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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2018  |  Volume : 29  |  Issue : 2  |  Page : 422-425
Reactive perforating collagenosis: An important differential diagnosis in hemodialysis patients

Department of Nephrology, Apollo Gleneagles Hospitals, Kolkata, West Bengal, India

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Date of Web Publication10-Apr-2018


This is a case report of a 68-year-old hypertensive, diabetic woman who was on regular thrice weekly hemodialysis (HD). She presented with gradually worsening left lower limb pain and swelling. Clinical examination revealed significant edema over the left calf and ankle joint with significant calf tenderness. Extensive workup including magnetic resonance imaging of the lower limb and venous Doppler failed to show any significant abnormality. In view of developing papular lesions over the area, skin biopsy was performed, which finally confirmed reactive perforating collagenosis. This diagnosis ensured reassurance to the patient with halting of further extensive and probably expensive testing. Simple conservative management and symptomatic relief improved the pain over the next few days. This case report highlights the importance of keeping a wide differential for calf tenderness in diabetic HD patients and projects reactive perforating collagenosis as one of the important but frequently missed entities in such a scenario.

How to cite this article:
Dey AK. Reactive perforating collagenosis: An important differential diagnosis in hemodialysis patients. Saudi J Kidney Dis Transpl 2018;29:422-5

How to cite this URL:
Dey AK. Reactive perforating collagenosis: An important differential diagnosis in hemodialysis patients. Saudi J Kidney Dis Transpl [serial online] 2018 [cited 2020 Jun 4];29:422-5. Available from: http://www.sjkdt.org/text.asp?2018/29/2/422/229281

   Introduction Top

Skin involvement in kidney failure can present with several manifestations; they can be related to the process that causes renal failure due to the uremic state or the therapeutic measures performed. Involvement of the skin and mucous membranes may be a part of a varied and complex multisystem involvement and may be present at the first medical examination. Pruritus, which is the most important clinical symptom, is frequently described in patients with end-stage renal disease (ESRD).[1] Historically, the incidence of renal pruritus varies between 50% and 90% in patients on hemodialysis (HD). Reactive perforating collagenosis is a relatively less common group of disorders that are most commonly associated with diabetes mellitus (DM) and chronic kidney disease (CKD). The reported incidence is 4.5%–11% in dialysis populations.[1],[2]

There are very few studies looking into the prevalence of varied forms of acquired perforating dermatosis. Case reports have, however, highlighted time and again the growing need for skin biopsy in patients with intractable and sometimes incessant problems associated with these skin complaints, especially in the diabetic subgroup of CKD–HD-dependent patients. This case report looks into this aspect and asserts the need for timely diagnosis of such conditions to effectively manage such cases and more importantly prevent unnecessary and costly investigations.

   Case Report Top

A 68-year-old woman, known hypertensive and diabetic for the past 12 years, on regular thrice weekly HD for the past three years presented with a history of insidious onset and gradually worsening left lower limb pain and swelling of two-week duration. She did not have any symptoms to suggest a connective tissue disorder. She gave no history of trauma or prolonged episodes of immobilization before the onset of these symptoms. She denied any febrile illness or shortness of breath before the symptoms. She had optimum sugar control with regular compliant oral antidiabetic therapy.

On physical examination, she appeared relatively well with blood pressure of 130/80 mm Hg. A right femoral arteriovenous graft was noted without any local tenderness or bruising. Distal right lower limb was unremarkable.

Local examination of the left lower limb revealed significant edema over the left calf and ankle joint. Cautious examination also revealed significant left calf tenderness without any restriction of movement in the adjacent joints. Skin over the left tibia showed papular lesions [Figure 1] without any pruritus or blister formation. Systemic examination was unremarkable.
Figure 1: Blisters and papular lesions over the left calf region.

