Panniculitis associated with severe alpha 1-antitrypsin deficiency. Treatment and review of the literature

Alpha‐1 antitrypsin deficiency panniculitis: clinical and pathologic characteristics of 10 cases

“…The rarity of AATD panniculitis makes it challenging to design any adequately powered therapeutic trials. Various medications including corticosteroids, nonsteroidal anti‐inflammatory drugs, colchicine, cyclophosphamide, danazol, antimalarials, and antibiotics have been reported as treatments for AATD panniculitis . The most commonly reported medications in this study were dapsone (50%) and therapeutic augmentation of AAT with purified alpha‐1 antitrypsin from pooled human plasma (50%).…”

“…Various medications including corticosteroids, nonsteroidal anti-inflammatory drugs, colchicine, cyclophosphamide, danazol, antimalarials, and antibiotics have been reported as treatments for AATD panniculitis. 4,8,18,21,22 The most commonly reported medications in this study were dapsone (50%) and therapeutic augmentation of AAT with purified alpha-1 antitrypsin from pooled human plasma (50%). Currently, the literature advocates using dapsone as the initial treatment and AAT replacement therapy as second-line therapy, likely secondary to cost associated with AAT replacement therapy.…”

Alpha1‐Antitrypsin Deficiency‐Associated Panniculitis: Case Report and Review of the Literature

“…An important new therapeutic option is administration of lyophilized partially purified human a|-AT obtained from healthy donors (3). Not only does it slow the progression of emphysema in a,-ATdeficient patients, it also controls the progression of the ulcerative panniculitis (7). Its use proved unnecessary in our patient.…”

Efficacy of alpha1-antitrypsin augmentation therapy in conditions other than pulmonary emphysema

“…From 1987 to 2010 seven cases of AAT deficiency-related refractory relapsing panniculitis, treated with AAT as the last resource, were published [62-67]. The two first cases were reported by clinicians in the Mayo Clinic, Rochester, Minn, in 1987 [62].…”

“…The two first cases were reported by clinicians in the Mayo Clinic, Rochester, Minn, in 1987 [62]. Ten years later, 2 other cases were reported by the Northwestern University Medical School of Chicago [63,64], and, from 2002, 3 new cases (1 each from the UK [65], Norway [66] and Germany [67]) were reported by several European authors.…”