Summary Multiple dermatofibromas (DFs) are rare benign fibrohistiocytic lesions characterized by asymptomatic hyperpigmented nodules. Several diseases, like systemic lupus erythematosus (SLE) and human immunodeficiency virus (HIV) infection, are found to be associated with multiple DFs. Here, we designed a systematic review by an electronic search of PubMed and Embase databases to select case reports of multiple DFs (29 May 2021). From 2,145 initially found articles, 96 studies (106 cases) were included. We classified multiple DFs (>1 DF lesion) into four subtypes and separately investigated clinical characteristics of each one: multiple eruptive DFs (MEDFs) (n = 45), multiple (n = 41), multiple clustered DFs (MCDFs) (n = 18), and giant combined (n = 2). The patients’ mean age was 38.3 ± 14.7 years. The majority were female (61.3 %). The lesions were commonly on the trunk and extremities (36.8 %). MEDFs (n = 36) had the most rapid disease onset (2.0 ± 6.6 years). Immunosuppression induced by either HIV (10.3 %) and hematologic malignancy (9.4 %) or immunosuppressive drugs (23.4 %) along with SLE (19.8 %) were the most observed associations. However, 66.7 % of the MCDF patients were otherwise healthy individuals. As for disease management, surgery and follow‐up were the preferred options. Most of the cases showed neither resolution nor development of new lesions in follow‐up.

Immunotherapy by diphenylcyclopropenone (DPCP) is generally started with 2% DPCP sensitization, however in recent years studies have questioned the necessity of sensitization that may cause patients severe reactions and troubles at the onset of therapy. The purpose of the present study was to evaluate the association between the severity of initial reaction to 2% DPCP sensitization in AA patients and clinical response. In this retrospective study, 110 AA patients who continued therapy for at least 6 months were enrolled. Hair loss and hair regrowth rates were calculated based on the Severity of Alopecia Tool (SALT) scoring system. Initial reaction to 2% DPCP sensitization after 2 weeks was graded as negative reaction (absence of any reaction), doubtful reaction (mild erythema, pruritus, and irritation for minutes after test), weak (erythema, mild edema, and scaling), and strong to extreme reaction (vesicles, bullae, ulcer, and discharge). The degrees of the initial reaction to 2% DPCP after 2 weeks were negative reaction 13 (11.81%), doubtful reaction 40 (36.36%), weak reaction 33 (30%), and strong to extreme reaction 24 (21.81%). Patients were divided into two groups: (A) patients with less than 12-month therapy (75 of 110), (B) patients with more than 12-month therapy (35 of 110). Initial reaction to 2% DPCP sensitization was not correlated with hair regrowth rate in either group (group A: Spearman's rho = 0.194, p = 0.095; group B: Spearman's rho = 0.063 p = 0.720). After 12-month treatment with DPCP, hair regrowth rate was significantly greater than 6-months therapy (group A: 17.03 ± 37.78, group B: 49.26 ± 36.34; p = 0.003).The severity of hair loss at the onset of treatment was significantly associated with the response rate in both groups (p-value <0.002). Based on our results, it is the initial severity of the disease and not the initial reaction to 2% DPCP sensitization that predicts the clinical response to DPCP immunotherapy.

Zusammenfassung Multiple Dermatofibrome (DF) sind seltene gutartige fibrohistiozytäre Läsionen, die durch asymptomatische hyperpigmentierte Knoten charakterisiert sind. Mehrere Erkrankungen wie systemischer Lupus erythematodes (SLE) und Infektionen mit dem humanen Immundefizienz‐Virus (HIV) sind mit multiplen DF assoziiert. Hier haben wir eine systematische Übersicht anhand einer elektronischen Recherche der Datenbanken PubMed und Embase konzipiert, um Fallberichte zu multiplen DF auszuwählen (29. Mai 2021). Von den 2145 ursprünglich gefundenen Artikeln wurden 96 Studien (106 Fälle) eingeschlossen. Multiple DF (> 1 DF‐Läsion) wurden in vier Subtypen eingeteilt und für jeden Subtyp wurden die klinischen Charakteristika separat untersucht: multiple eruptive DF (MEDF) (n = 45), multiple DF (n = 41), multiple gruppierte DF (MCDF) (n = 18) und große kombinierte DF (n = 2). Das Durchschnittsalter der Patienten betrug 38,3 ± 14,7 Jahre. Die Mehrzahl waren Frauen (61,3 %). Die Läsionen befanden sich häufig am Stamm und an den Extremitäten (36,8 %). MEDF (n = 36) hatten den schnellsten Krankheitsbeginn (2,0 ± 6,6 Jahre). Neben SLE (19,8 %) war eine durch HIV (10,3 %), maligne hämatologische Erkrankungen (9,4 %) oder Immunsuppressiva (23,4 %) induzierte Immunsuppression die am häufigsten beobachtete Assoziation. Allerdings waren 66,7 % der MCDF‐Patienten ansonsten gesund. Hinsichtlich des Managements der Erkrankung waren Operation und Nachkontrolle die bevorzugten Optionen. Bei den meisten Fällen trat während der Nachkontrolle weder eine Abheilung noch eine Entwicklung neuer Läsionen auf.

6. Makris C, Godfrey VL, Kr€ ahn-Senftleben G et al. Female mice heterozygous for IKK gamma/NEMO deficiencies develop a dermatopathy similar to the human X-linked disorder incontinentia pigmenti.