A 64-year-old woman with a past medical history of hypertension, poorly controlled type 2 diabetes mellitus, hyperlipidemia, and obstructive coronary artery disease with prior stent placement was referred to the hospital by her primary care physician after laboratory testing revealed acutely elevated bilirubin. On evaluation, she reported 3 months of progressive fatigue and decreased exercise tolerance associated with generalized pruritus and jaundice. Vital signs were all within normal range.
Examination of the eyes was remarkable for bilateral scleral icterus. Skin examination revealed hyperpigmented, umbilicated papules with a central core (Figure 1) and larger plaques with central crusting (Figure 2) predominately in the shins, ankles, knees, forearms bilaterally, and back. Lesions were clean-based without surrounding erythema or purulent discharge. Labs were remarkable for a total bilirubin of 17 mg/dL with a direct bilirubin of 9.2 mg/dL, alkaline phosphatase of 1244 U/L, and slight AST elevation. Coagulation profile and balance of the comprehensive metabolic panel was within normal limits. She was admitted for work-up of unexplained hyperbilirubinemia and further evaluation of skin lesions.
Figure1. Hyperpigmented umbilicated papule with central keratotic plug. Figure 2. Koebnerization resulting in large crusted plaque.
Dermatology was consulted for evaluation of diffuse skin lesions. The differential diagnosis included the following.
Answer: D. Acquired perforating collagenosis
Biopsy of the lesions was performed and the patient prescribed empiric topical corticosteroids for symptomatic relief. Pathology results showed altered collagen with keratin plug, findings congruent with perforating collagenosis. In this clinical presentation, these results confirmed the diagnosis of reactive perforating collagenosis.
Concurrent workup of the hyperbilirubinemia revealed negative rheumatologic and tumor markers. CT scan of the abdomen and pelvis with IV contrast was performed, revealing no hepatic masses. Magnetic resonance cholangeopancreatograhy (MRCP) showed no evidence of biliary duct dilation or pathology. As the etiology of liver disease remained unclear and given history of significant coronary disease and dyspnea without overt physical manifestations of volume overload, transthoracic echocardiogram was performed to evaluate heart function. Echocardiogram revealed diffuse hypokinesis with ejection fraction of 20%. Liver biopsy was consistent with hepatic congestion and the patient was diagnosed with congestive hepatopathy, a result of severe systolic heart failure. After initiation of heart failure medications, total bilirubin decreased to 3mg/dL. At the time of discharge, her skin lesions showed clinical and symptomatic improvement with topical steroids and improved glycemic control.
APD is the term used to encompass acquired primary skin disorders in adults that are characterized by elimination of dermal connective tissue through the epidermis.1 These disorders are reactive perforating collagenosis (RPC), elastosis perforans serpiginosa, perforating folliculitis, and Kyrle Disease. Acquired RPC is the most common form of APD reported, typically diagnosed in the 5th decade of life with equal sex distribution.2,3
APD are most frequently seen in patients with long-standing systemic diseases, such as diabetes mellitus, chronic renal insufficiency, and liver disease. APD have also been reported in autoimmune disease, thyroid dysfunction, and both solid organ and hematologic malignancies.4,5 Lesions in acquired RPC typically develop on the extremities and may also appear on the trunk. These lesions are characterized by umbilicated papules and plaques with peripheral crusting.6 Larger plaques have an identifiable central white crusting on a hyperpigmented base. Skin biopsy may be obtained in patients with typical clinical history to confirm diagnosis.
Our patient presented for recent onset liver disease and known history of long standing diabetes mellitus, both systemic conditions associated with RPC. Though skin lesions were typical manifestations of RPC, skin biopsy was done to rule out rheumatologic and malignant etiologies.
Treatment consists of symptomatic control and management of the underlying disease. Pruritus can be treated with antihistamines and emollient creams. Topical corticosteroids and topical retinoids may reduce local inflammation and improve appearance of lesions.7
1. Fei C, Wang Y, Gong Y, et al. Acquired reactive perforating collagenosis. Medicine (Baltimore). 2016;95 e4305.
2. Hong S-B, Park J-H, Ihm C-G, Kim NI. Acquired perforating dermatosis in patients with chronic renal failure and diabetes mellitus. J Korean Med Sci. 2004;19;283-288. /
3. Metterle L, Magro CM, Zang JB. Giant variant of acquired perforating dermatosis in a renal dialysis patient. JAAD Case Rep. 2017;3:42-44.
4. Kim S-W, Kim M-S, Lee JH, et al. A clinicopathologic study of thirty cases of acquired perforating dermatosis in Korea. Ann Dermatol. 2014;26 162-71.
5. Hemma T, Becker JC, Legat F, et al. Allopurinol in the treatment of acquired reactive perforating collagenosis. An Bras Dermatol. 2013;88:94-97.
6. Poliak SC, Lebwohl, MG, Parris A, Prioleau PG. Reactive perforating collagenosis associated with diabetes mellitus. NEJM. 1982;306:81-84.
7. Arzu A, Kayacetin A. Acquired reactive perforating collagenosis. Eurasisan J Med. 2012;44:51-53.