Penyakit Fox-Fordyce adalah kelainan kulit yang jarang terjadi, bahkan insidensnya tidak diketahui dengan pasti. Gambaran klinis penyakit tersebut berupa papul folikular multipel berbentuk kubah sewarna kulit yang sangat gatal. Daerah yang terkena adalah daerah yang mengandung banyak kelenjar apokrin, paling sering di aksila, serta cenderung bilateral. Etiologi dan faktor pencetusnya belum diketahui. Diduga udara panas, kelembaban, dan stres merupakan faktor pencetus eksaserbasi. Terdapat hipotesis bahwa manifestasi klinis disebabkan oleh sumbatan keratin dalam lumen infundibula folikel, pada tempat masuk duktus apokrin ke dinding folikular. Pengaruh hormon dan genetik masih diperdebatkan. Secara histopatologik, gambaran yang paling sering ditemukan berupa dilatasi dan hiperkeratosis infundibula folikel yang menyumbat akrosiringium apokrin. Diagnosis banding di antaranya adalah folikulitis, liken planus folikular, liken nitidus, dermatitis kontak, skabies, dan dermatitis kronik. Penyakit ini sulit diterapi. Terdapat beberapa laporan keberhasilan terapi, namun belum ada terapi yang dapat diterima secara luas. Menghindari keringat berlebih dan udara panas dapat mengurangi gejala. (MDVI 2011; 38/2:89-95)

Kata kunci: penyakit Fox-Fordyce, patogenesis, gambaran klinis, diagnosis banding, terapi

      Fox-Fordyce disease is a rare skin disorder. The incidence is unknown. Clinical feature consists of multiple dome-shaped, flesh-colored follicular papules, which extremely pruritic. It involves areas that are anatomically rich in apocrine glands, with most common site being the axillae, usually bilaterally. The etiology and triggering factors are unknown. Heat, humidity and stress are thougt to be exacerbating factors. There are some hypothesis that the clinical manifestation of Fox-Fordyce disease is caused by intraluminal keratin plug of follicle infundibulum at the apocrine duct opening in the follicular wall. Hormonal and genetic factors are still debatable. Histopathologically, the most common findings are dilatation and hyperkeratosis of follicle infundibulum which occluded the apocrine acrosyringium. The differential diagnosis includes folliculitis, follicular lichen planus, lichen nitidus, contact dermatitis, scabies, chronic dermatitis, etc. Fox- Fordyce disease is difficult to treat. There are some reports of succesful therapy, but none of those are widely accepted. Avoiding excessive sweat and heat can reduce symptoms.(MDVI 2011; 38/2:89-95)

Keywords: Fox-Fordyce disease, pathogenesis, clinical feature, differential diagnosis, therapy