistent pupillary membrane. Based on this case and a bibliographical overview, the light and electron microscopical peculiarities are presented.

[Squamous cell cancers in Hallopeau-Siemens recessive dystrophic epidermolysis bullosa]. / Spinozelluläre Karzinome bei Epidermolysis bullosa dystrophica recessiva Hallopeau-Siemens.

Recessive dystrophic epidermolysis bullosa is one of the most severe hereditary mechano-bullous diseases, characterized by scarring blister formation, nail dystrophy and onycholysis, cutaneous contractures, synechiae, mutilations of the hands and feet and oesophageal stenosis. With increasing age the patients may develop multiple, fast-growing and early-metastasizing squamous cell carcinomas. When epidermolysis is present, precise determination of which of the various forms is concerned is necessary soon after birth, to make it possible to advise parents about the prognosis of the disease and the likelihood of its occurrence in further children. In pregnancies at risk of severe epidermolysis bullosa a prenatal diagnosis should be performed. We present two siblings with recessive dystrophic epidermolysis bullosa, each of whom developed two squamous cell carcinomas.