CLINICAL

A 37 year-old woman presented with a history of systemic lupus erythematosis (SLE) for 15 years presented with multiple erythematous annular plaques of alopecia on the vertex and right side of the scalp. Her prior symptoms included arthralgias, arthritis, oral ulceration and discoid lesions of the arms after sun exposure. A punch biopsy of one of the scalp lesions was performed.

LAB INVESTIGATIONS

• CBC White cell count 3500/mm³
• Platelet count 85000/mm³
• ANA -Positive at 1:640 with a peripheral (rim) pattern
• Anti double stranded DNA by immunofluorescence -Positive
• Anti Sm antibody -Positive by radial immunodiffusion
• Anti Ro, Anti La antibodies -Negative
• Anti U, RNP antibody -Positive

PATHOLOGY

A 4mm punch biopsy was performed and processed by the Headington method.

There was a diminished number of hairs with an average of 15 hairs per transverse section (Fig 1). The infundibular and isthmic areas of the follicle had an interface dermatitis vacuolar type with colloid body formation (Fig. 2, 3) and a thickened basement membrane (highlighted on PAS-D stain)(Fig 4).

In addition there was a superficial and deep perivascular and periadenexal lymphoid infiltrate (Fig 5) with dermal mucin deposition seen on Alcian blue stain (Fig. 6).

Sections at the level of the eccrine gland coils showed an increase of catagen hairs (Fig 7) and follicular stelae with pigment incontinence. A peribulbar lymphoid infiltrate was noted (Fig 8).

DIAGNOSIS:

Discoid lesions of scarring alopecia in a patient with SLE.

DISCUSSION:

Patients with LE may develop LE specific and LE non-specific skin diseases. In addition they may develop dermatologic conditions unrelated to the LE.

Cutaneous lesions of SLE are common and occur in 80-90% of SLE patients at some point in their disease. The specific lesions are a malar rash, a photosensitive macular or maculopapular rash, oral lesions (including petechia, gingivitis, chelitis and ulceration) and discoid lesions. Discoid lesions occur in 15-30% of patients with SLE and approximately 5-10% of patients with SLE have discoid lesions as the presenting disease manifestation.

The non-specific lesions of SLE include alopecia, vascular lesions (including chillblains, Raynauds phenomenon, livedo reticularis, vasculitis), angioedema, rheumatoid nodules and calicinosis cutis.

Three types of alopecia have been described in patients with SLE

1. Discoid lesions with associated scarring alopecia
2. A diffuse non-scarring alopecia with transient hair loss related to the activity of the disease (a telogen effluvium like picture) and
3. Lupus hair which is an unusual non-scarring alopecia characterized by thin weakened hairs at the periphery of the scalp. The hairs fragment and result in a characteristic unruly appearance. In addition alopecia areata has been discovered in patients with SLE rarely scarring DLE and non-scarring AA like lesions may coexist in the same patient.

The patient above is unusual in that the histologic findings were both those of DLE associated alopecia as well as alopecia areata (AA) (increase of catagen hairs and peribulbitis). A study of 89 patients with scarring alopecia and DLE showed a lymphocytic infiltrate mainly directed to the mid portion of the follicle and a normal anagen:telogen ratio. The authors postulated that the loss of follicle may be due to the destruction of the stem cells which reside in the bulge area where the arrector pili muscle inserts.

TAKE HOME MESSAGE

1. Cutaneous lesions occur in the vast majority of patients with SLE and approximately 5-10% of patients with SLE have discoid lesions as the presenting disease manifestation.
2. Alopecia associated with SLE may be of 3 types and in occasional cases AA may coexist with SLE associated scalp lesions. A scalp biopsy may be helpful in identifying the specific type of alopecia in patients with SLE.

REFERENCES:

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