MANCHESTER, ENGLAND — Reactive perforating collagenosis has been reported in a patient with severe connective tissue disease for the first time, adding to the list of underlying disorders associated with this skin eruption.
A 17-year-old female was referred with a 9-month history of a rash on the arms, shoulders, and legs. It had appeared post partum, coinciding with the onset of painful symptoms of Raynaud's phenomenon, fatigue, anergia, and pauciarticular arthritis, Dr. Anne-Marie Tobin said at the annual meeting of the British Association of Dermatologists.
The patient also had recently had a tonsillectomy for recurrent sore throat and was being tested for microcytic anemia.
The rash consisted of keratotic papules and plaques, typical of a perforating dermatosis.
An initial skin biopsy suggested a reactive folliculitis, but a repeat biopsy revealed acanthosis and an underlying perivascular infiltrate said Dr. Tobin of the department of dermatology, Waterford (Ireland) Regional Hospital. It also showed collagen entrapment in the epidermis and elimination through an epidermal depression, confirming the diagnosis of perforating collagenosis.
Because the rash worsened and the lesions became tender and unsightly, a course of 18 sessions of bath photochemotherapy (PUVA) was undertaken, she said. The cumulative dose was 20 J/cm2, and the results were excellent. Her connective tissue disease remains intractable, however. Trials of corticosteroids, mycophenolate mofetil, azathioprine, and colchicine have all been unsuccessful.
She required admission to the hospital on three occasions for infusions of iloprost to alleviate digital ischemia, but the endothelin receptor antagonist bosentan is now being used and appears to have stabilized her Raynaud's symptoms.
Recently, she was admitted because of myalgias, fatigue, elevated serum creatinine kinase, and raised serum aldolase. “The rash has also recurred, and we are awaiting resolution of the myositis to recommence bath PUVA,” Dr. Tobin said.
Other treatments that have been used successfully in reactive perforating collagenosis include UVB, allopurinol, doxycycline, and rifampin.