[Survey in 2000: 3 cases]. / Scorbut en l'an 2000: 3 cas.

BACKGROUND: We report three new cases of patients presenting scurvy. In the year 2000 this rare disease still occurs in France. CASE REPORTS: The three patients, 2 men and a woman respectively 51, 50, and 73 years-old were alcoholics, and lived alone in difficult social conditions. Dietary survey indicated in the 3 cases inadequate vitamin C intake, and a regimen including solely bread, rice, pasta, and packet soup devoid of fresh vegetables and fruit. The cutaneous findings attributed to scurvy were: in the first patient, a woody inflammatory and painful oedema of the left leg associated with perifollicular petechial haemorrhages over the lower limbs, and hyperpigmentation of the facial skin with slate-gray spotty pigmentation of the tongue (pseudo-addisonian hyperpigmentation); in the second patient, an accentuation of a pre-existing acne becoming more inflammatory and extensive; and in the third patient, a diffuse petechial eruption on the abdomen and lower extremities. The diagnosis of scurvy was confirmed by low plasma ascorbic acid levels (< 6 mumol/l). All patients were treated with 1 to 2 g of oral ascorbic acid daily for 2 weeks resulting in rapid and dramatic response. DISCUSSION: Scurvy is a rare disease in industrialized nations. Its incidence is unknown because of absence of total census. Dietary vitamin C deficiency represents the main risk factor exposing for scurvy among adults, often alcoholics and living in social isolation. Cutaneous features supporting the diagnosis of scurvy are described in our observations. The recognition of these cutaneous abnormalities is important because their association can be misleading, and erroneously interpreted as a sign of systemic vasculitis, or connective tissue disease. The diagnosis of scurvy is confirmed by the measurement of plasma ascorbic acid levels. Treatment is simple and based on the administration of vitamin C, which results in dramatic improvement.

Relationship between the in vivo localization and the immunoblotting pattern of anti-basement membrane zone antibodies in patients with bullous pemphigoid.

OBJECTIVE: To compare the localization of anti-basement membrane zone (BMZ) antibodies bound in vivo with the antigenic specificities of circulating anti-BMZ antibodies in patients with bullous pemphigoid (BP). DESIGN: Comparison of the results of an examination of the skin specimens of the patients using direct immunoelectron microscopy and direct immunofluorescence on 1-mol/L sodium chloride-split skin with the results of an analysis of the corresponding serum samples using the immunoblot technique. SETTING: Immunodermatology department in a teaching hospital. PATIENTS: Thirty-six patients with typical BP and circulating anti-BMZ antibodies. RESULTS: Serum samples from 22 patients with BP indicated only BP antigen 1 in the results of immunoblot analysis. Using direct immunofluorescence, an analysis of the peribullous skin samples obtained from these 22 patients showed deposits of IgG exclusively located along the epidermal side of sodium chloride-split skin; the results of direct immunoelectron microscopic examination showed deposits of IgG located on the intracellular portion of hemidesmosomes in 18 (82%) of these 22 specimens, whereas 4 biopsy specimens had linear IgG deposits located both intracellularly and extracellularly along the keratinocyte plasma membrane. The results of immunoblot analysis of the serum samples from 5 patients with BP indicated BP antigen 2 alone; the results of direct immunoelectron microscopic examination of peribullous skin samples from these 5 patients showed linear intracellular and extracellular deposits along the keratinocyte membrane, corresponding to an epidermal fluorescence labeling pattern of peribullous sodium chloride-split skin in 2 patients and a combined (dermal and epidermal) pattern in 3 patients. CONCLUSION: The 2 different patterns of reactivity of anti-BMZ antibody deposits bound in vivo closely corresponded to the antigenic specificities indicated in the corresponding serum samples of the patients. These results are in accordance with those previously obtained in vitro and argue for identical binding profiles of circulating antibodies that are bound in vivo in BP.