Hintergrund und Ziel: Als seltene Form der Mycosis fungoides (MF), ist die follikulotrope MF (FMF) durch ein breites Spektrum klinischer Symptome gekennzeichnet. Dazu gehören, neben den vorherrschenden follikulären Läsionen, auch viele atypische Manifestationen. Das Ziel der vorliegenden Studie war eine klinische Bewertung von FMF-Patienten, unter besonderer Berücksichtigung von vernachlässigten dermatologischen Merkmalen. Patienten und Methodik: Insgesamt wurden 27 FMF-Patienten aus dem 572 Patienten umfassenden MF-Register unserer Abteilung retrospektiv bezüglich ihrer Demographie sowie der klinischen Merkmale, Behandlungsformen, Nachsorge und Therapieergebnisse bewertet. Ergebnisse: Neben den bekannten klinischen Symptomen der FMF fanden wir Lichen-spinulosus-artige Läsionen mit begleitender Hypopigmentierung (n = 3) und Alopezie (n = 2), infiltrierte/erhabene, erythematöse Plaques im Gesicht, die zunächst als Lupus tumidus angesehen wurden (n = 2), pseudotumorale Läsionen, die klinisch eine MF im Tumorstadium vortäuschten (n = 1), dauerhafte Exkoriationen (n = 1), erythematöse, Rosazea-artige Papeln im Gesicht (n = 1) sowie kuppelförmige, asymptomatische, mit Muzin gefüllte (in der Histologie) Papeln/Knoten (n = 2), die andere krankheitsbedingte Läsionen überlagerten. Es kamen mehrere Therapieansätze mit unterschiedlichem Ergebnis zur Anwendung. Acht (29,6 %) Patienten hatten FMF im Spätstadium. Schlussfolgerungen: Das Bewusstsein für vernachlässigte klinische Symptome kann wesentlich dazu beitragen, verspätete Diagnosen dieser aggressiven MF-Variante zu verringern. SummaryBackground and Objective: A rare variant of mycosis fungoides (MF), folliculotropic MF (FMF) is characterized by a broad clinical spectrum that primarily includes follicle-based lesions but also many atypical clinical manifestations. The objective of the present study was to conduct a clinical analysis of patients with FMF, with a particular focus on highlighting underrecognized dermatological features.

Background. Hidradenitis suppurativa (HS) is a chronic, relapsing and debilitating inflammatory disease associated with profound morbidity. Aim. In this multicentre study, we investigated the demographic and clinical features of HS, and determined risk factors of disease severity. Methods. In total, 1221 patients diagnosed with HS from 29 centres were enrolled, and the medical records of each patient were reviewed. Results. The mean age of disease onset was 26.2 AE 10.4 years, and almost 70% (n = 849) of patients were current or former smokers. Mean disease duration was 8.9 AE 8.4 years with a delay in diagnosis of 5.8 AE 3.91 years. Just over a fifth (21%; n = 256) of patients had a family history of HS. The axillary, genital and neck

Background:The frequency of clinicoepidemiological variants of Kaposi's sarcoma (KS) differs markedly throughout the world. The iatrogenic variant is mainly associated with the use of immunosuppressive therapy.Aims:We aimed to investigate the distribution of KS variants in our practice and elucidate the underlying causes of iatrogenic KS.Methods:Consecutive KS patients seen in a single tertiary center were grouped according to the tumor variants and iatrogenic KS patients were evaluated about associated conditions.Results:Among 137 patients, classic variant was the most frequent presentation (n = 88), followed by iatrogenic (n = 37) variant. Among the iatrogenic group, ten were transplant recipients. In 16 iatrogenic KS patients, systemic corticosteroid was used, in four for myasthenia gravis (MG) and in three for rheumatoid arthritis. In three patients, KS developed under topical corticosteroid (TC) treatment. Among iatrogenic KS patients, ten of them had a second primary neoplasm and one had congenital immunodeficiency syndrome.Conclusions:Our study revealed one of the highest rates for iatrogenic KS (27%) reported in the literature. Besides well-known causes, relatively frequent association with MG was remarkable. Usage of different forms of TCs was the cause of KS in a few cases.

Cold-associated perniosis of the thighs ("equestrian-type" chilblain): a reappraisal based on a clinicopathologic and immunohistochemical study of 6 cases.

Background Xeroderma pigmentosum (XP) is a rare genodermatosis with a lifelong propensity to develop malignant skin tumors. Methods In this retrospective study, 24 XP patients were evaluated with regard to frequency and clinicopathological features of benign and malignant skin tumors. Results Seventeen patients had at least one malignant skin tumor diagnosed: basal cell carcinoma (BCC) in 13 patients (n = 72), basosquamous carcinoma in three patients (n = 4), squamous cell carcinoma in six patients (n = 13), keratoacanthoma in three patients (n = 15), and melanoma in six patients (n = 18). Most melanomas (n = 15) were in situ lesions. Several benign skin tumors were noted such as tricholemmoma (n = 1), trichoepithelioma (n = 1), trichoblastoma (n = 1), follicular infundibulum tumor (n = 1), keratoacanthoma‐like follicular lesion (n = 1), adnexal tumors with folliculosebaceous (n = 1) and tricholemmal differentiation (n = 1), and neurofibroma (n = 1). Benign vascular proliferations including pyogenic granulomas (n = 8), widespread telangiectasias, and senile angioma‐like lesions were also observed in 3, 5, and 5 patients, respectively. Conclusions Similar to many reports, BCC was found to be the most common malignant skin tumor. The high prevalence of benign adnexal tumors of follicular differentiation, some of them showing mixed histopathological features and various vascular proliferations in our series raises the question of whether they indicate a formerly undescribed association with XP.