The Journal of Dermatology Vol. 18: 167-170, 1991
A Case of Erythromelanosis Follicularis Faciei with a Unique Distribution Taisuke Seki, Shozo Takahashi and Masaaki Morohashi Abstract A If-year-old japanese boy with erythromelanosis follicularis faciei (EFF) with an unusual distribution is described. The clinical features of classic EFF consist of reddish-brown discoloration of the skin with fine follicular hyperkeratosis in the preauricular areas, and occasionally on the temples and the lateral aspects of the neck. The present case is unique in that similar lesions also occurred on the auricles and eyebrows. Both light and electron microscopic findings of the lesions were consistent with the diagnosis of EFF. Key words:
erythromelanosis follicularis faciei; auricle; eyebrow
Introduction Erythromelanosis follicularis faciei (EFF) was first described by Kitamura et al. in 1960 (1). The typical clinical feature is reddishbrown discoloration of the skin with follicular hyperkeratosis, located mainly in the preauricular regions, but occasionally over the temples and the lateral aspects of the neck. To our knowledge, however, the presence of the lesions on the auricles and the eyebrows, as seen in this patient, has not been documented. We will also report the results of both light and electron microscopic studies of the lesions.
Fig. 1. Diffuse reddish-brown discoloration with fine follicular hyperkeratotic papules of the right preauricular region.
Case Report A 16-year-oldjapanese boy visited our Dermatology Clinic in july of 1987 complaining of bilateral reddish-brown discoloration of the cheeks, auricles, and eyebrows. When he was 8 years old, he first noticed asymptomatic erythema and papules on both eyebrows. Since the age of 10, similar lesions have gradually spread over the cheeks and the ears. The lesions have always been asymptomatic. There Received November 30, 1990; accepted for publication February 4,1991. Department of Dermatology, Faculty of Medicine, Toyama Medical and Pharmaceutical University, Toyama, Japan. Reprint requests to: Taisuke Seki, M.D., Department of Dennatology, Faculty of Medicine, Toyama Medical and Phannaceutical University, 2630 Sugitani, Toyama-shi 930-01. Japan.
is no family history of similar skin diseases. The patient has been otherwise healthy and denies a history of jaundice, prolonged fever and malaise, severe sunburns, or generalized skin eruptions. His laboratory examinations, performed in 1986, were unremarkable exept for elevated GOT. His father has a history of chronic hepatitis of unknown etiology. Examination of his skin revealed diffuse, irregular, bilateral, reddish-brown discoloration of the skin wi"th follicular hyperkeratosis involving the cheeks, preauricular areas (Fig. 1), ears (Fig. 2), and the eyebrows (Fig. 3). On diascopy, the redness disappeared, leaving only a brownish hue suggestive ofhyperpigmentation. Follicular hyperkeratosis was absent elsewhere, including the upper arms.
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Fig. 2. Reddish-brown discoloration with fine follicular hyperkeratotic papules on the left auricule.
Fig. 3. Reddish-brown discoloration with fine follicular hyperkeratotic papules in the right eyebrow region.
Fig. 4. Mild hyperkeratosis, acanthosis, follicular horny plugs, and hyperpigmentation in the basal layer in the epidermis. Dilatation of superficial dermal blood vessels and a minimal lymphocytic perivascular infiltration in the dermis (xl00). The following laboratory examinations performed at his outpatient visit were within normal limits: complete blood cell count, erythrocyte sedimentation rate, renal function tests, serum cholesterol, and triglyceride. His liver function tests revealed an elevated alkaline phosphatase (I8.9RAU, normal 2.7-10), elevated COT (78KU, normal 8-40), elevated CPT (85KU, normal 5-35), and elevated y-CTP (266IU/I, normal 6-80). A biopsy specimen was obtained from the right preauricular region. Mild hyperkeratosis, acanthosis, follicular horny plugs, and hyperpigmentation in the basal layer were observed in the epidermis.
