with altered activity may still lead to clinical disease. 4,5 A1AT deficiency panniculitis has been treated with corticosteroids, dapsone, colchicines, and doxycycline. 1,2,4 The latter acts predominantly through its anticollagenase properties. In severe resistant cases, such as in our patient, intravenous supplement of exogenous A1AT concentrate can be highly effective. 6 This is a human purified enzyme that is collected from a pool of healthy donors. Initial treatment is at a dose of 60 mg per kg weekly; however, the dose and frequency of infu- sion therapy can be adjusted according to clinical response. Although this therapy appears to be highly effective, its use may be limited because of the relative high cost and selected availability in specialized emphysema centres. In severe cases of A1AT deficiency panniculitis, particularly when cirrhosis is present, liver transplantation can be considered. It is important to include A1AT levels and pheno- type testing as part of routine screening for pannic- ulitis, particularly in the ulcerative and necrotic forms, and especially if associated with unexplained emphysema and/or cirrhosis in a young patient. Intravenous therapy with A1AT may be extremely effective in such cases. Firas Al-Niaimi, MSc, MRCP, a and Calum Lyon, MA MB BChir FRCP b Departments of Dermatology, Salford Royal Hospi- tal Trust, a Manchester, and York Hospital, b York, United Kingdom Funding sources: None. Conflicts of interest: None declared. Correspondence to: Firas Al-Niaimi, Specialist Reg- istrar, Department of Dermatology, Salford Royal Hospital, Stott Lane, Manchester, M50 3RA, United Kingdom E-mail: firas55@hotmail.com REFERENCES 1. Hendrick SJ, Silvermann AK, Solomon AR, Headington JT. Alpha 1 antitrypsin deficiency associated with panniculitis. J Am Acad Dermatol 1988;18:684-92. 2. Edmonds BK, Hodge JA, Rietschel RL. Alpha 1-antitrypsin deficiencyeassociated panniculitis: case report and review of the literature. Pediatr Dermatol 1991;8:296-9. 3. Geller JD, Su WP. A subtle clue to the histopathologic diagnosis of early alpha 1-antitrypsin deficiency panniculitis. J Am Acad Dermatol 1994;31(2 pt 1):241-5. 4. Su WPD, Smith KC, Pittelkow MR, Winkelmann RK. Alpha 1-antitrypsin deficiency panniculitis: a histopathologic and immunopathologic study of four cases. Am J Dermatopathol 1987;9:483-90. 5. Smith KC, Pittelkow MR, Su WPD. Panniculitis associated with severe alpha 1-antitrypsin deficiency. Arch Dermatol 1987;123: 1655-61. 6. Chowdhury MM, Williams EJ, Morris JS, Ferguson BJ, McGregor AD, Hedges AR, et al. Severe panniculitis caused by homozygous ZZ alpha1-antitrypsin deficiency treated successfully with human purified enzyme (Prolastin). Br J Dermatol 2002;147: 1258-61. doi:10.1016/j.jaad.2009.11.587 FoxeFordyce disease in monozygotic female twins To the Editor: FoxeFordyce disease (apocrine mili- aria) is a rare pruritic and chronic inflammatory condition that was first reported in 1902. 1 The term apocrine miliaria was suggested after the demon- stration of infundibulum ducteplugging of the apo- crine gland, resulting in bilateral apocrine sweat retention. It is characterized by multiple small follic- ular papules on apocrine glandebearing regions, mainly the axillae. Although it was reported more than a century ago, its etiopathogenesis is not been fully understood. However, hormonal influence seems to be important, and familial cases have seldom been reported. We describe a 22-year-old woman with a 10-year history of dome-shaped, skin- colored, or slightly pigmented follicular papules on both axillae (Fig 1, A). Her identical twin sister presented with the same clinical condition (Fig 1, B). They reported the appearance of lesions after their menarche when they coincidently shaved their axillae for the first time. Itching was present only at the beginning. They denied using oral contracep- tives and topical or systemic medicines. There were no other cases in the family. A histopathologic examination revealed hyper- keratosis of follicular infundibula, spongiosis, and Fig 1. A and B, Clinical aspects of the two sisters’ axillae: multiples dome-shaped, skin-colored papules. JAM ACAD DERMATOL VOLUME 65, NUMBER 1 Letters 229