INTRODUCTION: Hidradenitis suppurativa (HS) is an inflammatory skin disease, which usually occurs after puberty. Even if the pathogenesis of HS is still unproven, numerous studies hypothesized that the disease is triggered by genetic and environmental factors, with hormones playing a central role. Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital malformation in which a failure of Müllerian duct development results in absent uterus and fallopian tubes and malformations of the vagina. CASE PRESENTATION: A 38-year-old Caucasian female referred at our outpatient clinic affected by HS. Before the appearance of HS manifestations, the patient referred to a gynecologist due to difficulties in getting pregnant. The sonographic findings were consistent with MRKH syndrome. An ovarian stimulation for in vitro fertilization was started. However, after 3 months the patient showed the first HS onset. After the hormonal treatment ended, a combined treatment with rifampicin and clindamycin was started, showing a great clinical improvement at week 10. DISCUSSION/ CONCLUSION: HS usually appears after puberty, exhibits premenstrual flare, improves in pregnancy, and worsens postpartum, which indicates a role of hormones and particularly of androgens in its pathophysiology. Contrasting data are reported in the literature. Our case wants to strengthen the idea that hormones could play an important role, probably with other endogenous or exogenous factors. Multidisciplinary investigations will clarify which part is played by each factor in the pathogenesis of HS.
INTRODUCTION: Hidradenitis suppurativa (HS) is an inflammatory skin disease, which usually occurs after puberty. Even if the pathogenesis of HS is still unproven, numerous studies hypothesized that the disease is triggered by genetic and environmental factors, with hormones playing a central role. Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital malformation in which a failure of Müllerian duct development results in absent uterus and fallopian tubes and malformations of the vagina. CASE PRESENTATION: A 38-year-old Caucasian female referred at our outpatient clinic affected by HS. Before the appearance of HS manifestations, the patient referred to a gynecologist due to difficulties in getting pregnant. The sonographic findings were consistent with MRKH syndrome. An ovarian stimulation for in vitro fertilization was started. However, after 3 months the patient showed the first HS onset. After the hormonal treatment ended, a combined treatment with rifampicin and clindamycin was started, showing a great clinical improvement at week 10. DISCUSSION/ CONCLUSION: HS usually appears after puberty, exhibits premenstrual flare, improves in pregnancy, and worsens postpartum, which indicates a role of hormones and particularly of androgens in its pathophysiology. Contrasting data are reported in the literature. Our case wants to strengthen the idea that hormones could play an important role, probably with other endogenous or exogenous factors. Multidisciplinary investigations will clarify which part is played by each factor in the pathogenesis of HS.
Authors: H Kurzen; I Kurokawa; G B E Jemec; L Emtestam; K Sellheyer; E J Giamarellos-Bourboulis; I Nagy; F G Bechara; K Sartorius; J Lapins; D Krahl; P Altmeyer; J Revuz; C C Zouboulis Journal: Exp Dermatol Date: 2008-05 Impact factor: 3.960