Dilatation of superficial dermal blood vessels and a minimal lymphocytic perivascular infiltration were observed in the dermis (Fig. 4). Electron microscopic examination revealed almost normal findings except for the melanosomes; most of them were found in the form of melanosome complexes and were abundant in the cytoplasm of basal cells (Fig. 5). Discussion Kitamura et al. described this disorder, seen in 6 Japanese men between 17 to 22 years old, as erythromelanosis follicularis faciei (EFF).
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Fig. 5. Melanosomes, most of which were found in the form of melanosome complexes, were abundant in the cytoplasm of basal cells (x13,OOO).
Later, 14 more cases of this disorder have been reported. In the first report by Kitamura et al. all 6 cases had symmetrical eruptions on the face and neck. The clinical picture was that of a reddish-brown discoloration with follicular papules occurring over both sides of the face and neck. In 1966, Mishima and Rudner (2) reported 4 cases of EFF in Caucasian men between 37 and 43 years old. The clinical findings were identical to those reported by Kitamura et al. Although EFF was initially regarded as a disease of men, Anderson (3) reported a case of EFF in a woman in 1980. This was followed by a case report of EFF in a 21-year-old woman by Ingber et al. (4), and, in 1985, Alcalay (5) added 5 more cases. Thus, it is apparent that EFF affects both sexes. EFF usually involves the preauricular areas, the cheeks, and the lateral aspects of the neck bilaterally. Unilateral involvement has been documented in only one case (6). However, to the best of our knowledge, the involvement of the auricles and the eyebrows has never been reported.
Because of the presence of follicular hyperkeratosis on and around the eyebrows, we also entertained the differential diagnosis of ulerythema ophryogenes. However, the absence of hair loss on the lateral half of the eyebrows, perifollicular atrophy, or scarring persuaded us against this possibility. Other diseases, such as keratosis pilaris of the face, erythrose peribuccale pigmentaire (Brocq), and keratospilaire rouge could also be considered. However, these conditions are more or less descriptive in nature and have not been established as entities. Furthermore, these and other differential diagnoses do not present the combination of hyperpigmentation, follicular hyperkeratosis, and erythema of the skin. Electron microscopic examination of EFF was described by Borkovic et al., who noted abnormalities of some melanosomes. There were tentral homogeneous masses of pigment and peripheral stippled pigment in these melanosomes. In addition, a few melanosomes were abnormally large in size. These abnormalities were observed in the cytoplasm of both
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melanocytes and epidermal cells. In our examination, melanosomes were abundant in the keratinocytes of the basal layer; many of them were found in the form of melanosome complexes. However, the melanosome complexes may not necessarily be pathogenomonic of EFF; they are also frequently observed in normal Japanese individuals without skin lesions. We presume that EFF may be a much more prevalent condition than is suggested by the small number of cases reported in the literature. This may be because very few patients with EFF actually seek medical evaluation, since it is a relatively mild cosmetic problem; above all, it is asymptomatic. The pathogenesis, epidemiology, and therapy of EFF remain unknown. Future studies should include epidemiological surveys of EFF, the response of
EFF to various therapeutic modalities, and more ultrastructural and biochemical investigations. References 1) Kitamura K, Kato H, Mishima Y, Sonoda S: Erythromelanosis follicularis faciei, DerHautarzi, 9: 391-393, 1960. 2) Mishima Y, Rudner E: Erythromelanosis follicularis faciei et colli, Dermatologica, 132: 269-287,1966. 3) Andersen BL: Erythromelanosis follicularis faciei et colli, BrJ Dermatol, 102: 323-325, 1980. 4) Ingber A, Hodak E, Sandbank M: A new case of erythromelanosis foIlicularis faciei et colli in a female patient, Z Hautkr, 61: 1409-1410,1986. 5) AIcalayJ, Ingber A, Halevi S, David M, Sandbank M: Erythromelanosis follicularis faciei in women, Br J Dermatol, 114: 267, 1986. 6) Borkovic SP, Schwartz RA, Scott MN: Unilateral erythromelanosis foIlicularis faciei et colli, Cutis, 33: 163-169,1984.
