SW Kim, et al Ann Dermatol Vol. 26, No. 2, 2014 http://dx.doi.org/10.5021/ad.2014.26.2.162 ORIGINAL ARTICLE A Clinicopathologic Study of Thirty Cases of Acquired Perforating Dermatosis in Korea Seo Wan Kim, Mi Sun Kim, June Hyunkyung Lee, Sook-Ja Son, Kui Young Park1, Kapsok Li1, Seong Jun Seo1, Tae Young Han Department of Dermatology, Eulji General Hospital, Eulji University, 1Department of Dermatology, Chung-Ang University College of Medicine, Seoul, Korea Background: Acquired perforating dermatosis (APD) is tive perforating collagenosis (n=23, 73.3%). Conclusion: In histopathologically characterized by transepidermal elimi- this study, most patients had a systemic association to the nation of materials from the upper dermis. APD can be diseases. Therefore, we suggest that further evaluation is divided into four diseases: Kyrle’s disease, perforating necessary for patients who present with APD. This includes folliculitis, elastosis perforans serpiginosa, and reactive reviewing patient’s comprehensive past medical history, perforating collagenosis. APD is usually associated with clinical exam, and additional diagnostic testing to check for systemic diseases, especially diabetes mellitus or chronic the possibility of associated systemic diseases. (Ann renal failure. So far, there have only been a few Korean Dermatol 26(2) 162∼171, 2014) studies of APD, which have a limited number of patients. Objective: The aim of this study is to evaluate the clinical and -Keywords- histopathologic characteristics of 30 cases of APD and to Acquired perforating dermatosis, Elastosis perforans serpi- examine the association with systemic diseases. Methods: ginosa, Kyrle’s disease, Perforating folliculitis, Reactive We retrospectively reviewed the medical records and biopsy perforating collagenosis specimens of 30 patients who were diagnosed with APD. Results: The mean age was 55.5 years, and the average duration of the lesion was 7.8 months. The lower extremities INTRODUCTION (73.3%) were the most frequently occurring sites of the lesion. Twenty-five patients (83.3%) had pruritus, and Acquired perforating dermatosis (APD) is histopatholo- Koebner’s phenomenon was present in 11 patients. Patients gically characterized by transepidermal elimination of of 63.3% had at least one systemic disease. Diabetes mellitus various dermal substances. APD can be divided into four (n=17, 56.7%) and chronic renal failure (n=10, 33.3%) diseases, according to the types of epidermal disruption were the most commonly associated conditions. Most and the natures of the eliminated materials: Kyrle’s disease patients received topical steroids (93.3%) and antihistamines (KD), perforating folliculitis (PF), elastosis perforans serpig- (80.0%). The most common histopathologic type was reac- inosa (EPS), and reactive perforating collagenosis (RPC)1. APD is usually associated with diabetes mellitus (DM)2 or chronic renal failure (CRF)3. In recent years, it has also Received November 20, 2012, Revised March 15, 2013, Accepted for been reported to be associated with other systemic publication March 15, 2013 diseases, such as non-diabetic hemodialysis4, hepatic5 and Corresponding author: Tae Young Han, Department of Dermatology, Eulji 5 6 General Hospital, Eulji University, 68 Hangeulbiseong-ro, Nowon-gu, endocrinologic disorders , hepatocellular carcinoma , 7 8 Seoul 139-711, Korea. Tel: 82-2-970-8580, Fax: 82-2-974-1577, E-mail: Hodgkins’ lymphoma , acute leukemia , acquired im- [email protected] mune deficiency syndrome (AIDS)9, tuberculosis10, pulmo- This is an Open Access article distributed under the terms of the nary aspergillosis11, neurodermatitis5, atopic dermatitis12, Creative Commons Attribution Non-Commercial License (http:// scabies13, and pregnancy14. creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, To date, there have been only a few studies of APD, provided the original work is properly cited. including case reports in Korean literature, which have a 162 Ann Dermatol Table 1. Clinical features of 30 patients Case Sex/age APD Koebner Associated Distribution Symptom Clinical appearance Treatment Effectiveness No. (yr) duration phenomenon conditions 1 M/50 1 mo Trunk Pruritus - Hyperkeratotic NIDDM, CRF, CHF Oral antihistamine, topical steroid Slightly papules improved 2 F/44 1 mo Trunk, LEx Pruritus - Keratotic plugged, NIDDM, CRF, Oral antihistamine, NB-UVB, topical steroid Improved umbilicated papules hypothyroidism 3 M/70 1 yr LEx - - Hyperkeratotic - Topical steroid Slightly papules improved 4 M/71 2 yr LEx - - Hyperkeratotic - Topical steroid Improved papules 5 F/61 3 mo Trunk Pruritus - Hyperkeratotic NIDDM, CRF (HD), Cryotherapy, oral antihistamine, topical steroid Improved papules xerotic