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Asymptomatic Red and Brown Lesions on the Face and Neck of a 15-Year-Old Boy—Diagnosis

Asymptomatic Red and Brown Lesions on the Face and Neck of a 15-Year-Old Boy—Diagnosis Diagnosis: Erythromelanosis follicularis faciei et colli. Microscopic findings and clinical course Histopathologic examination of the skin biopsy specimen revealed mild epidermal hyperplasia and increased melanin in basal keratinocytes. A mild, superficial, perivascular, lymphocytic infiltration surrounded the dilated papillary vasculature. Slightly dilated hair follicles containing lamellar keratin plugs, tortuous hair, and perifollicular fibrosis were noted. The histologic findings supported the clinical diagnosis of erythromelanosis follicularis faciei et colli. The patient was treated with tretinoin cream, 0.05%. The pigmented and erythematous lesions had slightly faded after a 3-month course of treatment, but the follicular papules persisted. Discussion Erythromelanosis follicularis of the face was first described in 6 young Japanese patients by Kitamura et al1 in 1960. It was defined by a clinical triad of erythema, hyperpigmentation, and follicular papules. Involvement of the neck was later described in the United States by Mishima and Rudner.2 This new entity has since been called erythromelanosis follicularis faciei et colli. For many years, erythromelanosis follicularis faciei et colli was thought to occur only in young Asian men. Later, several authors reported the disease in light-skinned men1 and, more rarely, in women.3,4 The clinical picture is distinctive: red-brown pigmentation, telangiectasia, and follicular papules symmetrically distributed in the preauricular area and subsequently extending to the cheek and neck. A unilateral distribution has been reported.3 The dermatosis is usually asymptomatic. However, there have been reports of localized hyperhidrosis and burning experienced on heat exposure.5 Keratosis pilaris of the upper extremities and trunk is a frequently associated finding. Erythromelanosis follicularis differs from keratosis rubra pilaris atrophicans faciei (Brocq) in that it lacks scarring, atrophy, and eyebrow loss. The pathogenesis is unknown, but the possibility of autonomic nerve dysfunction has been proposed.2 Genetic predisposition with probable autosomal recessive transmission was suggested after 2 cases were reported in a Spanish family in which the parents were consanguineous.6,7 On histologic examination, the epidermis is acanthotic and hyperkeratotic without parakeratosis, and there is increased pigmentation of the basal keratinocytes. There are follicular plugging and a slight lymphocyte infiltrate with dilatation of the dermal vessels. Electron microscopic examination has been reported to show centrally localized homogeneous masses of pigment and peripheral stippled pigment within abnormally large melanosomes. The abnormalities occur within the cytoplasm of both melanocytes and keratinocytes and are confined to lesional skin. Direct immunofluorescence examination has been reported to show granular deposition of IgM in the dermoepidermal junction.8 Neither electron microscopy nor immunofluorescence was performed in our patient. The treatment of erythromelanosis follicularis faciei et colli is not clearly defined. Topical retinoic acid, ammonium lactate, and hydroquinone tend to reduce erythema, pigmentation, and follicular prominence.3 Long-pulsed dye laser treatment has been reported to give a satisfactory result, but the number of cases is limited.9 Return to Quiz Case. References 1. Kitamura KKato HMishima YSonoda S Erythromelanosis follicularis faciei [in German]. Hautarzt 1960;11391- 393PubMedGoogle Scholar 2. Mishima YRudner E Erythromelanosis follicularis faciei et colli. Dermatologica 1966;132 (3) 269- 287PubMedGoogle ScholarCrossref 3. McGillis STTuthill RJRatz JLRichards SW Unilateral erythromelanosis follicularis faciei et colli in a young girl. J Am Acad Dermatol 1991;25 (2, pt 2) 430- 432PubMedGoogle ScholarCrossref 4. Ingber AHodak ESandbank M A recent case of erythromelanosis follicularis faciei et colli in a female [in German]. Z Hautkr 1986;61 (19) 1409- 1410PubMedGoogle Scholar 5. Whittaker SJGriffiths WAD Erythromelanosis follicularis faciei et colli. Clin Exp Dermatol 1987;12 (1) 33- 35PubMedGoogle ScholarCrossref 6. Yañez SVelasco JAGonzález MP Familial erythromelanosis follicularis faciei et colli—an autosomal recessive mode of inheritance. Clin Exp Dermatol 1993;18 (3) 283- 285PubMedGoogle ScholarCrossref 7. Acay MC Erythromelanosis follicularis faciei et colli: a genetic disorder? Int J Dermatol 1993;32 (7) 542PubMedGoogle ScholarCrossref 8. Alcalay JIngber AHalevi SDavid MSandbank M Erythromelanosis follicularis faciei in women. Br J Dermatol 1986;114 (2) 267PubMedGoogle ScholarCrossref 9. Kurita MMomosawa AOzaki MBan IHarii K Long-pulsed dye laser for the treatment of erythromelanosis follicularis faciei: report of two clinical cases. Dermatol Surg 2006;32 (11) 1414- 1417PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Asymptomatic Red and Brown Lesions on the Face and Neck of a 15-Year-Old Boy—Diagnosis

Archives of Dermatology , Volume 147 (2) – Feb 1, 2011

Asymptomatic Red and Brown Lesions on the Face and Neck of a 15-Year-Old Boy—Diagnosis