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Laboratory findings included hemoglobin of 9.7 g/dL and white blood cell count of 6450 with normal differential. Peripheral smear showed normocytic, normochromic anemia. Serum uric acid level was normal. Serum sodium and potassium were 136 and 4.6 mmol/L, respectively. Any evidence of myositis was ruled out with creatine phosphokinase level of 35 units/L.

Local X-ray did not show any obvious fracture. In view of the clinical picture, anti-cyclic citrullinated peptide testing was carried out which came to be negative. At this point, due to significant calf tenderness, Doppler of the venous system of the left lower limb was undertaken. It failed to show any features suggestive of deep vein thrombosis.

At this juncture, orthopedician referral was taken and magnetic resonance imaging of the left lower limb was carried out which did not show any lesions.

Clinical examination at this time showed worsening nagging pain disrupting the quality of life of the patient. In view of papular lesions, dermatology opinion was sought. Skin biopsy was performed after obtaining informed consent. The patient was apprehensive and anxious due to inability to reach a specific diagnosis and persisting symptoms.

Subsequently, the skin biopsy with hematoxylin and eosin staining revealed skin tissue with hyperkeratosis, papillomatosis, acanthosis of the epidermis containing a shallow cup-shaped invagination filled with basophilic material, and degenerated collagen bundles. The dermis showed vertically oriented bundles of collagen at the base of invagination and mild perivascular chronic inflammation. These features were suggestive of reactive perforating collagenosis [Figure 2].
Figure 2: Skin biopsy showing reactive perforating collagenosis (H & E).

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She was subsequently started on topical emollients, ice cold compressions, symptomatic pain relief with analgesics, and fusidic acid ointment for local application. No evidence of drug-related side effects was documented during this period.

She was discharged with the above medications along with her regular antihypertensive and oral hypoglycemic medicines. She continued regular HD on an outdoor patient basis. Over the next three to four weeks, she gradually improved symptomatically. Two months after this, she is now pain free and is on regular follow-up with us and compliant with her medications.

   Discussion Top

There has been a recent escalation in the quantum of patients with CKD. With the improvement in understanding of renal physiology and improvement in management protocols including better HD modalities and renal transplantation, survival of patients with CKD is showing much improvement. Hence, with this improvement in longevity of patients, there has been unearthing of various pathological entities, which were hitherto unknown or unexplored.

Skin manifestations in HD patients are one such unaddressed and unexplored arena. Skin changes in both dialysis and transplant patients have been a topic of discussion in several studies.[3],[4],[5],[6] However, in the past few years, there has been some progress regarding therapy, which might have changed the type of skin change associated with these two conditions. Some of the skin manifestations of patients on renal replacement therapy may also be a result of dialysis or posttransplant immunosuppressant treatment.

Pruritus is frequently described in patients with ESRD. Acquired perforating dermatoses, such as perforating folliculitis, or other perforating dermatoses, such as reactive perforating collagenosis,[5] may be associated with ESRD.[6],[7],[8],[9] This condition affects more than 10% of the patients on HD, and it seems to be more common in people of African origin.[6],[7],[8]

Rapini et al[9] recommended the term “acquired perforating dermatosis” to be used for the broad spectrum of perforating skin disorders occurring in adult patients with systemic diseases.

As such, acquired perforating dermatosis is a heterogeneous group of disorders with transepidermal elimination of collagen, elastic tissue, or necrotic connective tissue. While CKD and DM are most commonly associated (73 and 50%, respectively, in one study),[10] acquired perforating dermatosis has been reported in patients with other systemic disorders, such as hepatic dysfunction, thyroid disorders, human immunodeficiency virus, and malignancies (namely, lymphoma and pancreatic carcinoma).[10] There is no gender or racial predilection.

