Extracorporeal photochemotherapy for generalized deep morphea.

Ten years ago, a 49-year-old white patient presented with a reticulated erythema and induration of her abdominal skin. Over the next 5 years, the erythema and induration progressed to include the skin over her lower extremities, chest, back, and upper extremities. With the onset of new lesions, she frequently experienced pruritus and tenderness at the areas of involvement. She also noted decreased ranges of motion in her shoulders. After a 2-year period without progression, she again developed worsening of her symptoms with progression of existing lesions and new areas of involvement accompanied by arthralgias in her shoulders, ankles, knees, wrists, and elbows and myalgias in her thighs and calves. Her symptoms interfered with her mobility and her ability to work a full day owing to severe fatigue and pain. She described frequently awakening at night with pain and discomfort in her skin and joints. Her medical, surgical, and family histories were noncontributory. There was no history of exposure to toxins, including bleomycin, polyvinyl chloride, or tryptophan. A review of systems was negative for dysphagia and Raynaud phenomenon. On physical examination, the bilateral extensor surfaces of the upper extremities from the deltoids to the triceps demonstrated a cobblestoned appearance. Hyperpigmentation was noted over the bilateral extensor forearms. Mild cobblestoning was present over the medial surface of the bilateral breasts and the bilateral buttocks. There were coalescing indurated, bound-down, reddish-purple, hypopigmented and hyperpigmented plaques over the abdomen (Figure 1). Thick, cobblestoned, erythematous, nearly circumferential plaques were found on the bilateral thighs with severe, deep erythematous bands over the sartorius muscles bilaterally. Findings from a comprehensive metabolic profile and complete blood cell count were within reference range, including a normal eosinophil count. An antinuclear antibody test was positive with a titer of 1:320 and a speckled pattern. Findings from an upper endoscopy were unremarkable. Clinical presentation and findings from a punch biopsy specimen from the abdomen were consistent with generalized and deep morphea. Previous treatments were numerous and included several topical corticosteroids, calcipotriene, and tacrolimus. Oral tetracycline antibiotics were prescribed for 6 years, including doxycycline, once daily, and minocycline, twice daily. Multiple prednisone tapers with a maintenance dose of 5 mg every other day were prescribed over a 2-year period. This was followed by several other unsuccessful therapeutic trials: psoralen–UV-A phototherapy 3 times a week for 7 months; azathioprine, 50 mg twice daily for 6 months; and mycophenolate mofetil for 1 year, up to a maximum oral dose of 6 g/d. Despite these therapeutic trials, the patient continued to experience progression of her disease.