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[Cutaneous calciphylaxis treated by autologous keratinocytes graft and subtotal parathyroidectomy].

BACKGROUND: Cutaneous calciphylaxis, seen most often in dialysis patients, is characterised by skin necrosis, and is a disabling and lifethreatening disease. Despite intensive topical treatment, recourse to parathyroidectomy is often necessary. We report the case of a female patient with skin necrosis due to calciphylaxis: pain and necrotic lesions were controlled by grafting of cultured autologous keratinocytes (Epibase). CASE REPORT: A 75-year-old woman with a 5-year history of dialysis-dependent chronic renal failure secondary to nephroangiosclerosis presented a very painful necrotic ulceration on her left leg. In spite of an autologous patch grafts, the lesions rapidly deteriorated. Laboratory data showed high levels of calcium, phosphate and parathyroid hormone and imaging suggested parathyroidal adenoma. Although cutaneous biopsy was not performed, the diagnosis of cutaneous necrosis due to calciphylaxis with tertiary hyperparathyroidism was established. Treatment consisted of a low-calcium and low-phosphorus diet with autologous keratinocytes grafts (Epibase). After the third application of keratinocytes, the pain disappeared, necrosis ceased and wound healing began. Subtotal parathyroidectomy was performed two months after the start of grafts. At three months, the patient was cured. DISCUSSION: . Calciphylaxis is an obstructive vascular disease secondary to calcification of the arterioles leading to ischemic tissue necrosis. Prompt diagnosis is essential since this disease is disabling and life-threatening due to sepsis and ischemic complications. In our case, autologous keratinocyte grafts allowed pain relief to be achieved within large expanses of cutaneous necrosis after correction of calcium and phosphorus levels, thereby allowing parathyroidectomy to be performed under optimal conditions.

Aged↗

Malignant melanoma and dermatofibrosarcoma in a 60-year-old patient with lifelong acrodermatitis enteropathica.

The life course of acrodermatitis enteropathica is recorded in a 62-year-old white man. Initially saved in infancy by breast-feeding and good medical care, later in his twenties he responded well to diiodohydroxyquinoline (Diodoquin) therapy, his only residua being dermatitis, hoarseness, and short stature. Subsequently untreated, this patient years later developed not only a dermatofibrosarcoma but also a large amelanotic malignant melanoma. Both were successfully excised. Subsequently, oral zinc therapy initiated for the first time cleared his acrodermatitis, which had been present for 60 years. It is suggested that this patient's malignancies developed as a result of an immune deficiency state typically found in acrodermatitis enteropathica. On this basis, acrodermatitis enteropathica may be viewed as having a malignant potential over the long term. The zinc-dependent nature of the immune deficit, however, suggests that lifelong daily zinc supplementation is an appropriate prophylactic measure.

Acrodermatitis↗

Bullous lichen sclerosus et atrophicus: treatment by tangential excision.

The bullous variant of lichen sclerosus et atrophicus (LSA) is a rare disorder for which there is no satisfactory treatment. A patient is reported with extensive plaques of bullous LSA on the pretibial skin that did not respond to sharp debridement, vigorous local care, or intralesional corticosteroid injections. Tangential partial-thickness excision of diseased tissues resulted in an extended remission. To our knowledge this approach has not been previously employed for bullous LSA, and it offers an encouraging surgical alternative to other therapeutic modalities.

Aged↗

Treatment of lichen sclerosus with potassium para-aminobenzoate.

In five patients with symptomatic lichen sclerosus, significant clinical improvement was obtained following the administration of potassium para-aminobenzoate. Improvement was characterized by a marked decrease or absence of symptoms and a flattening of skin lesions.

Adult↗