A Case of Erythromelanosis Follicularis Faciei with a Unique Distribution Taisuke Seki, Shozo Takahashi and Masaaki Morohashi Abstract A If-year-old japanese boy with erythromelanosis follicularis faciei (EFF) with an unusual distribution is described. The clinical features of classic EFF consist of reddish-brown discoloration of the skin with fine follicular hyperkeratosis in the preauricular areas, and occasionally on the temples and the lateral aspects of the neck. The present case is unique in that similar lesions also occurred on the auricles and eyebrows. Both light and electron microscopic findings of the lesions were consistent with the diagnosis of EFF. Key words:
erythromelanosis follicularis faciei; auricle; eyebrow
Introduction Erythromelanosis follicularis faciei (EFF) was first described by Kitamura et al. in 1960 (1). The typical clinical feature is reddishbrown discoloration of the skin with follicular hyperkeratosis, located mainly in the preauricular regions, but occasionally over the temples and the lateral aspects of the neck. To our knowledge, however, the presence of the lesions on the auricles and the eyebrows, as seen in this patient, has not been documented. We will also report the results of both light and electron microscopic studies of the lesions.
Fig. 1. Diffuse reddish-brown discoloration with fine follicular hyperkeratotic papules of the right preauricular region.
Case Report A 16-year-oldjapanese boy visited our Dermatology Clinic in july of 1987 complaining of bilateral reddish-brown discoloration of the cheeks, auricles, and eyebrows. When he was 8 years old, he first noticed asymptomatic erythema and papules on both eyebrows. Since the age of 10, similar lesions have gradually spread over the cheeks and the ears. The lesions have always been asymptomatic. There Received November 30, 1990; accepted for publication February 4,1991. Department of Dermatology, Faculty of Medicine, Toyama Medical and Pharmaceutical University, Toyama, Japan. Reprint requests to: Taisuke Seki, M.D., Department of Dennatology, Faculty of Medicine, Toyama Medical and Phannaceutical University, 2630 Sugitani, Toyama-shi 930-01. Japan.
is no family history of similar skin diseases. The patient has been otherwise healthy and denies a history of jaundice, prolonged fever and malaise, severe sunburns, or generalized skin eruptions. His laboratory examinations, performed in 1986, were unremarkable exept for elevated GOT. His father has a history of chronic hepatitis of unknown etiology. Examination of his skin revealed diffuse, irregular, bilateral, reddish-brown discoloration of the skin wi"th follicular hyperkeratosis involving the cheeks, preauricular areas (Fig. 1), ears (Fig. 2), and the eyebrows (Fig. 3). On diascopy, the redness disappeared, leaving only a brownish hue suggestive ofhyperpigmentation. Follicular hyperkeratosis was absent elsewhere, including the upper arms.
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Fig. 2. Reddish-brown discoloration with fine follicular hyperkeratotic papules on the left auricule.
Fig. 3. Reddish-brown discoloration with fine follicular hyperkeratotic papules in the right eyebrow region.
Fig. 4. Mild hyperkeratosis, acanthosis, follicular horny plugs, and hyperpigmentation in the basal layer in the epidermis. Dilatation of superficial dermal blood vessels and a minimal lymphocytic perivascular infiltration in the dermis (xl00). The following laboratory examinations performed at his outpatient visit were within normal limits: complete blood cell count, erythrocyte sedimentation rate, renal function tests, serum cholesterol, and triglyceride. His liver function tests revealed an elevated alkaline phosphatase (I8.9RAU, normal 2.7-10), elevated COT (78KU, normal 8-40), elevated CPT (85KU, normal 5-35), and elevated y-CTP (266IU/I, normal 6-80). A biopsy specimen was obtained from the right preauricular region. Mild hyperkeratosis, acanthosis, follicular horny plugs, and hyperpigmentation in the basal layer were observed in the epidermis.
