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A 27-year-old Chinese man presented to the dermatology department with a 1-year history of widespread, asymptomatic, hyperpigmented macules and patches. Lesions previously began on the trunk and gradually extended to the neck, face and extremities. However, the palms, soles, nails and mucous membranes were uninvolved. He was otherwise healthy without taking any medications. The patient denied any history of prolonged sun exposure, and no family members had an onset of similar eruptions. Physical examination revealed numerous blue-grey oval macules and patches on the trunk, neck, face and extremities with a Wagyu beef-like appearance (Fig. 1A, B). On the forearms, some hyperpigmented macules were surrounded by an erythematous border (Fig. 1C). Darier’s sign was not elicited for the lesions. Routine laboratory investigations, including complete blood count, biochemical parameters, antinuclear antibodies, viral hepatitis panel and human immunodeficiency virus (HIV) were all normal.
(A) Diffuse hyperpigmented lesions on the anterior aspect of the body. (B) Diffuse hyperpigmented lesions on the posterior aspect of the body. (C) Partial lesions of the upper limb with an erythematous border. (D) Focal vacuolar alteration of the basal layer, with abundant melanophages and mild lymphocytic infiltration in the dermis (original magnification × 100, hematoxylin-eosin stain stain).
What is your diagnosis?
1: Lichen planus pigmentosus
2: Idiopathic eruptive macular pigmentation
3: Maculopapular mastocytosis
4: Erythema dyschromicum perstans
See next page of answer.
Contributor Information
Hong-Kai Gao, Email: angaohongkai@163.com.
Cheng Tan, Email: tancheng@yeah.net.
Acta Derm Venereol. 2026 Feb 3;106:0105.
A Wagyu Beef-like Appearance of Hyperpigmentation: A Commentary
It is common to differentiate EDP from other pigmentary skin disorders with the guidance of a global consensus statement on AD, lichen planus pigmentosus (LPP) and idiopathic eruptive macular pigmentation (IEMP) (1). EDP, AD and LPP may have remarkable histopathological resemblances during some periods of the disease. Hyperpigmented macules in EDP usually present a peripheral erythematous margin, which differs from AD (1-2). Unlike EDP, the lesions in LPP always involve the sun-exposed areas, the flexural regions of the skin and the mucosa without an obvious erythematous border. Hyperkeratosis, epidermal thinning and lichenoid infiltrate are commonly observed in LPP (3). Although similarly without preceding inflammation, IEMP mainly affects young individuals and is often distributed on the trunk. Pathologically, it is highlighted with basal layer hyperpigmentation without interface pathology, which differs from EDP.
The aetiology of EDP is not fully elucidated. Infections (HIV and hepatitis C virus), toxic chemicals and drugs (ammonium nitrate, proton pump inhibitors, chlorothalonil, ethambutol, fluoxetine, adalimumab, ribociclib, osimertinib, erlotinib, etc.) are considered as the potential triggers (1, 4–6). EDP has been reported to be associated with abnormal immune responses mediated by CD8+ T lymphocytes, HLA-DR4+ and intercellular adhesion molecules (7).
There is no consistently effective therapy for EDP. The condition persists for years and then gradually disappears much more slowly in adults, in contrast with the course of the disorder in young patients, 50–69% of idiopathic prepubertal patients tend to resolve spontaneously (8). As there is no effective standardized regimen for EDP, many modalities (clofazimine, isotretinoin, dapsone, griseofulvin, steroids, topical tacrolimus, topical ruxolitinib and narrowband-UVB) have been tried with unsatisfactory results (9). Awareness of this uncommon, psychologically devastating pigmentary skin disorder can prompt a correct diagnosis, warranting proper counselling and management.
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