Acquired reactive perforating collagenosis

ARTICLE

A 67-year-old woman presented with a 6-month history of painful and pruritic eruptions on the extensor surface of the legs and gluteal region. She had been under oral medication for non-insulin-dependent diabetes mellitus, hypertension and congestive cardiac failure for 7 years. Examination revealed multiple, tender, umbilicated red-brown papules of different sizes, mostly with adherent keratotic plugs although a few were ulcerated. Koebner phenomenon was evident (Fig. 1). In addition to systolic murmur, hepatomegaly, ascides and pretibial edema were present. She had retinopathy, peripheric neuropathy, dilated cardiomyopathy, pulmonary hypertension and antral gastritis as well.

Laboratory findings including complete blood count, sedimentation rate, thyroid function tests, serological and biochemical analysis were normal except for fasting blood glucose (301 mg/dl; normal: 70-110), lactic dehydrogenase (586 IU/L; normal: 218-472) and serum parathormone levels (108 pg/ml; normal: 9-55). Urinalysis yielded 3 + glucose. Examination of feces for occult blood and parasitic infections were normal as well as 24-hour creatinine clearance.

Acquired reactive perforating collagenosis

In microscopic examination of the well-developed skin lesion biopsy, a dome shaped papule with a crater which was filled with keratinous material, neutrophils, and neutrophilic debris was observed (Fig. 2A). Epithelial cells lining the crater and adjacent epithelium were hyperplastic. At the base of the crater, collagen bundles were detected passing through into the epidermis (Fig 2B and C).

A moderately potent topical steroid and oral antihistamines were used with no beneficial results for the skin lesions. We tried 0.1% retinoic acid cream for 2 weeks and had to start oral isotretinoin, 40 mg daily, thereafter because of uncontrolled itching. Pruritus was controlled within 2 weeks, but we could not continue the treatment since the patient was hospitalized because of cardiac failure and died 48 hours later.

Comments

Since first described in 1967 [1], reactive perforating collagenosis (RPC) has been characterized by transepidermal elimination of collagen histologically, altered by superficial trauma. It is a rare entity presenting as pinhead-sized papules which develop umbilicated keratotic plugs on the extensor aspects of the limbs. A sporadic acquired form of the disease occurs in adulthood, particularly among patients suffering from diabetes mellitus or renal failure [2-4].

Various systemic disorders accompanied by pruritus and scratching were reported to be associated with ARPC such as diabetes mellitus, hyperparathyroidism, hypothyroidism and kidney or liver disorders [3-5]. Recently, several cases were described occurring in a zosteriform distribution [6], or associated with adenocarcinoma of the biliary duct [7] and periampullary carcinoma [8] or with ichtiyosis and liver metastasis of unknown primary [9]. Our case was associated with diabetes mellitus and hyperparathyroidism, meeting the diagnostic criteria of Faver et al. [3] for ARPC. In differential diagnosis, erythema induratum, sarcoidosis, deep fungal infections, prurigo nodularis, Kaposi's sarcoma and pityriasis lichenoides et varioliformis acuta must be considered.

There are several treatment modalities proposed for ARPC with different results, including topical steroids and UVB [3], topical retinoic acid and isotretinoin [10] or allopurinol [11]. In this case, itching responded well to isotretinoin within a few days whereas topical retinoic acid was not satisfactory in a 2 week period. We believe that the duration of treatment must be longer and proper control of diabetes mellitus or an associated disease, which is the main cause of pruritus, such as the onset of hyperparathyroidism probably resulting from nephropathy or malignancy, is absolutely necessary. *

* Presented as a poster at 9th EADV Congress, 11-15 October 2000, Geneva.

Article accepted on 2/4/01

REFERENCES

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