Chronic cutaneous lupus erythematosus presenting as atypical acneiform and comedonal plaque: case report and literature review

Introduction

Chronic cutaneous lupus erythematosus (CCLE) usually affects sun-exposed areas, presenting with erythematous patches of various sizes, infiltrated coin-shaped (“discoid”) plaques with hyperkeratosis in the center, and follicular plugs (adherent scale into dilated hair follicles). CCLE lesions heal with atrophy and scarring, hyperpigmentation and telangiectasia. On hair-bearing skin this results in permanent hair loss (scarring alopecia).^1,2

Women are preferentially affected in a 2–3:1 ratio, with an onset of the disease typically in the fourth decade of life. ³

Cutaneous manifestations of CCLE are usually characteristic enough to allow straightforward diagnosis. However, there are some unusual clinical variants, such as hypertrophic CCLE, mucosal CCLE and lichenoid CCLE, which may mimic other dermatological conditions.^4,5 In addition, in 1972 Haroon et al. reported the first case of an unusual presentation of CCLE with hypertrophic follicular scars seen in acne vulgaris. ⁶

Acneiform presentation is one of the most rarely reported and one of the most confusing, as it resembles a very common inflammatory skin disease and therefore can be easily missed clinically. ⁴ A brief literature review using PubMed’s advanced search with Medical Subject Headings (MeSH) terms: “cutaneous lupus erythematosus” OR “systemic lupus erythematosus” AND “acneiform eruptions” and “cutaneous lupus erythematosus” OR “systemic lupus erythematosus” AND “acne vulgaris” found only nine other case reports of acneiform lupus.

Here we describe a case of CCLE presenting with pruritic and localized acneiform and comedonal plaque that was initially misdiagnosed and treated as acne vulgaris.

Case report

A 32-year-old woman presented with a two-year history of pruritic acneiform and comedonal eruption on the right chin area, previously treated as acne with isotretinoin 40 mg/day for three months with no improvement. There were no systemic symptoms. The patient did not have any diseases or use continuous medication.

On examination the patient presented with erythematous-infiltrated plaque, papules, open comedones, pitting scars and hypopigmented atrophic scars on the right chin area (Figure 1). The patient also presented with erythematous plaques, some with atrophy, on the ear concha and abdomen, and scalp hair loss.

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Figure 1

Erythematous-infiltrated plaque, papules and open comedones, pitting scars and hypopigmented atrophic scars on the right chin area.

Since acne vulgaris is a bilateral, symmetrical dermatosis with lesions ranging from comedones to papules and pustules, such a diagnosis was discarded and a skin biopsy was requested considering the hypotheses of CCLE, cutaneous sarcoidosis and facial granuloma.

An incisional skin biopsy was performed at the chin and scalp lesions. The scalp histopathology showed dense superficial and deep perivascular and periadnexal lymphocytic infiltration, and signs of lichenoid infiltrate, with final diagnosis of cicatricial alopecia caused by lupus erythematosus (LE). The chin lesion showed basal layer vacuolar degeneration (Figure 2), superficial and deep perivascular, and periadnexal mononuclear infiltration at the dermis and exuberant follicular hyperkeratosis and plugging (Figure 3). In addition, some melanophages were found. These findings are consistent with CCLE. Direct immunofluorescence (DIF) was performed and showed presence of homogeneous continuous deposition of immunoglobulin M (IgM) in the basement membrane zone (BMZ), confirming the diagnosis (Figure 4). DIF also showed positivity stain of upper dermal vessels.

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Figure 2

Basal layer vacuolar degeneration of the epidermis.

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Figure 3

Exuberant follicular plugging and superficial and deep mononuclear infiltration.

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Figure 4

Direct immunofluorescence with homogeneous continuous deposition of immunoglobulin M in the basement membrane zone and upper dermal blood vessels.

Additional tests ruled out systemic involvement, with blood inflammatory markers, antibody profile, serology for human immunodeficiency virus (HIV), Hepatitis B, C, venereal disease research laboratory (VDRL), and radiological investigation all negative.

The patient was treated with 40 mg of prednisone for one month with a progressive dose reduction for four months, and 250 mg of chloroquine diphosphate. Three months later the patient showed significant improvement with less inflammation and pruritus. The patient continued the use of chloroquine and after four years the lesion is stable, with some scarring, but without pruritus, inflammation, or new lesions (Figure 5).

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Figure 5

Four-year follow-up. There is only scarring.

