Dark brown scaly plaques on face and ears

Journal of Family Practice, June, 2006 by Nayla Idriss, Shahbaz A. Janjua, Amor Khachemoune

A 27-year-old woman, otherwise healthy, presented for evaluation of a mildly pruritic eruption of plaques on her face and ears. The eruption started as a few small, scaly red papules on her cheeks and nose about 3 months earlier. The papules slowly expanded to form well-demarcated, rounded, dark brown plaques covered with superficial scale. Similar lesions appeared on the concha of both ears. The older lesions started to regress after 2 months. Complete regression was followed by residual scarring and hyper-pigmentation.

Physical examination revealed multiple well-defined, erythematous, hyper-pigmented, round plaques covered with adherent scale affecting her nose, cheeks, and the concha of both ears (FIGURE 1). The mucosae and the rest of her skin and adnexa were unaffected. She had no personal or family history of similar skin findings or autoimmune disorders. She also had no history of any drug intake prior to the eruption. Her routine blood tests and urinalysis results were unremarkable, and the serological analysis for antinuclear antibodies had negative results.

[FIGURE 1 OMITTED]

A punch biopsy was taken from one of the lesions for histopathology. The epidermal histopathological findings were remarkable for hyperkeratosis, follicular plugging, pigment incontinence, and vacuolization of the basal cell layer. There was a predominantly lymphocytic infiltrate of the subepidermal and perivascular dermal areas.

* What is your diagnosis?

* How would you treat this patient?

* Diagnosis: Discoid lupus erythematosus

Discoid lupus erythematosus (DLE) is an autoimmune inflammatory disorder of the skin that often leads to scarring and alopecia. It may be localized to sun-exposed areas such as the face, ears, and scalp but occasionally is much more extensive, involving the trunk and extremities. Most patients are otherwise healthy, and DLE may be the only clinical finding.

Although its prevalence is not known, DLE is not uncommon. It affects females twice as often as males, and patients fall between the ages of 25 to 45 years, with no racial predilection. While about 15% to 20% of patients with systemic lupus erythematosus (SLE) manifest DLE lesions, only about 5% to 10% of patients with DLE go on to develop SLE. (1)

Like SLE, DLE is believed to be an autoimmune disorder. Unlike SLE, however, DLE patients do not have similar serologic abnormalities. Skin trauma and ultraviolet light exposure have been reported to induce or exacerbate the lesions of DLE. Sex hormones may also play a role: exacerbation may occur during pregnancy, during menstrual or premenstrual periods, or while taking oral contraceptives. Drugs such as procainamide, hydralazine, isoniazid, diphenylhydantoin, methyldopa, penicillamine, guanides, and lithium may also precipitate DLE lesions. (2)

DLE usually begins as dull red macules with adherent scales on sun-exposed areas. If the overlying scales are removed, an undersurface of horny plugs is revealed. These plugs fill the follicles and resemble carpet tacks or a cat's tongue (langue au chat). The macules slowly expand to form large plaques.

Usually only a mild pruritus and tenderness is seen with DLE lesions. As the lesions progress, the scale may thicken and pigmentary changes become evident. The lesions heal with atrophy, scarring, telangiectasias, and changes in pigmentation. Morphological appearance of older lesions may vary from erythematous plaques to hyperkeratotic, dark gray plaques with centrally depressed scars. (3) Scalp involvement in DLE results in more sclerotic and depressed scars with subsequent scarring alopecia. (4)

Differential diagnosis is wide. The differential diagnosis of DLE is extensive and includes actinic keratosis, dermatomyositis, granuloma annulare, granuloma faciale, keratoacanthoma, lichen planus, subacute cutaneous lupus erythematosus, psoriasis, rosacea, sarcoidosis, squamous cell carcinoma, syphilis, and nongenital warts.

* Histopathology

Histopathological findings include hyperkeratosis, parakeratosis, follicular plugging, telangiectasias, and atrophy of the epidermis. Liquefaction or hydropic degeneration of the basal layer leads to pigmentary incontinence. A perivascular and perifollicular mononuclear inflammatory cell infiltrate is present in the superficial and deep dermis. (5,6)

Direct immunofluorescence demonstrates immunoglobulins and complement deposits at the dermoepidermal junction. (5) This test was not recommended for this patient, as the diagnosis of DLE had already been confirmed on clinical and histopathologic grounds.

* Workup: Exam, biopsy

In addition to a routine history and physical examination, the workup for DLE should include a complete blood count, antinuclear antibody levels, anti-Ro, anti-La, hepatic and renal function tests, and urinalysis. Consider a diagnosis of SLE by using the American College of Rheumatology criteria. A biopsy for histopathology of a fresh lesion or a biopsy for immunofluorescence of an old lesion can confirm the diagnosis.

* Therapeutic options are varied