L. Kowalzick

Chronic Graft-versus-Host-Disease-Like Dermopathy in a Child with CD4+ Cell Microchimerism

We report the case of an 11-year-old boy suffering from a severe progressive chronic skin disease with clinical features of progressive systemic scleroderma, systemic lupus erythematosus and dermatomyositis. Skin biopsies revealed fibrosis and lichenoid changes and muscle biopsy a myositis. Immunohistology of the skin showed a lichen-ruber-like pattern. Despite repeated extensive investigations, no autoantibodies were detectable. Some of these findings looked like those described in juvenile dermatomyositis. Finally, it could be demonstrated that the boy showed microchimerism with approximately 1% maternal CD4+ lymphocytes in his peripheral blood leukocytes. Furthermore maternal cells could be demonstrated in inflamed muscle tissue. So a graft-versus-host-disease-like pathomechanism appears to be likely. Several systemic therapies have been used with limited success to improve the condition including corticosteroids, azathioprine, cyclosporine A and mycophenolate mofetil. A distinct improvement of erythemas and sclerosis could be achieved by means of low-dose UVA1 phototherapy which was applied with escalating single doses of 3–12 J/cm2 for 35 consecutive days.

Elevated Serum Levels of Soluble Adhesion Molecules ICAM-1 and ELAM-1 in Patients with Severe Atopic Eczema and Influence of UVA1 Treatment

BACKGROUND ICAM-1 is known to be strongly expressed on keratinocytes in lesional atopic eczema correlating with the degree of inflammation. ELAM-1 was found to be expressed on dermal vascular endothelium in lesional atopic eczematous skin. OBJECTIVE The present study was performed to investigate whether elevated serum levels of soluble forms of these molecules are detectable in patients with severe atopic eczema and whether these parameters could be useful markers for disease activity. METHODS Serum levels of soluble ICAM-1 (sICAM-1) and ELAM-1 (sELAM-1) were measured by ELISA in 18 patients with severe atopic eczema before and after UVA1 therapy. RESULTS Before onset of treatment, serum sICAM-1 (565 +/- 99 ng/ml) and sELAM-1 (89.7 +/- 29.9 ng/ml) levels were significantly (p < 0.001) elevated compared to 22 healthy control persons (296 +/- 46 and 48.8 +/- 22.7 ng/ml). After achievement of significant clinical improvement after 3 weeks of UVA1 therapy, there was neither a decrease in serum sICAM-1 nor in sELAM-1 levels. The posttherapeutic serum sICAM-1 and sELAM-1 values remained elevated (p < 0.001) above the normal range. CONCLUSION Based on these data we suggest that (1) serum sICAM-1 and sELAM-1 are elevated in patients with severe atopic eczema, (2) sICAM-1 does not decrease together with reduction of ICAM-1-positive keratinocytes in atopic eczema following clinical improvement and might therefore be mainly of a different origin, i.e. leukocytes/endothelial cells, and that (3) sICAM-1 and sELAM-1 seem not to be suitable markers of actual disease activity in severe atopic eczema.

[Long term treatment of psoriasis with TNF-alpha antagonists. Occurrence of malignant melanoma].

A 51-year-old white man developed de novo a cutaneous malignant melanoma (Stage Ia) after a 30-month treatment period with TNF-alpha-antagonists, consecutively using infliximab, adalimumab and etanercept because of a recalcitrant moderate to severe plaque psoriasis. The patient previously had been treated fumarates for 4 years, cyclosporine A for 2 months and methotrexate for 5 weeks. He also received cycles of cream PUVA and UVB before and then between systemic medications. A possible causal connection between development of melanoma and immunosuppressive treatment is discussed in the light of recent literature. The termination of TNF-alpha-antagonist therapy following development of melanoma is recommended.

Sarkoidale allergische Kontaktdermatitis auf Palladium nach Ohrpiercing

Granulomatöse Gewebereaktionen auf Metallschmuck aus Gold, Silber, Nickel und Palladium sind selten, jedoch schon seit langem bekannt. Wir berichten im Folgenden über eine Patientin, die im Anschluss an Piercing der Ohren mit palladiumhaltigen Ohrstickern knotige Infiltrationen entwickelte. Das Vorliegen einer kontaktallergischen Reaktion konnte durch identische histologische Veränderungen im Bereich einer Epikutantestung mit Palladium aufgezeigt werden.Granulomatous tissue reactions due to jewelry made of gold, silver, nickel and palladium are rare but nevertheless have been known for a long time. A female patient developed nodular infiltrates after having been pierced with ear stickers containing palladium. A contact allergic reaction could be demonstrated as the underlying cause by inducing similar histological changes following patch testing with palladium.