Clinically, patients present with hyperkeratotic papules with a central crater filled with crust, on the trunk and extensor surfaces, usually with linear distribution.[11] Simultaneous transepidermal elimination of collagen and elastin has been detected.[5] The etiology of this process is not clear. However, the proposed mechanisms include diabetic microangiopathy, microtrauma caused by chronic pruritus, deregulation of Vitamins A and D, abnormality of collagen fibers and/or elastin, or local inflammation and conjunctive tissue degradation, caused by dermal microdeposit of substances, such as uric acid and calcium pyrophosphate.[7],[12] Topical and intralesional steroids, topical and systemic retinoids, cryotherapy, and ultraviolet radiation have been some of the described treatments.[7],[11]

There have been isolated case reports that have suggested that reactive perforating collagenosis is more common in diabetic HD patients. This case report also fulfills these criteria. However, no case report till date has addressed reactive perforating collagenosis as a differential diagnosis of calf tenderness. In doing so, this case report in no way undermines the importance of detailed evaluation of the more common causes of calf tenderness in HD patients. Although uncommon, keeping this entity in mind will in fact broaden the differential causes of calf tenderness in this group of patients. This case report is unique since the diagnosis of this condition in this particular scenario ensured alleviation of apprehension and unnecessary further costly investigations. In the Indian health structure where investigation costs have to be borne by the patients themselves, it is important to apprise nephrologists of this not so uncommon entity. This will go a long way in ensuring timely diagnosis and management.

Conflict of interest: None declared.

   References Top

De Marchi S, Cecchin E, Villalta D, et al. Relief of pruritus and decreases in plasma histamine concentrations during erythropoietin therapy in patients with uremia. N Engl J Med 1992;326:969-74.  Back to cited text no. 1
Szepietowski JC, Schwartz RA. Uremic pruritus. Int J Dermatol 1998;37:247-53.  Back to cited text no. 2
Altmeyer P, Kachel HG, Jünger M, Koch KM, Holzmann H. Skin changes in long-term dialysis patients. Clinical study. Hautarzt 1982; 33:303-9.  Back to cited text no. 3
Avermaete A, Altmeyer P, Bacharach-Buhles M. Skin changes in dialysis patients: A review. Nephrol Dial Transplant 2001;16:2293-6.  Back to cited text no. 4
Griffon-Euvrard S, Bustamante R, Thiovolet J. Skin manifestations in patients with renal chronic renal failure on regular hemodyalysis. Med Cutan Ibero Lat Am 1976;4:401-13.  Back to cited text no. 5
Picó MR, Lugo-Somolinos A, Sánchez JL, Burgos-Calderón R. Cutaneous alterations in patients with chronic renal failure. Int J Dermatol 1992;31:860-3.  Back to cited text no. 6
Morton CA, Henderson IS, Jones MC, Lowe JG. Acquired perforating dermatosis in a British dialysis population. Br J Dermatol 1996;135:671-7.  Back to cited text no. 7
Patterson JW. The perforating disorders. J Am Acad Dermatol 1984;10:561-81.  Back to cited text no. 8
Rapini RP, Herbert AA, Drucker CR. Acquired perforating dermatosis. Evidence for combined transepidermal elimination of both collagen and elastic fibers. Arch Dermatol 1989;125: 1074-8.  Back to cited text no. 9
Saray Y, Seçkin D, Bilezikçi B. Acquired perforating dermatosis: Clinicopathological features in twenty-two cases. J Eur Acad Dermatol Venereol 2006;20:679-88.  Back to cited text no. 10
Farrell AM. Acquired perforating dermatosis in renal and diabetic patients. Lancet 1997; 349:895-6.  Back to cited text no. 11
Haftek M, Euvrard S, Kanitakis J, Delawari E, Schmitt D. Acquired perforating dermatosis of diabetes mellitus and renal failure: Further ultrastructural clues to its pathogenesis. J Cutan Pathol 1993;20:350-5.  Back to cited text no. 12

Correspondence Address:
Dr. Ayan Kumar Dey
Department of Nephrology, Apollo Gleneagles Hospitals, Kolkata, West Bengal
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DOI: 10.4103/1319-2442.229281

PMID: 29657213

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