Lichen sclerosus et atrophicus in sclerodermatous chronic graft‐versus‐host disease
Authors: Córdoba Susana , Vargas Erich , Fraga Javier , Aragüés Maximiliano , Fernández‐Herrera Jesús , García‐Díez Amaro 
Published: March 2003

In May 1994, a 40‐year‐old woman with chronic myeloid leukemia received an allogeneic bone marrow transplant (BMT) from her human leukocyte antigen (HLA) identical sister, after a conditioning regimen with cyclophosphamide and busulfan. Graft‐versus‐host disease (GVHD) prophylaxis consisted of cyclosporine (CsA) and methotrexate. Facial and palmoplantar erythema and moderate cholestasis developed on day 14 after the BMT. A diagnosis of acute GVHD was made and she was successfully treated with low doses of corticosteroids. On day 150 after the BMT, despite the prophylactic treatment of GVHD with CsA (150 mg/12 h), she developed several burning white plaque‐like striae over the buccal mucosa and numerous itching violaceous lichenoid papules on the fingertips. Biopsy specimens obtained from both the skin of the fingertips and the oral mucosa (Fig. 1) revealed patchy to diffuse subepithelial lymphocytic inflammation and necrosis of individual squamous cells, consistent with a diagnosis of chronic lichenoid GVHD. Despite therapy with CsA, topical and systemic corticosteroids (prednisone 60 mg/24 h), the oral lichenoid lesions persisted. On day 750 after the BMT, 2 months after withdrawal of immunosuppressive therapy, she developed several erythematous, pruriginous, and slight indurated lesions over the neck. These lesions coalesced into plaques, adopting a white atrophic‐like appearance with follicular plugs similar to lichen sclerosus et atrophicus (Fig. 2). Histopathologic examination showed hyperkeratosis with follicular plugging, atrophy of the stratum Malpighii with hydropic degeneration of the basal cells, homogenization of the collagen, incontinence of the pigment, and a discrete lymphoplasmocytic inflammatory infiltrate in the upper dermis (Fig. 3). Systemic corticosteroid therapy was re‐introduced. On day 850 after the BMT, physical examination revealed patchy hyperpigmentation affecting the back and limbs, and diffuse thickening and hardening of the skin of the legs, forearms, and dorsa of the hands, resulting in

Diffuse subepithelial lymphocytic inflammation and satellite cell necrosis of squamous cells in oral mucosa (hematoxylin and eosin, ×25)

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Atrophic and confluent plaques with follicular plugs on the neck

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Follicular plugging, atrophy of the stratum Malpighii, hydropic degeneration of basal cells with a split visible at the dermoepidermal junction, and homogenization of the collagen in the upper dermis (hematoxylin and eosin, ×25)

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a slight limitation of movements. Various smooth, shiny, and indurated plaques, characteristic of morphea, were present on the flexural areas of the arms (at vein puncture sites). Lichenoid lesions over the oral mucosa and the atrophic plaques on the neck were still present. No dysphagia was observed. Liver function tests revealed a pattern of cholestasis. Antinuclear antibodies were detected (1 : 80); no antibodies against centromere antigens and DNA topoisomerase I (SCL70) were found. Reduced lacrimal secretion (Schirmer test <10 mm in 5 min) and a reduced forced expiratory volume in 1 s (FEV1) were documented. A biopsy specimen from a sclerodermatous area showed a thickened reticular and papillary dermis with closely packed collagen bundles and abundant melanophages in the dermis (Fig. 4). Azathioprine was added to the corticosteroid therapy in an attempt to arrest the progression of the disease. Six months after starting treatment, cutaneous lesions still persist, but the patient has not developed any other systemic symptoms.

Thick collagen bundles in reticular and papillary dermis (hematoxylin and eosin, ×10)

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