Coexistence of morphea and lichen sclerosus et atrophicus in a zosteriform pattern

A 23-year-old female presentedwith a 3-month history of an asymptomatic, slowly expanding area of dyschromia with textural changes over the left side of her face and neck. Physical examination showed a band of hyperpigmented, indurated plaque that ran from the left anterior to the posterior aspect of her neck (Figure 1). A slightly hypopigmented patch was aligned, in parallel to the plaque, on the adjacent upper portion of her neck. A small part of her lower left cheek andpostauricular areawere also involved.A skinbiopsywasobtained from the junction of the hyperand hypopigmented areas on her neck. Under low-power magnification, both areas showed the presence of thickened collagen bundles with entrapped, infiltrated eccrine glands (Figure 2A). There was epidermal hyperkeratosis with follicular plugging. The papillary dermis showed rather homogenous changes with pigment incontinence, suggesting a previous interface inflammatory process (Figure 2B). An orcein stain indicated the loss of elastic fibers in the superficial dermis (Figure 2C and D). These findings were compatible with morphea showing superficial changes reminiscent of lichen sclerosus et atrophicus (LSA). Laboratory tests scored positive for speckled-type antinuclear antibodies at a 1:80 dilution, and anti-Ro antibodies at a 1:240 dilution. Her genital area was normal in appearance. There was no evidence of sicca syndrome, Raynaud’s phenomenon, or any symptoms suggestive of systemic scleroderma. A complete blood cell count, biochemistry profile, and urinalysis were all unremarkable for the patient. On the basis of the clinical and laboratory examination, we inferred that the patient had coexistence of morphea and extragenital LSA. She received treatment with hydroxychloroquine (200 mg/day) and topical steroid of fluocinonide. After 4 months of serial follow-up, the color and induration of the lesions gradually improved without involving any other organs. Morphea and LSA are two cutaneous disorders with different clinical and pathologic presentations. The relationship between the two diseases has been extensively debated for decades, and frequent reports of an association between the two conditions have led some clinicians to hypothesize the existence of a common pathologic link. Wallace1 studied 380 LSA patients and reported that histologically-confirmed morphea was present in 13 of these patients. Uitto et al2 were the first to describe a series of 10 patients in which the two entities coexisted. In seven of the 10 cases, biopsies indicated that features of both morphea and LSA presented with equal intensity. Subsequently, sporadic cases of these two coexisting diseases have been published. Most recently, Peterson et al3 proposed a revised classification of morphea to include LSA as a subtype owing to the histologic similarities between the two conditions. However, many investigators have argued that