Studies of cicatricial pemphigoid autoantibodies using direct immunoelectron microscopy and immunoblot analysis.

We studied 11 consecutive patients with classical cicatricial pemphigoid (CP) using direct immunoelectron microscopy (IEM) and Western immunoblotting analysis. Direct IEM performed in the skin or gingival mucosa revealed in all 11 CP patients that immunoglobulins and complement deposits were usually thick and discontinuous along the dermoepidermal junction, mostly localized on the lamina densa and occasionally in the lamina lucida. By direct IEM, the ultrastructural aspect in CP differs from the pattern observed in bullous pemphigoid (BP) and from that of chronic epidermolysis bullosa acquisita (EBA). Nine CP patients were studied by Western immunoblotting and, of these nine, only two had detectable anti-basement membrane zone (BMZ) antibodies by indirect immunofluorescence on salt-split skin. By immunoblotting performed on protein extracts of heat-separated epidermis, eight out of the nine CP sera specifically reacted with two protein bands of approximately 230-240 kD and 180 kD, similar to those recognized by BP sera in co-migration experiments. By immunoblotting on skin BMZ extracts, none of these nine CP sera recognized the 290-kD major polypeptide of EBA antigen. Taken together, these results suggest that, in CP, the target-antigen, as identified on immunoblots, is similar to BP antigen, but with an abnormal expression within the dermoepidermal junction of patients, which may in part explain the scarring course of the disease.

Papillon-Lefèvre syndrome. Ultrastructural study and successful treatment with acitretin.

Four siblings affected by Papillon-Lefèvre syndrome (PLS) ranged in age from 2 to 11 years. The parents were unaffected and parental consanguinity was present. The 2-year-old girl showed the early manifestations of PLS; that is, slight gingival swelling and erythema occurring simultaneously with minimal scaling of palms and soles. The other siblings, aged 5, 8, and 11 years, showed severe periodontopathy with tooth loss and marked palmoplantar keratoderma with a centripetal extension of the keratoses to the limbs and trunk. These three older siblings were treated with acitretin (Ro 10-1670), the free acid of etretinate, with complete clearing of the skin and healing of gingival pockets. Treatment was given for 16 months; teeth that erupted during therapy were free of periodontopathy and remained firmly anchored to the alveolar bone. In two of the children ultrastructural examination of involved skin was performed before and during acitretin treatment. Before treatment a large number of lipidlike vacuoles were found in corneocytes and in granulocytes; tonofilaments were reduced in number, and keratohyaline granules frequently showed a rectangular or globular shape. During treatment with acitretin these abnormalities diminished markedly. Thus, etretin is effective in treating PLS and, if treatment is started at an early age, should allow patients with PLS to have normal adult dentition.

[Hand-foot-mouth syndrome recurring during common variable deficiency]. / Syndrome "mains-pieds-bouche" récidivant au cours d'un déficit commun variable.

BACKGROUND: Common variable immunodeficiency is characterized by hypogammaglobulinemia and recurrent bacterial infections. More uncommonly, these patients develop chronic enterovirus infectious meningoencephalitis. Recurrent enterovirus skin infection has not been reported to date in subjects with common variable immunodeficiency. CASE REPORT: A 26-year-old man had suffered repeated episodes of otorhinolaryngological and pulmonary infections since childhood. He experienced three episodes of vesicular cutaneous eruption involving the palms of both hands, the plantar aspect of the feet and the buccal mucosa. The patient was hospitalized in March 1995 at the third episode. Temperature was 38 degrees C. He had maculopapulous and vesicular eruptions on the palm of the hands and plantar aspect of the feet with irritation in some areas. Petichial lesions were seen on the palate. PCR demonstrated viral DNA and cell cultures of a lesion biopsy were positive for enterovirus. Gammaglobulinemia was 4 g/l with low B cell count. DISCUSSION: Viral infections are uncommon in patients with common variable immunodeficiency as cellular immunity remains normal. Severe viral infections caused by enteroviruses have however been reported, generally associating chronic, and generally fatal, meningoencephalitis. Our case would be the first case of a recurrent hand, foot and mouth disease in such patients.

Facial lipoatrophy: report of five cases after injection of synthetic filler into naso-labial folds.

BACKGROUND: Facial lipoatrophies are rare disfiguring syndromes, most commonly characterized by loss of buccal fat pads. AIMS: To demonstrate a relationship between facial lipoatrophy and a new synthetic filler device (Profill) used to correct naso-labial folds. METHODS: Five cases of lipoatrophy, appeared 9 months or later after Profill injections. No systematic abnormalities were found. In one case biopsy reported a destruction of adipocytes with intense fibrosis in the hypodermis. DISCUSSION: The culpability of this new biodegradable product is highlighted. To our knowledge, this is the first time this adverse reaction has been reported. CONCLUSION: Serious adverse reactions may appear months after Profill injection, skin testing is not always predictive or sufficient. Clinical studies with long-term follow-up need to be carried out before new filler devices are launched on the market.