Dermatology Online Journal

Erythromelanosis follicularis faciei et colli: Report of involvement in two female patients
Ertam I MD¹, Unal I MD², and Alper S MD²
Dermatology Online Journal 11 (2): 23

1. Ege University School of Medicine, Department of Dermatology. iertam@yahoo.com2. Prof. Ege University School of Medicine, Department of Dermatology, Bornova/IZMIR/TURKEY

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Abstract

Erythromelanosis follicularis faciei is a rare disease characterized by reddish brown pigmentation and follicular papules localized on certain areas such as the face and neck. Young men are usually affected, but young women or children may be affected. Bilateral distribution is usual, but it may occur unilaterally. Histopathologically, hyperkeratosis, increased melanization and dilatation of the hair follicle are characteristic. Two female patients with typical localizations and clinical findings of the disease are reported here.

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Erythromelanosis follicularis faciei et colli (EFFC), is a rare disease characterized by hyperpigmentation, erythema, and follicular papules on preauricular and cheek areas. There are approximately fifty reported cases. The etiology of this condition is unknown; it rarely affects women [1, 2, 3]. EFFC is a disease more commonly seen in men. It is characterized by reddish-brown pigmentation, telangiectatic vessels, and pale follicular papules. There is apparently no effective therapy for this condition.

Case-1

Figure 1	Figure 2

A 17-year-old woman presented with erythematous and pigmented macules and papules on the face and arms. The lesions began to appear 5 years prior and continued to spread over time. Examination revealed skin-colored follicular papules on an erythematous telangiectatic base starting from both malar areas and extending to the preauricular and mandibular areas and neck (Fig. 1). In addition, follicular keratotic papules were observed on extensor surfaces of both upper extremities, on both lateral aspects of the back, and on the medial aspects of femoral regions. Systemic examination of the patient was normal. There were no other family members with such complaints. The histopathological examination of a biopsy taken from the face skin revealed thick orthokeratotic keratin layer and an epidermis with mild papillomatosis. An increased number of melanocytes and increased melanin pigment was present in the basal layer; focal lentiginous hyperplasia was also present. Superficial blood vessels were dilated. Focal collagen degeneration was present in the perifollicular area and there was a perivascular mixed inflammatory cell infiltration (Fig. 2).

Figure 3

The histopathological examination of the biopsy taken from the arm revealed hyperkeratosis, enlargement of follicles and follicular shafts, and follicular plugging. Focal lentiginosis hyperplasia and proliferate vessel cross-sections in the dermis were also observed. Findings were consistent with keratosis pilaris (Fig. 3).

Case-2

Figure 4	Figure 5

A 19-year-old woman presented with lesions on her face and neck that began 4 years ago. The dermatological examination revealed skin-colored follicular papules on an erythematous telangiectatic base starting from both malar regions and extending to the preauricular and mandibular areas and to the neck (Fig. 4). Moreover, follicular keratotic papules were observed on the extensor surfaces of her upper extremities (Fig. 5), on both lateral aspects of the back, and on the medial aspects of femoral regions. The systemic examination of the patient was normal. Family history has not been described. Histopathological examination of skin biopsy specimens taken from the face and arms revealed the same findings as case-1 and the diagnosis was established accordingly.

Both patients were treated with salicylic acid (2 %) and retinoic acid (0.01 %) cream. No side effects were observed during treatment except for slight irritation. Although lesions were inhibited in the patients, they recurred after the treatment was discontinued.

Discussion

EFFC, first described by Kitamura in 1960, is seen in Asia mostly involving men [6, 8]. There are also reports of the disease in women [1, 3]. EFFC is an erythematous pigmentary disease that affects follicles. It emerges as sharply marginated reddish-brown lesions in preauricular and maxillary areas [6]. No lesions are seen on the forehead [3]. The skin color may change from pink to brown, and telangiectasia can be observed on the skin [2, 3, 6]. The reddish brown area becomes pale and light brown in diascopy [6]. Pityriasiform desquamation and slight itching may occur. The disease is usually accompanied by keratosis pilaris on the arms and shoulders [3, 4, 6]. Both patients had keratosis pilaris and only case-1 had subjective complaints of slight itching.

The disease is of unknown etiology. Cases often emerge sporadically, however, there are also studies reporting that rarely people from the same family have this disease [5, 9].

Yanez et al. reported that the disease may have an autosomal recessive mode of inheritance. None of our patients had similar complaints among family members.

In EFFC, a slight hyperkeratosis along with pigmentation in the epidermis is observed histopathologically. Hair follicles are enlarged especially in the infundibular area. Sebaceous glands are hypertrophic, and lymphocytic infiltration is present around adnexa [6]. Hyperkeratosis and dilatation of the blood vessels in the upper dermis can be the major characteristic histopathological findings of EFFC. Pigmentation appears to have too much variation [10].

Civatte's poikiloderma, lichen pilaris faciei, ulerythema ophryogenesis and Broq's erythrosis pigmentosa peribuccalis should be considered while the distinctive diagnosis for the disease is established.

There is no effective treatment for the disease as yet. Keratolytic, moisturizing and covering creams can only provide partial protection. Retinoic acid, metronidazole, ammonium lactate cream, and hydroquinone can also be used topically [5].

Application of retinoic and salicylic acids and topical moisturizers produced satisfactory results in our patients during treatment. However, lesions reocurred soon after the treatment was discontinued.

References

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8. Bleehen SS. Disorders of skin colour.In : Champion RE, Burton JL, Burns D&Breathnach eds. Textbook of Dermatology 6th ed., Milan, Blackwell Science Publications, 1998:1753-1815.

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