eczema 6 F/32 3 mo Trunk, LEx Pruritus - Keratotic plugged, Pregnancy NB-UVB, oral antihistamine, topical steroid Improved umbilicated papules 7 M/42 2 mo LEx Pruritus + Keratotic plugged, NIDDM, CRF (HD) NB-UVB, TA ILI, oral antihistamine Improved umbilicated papules 8 F/63 1 yr Trunk, UEx Pruritus - Serpiginous - Oral antihistamine, topical steroid Improved hyperkeratotic plaques 9 F/78 2 mo UEx Pruritus - Keratotic plugged, NIDDM, HTN, xerotic Oral antihistamine, topical steroid Improved umbilicated papules eczema 10 F/57 1 yr Trunk, LEx Pruritus + Keratotic plugged, NIDDM NB-UVB, TA ILI, oral antihistamine, topical Improved umbilicated papules steroid 11 M/83 3 mo LEx - - Hyperkeratotic CRF, HTN Topical steroid Slightly papules improved 12 M/39 1 yr UEx, LEx Pruritus + Keratotic plugged, IDDM, CRF, HTN, Oral antihistamine, topical steroid Resistant umbilicated papules hepatitis 13 F/59 2 mo Trunk Pruritus + Keratotic plugged, - TA ILI, Oral antihistamine, topical steroid Improved umbilicated papules 14 F/40 1 mo Trunk Pain - Keratotic plugged, -TA ILI Improved umbilicated papules 15 M/68 3 yr Trunk, UEx, Pruritus - Keratotic plugged, NIDDM, CRF (HD), TA ILI, Oral antihistamine, topical steroid Improved LEx umbilicated papules stasis dermatitis Acquired Perforating Dermatosis 16 F/48 2 mo Trunk, UEx Pruritus + Keratotic plugged, NIDDM, atopic TA ILI, Oral antihistamine, topical steroid Improved Vol. 26, No. 2,2014 26,No. Vol. umbilicated papules dermatitis 17 F/42 4 mo Trunk, UEx, Pruritus + Keratotic plugged, - Oral antihistamine, topical steroid Improved LEx umbilicated papules 18 F/30 3 mo Face, scalp, Pruritus Keratotic plugged, IDDM, CRF, atopic Oral antihistamine, topical steroid Slightly trunk umbilicated papules dermatitis improved 19 F/69 3 mo Trunk, LEx Pruritus + Keratotic plugged, HTN, xerotic eczema Oral antihistamine, topical steroid Improved umbilicated papules 20 M/81 1 mo LEx Pruritus, - Keratotic plugged, NIDDM, HTN, xerotic Oral antihistamine, TA ILI, topical steroid Improved 163 pain umbilicated papules eczema SW Kim, et al et SW Kim, 164 Ann Dermatol Table 1. Continued Case Sex/age APD Koebner Associated Distribution Symptom Clinical appearance Treatment Effectiveness No. (yr) duration phenomenon conditions 21 F/55 5 yr Trunk, UEx, Pruritus - Keratotic plugged, - TA ILI, Oral antihistamine, topical steroid Improved LEx umbilicated papules 22 F/75 1 mo Trunk, UEx, Pruritus + Keratotic plugged, - Oral antihistamine, topical steroid Slightly LEx umbilicated papules improved 23 F/55 2 mo Scalp, trunk, Pruritus - Hyperkeratotic NIDDM, xerotic eczema NB-UVB, TA ILI, oral antihistamine, oral Improved LEx papules acitretin, topical steroid 24 F/36 1 yr Upper LEx Pruritus + Keratotic plugged, - Oral antihistamine, topical steroid, topical Improved umbilicated papules calcineurin inhibitor 25 F/39 3 mo UEx, LEx Pruritus - Follicular infiltrating NIDDM, CRF (PD) Topical steroid Slightly papules improved 26 M/63 3 mo Trunk, UEx, Pruritus + Keratotic plugged, NIDDM Oral antihistamine, oral acitretin, oral Improved LEx umbilicated papules cyclosporine, Topical steroid 27 M/54 1 mo LEx Pruritus + Keratotic plugged, NIDDM, atopic NB-UVB, TA ILI, oral antihistamine, oral Improved umbilicated papules dermatitis acitretin, topical steroid 28 M/57 9 mo Trunk, UEx, Pruritus - Keratotic plugged, NIDDM, CRF (PD), NB-UVB, oral antihistamine, topical steroid Improved LEx umbilicated papules HTN, hepatitis, COPD 29 M/56 1 mo LEx - - Keratotic plugged, NIDDM Topical steroid Slightly umbilicated papules improved 30 F/47 3 mo LEx Pruritus - Hyperkeratotic - Oral antihistamine, TA ILI, topical steroid Improved papules APD: acquired perforating dermatosis, M: male, F: female, LEx: lower extremities, UEx: upper extremities, NIDDM: non-insulin-dependent diabetes mellitus, CRF: chronic renal failure, CHF: congestive heart failure, HD: hemodialysis, HTN: hypertension, IDDM: insulin-dependent diabetes mellitus, PD: peritoneal dialysis, COPD: chronic obstructive pulmonary disease, NB-UVB: narrow-band ultraviolet B, TA ILI: intralesional triamcinolone injection. Acquired Perforating Dermatosis limited number of patients and make it difficult for a Masson trichrome stains were used to evaluate the literature review. We report the clinical and histopatho- elimination of elastic or collagen fibers. The samples were logical features of 30 cases with APD and clarify the classified as KD, PF, EPS and RPC, according to the association with pathogenesis of APD and comorbidities. pathologic features and eliminated materials. To our knowledge, this is the first report of its kind and the largest study on the clinicopathological features of APD, RESULTS including all classical types of APD performed in