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Sclerodermatous chronic graft-versus-host disease presenting with dysphagia

European Journal of Dermatology. Volume 16, Number 1, 92-3, January-February 2006, Letter to the editor

Author(s) : Risa Tamagawa, Hideya Takenaka, Norito Katoh, Chihiro Shimazaki, Hitoshi Bamba, Saburo Kishimoto , Department of Dermatology, Kyoto Prefectural University of Medicine Graduate School of Medical Science, 465, Kajii-cho, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan, Division of Hematology and Oncology, Department of Medicine, Department of Otolaryngology-Head and Neck Surgery, Kyoto Prefectural University of Medicine Graduate School of Medical Science. 465, Kajii-cho, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, Japan.

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Auteur(s) : Risa Tamagawa¹, Hideya Takenaka¹, Norito Katoh¹, Chihiro Shimazaki², Hitoshi Bamba³, Saburo Kishimoto¹

¹Department of Dermatology, Kyoto Prefectural University of Medicine Graduate School of Medical Science, 465, Kajii-cho, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan
²Division of Hematology and Oncology, Department of Medicine
³Department of Otolaryngology-Head and Neck Surgery, Kyoto Prefectural University of Medicine Graduate School of Medical Science. 465, Kajii-cho, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, Japan

We read with interest the article by Marzano et al. [1] concerning clinical and laboratory investigations on localized scleroderma. Orthopedic complications, such as subcutaneous tissue and muscle atrophy and impaired joint motility, are sometimes seen in generalized morphea and morphea profunda. Occasionally, the mucocutaneous manifestations of chronic graft-versus-host disease (GVHD) resemble scleroderma, and here we report a rare case of sclerodermatous GVHD presenting with impaired joint motility and dysphagia, which might have been caused by sclerosis of the dermis and subcutaneous tissues after scar formation due to catheter placement on the neck.A Japanese woman was diagnosed with acute lymphocytic leukemia at the age of 27, and underwent bone marrow transplantation (BMT) from a human leukocyte antigen-identical unrelated donor one year after disease onset. Acute GVHD appeared on the sixteenth day following BMT, but responded to treatment with prednisolone and immunosuppressors. Thereafter, GVHD was well controlled. However, at 33 years of age, sclerotic lesions appeared around scars at the bilateral aspects of the neck (( figure 1 )A); these scars were caused by intravenous hyperalimentation treatment given eight years earlier. The sclerotic lesions increased, and the patient complained of dysphagia for solids. One year later, sclerotic lesions appeared in her upper extremities, and flexion contractures of the elbows also developed.The full blood count was normal, and the only abnormal biochemical finding was mild liver dysfunction, which was thought to be a sign of chronic GVHD. The rheumatoid factor level was higher than 22.9 IU/ml, but other immunologic tests for antinuclear, anti-Scl-70, anticentromere, anti-SS-A/B and anti-DNA antibodies gave negative or normal values. A neck skin biopsy specimen showed that the collagen bundles had become hyalinized, coarse and condensed from the middle layer of the dermis to the fascia. The fascia itself was intact, but atrophy of the epidermis and slight perivascular lymphocytic infiltration in the upper dermis were apparent (( figure 1 )B and C). Endoscopic findings of the esophageal mucosa were normal. A barium esophagogram revealed small movements of the larynx and failure of the upper esophagus to open. Cervical ultrasonography showed thickening of subcutaneous tissues around the trachea, which was thought to be fibrosis (( figure 1 )D). A diagnosis of sclerodermatous GVHD was made based on the clinical and histologic findings, and therapy of 5 mg/day prednisone and topical corticosteroids was initiated. However skin sclerosis has progressed gradually.Histological findings in sclerodermatous GVHD show that hyperplasia of collagen fibers occurs in the upper layer of the dermis, and hydropic changes in the basal cell layer and exocitosis are also observed [2]. Our case showed atypical histological findings as sclerosis and epidermal changes began to develop. Antecedent cutaneous inflammatory conditions such as acute GVHD reportedly serve as a trigger for onset of sclerodermatous GVHD [3], and in our patient injury to the deep subcutaneous tissue occurred due to intravenous hyperalimentation treatment in the internal jugular veins. The deep dermis and subcutaneous tissue are thought to be possible targets of inflammation following tissue injury, and collagen synthesis may mainly occur in these tissues.Over 10% of patients with chronic GVHD develop esophageal disease [4], resulting in mucosal inflammation and leading to submucosal fibrosis and occasionally to formation of webs and strictures. Dysphagia usually occurs if abnormalities in the esophageal structure are present. In our patient, dense fibrosis around the trachea may have restricted movement of the larynx and upper esophagus, although the esophageal structure itself was intact. This case indicates that injury in deep parts of the skin can be a trigger of sclerodermatous GVHD, and that skin sclerosis that reaches to deep tissues can cause abnormalities of other organs.

References

1 Marzano AV, Menni S, Parodi A, et al. Localized Scleroderma in adults and children. Clinical and laboratory investigations of 239 cases. Eur J Dermatol 2003; 13: 171-6.

2 Lerner KG, Kao GF, Stob R, et al. Histopathology of graft-vs.-host reaction (GvHR) in human recipients of marrow from HL-A-matched sibling donors. Transplant Proc 1974; 6: 367-71.

3 Penas PF, Jones-Caballero M, Aragues M, et al. Sclerodermatous graft-versus-host disease: clinical and pathological study of 17 patients. Arch Dermatol 2002; 138: 924-34.

4 Wolford JL, McDonald GB. A problem-oriented approach to intestinal and liver disease after marrow transplantation. J Clin Gastroenterol 1988; 10: 419-33.