Folliculocentric lichen sclerosus et atrophicus

A 71-year-old black woman presented to the dermatology clinic with a several-year history of increasing numbers of itchy white spots spreading over the chest, back, elbows, and legs. In 2004, the patient developed clusters of mildly pruritic hypopigmented and depigmented macules on her back and left shoulder along with depigmented flat papules on the lower extremities. The patient's condition was unresponsive to topical triamcinolone and tacrolimus, after which she was lost to follow-up for 3 years until her entire chest became involved. Her medical history was significant for hypertension and diabetes mellitus and her medications included amlodipine, aspirin, atorvastatin, hydrochlorothiazide, irbesartan, and metformin. Family history, social history, and review of systems were unremarkable. Physical examination revealed multiple shiny white perifollicular macules, some with slight scale, on the trunk (Figure 1) and extremities (Figure 2). On the left shoulder and back were 2 shiny plaques with hypopigmented macules and follicular scale within. Punch biopsies from the abdomen and back showed atrophy of the epidermis, edema and sclerosis of the papillary dermis, and a patchy lichenoid lymphocytic infiltrate (Figure 3). Despite inspecting multiple step sections, we were unable to histopathologically confirm the clinically apparent folliculocentric distribution. Based on the clinical and histological findings, extragenital lichen sclerosus et atrophicus (LSA) was diagnosed and the patient was treated with clobetasol proprionate 0.05% ointment. While being prepared for narrowband UV-B therapy, a serologic screen revealed antinuclear antibodies at 1:640 with a homogeneous pattern. A subsequent rheumatologic evaluation was negative for autoimmune disorders; pertinent negative serologies included anti-double-stranded DNA, smooth muscle, mitochondrial, thyroid, and parietal cell antibodies.