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A Cross-sectional Study of 128 Cases

Pierre Wolkenstein, MD, PhD; Jacques Zeller, MD; Jean Revuz, MD; Emmanuel Ecosse, PhD; Alain Leplège, MD, PhD

Arch Dermatol. 2001;137:1421-1425.

Background  Neurofibromatosis type 1 affects quality of life (QoL) through association with severe complications, impact on cosmetic features, and uncertainty of the effects of the disorder.

Objective  To evaluate the impact of the severity and visibility of neurofibromatosis type 1 on QoL.

Design  Monocenter, cross-sectional study.

Setting  One French academic dermatological and neurofibromatoses clinic.

Patients  A total of 128 adult patients with neurofibromatosis type 1.

Main Outcome Measures  Evaluation of severity and visibility using, respectively, the Riccardi and Ablon scales. Evaluation of skin disease–specific and general QoL using, respectively, Skindex-France and SF-36 (Short Form 36 health survey) profiles controlled for sex, age, severity, and visibility.

Results  In a multiple regression model controlling for sex, age, and visibility, visibility remained independently associated with the alteration of 3 aspects of the skin disease–specific QoL (Skindex-France): emotions, physical symptoms, and functioning (P = .03, P = .009, and P = .002, respectively). Patients with more severe neurofibromatosis reported more effects on the following domains of their general health QoL (SF-36): physical function, bodily pain, general health perception, and vitality (P = .006, P = .03, P = .01, and P = .04, respectively).

Conclusions  Neurofibromatosis type 1 has a significant impact on QoL through alteration of health and appearance. The consequences of visibility and severity from the viewpoint of patients can be evaluated using Skindex and the SF-36, respectively.

From the Department of Dermatology and Réseau NF-Mondor, Hôpital Henri-Mondor, Assistance Publique des Hôpitaux de Paris, Paris XII University (Drs Wolkenstein, Zeller, and Revuz), the Centre d'Investigation Clinique, Hôpital Henri-Mondor, Assistance Publique des Hôpitaux de Paris (Dr Wolkenstein) Créteil, France; and Inserm Unit 292, Hôpital de Bicêtre, Le Kremlin-Bicêtre, France (Drs Ecosse and Leplège).

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