Discussion

CCLE has several clinical manifestations and acneiform presentation is very rare. ⁴ In the literature we found nine cases of acneiform presentation of LE.^1,4–10 Three cases were systemic lupus erythematosus (SLE), with acne-like lesions as one of the manifestations, and the seven others (including our case) showed no sign of systemic manifestations, diagnosed as CCLE. All three patients who had SLE tested positive for ANA (homogeneous, nucleolar and fine speckled), and only one patient with CCLE, had a low titer of ANA. These cases are summarized in Table 1.

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Table 1

Summary data from nine cases found in literature review and present case (number 10)

Case	Age/ gender	Site of lesions	Time to diagnosis (years)	Pruritus	Thickened basement membrane	Direct immunofluorescence	SLE/CCLE	Antinuclear antibody
1	38/M	Face, chest, back	7	Yes	–	Negative	CCLE	Negative
2	32/M	Face	3	No	–	IgG + C3	CCLE	Negative
3	33/F	Scalp	–	No	–	–	CCLE	–
4	35/F	Face, ears	2	Yes	Yes (PAS)	–	CCLE	–
5	32/F	Back, shoulders, upper arm	4	Yes	–	Positive	CCLE	Negative
6	29/F	Face, shoulders, upper back	7	Yes	–	Negative	SLE	1/2560 Homogeneous
7	24/F	Face and neck	–	Yes	Yes	IgG	SLE	1/5120 Nucleolar
8	60/M	Face	8	No	Yes (PAS)	Lupic band	CCLE	1/80 fine speckled
9	30/F	Face	2	No	–	IgM + C3	SLE	1/80 fine speckled
10a	32/F	Face	2	Yes	No	IgM	CCLE	Negative

Ages varied from 24 to 60 years old, with a median of 32 years old, which is consistent with the literature. Seven were females and three were males, with a ratio of 2.3:1. Most cases showed acneiform lesions preferably on the face, a common site of typical CCLE. ³

Pruritus is not a prominent feature of CCLE. ¹ With our patient however, itching was a striking manifestation. Out of 10 patients, six complained of pruritus. An article suggests that itching and photosensibility in acneiform lesions are important features to guide diagnostic suspicion of CCLE. ⁷

Laboratory tests are recommended to exclude the possibility of SLE. ANA may be detected in the sera of patients, with varying frequency and depending on the particular subtype. ¹¹ Serologically, CCLE patients have a lower incidence of ANA, double-stranded DNA (dsDNA), Sm, U1RNP, and Ro/SSA antibodies, as compared to other CCLE subtypes. ¹² Our patient tested negative for ANA, and out of the other nine cases only one patient with CCLE had a low titer of ANA (1:80 fine speckled).

To confirm the diagnosis of CCLE, histopathological examination of skin biopsy specimens is recommended. The histology of lesions of CCLE shows some typical characteristics, including vacuolar degeneration of the basal layer (interface dermatitis), as well as perivascular and periadnexal lymphocytic infiltrates, characteristics found in our case. The epidermis exhibits variable acanthosis and atrophy. In older lesions thickening of the basement membrane becomes obvious in the periodic acid-Schiff stain (PAS).^2,3 This case showed exuberant follicular hyperkeratosis and pluggings, which corresponds to the clinical lesions of comedones and papules. One hypothesis for acneiform scaring is that it may be the result of destructive process of pilosebaceous units by periadnexal mononuclear cell infiltration. ⁵

DIF of lesional biopsies can supplement non-definitive histologic findings. ¹² Immunofluorescence deposits of immunoglobulins (Ig)G, IgM, and in rare cases IgA, as well as deposits of the complement C3, are found along the dermoepidermal junction (DEJ).^2,11 The likelihood of positive results in lesional skin ranges from 50% to 90% and recent studies have shown a prevalence of IgM.^12,13 A study performed by Kontos et al. with 199 DIF of LE (95 with CCLE, 50 with subacute LE, 32 with SLE), demonstrated a marked predominance of IgM along the DEJ (75% of patients). It has been reported that IgM is more sensitive, whereas IgG is more specific in LE. There are several deposit patterns in the BMZ, the most common in CCLE are IgG and IgM with a homogeneous, granulous or reticulate pattern. ¹³ The DIF performed in this case showed homogeneous deposits of IgM along the DEJ. DIF was performed in seven of nine patients in the previous case reports, and was positive in six. Besides BMZ, another possible site of immune deposition is the superficial dermal blood vessel wall, more characteristic of SLE. ¹⁴ Even though is not described as frequently for CCLE cases, our patient also showed fluorescence of upper dermal vessels. A study with 51 DIF of CCLE patients showed nine cases of positive stain of blood vessels, five in association with BMZ and four isolated positivity of vessels. ¹⁵

The present case and literature review show that CCLE should be considered in acneiform and comedonal lesions, especially in a localized lesion, which can be itchy and does not improve with conventional treatment for acne vulgaris.