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Bringing pigmented lupus to light

DII small banner By Warren R. Heymann, MD
Dec. 7, 2017

Cutaneous lupus. Biopsy of the left distal thigh shows interface dermatitis, dyskeratosis, superficial perivascular lymphocytic infiltrate, focal red blood cell extravasation, and pigment incontinence.
Credit: JAAD

Lettie (not her real name) was an elderly, charming, courtly, soft-spoken African-American woman whose facial skin was getting progressively darker. Although asymptomatic, she was embarrassed by it, and was reluctant to go to church. I was considering the usual suspects of post-inflammatory changes of a lichenoid drug eruption, lichen planus actinicus, a resolved photocontact dermatitis, exogenous ochronosis, or argyria. Only after I drew routine laboratory studies and found marked thrombocytopenia, did I entertain the diagnosis of lupus, which was subsequently confirmed by biopsy and serologies.

We all have cases that alter our perspective on a particular disease. I always recall Lettie’s delayed diagnosis of lupus when assessing facial hyperpigmentation. When we think of chronic cutaneous lupus erythematosus (CCLE), most of us will conjure an image of discoid lesions with scales, follicular plugs, atrophy, hypopigmentation and hyperpigmentation. Realistically, there are more than 20 various clinical forms of CCLE (1), however, the macular hyperpigmented form seems to be something of a secret.

I never could find the right name for this variant of lupus until I read the article by Khuller et al, describing the “pigmented macular variant of chronic cutaneous lupus erythematosus.’ They detailed three middle-aged women who presented with lichenoid to slate-grey pigmentation on the forehead, malar region, helicies, and chin. One patient had pigmented macules on the dorsal hands. Histologic examination was characteristic for lupus (vacuolar interface dermatitis, a perivascular and perifollicular mononuclear infiltrate, and pigment incontinence). All three women had positive direct immunofluorescence; serologic lupus studies were confirmatory in two of the three women. None had systemic symptoms. The patients demonstrated improvement with hydroquinone, fluticasone, and sunscreen. The authors aptly stated: “Pigmented macules in the absence of erythema, follicular plugging, adherent scaling and scarring typical of DLE [discoid lupus erythematosus] represent an uncommon morphological subtype of CCLE.” (2)

Boyd reported the case of a 72-year-old woman with a slowly expanding lesion of her right cheek that measured 3 cm; it displayed variable pigmentation ranging from brown to gray to black. Her ANA was positive at 1:160 and the biopsy confirmed lupus. He then performed a retrospective analysis of 11 similar cases (9 women, 2 men, average age 68 years). Most presented with single, hyperpigmented macular lesions. Photosensitivity was rare and there were no other stigmata of lupus. Patients responded well to topical corticosteroids. (3) Looking at this patient I surmise that most dermatologists (including myself) would have sent in a biopsy to rule out a lentigo maligna, without even thinking about lupus.

Of course this needs to be differentiated from hydroxychloroquine-induced pigmentation in those patients with lupus who are being treated with antimalarials. A recent study has supported the hypothesis that ecchymosis, platelet antiaggregants and oral anticoagulants may be the main predisposing factors to hydroxychloroquine-induced hyperpigmentation. (4)

In his article, Boyd cited two other similar cases of hyperpigmented CCLE, and found it “puzzling whey this subset of CCLE patients has not been previously described.” That’s a great question. My guess is that it is more common than we suspect — we just don’t recognize it, or perhaps just treat it as hyperpigmentation with bleaching agents. The diagnosis of the pigmented macular variant of chronic cutaneous lupus erythematosus is worth remembering when assessing hyperpigmented patches in a photodistribution.

Lettie appears to have been unusual in that she had systemic disease. The literature suggests that the prognosis is good. It was for her. With topical steroids and sunscreen, the hyperpigmentation diminished.  Feeling better about her appearance, she went to church, met a widow, and got married.

1. Pramatarov KD. Chronic cutaneous lupus erythematosus – clinical spectrum. Clin Dermatol 2004; 22: 113-20.
2. Khullar G, et al. Pigmented macular variant of chronic cutaneous lupus erythematosus: An under-recognized subset in dark skin. Clin Exp Dermatol 2017; 42: 793-5.
3. Boyd AS. Localized chronic cutaneous lupus erythematosus masquerading as pigmented lesions: A new clinical subset? Lupus 2006; 15: 292-5.
4. Bahloul E, et al. Hydroxychloroquine-induced hyperpigmentation in systemic diseases: Prevalence, clinical features, and risk factors: A cross-sectional study of 41 cases. Lupus 2017; 26: 1304-8.

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