CLINICAL HISTORY:

Signs and symptoms:  An 81 year old white male with a significant past medical history of chronic renal failure, bladder cancer and hemochromatosis presented with a three to four week history of “sores all over his body.” He complained of pruritus, scratching and picking at the lesions. He also complained of chronic decreased urine output and swelling in both feet.

Previous Treatment:  He had previously been treated with diphenhydramine and hydroxyzine with little improvement of the pruritus or skin lesions.

Other information:  The rest of the review of systems was unremarkable. He has been on hemodialysis for approximately 5 years for which he has a fistula in his left arm for dialysis access and has no history of diabetes mellitus. His medications at presentation included tramadol, diclofenac, alprazolam, metoprolol, losartan, omeprazole, diphenhydramine, and hydroxyzine.

PHYSICAL EXAM:

Examination revealed multiple crusted and excoriated erosions and ulcers on the dorsal hands, arms, legs, and feet bilaterally. The erosions involved the patient’s left side more extensively, especially his left arm and dorsal hand. A few scattered excoriated papules with central keratotic plug were also present. An arteriovenous fistula was noted in the left brachium.

LABORATORY TESTS:

CBC : WBC 4.5, HGB 12.5, HCT 36.5, Platelet 110
BUN 20, Creatinine 3.8, Albumin 2.6, ESR 22
Alk Phos, Calcium, Glucose, AST, ALT, Sodium, Potassium, ANA and were all WNL 24 hour urine porphyrins were not done due to patient’s inability to produce urine

DERMATOHISTOPATHOLOGY:

A punch biopsy of the right dorsal hand at the base of the fifth finger revealed a crater-like ulcer in the epidermis with scattered extravasation of collagen fibers through the ulceration. Neutrophils present at the base of the ulcer. A patchy perivascular lymphocytic infiltrate was seen within the dermis.

DIFFERENTIAL DIAGNOSIS:

1.   Porphyria cutanea tarda
2.   Acquired perforating dermatosis secondary to renal failure
3.   Prurigo nodularis
4.   Kyrle's disease
5.   Excoriations secondary to prurigo simplex

SCROLL DOWN FOR ANSWER AND DISCUSSION.

CORRECT DIAGNOSIS:

Acquired perforating dermatosis secondary to renal failure

DISCUSSION:

The perforating dermatoses have classically been divided into four main entities: Kyrle’s disease, perforating folliculitis, reactive perforating collagenosis, and elastosis perforans serpiginosa. Acquired perforating dermatosis (APD), as described by Rapini et al., has recently become recognized as a separate disease entity within the category of perforating dermatoses. APD is seen almost exclusively in patients who have chronic renal failure, diabetes mellitus or both, but has been reported in patients with neither disease. APD has been reported to occur in approximately 10% of hemodialysis patients, with onset of lesions anywhere from 1 to 5 years after the start of dialysis.
Clinically, APD present as pruritic papules often with a central keratotic plug, commonly appearing on the extensor extremities and trunk. The lesions may progress to nodules or plaques that may resemble prurigo nodularis, and Koebnerization may be seen. The exact mechanism behind APD in renal failure and diabetic patients is not known. Some authors have described APD as a reaction to trauma and scratching related to the associated pruritus, while others have theorized that it may be a result of microangiopathy caused by the associated renal failure or diabetes. Diagnosis is made based on clinical and histopathological examination.
Histologic examination lies somewhere along a spectrum of patterns that may be found in any of the other major perforating disorders including Kyrle’s disease, perforating folliculitis, elastosis perforans serpiginosa, and reactive perforating collagenosis. The most common pattern is one similar to that of perforating folliculitis showing a disruption in the follicular epithelium often filled with a plug of compact orthokeratosis and parakeratosis. Altered collagen and elastic fibers are commonly seen adjacent to the perforation and can be seen extruding through the perforation. A mixed inflammatory infiltrate of neutrophils, histiocytes and lymphocytes is often seen in a perifollicular distribution. There may be some histologic overlap with the features of prurigo nodularis.

TREATMENT:

Treatment of APD is often difficult and inadequate. To date, few controlled studies have been done on the various methods used to treat APD. Antihistamines provide little to no relief of the often associated severe pruritus. Some studies and case reports have shown that UVB may provide significant improvement of the pruritus and skin lesions of APD. Other studies have shown response to topical steroids, topical retinoic acid, as well as systemic allopurinol, etretinate, isotretinoin, and the tetracycline family of antibiotics. Complete resolution of lesions and symptoms has been reported after renal transplantation in some patients.
The patient was initially treated with a high potency steroid and oral antihistamines to control itching. We discussed using narrow-band UVB, however the patient died one month later.

REFERENCES:

Elder, D et al. Lever’s Histopathology of the Skin. 9th Edition. Lippincott Williams & Wilkins. 2005: 404-408.

Hong, SB; Park, JH; Ihm, CG; Kim, NI. Acquired Perforating Dermatosis in Patients with Chronic Renal Failure and Diabetes Mellitus. J Korean Med Sci 2004; 19:283-8.

James, W et. Al. Andrews’ Diseases of the Skin. 10th edition: 774-5.

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.

Saray, Y; Seckin, D; Bilezikci, B. Acquired perforating dermatosis: clinicopathological features in twenty-two cases. J Euro Acad of Dermol and Venereol. 2006 Jul;20(6): 679-88.

Ohe, Shuichi; et. al. Treatment acquired perforating dermatosis with narrowband ultraviolet B.
J Am Acad Dermatol 2004; 50(6):892-4.

Additional Comment:

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