Abstract

Diagnosis: Erythromelanosis follicularis faciei et colli. Microscopic findings and clinical course Histopathologic examination of the skin biopsy specimen revealed mild epidermal hyperplasia and increased melanin in basal keratinocytes. A mild, superficial, perivascular, lymphocytic infiltration surrounded the dilated papillary vasculature. Slightly dilated hair follicles containing lamellar keratin plugs, tortuous hair, and perifollicular fibrosis were noted. The histologic findings...
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References (7)

Publisher
American Medical Association
Copyright
Copyright © 2011 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archdermatol.2010.424-b
Publisher site
See Article on Publisher Site

Abstract

Diagnosis: Erythromelanosis follicularis faciei et colli. Microscopic findings and clinical course Histopathologic examination of the skin biopsy specimen revealed mild epidermal hyperplasia and increased melanin in basal keratinocytes. A mild, superficial, perivascular, lymphocytic infiltration surrounded the dilated papillary vasculature. Slightly dilated hair follicles containing lamellar keratin plugs, tortuous hair, and perifollicular fibrosis were noted. The histologic findings supported the clinical diagnosis of erythromelanosis follicularis faciei et colli. The patient was treated with tretinoin cream, 0.05%. The pigmented and erythematous lesions had slightly faded after a 3-month course of treatment, but the follicular papules persisted. Discussion Erythromelanosis follicularis of the face was first described in 6 young Japanese patients by Kitamura et al1 in 1960. It was defined by a clinical triad of erythema, hyperpigmentation, and follicular papules. Involvement of the neck was later described in the United States by Mishima and Rudner.2 This new entity has since been called erythromelanosis follicularis faciei et colli. For many years, erythromelanosis follicularis faciei et colli was thought to occur only in young Asian men. Later, several authors reported the disease in light-skinned men1 and, more rarely, in women.3,4 The clinical picture is distinctive: red-brown pigmentation, telangiectasia, and follicular papules symmetrically distributed in the preauricular area and subsequently extending to the cheek and neck. A unilateral distribution has been reported.3 The dermatosis is usually asymptomatic. However, there have been reports of localized hyperhidrosis and burning experienced on heat exposure.5 Keratosis pilaris of the upper extremities and trunk is a frequently associated finding. Erythromelanosis follicularis differs from keratosis rubra pilaris atrophicans faciei (Brocq) in that it lacks scarring, atrophy, and eyebrow loss. The pathogenesis is unknown, but the possibility of autonomic nerve dysfunction has been proposed.2 Genetic predisposition with probable autosomal recessive transmission was suggested after 2 cases were reported in a Spanish family in which the parents were consanguineous.6,7 On histologic examination, the epidermis is acanthotic and hyperkeratotic without parakeratosis, and there is increased pigmentation of the basal keratinocytes. There are follicular plugging and a slight lymphocyte infiltrate with dilatation of the dermal vessels. Electron microscopic examination has been reported to show centrally localized homogeneous masses of pigment and peripheral stippled pigment within abnormally large melanosomes. The abnormalities occur within the cytoplasm of both melanocytes and keratinocytes and are confined to lesional skin. Direct immunofluorescence examination has been reported to show granular deposition of IgM in the dermoepidermal junction.8 Neither electron microscopy nor immunofluorescence was performed in our patient. The treatment of erythromelanosis follicularis faciei et colli is not clearly defined. Topical retinoic acid, ammonium lactate, and hydroquinone tend to reduce erythema, pigmentation, and follicular prominence.3 Long-pulsed dye laser treatment has been reported to give a satisfactory result, but the number of cases is limited.9 Return to Quiz Case. References 1. Kitamura KKato HMishima YSonoda S Erythromelanosis follicularis faciei [in German]. Hautarzt 1960;11391- 393PubMedGoogle Scholar 2. Mishima YRudner E Erythromelanosis follicularis faciei et colli. Dermatologica 1966;132 (3) 269- 287PubMedGoogle ScholarCrossref 3. McGillis STTuthill RJRatz JLRichards SW Unilateral erythromelanosis follicularis faciei et colli in a young girl. J Am Acad Dermatol 1991;25 (2, pt 2) 430- 432PubMedGoogle ScholarCrossref 4. Ingber AHodak ESandbank M A recent case of erythromelanosis follicularis faciei et colli in a female [in German]. Z Hautkr 1986;61 (19) 1409- 1410PubMedGoogle Scholar 5. Whittaker SJGriffiths WAD Erythromelanosis follicularis faciei et colli. Clin Exp Dermatol 1987;12 (1) 33- 35PubMedGoogle ScholarCrossref 6. Yañez SVelasco JAGonzález MP Familial erythromelanosis follicularis faciei et colli—an autosomal recessive mode of inheritance. Clin Exp Dermatol 1993;18 (3) 283- 285PubMedGoogle ScholarCrossref 7. Acay MC Erythromelanosis follicularis faciei et colli: a genetic disorder? Int J Dermatol 1993;32 (7) 542PubMedGoogle ScholarCrossref 8. Alcalay JIngber AHalevi SDavid MSandbank M Erythromelanosis follicularis faciei in women. Br J Dermatol 1986;114 (2) 267PubMedGoogle ScholarCrossref 9. Kurita MMomosawa AOzaki MBan IHarii K Long-pulsed dye laser for the treatment of erythromelanosis follicularis faciei: report of two clinical cases. Dermatol Surg 2006;32 (11) 1414- 1417PubMedGoogle ScholarCrossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Feb 1, 2011

Keywords: neck,erythromelanosis follicularis faciei et colli

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