Coexistence of erythema dyschromicum perstans, frontal fibrosing alopecia, and facial papules

Irem Ozdemir1, Funda Tamer1, Betul Ogut2, Ozlem Erdem2

1Department of Dermatology, Gazi University, School of Medicine, Ankara, Turkey, 2Department of Pathology, Gazi University, School of Medicine, Ankara, Turkey

Corresponding author: Funda Tamer, MD, E-mail: fundatmr@yahoo.com

How to cite this article: Ozdemir I, Tamer F, Ogut B, Erdem O. Coexistence of erythema dyschromicum perstans, frontal fibrosing alopecia, and facial papules. Our Dermatol Online.2024;15(1):88-89.
Submission: 02.08.2023; Acceptance: 28.10.2023
DOI: 10.7241/ourd.20241.22

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© Our Dermatology Online 2024. No commercial re-use. See rights and permissions. Published by Our Dermatology Online.


Sir,

Erythema dyschromicum perstans is a chronic skin disorder manifesting with hyperpigmented macules on the trunk, extremities, neck, and face [1]. It has been suggested that various factors such as radiocontrast agent exposure and cobalt allergy might play role in the development of erythema dyschromicum perstans. However, the exact cause of erythema dyschromicum perstans has not been elucidated yet [2,3]. The disease affects males and females equally and may occur at any age [4]. The definitive diagnosis of erythema dyschromicum perstans is usually made based on clinical and histopathological features. The treatment of erythema dyschromicum perstans may be troublesome since the disease may have a progressive course [1].

A 72-year-old Caucasian female presented with a seven-year history of asymptomatic hyperpigmented macules on the face without photoaggravation and hair loss (Figs. 1a1c). Two years after the appearance of the symptoms, a skin biopsy was performed from a hyperpigmented macule on the neck, and the histopathological examination was consistent with erythema dyschromicum perstans (Figs. 1d1f). The patient was previously treated with oral dapsone 50–100 mg/day for three years, hydroxychloroquine 200 mg/day for three months, and cyclosporine 200 mg/day for eight months without a significant clinical response.

Figure 1: Grayish, hyperpigmented macules on the neck (a). Frontotemporal alopecia, regression of the hairline, and loss of the eyebrows. Eyebrow tattooing was also noted (b). Band-like frontotemporal alopecia, hyperpigmented macules, and skin-colored papules on the left malar region (c). Histopathological examination of the grayish, hyperpigmented macule on the neck revealed melanin incontinence in the papillary dermis (H&E, 100x) (d), lymphocytic inflammation in a lichenoid pattern in the papillary dermis (H&E, 100x) (e) and sparse apoptotic keratinocytes in the epidermis (arrow) (H&E, 200x) (f). Dermatoscopic examination of the hyperpigmented macule on the neck (g). Dermatoscopic examination of the alopecic area on the scalp (h). Dermatoscopy was performed with a FotoFinder Medicam 1000 (FotoFinder Systems GmbH, Bad Birnbach, Germany) under 10x magnification.

The present dermatological examination revealed grayish, hyperpigmented macules distributed symmetrically on the face, neck, and inframammary region. In addition, symmetrical, band-like frontotemporal alopecia, regression of the hairline, and loss of the eyebrows were detected. Skin-colored, monomorphic papules on the cheeks were also noted. The patient’s Fitzpatrick skin phototype was III. A dermatoscopic examination revealed small, gray to brown dots mostly in an irregular linear arrangement with a pinkish-brown background on the neck, and a perifollicular scale and loss of orifices on the scalp (Figs. 1g and 1h).

Erythema dyschromicum perstans usually presents with gray to brown colored, asymptomatic macules on the trunk, proximal extremities, neck, and face. The lesions may occur on both sun-exposed and sun-protected body areas. Moreover, they show symmetrical distribution [1,2]. Various factors such as radiocontrast agent exposure, cobalt allergy, and human immunodeficiency virus (HIV) infection have been implicated in the development of erythema dyschromicum perstans. However, the exact cause of the disease remains unknown [2]. Systemic and topical steroids, dapsone, clofazimine, isotretinoin, narrow-band ultraviolet B therapy have been used in the treatment of erythema dyschromicum perstans [1]. Nevertheless, no effective treatment has been widely accepted for the management of erythema dyschromicum perstans [2]. On the other hand, it is controversial whether erythema dyschromicum perstans and lichen planus pigmentosus are different disorders or variants of the same disorder [3]. Both erythema dyschromicum perstans and lichen planus pigmentosus are acquired skin diseases characterized by dermal hyperpigmentation [4]. Furthermore, lichen planus pigmentosus of the face has been associated with frontal fibrosing alopecia in which facial papules might indicate poor prognosis [5]. Hereby, we present a 72-year-old female patient who had erythema dyschromicum perstans, frontal fibrosing alopecia, and facial papules at the same time. We suggest that frontal fibrosing alopecia and facial papules should also be considered in the management of patients diagnosed with erythema dyschromicum perstans.

Consent

The examination of the patient was conducted according to the principles of the Declaration of Helsinki.

The authors certify that they have obtained all appropriate patient consent forms, in which the patients gave their consent for images and other clinical information to be included in the journal. The patients understand that their names and initials will not be published and due effort will be made to conceal their identity, but that anonymity cannot be guaranteed.

REFERENCES

1. Leung N, Oliveira M, Selim MA, McKinley-Grant L, Lesesky E. Erythema dyschromicum perstans:A case report and systematic review of histologic presentation and treatment. Int J Womens Dermatol. 2018;4:216-22.

2. Vinay K, Bishnoi A, Kamat D, Chatterjee D, Kumaran MS, Parsad D. Acquired dermal macular hyperpigmentation:An update. Indian Dermatol Online J. 2021;12:663-73.

3. Gupta V, Sharma VK. Ashy dermatosis, lichen planus pigmentosus and pigmented cosmetic dermatitis:Are we splitting the hair?Indian J Dermatol Venereol Leprol. 2018;84:470-4.

4. Wang RF, Ko D, Friedman BJ, Lim HW, Mohammad TF. Disorders of hyperpigmentation. Part I. Pathogenesis and clinical features of common pigmentary disorders. J Am Acad Dermatol. 2023;88:271-88.

5. VerzìAE, Lacarrubba F, Dall’Oglio F, Micali G. Association of frontal fibrosing alopecia with facial papules and lichen planus pigmentosus in a Caucasian woman. Skin Appendage Disord. 2020;6:379-83.

Notes

Source of Support: This article has no funding source.

Conflict of Interest: The authors have no conflict of interest to declare.

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