Successful Management of Erythema Dyschromicum Perstans Following Topical Ruxolitinib Therapy

March 2023 | Volume 22 | Issue 3 | 297 | Copyright © March 2023


Published online February 13, 2023

Dhipthika Srinivasan BAa, Alice B. Gottlieb MD PhDb

aA.T. Still University School of Osteopathic Medicine in Arizona Mesa, AZ
bThe Icahn School of Medicine at Mount Sinai, New York, NY

Abstract
Erythema dyschromicum perstans (EDP) is a rare cutaneous disorder in which patients develop gray or blue-brown macules or patches on their bodies.1 This condition does not appear to have a gender or age predilection. The diagnosis of EDP is essentially clinical, with histopathology findings being nonspecific. To date, treatment for EDP varies. The use of several therapies, including dapsone, clofazimine, retinoid A, tacrolimus, and ultraviolet light have been reported but with minimal effectiveness.5 We report a case of EDP occurring in a patient following the COVID-19 vaccine that was given topical ruxolitinib with success in treatment. To our knowledge, this is the first report of the use of topical ruxolitinib in treatment of EDP with successful management.

J Drugs Dermatol. 2022;22(3): doi:10.36849/JDD.7156

Citation: Srinivasan D, Gottlieb A. Successful management of erythema dyschromicum perstans following topical ruxolitinib therapy. J Drugs Dermatol. 2023;22(3):297-299. doi:10.36849/JDD.7156

INTRODUCTION

Erythema dyschromicum perstans (EDP) is a rare cutaneous condition characterized by gray or blue-brown macules or patches that usually presets over the trunk, neck, and extremities.1 EDP occurs in both children and adults, has a higher incidence in woman, and is common in Latin American, Asian, and Indian populations.2 EDP can present similarly to other pigmentation disorders such as lichen planus pigmentosus (LPP) resulting in an inaccurate diagnosis and treatment plan and opinions vary whether it is its own distinctive case or a form of LPP.3 The etiology of EDP is unknown, although non-conclusive associations with endocrinopathies, exposure to chemical substances, vitiligo, and chronic hepatitis C infection have been shown.4 Many treatments have been proposed that show inconsistent efficacy, including clofazimine, dapsone, tacrolimus, retinoid A, amongst other treatments but there has been no research conducted on ruxolitinib use.5 We report a novel case of EDP caused from the COVID-19 vaccine with successful treatment with topical ruxolitinib.

METHODS

A 57-year-old Hispanic female presented to our clinic with a rash that began a few weeks after receiving the COVID-19 vaccine. At presentation, the patient had gray-blue discoloration of patches on the chest and bilateral upper extremities (Figures 1-3). The patient's medical history and review of symptoms were typical and the patient’s family history was noncontributory. The patient described no itchiness, erythema, tingling, muscle weakness, or joint pains. The patient was prescribed triamcinolone 0.1% ointment and clotrimazole 1% cream by her primary care physician prior to this visit. She reported no improvement in the lesions and ongoing concern of gray-blue discoloration. A 4 mm punch biopsy of the border of the pigmentation was performed on the left upper arm. The pathology reports demonstrated a superficial, perivascular lymphocytic infiltrate with vacuolar interface change in the papillary dermis consistent with findings of EDP. The patient then discontinued triamcinolone and clotrimazole and was prescribed topical ruxolitinib. The patient presented back after 1 month of use of topical ruxolitinib 1.5% cream twice daily and the results showed improvement of the lesions as compared to the patient's initial visit (Figures 4-5). The patient continued to use ruxolitinib with follow up pending 3 months.

DISCUSSION

Considered a chronic disorder of the skin, EDP is characterized by ashy gray macules usually seen on the chest, face, and upper extremities.1,2 The histology of EDP is nonspecific and can be further subdivided into active (early) or inactive (late) lesions.6 The active lesion shows basal vacuolar degeneration and edematous papillary dermis with lymphocytic infiltration.6 The inactive lesion shows melanophages and pigment incontinence in the dermis without inflammation.6 There is also an increase in melanin which can be found in the epidermis that causes the grey-blue discoloration seen in patients.