Isomorphic Sclerotic-Type Cutaneous Chronic Graft-Versus-Host Disease: Report and Review of Chronic Graft-Versus-Host Disease in a Cutaneous Immunocompromised District
A 74-year-old man with a history of mantle cell lymphoma had received two prior hematopoietic cell transplants. The patient experienced several weeks of asymptomatic, yet progressive, stiffening of the skin on the distal right extremity and lower abdomen. The patient also noted that the skin on the lateral lower chest and upper abdomen had a rippled appearance.\nThe mantle cell lymphoma was diagnosed in 2005. In September 2008, the patient underwent an autologous stem cell transplant using BEAM (carmustine, etoposide, cytarabine, and melphalan) as a conditioning regimen. The patient achieved a complete remission.\nThe patient’s lymphoma relapsed in July 2009 and he participated in a clinical trial receiving intranodal injections of an immunotherapy Ad-ISF35 (immune stimulating factor 35). The patient was withdrawn from the study after injection #3 because of lack of clinical improvement. From October 2009 to April 2010, the patient completed six cycles of bendamustine and rituximab. The patient achieved a complete clinical response, but had residual inguinal lymphadenopathy; subsequently, new nodes were discovered.\nThe patient underwent an allogeneic hematopoietic peripheral blood stem cell transplant using a sex-matched unrelated donor in May 2010; the conditioning regimen was fludarabine, melphalan, and rituximab. The patient engrafted without complications and again achieved a complete remission. The patient subsequently developed biopsy-confirmed acute cutaneous GVHD that resolved after treatment with topical corticosteroids.\nIn early February 2013, the patient noted that his right forearm and hand were becoming stiff and indurated. Similarly, the area of his abdomen that came in contact with the waistband of his pants was becoming indurated. Also, the area of his lateral abdomen above his waistband became rippled. The observed changes continued to progress.\nCutaneous examination in late March 2013 showed the patient’s right forearm to be hyperpigmented and indurated; similarly, his dorsal hand was also firm and there was no elasticity of the overlying skin (Fig. ). The patient’s lower abdomen, particularly the areas that came in contact with the waistband of his pants and belt, was smooth and hyperpigmented with areas of erythema; in addition, the lower abdomen had developed a confluent morphea-like sclerotic dermal induration (Figs. a, b, , ). The skin of the patient’s lateral lower chest and abdomen had a “cellulite” appearance (Figs. , ).\nBiopsy from the right forearm, the waistband area, and the lateral upper abdomen all showed similar findings: sclerotic and thickened collagen in the reticular dermis. In the waistband area, these changes were predominantly in the mid reticular dermis; there was also loss of the perieccrine fat and a sparse lymphocytic superficial perivascular infiltrate. In the forearm and the upper abdomen, the sclerosis began in the deep reticular and extended into the subcutaneous fat; lymphoplasmacytic inflammation was noted at the junction of the deep reticular dermis and subcutaneous fat in the forearm, and extravasated erythrocytes were observed in the reticular dermis of the abdomen.\nCorrelation of the clinical presentation and pathology findings established the diagnosis of chronic sclerotic-type cutaneous GVHD; the localization of GVHD to the waistband area demonstrates an isomorphic response to the repetitive irritation, friction, and pressure from the patient’s belt and pants. The patient was treated with oral prednisone (initially at 10 mg per day for 2 weeks, and tapered to 5 mg per day and 2.5 mg per day for 2 weeks each) and topical tacrolimus 0.1% gel twice daily. Clinical improvement was observed, with the dermal plaques becoming less firm.