Prolongierte Psoriasistherapie mit TNF-α-Antagonisten

A 51-year-old white man developed de novo a cutaneous malignant melanoma (Stage Ia) after a 30-month treatment period with TNF-alpha-antagonists, consecutively using infliximab, adalimumab and etanercept because of a recalcitrant moderate to severe plaque psoriasis. The patient previously had been treated fumarates for 4 years, cyclosporine A for 2 months and methotrexate for 5 weeks. He also received cycles of cream PUVA and UVB before and then between systemic medications. A possible causal connection between development of melanoma and immunosuppressive treatment is discussed in the light of recent literature. The termination of TNF-alpha-antagonist therapy following development of melanoma is recommended.

Mycophenolate Mofetil in Epidermolysis bullosa acquisita

Letters to Dermatology predominating on the lower limbs. Histology shows capillary dilatations in the superficial dermis, perivascular lymphocytic infiltrate and red blood cell extravasation. The prognosis is usually good with regression in a few months. However, progression of chronic PPD to MF has been reported [1] as well as a purpuric patchy presentation of some early MF [2]. Evolution to MF may be suspected when the eruption lasts several years, is extensive, frequently relapses as in this case and has a reticular arrangement [1]. Here, the diagnosis of PPD has not been histologically documented initially. Thus, it is not possible to know if histological signs of MF were present from the beginning. However, histology, immunohistochemistry and TCR gene analysis are not always sufficient to distinguish PPD from early MF [3, 4]. A long delay before the diagnosis of MF has been reported by Barnhill and Braverman [5] with an average of 8.4 years for their 3 cases. A 16-year delay before the diagnosis of MF has been observed in a peculiar form of PPD evolving into generalized pigmentation and ichthyosis [6]. To our knowledge, such a long delay as in this patient –24 years – has not yet been previously reported [7].

Failure of Etretinate in Epidermodysplasia verruciformis

In a patient suffering from benign lesions of epidermodysplasia verruciformis due to human papillomavirus type 5 little clinical improvement could be observed after 6 months of oral etretinate. The drug was given 75 mg/day for 1 month before dosage was reduced to 50 mg/day. Control biopsy after 3 months of treatment still revealed large quantities of virions and typical cytopathic effects in light and electron microscopic examination.

Fotemustine and interferon α2b in metastatic malignant melanoma

The efficacy of treatment with fotemustine and interferon (IFN) α was evaluated in metastatic melanoma. A group of 50 patients with metastatic malignant melanoma were treated with a combination of IFNα2b and the nitrosourea fotemustine. The patients received 10 MU IFN three times weekly for 3 weeks and fotemustine at a dose of 100 mg/m2 on days 8, 15 and 22. After a 5-week rest period, patients with stabilized or responding disease received a maintenance therapy consisting of 10 MU IFN three times a week for 1 week followed by administration of fotemustine (100 mg/m2) on day 8. This cycle was repeated every 4 weeks until progression occurred. If there was complete remission (CR), treatment was stopped after an additional three cycles. Toxicity and clinical response were scored according to WHO criteria. Objective response was seen in 14 patients (28%; 95% confidence interval 15.6%–40.4%) with four CR and ten partial responses (PR). The median duration of CR was 73 weeks, that of PR 26 weeks. Toxicity was acceptable, enabling treatment on an outpatient basis. The combination of fotemustine with IFNα is effective and well tolerated, but there is no evident advantage over fotemustine monotherapy in the treatment of metastatic melanoma.

Progress of multiple cutaneous and subcutaneous melanoma metastases of the face during imiquimod treatment.

A 68‐year‐old man developed a local recurrence with multiple cutaneous and subcutaneous nodules three months after excision of a primary malignant melanoma of the temple. Despite extensive surgery and adjuvant irradiation, another local recurrence occurred. Following further local progression during dacarbazine chemotherapy, topical treatment with imiquimod was begun and the chemotherapy was changed to fotemustine. During this treatment further local progression occurred and two months later regional lymph node and distant metastasis were detected. The patient died from his tumor disease eighteen months after the first diagnosis of malignant melanoma.

Disseminierte kutane Beteiligung bei Churg-Strauss-Syndrom unter Omalizumab-Therapie

We report on a 48-years old caucasian female patient with severe recalcitrant IgE-mediated bronchial asthma who developed a Churg-Strauss syndrome with rapid progression of marked cutaneous involvement of the erythema migrans type 7 weeks after start of a therapy with omalizumab, a humanized monoclonal antibody directed against IgE (Xolair ® ).