ERYTHROMELANOSIS FOLLICULARIS FACIEI ET COLLI

Three Iranian men of light complexion, aged 15, 18, and 28, were seen in 1992. All three patients were referred with the chief complaint of increasing pigmentation over both cheeks since childhood. The pigmentation had gradually extended to involve the preauricular areas bilaterally. There was no history of pruritus or discomfort. Two patients related an increase in pigmentation to extremes of temperature, and one patient also complained of a burning sensation when exposed to sunlight. The third patient cited no change at different temperatures. All three patients had visited a few local physicians and had received sunblocks and topical steroids with no definite diagnosis. No improvement was noticed by use of the prescribed medications. Family history of pigmentary disturbance was negative in all three cases. Examination in all three cases revealed a well-demarcated reddish-brown pigmentation over both cheeks extending to the preauricular area (Fig. 1). In one patient part of the auricle was also involved. No extension to the neck area was seen in any of the cases. Pinhead sized white follicular papules producing a granular texture were clearly evident over the involved areas (Fig. 2). Vellus hair growth was affected in all three cases. No evidence of scar formation or atrophy was noted. Diascopy in all three patients revealed blanching of the erythematous component of the pigmentation with no change in the brown discoloration. Keratosis pilaris was evident over both arms in all three patients, and in one patient mild keratosis pilaris was also noted on the legs. A biopsy was performed in two patients. Histopathologic examination of both biopsy specimens revealed a similar picture. There was slight hyperkeratosis with no parakeratosis and only mild acanthosis. Follicular hyperplasia and plugging were evident. Fontana staining revealed intense melanin pigmentation of the basal layer. Mild perivascular mononuclear cell infiltration was observed in the upper dermis (Fig. 3).