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.

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EinleitungBei der Sklerodermie en coup de sabre handelt es sich um eine Bindegewebserkrankung unbekannter ¾tiologie. Sie ist klinisch gekennzeichnet durch eine "Säbelhieb"-artige Sklerose der frontoparietalen Haut. Die Erkrankung stellt eine Sonderform der Morphea dar, die ein Spektrum umfasst, das über die faziale Hemiatrophie bis hin zu Manifestation an anderen Lokalisationen reicht und als Parry-Romberg-Syndrom beschrieben wird. Die Erkrankung, die in 15 % der Fälle in der Kindheit beginnt, kann zu progressiven lang anhaltenden Indurationen der Haut und des subkutanen Gewebes sowie zu einer Atrophie bzw. Zerstö-rung darunter liegender Strukturen wie Muskelgewebe und Knochen führen. Bei Herden en coup de sabre wurden auch ophthalmologische und neurologische Auffälligkeiten beschrieben [1].Zahlreiche therapeutische Ansätze, wie die systemische Gabe von Penicillin, Penicillinamin, Kortikosteroiden, Resorchin, Methotrexat oder Gamma-Interferon, wurden eingesetzt, ohne dass eines dieser Medikamente sicher wirksam oder nebenwirkungsfrei wäre [2,3]. Außerdem werden phototherapeutische Verfahren wie die Photochemotherapie (PUVA) und BreitspekSklerodermie en coup de sabre: Erfolgreiche Therapie mit topischem Calcipotriol und Medium-Dose

Background: Folliculotropic mycosis fungoides (FMF) is a variant of cutaneous T-cell lymphoma that has clinical overlap with a variety of inflammatory follicular unit disorders. However, we describe distinctive presentations of FMF with acneiform features that can be diagnostically challenging, leading to diagnostic delay. Objective: To highlight the importance of histopathologic and immunohistochemical evaluation for diagnostic confirmation of presumed inflammatory follicular unit–based disorders that are unusual in presentation or unresponsive to standard therapies. Methods: A cross-sectional retrospective study of 5 consecutive patients with a histopathologic diagnosis of FMF was conducted. The clinical, histopathologic, immunophenotypic, and molecular genetic features of cases are presented. Results: We describe 5 patients with clinical and histopathologic presentations of FMF masquerading as hidradenitis suppurativa, furunculosis, or acne vulgaris (age range 34–66 years, 4:1 female to male). Clinical morphologies included open and closed comedones, inflammatory pustules, papules and nodules, follicular papules with keratotic plugging, cysts, and scarring involving the face, trunk, and intertriginous areas. All patients failed to respond to standard therapies, including topical and oral antibiotics, topical and oral retinoids, or topical corticosteroids, before receiving the diagnosis of FMF. Lesional skin biopsies showed a perifollicular CD4-positive T-lymphocytic infiltrate with pilotropism, intrafollicular mucin deposition, foreign-body granulomatous inflammation, acute inflammation, and follicular epithelial necrosis. None had concurrent systemic mycosis fungoides. Limitations: Small retrospective cohort study. Conclusion: We present these cases to expand the clinical and histopathologic spectrum of FMF that may strikingly resemble acneiform disorders and to highlight the importance of diagnostic reconsideration with histopathologic evaluation.