Dilatation of superficial dermal blood vessels and a minimal lymphocytic perivascular infiltration were observed in the dermis (Fig. 4). Electron microscopic examination revealed almost normal findings except for the melanosomes; most of them were found in the form of melanosome complexes and were abundant in the cytoplasm of basal cells (Fig. 5). Discussion Kitamura et al. described this disorder, seen in 6 Japanese men between 17 to 22 years old, as erythromelanosis follicularis faciei (EFF).
A Case of EFF with a Unique Distribution
169
Fig. 5. Melanosomes, most of which were found in the form of melanosome complexes, were abundant in the cytoplasm of basal cells (x13,OOO).
Later, 14 more cases of this disorder have been reported. In the first report by Kitamura et al. all 6 cases had symmetrical eruptions on the face and neck. The clinical picture was that of a reddish-brown discoloration with follicular papules occurring over both sides of the face and neck. In 1966, Mishima and Rudner (2) reported 4 cases of EFF in Caucasian men between 37 and 43 years old. The clinical findings were identical to those reported by Kitamura et al. Although EFF was initially regarded as a disease of men, Anderson (3) reported a case of EFF in a woman in 1980. This was followed by a case report of EFF in a 21-year-old woman by Ingber et al. (4), and, in 1985, Alcalay (5) added 5 more cases. Thus, it is apparent that EFF affects both sexes. EFF usually involves the preauricular areas, the cheeks, and the lateral aspects of the neck bilaterally. Unilateral involvement has been documented in only one case (6). However, to the best of our knowledge, the involvement of the auricles and the eyebrows has never been reported.
Because of the presence of follicular hyperkeratosis on and around the eyebrows, we also entertained the differential diagnosis of ulerythema ophryogenes. However, the absence of hair loss on the lateral half of the eyebrows, perifollicular atrophy, or scarring persuaded us against this possibility. Other diseases, such as keratosis pilaris of the face, erythrose peribuccale pigmentaire (Brocq), and keratospilaire rouge could also be considered. However, these conditions are more or less descriptive in nature and have not been established as entities. Furthermore, these and other differential diagnoses do not present the combination of hyperpigmentation, follicular hyperkeratosis, and erythema of the skin. Electron microscopic examination of EFF was described by Borkovic et al., who noted abnormalities of some melanosomes. There were tentral homogeneous masses of pigment and peripheral stippled pigment in these melanosomes. In addition, a few melanosomes were abnormally large in size. These abnormalities were observed in the cytoplasm of both
170
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melanocytes and epidermal cells. In our examination, melanosomes were abundant in the keratinocytes of the basal layer; many of them were found in the form of melanosome complexes. However, the melanosome complexes may not necessarily be pathogenomonic of EFF; they are also frequently observed in normal Japanese individuals without skin lesions. We presume that EFF may be a much more prevalent condition than is suggested by the small number of cases reported in the literature. This may be because very few patients with EFF actually seek medical evaluation, since it is a relatively mild cosmetic problem; above all, it is asymptomatic. The pathogenesis, epidemiology, and therapy of EFF remain unknown. Future studies should include epidemiological surveys of EFF, the response of
EFF to various therapeutic modalities, and more ultrastructural and biochemical investigations. References 1) Kitamura K, Kato H, Mishima Y, Sonoda S: Erythromelanosis follicularis faciei, DerHautarzi, 9: 391-393, 1960. 2) Mishima Y, Rudner E: Erythromelanosis follicularis faciei et colli, Dermatologica, 132: 269-287,1966. 3) Andersen BL: Erythromelanosis follicularis faciei et colli, BrJ Dermatol, 102: 323-325, 1980. 4) Ingber A, Hodak E, Sandbank M: A new case of erythromelanosis foIlicularis faciei et colli in a female patient, Z Hautkr, 61: 1409-1410,1986. 5) AIcalayJ, Ingber A, Halevi S, David M, Sandbank M: Erythromelanosis follicularis faciei in women, Br J Dermatol, 114: 267, 1986. 6) Borkovic SP, Schwartz RA, Scott MN: Unilateral erythromelanosis foIlicularis faciei et colli, Cutis, 33: 163-169,1984.
