[Leprosy disclosed by polyarthritis]. / Lèpre révélée par une polyarthrite.

BACKGROUND: We report a case of leprosy observed in a French woman who had lived in Africa 30 years earlier. The clinical presentation was misleading, suggesting connective tissue disease. CASE REPORT: A 69-year-old woman was hospitalized in April 1996 for inflammatory joint disease. The first manifestations had developed three years earlier and the patient had been on systemic corticosteroid therapy associated with anti-malarials since 1993. The clinical presentation progressively included neurological and skin manifestations. Histology examination gave the diagnosis of lepromatous leprosy. Three-drug anti-leprosy treatment in one oral dose was initiated. DISCUSSION: Chronic Mycobacterium leprae infection usually leads to overt leprosy with neurological and cutaneous involvement. Rheumatological forms are less common and found almost exclusively during leprous reactions. The association of inflammatory join pain with neurological and skin manifestations wrongly suggested vasculitis. In addition, the general corticosteroid therapy certainly was implicated in disease activation and progression to a purely lepromatous form.

Linear cutaneous neuromas (dermatoneurie en stries): a limited phakomatosis with striated pigmentation corresponding to cutaneous hyperneury (featuring multiple endocrine neoplasia syndrome?).

A 60-year-old woman developed a progressive linear pigmentation on the trunk. Skin biopsy demonstrated an increase in the number and size of neural fibers in the dermis. Clinical and paraclinical investigations of this unusual disease showed findings similar to the hereditary type of Sipple syndrome (multiple endocrine neoplasia syndrome, Type 2b). Such findings as Marfanoid habitus, abnormal electromyography and hypertrophy of the corneal nerves suggest that our patient's disease and Sipple syndrome are identical. However, such other symptoms of Sipple syndrome as presence of endocrine tumors are absent. Questions of long-term prognosis and physiopathogenicity are raised, since no nerve growth factor was detected in this patient. The name of linear cutaneous neuromas is suggested for the patient's lesions, and a possible association with the pigmentation and cutaneous neuromas of the MEN 2b syndrome.

[Pilomatrixoma (Malherbe's calcifying epithelioma). Apropos of 13 cases observed in 6 years]. / Le pilomatricome (épithélioma calcifié de Malherbe). A propos de treize cas observés en six ans.

Thirteen pilomatrixomas were seen over a six year period. These small benign cutaneous tumors are infrequent though not exceptional and occur mainly in children (mean age six years). The tumor's hard consistency, irregular shape and frequently cephalic location (nearly 50% of cases) are suggestive. Diagnosis is established upon histological examination which demonstrates irregularly shaped clusters of basophilic epithelial cells; these develop into highly suggestive momified areas with shadow cells and secondary calcification. Attention is drawn to the presence of hemorrhagic modifications in recent tumors and telangiectasia. Pilomatrixomas must be considered as suborganoid hamartomas arising from the primary hair-matrix germ cells. The treatment is minimal surgical excision.

[Diffuse poikiloderma with acrokeratosis, preceded by bullous lesions: Weary-Kindler's disease. Nosologic and histopathologic discussion]. / Poikilodermie diffuse avec acrokératose et précession de lésions bulleuses: maladie de Weary-Kindler. Discussion nosologique et histopathologique.

The authors report the case of a six and a half year old girl, with extensive poikilodermatous condition and acrokeratosis, preceded by early blistering and light sensitivity. The sequence of cutaneous symptoms suggests the diagnosis of hereditary and bullous acrokeratotic poikiloderma of Weary and Kindler; forty-two cases have already been reported in the literature and our patient would better fit into Kindler's type. The original findings under electron microscopy and direct immunofluorescence (colloid bodies, IgM deposition) that are reported herein are discussed from a pathogenic point of view.

[Thaumetopoea pityocampa caterpillar and man. Morphology of its venom apparatus. Epidemiological investigation (author's transl)]. / La chenille processionnaire (Thaumetopoea pityocampa Schiff. lépidoptères) et l'homme. Etude morphologique de l'appareil urticant. Enquête épidémiologique.

The combination of a mechanical phenomenon (penetration of the hair) and a chemical phenomenon (discharge of a toxic substance) accounts for the pathological symptomatalogy induced by the processional pine caterpillar. Epidemiological study has pin-pointed the regions infested by the pine caterpillar and has described in detail the dermatitis inflicted by this caterpillar with special emphasis on the frequency of ocular lesions. Also, a close correlation was noted between the geographical density of the populations of Thaumetopoea pityocampa Schiff and the geographical density of the clinical cases recorded. Skin tests have confirmed that the urticant substance is histamino-liberating. A morphological study of the urticant apparatus of this caterpillar ("mirror" and urticant hairs) was undertaken with the scanning electron microscope. The progressive coming into place of the bristles on the cuticular pads of the "mirror" was followed during the last three larval stages (L3, L4 et L5). The urticant hair, hollow on the inside, carries pointed spikes directed towards its distal end. There is no hole or pores on the hair and the necessity to grind the bristles in order to extract the urticant substance leads us to believe that the hair is really a bulb which must be broken, for example in the skin, in order to release its urticant substance (histamino-liberating).

[Cutaneous metastases in digestive cancers (author's transl)]. / Métastases cutanées des cancers digestifs.

Cutaneous metastases are seldom encountered in digestive cancers. The mechanism of their formation explains their low rate. The metastases spread mainly by the lymphatic system (especially in diffuse, spotty or pseudo-elephantiasic forms and in regional forms), however numerous lymph node filters found through out the lymphatic system limit the progression of neoplasic cells. On the contrary, metastases most often spread by retrograde involvement due to blocking of the lymphatic system. As for hematogenous metastases (generalized nodular forms or aberrant localizations), they are observed even less frequently than lymphatic ones; metastatic embolisms may effectively become blocked in capillaries, especially in the lungs and the liver, which constitutes a double barrier before the skin may be involved.

[The problem of "Duhring-Brocq's" disease in children (author's transl)]. / Le problème de la "maladie de Duhring-Brocq" chez l'enfant.

Inquiry in the french dermatological main centers and a survey of the literature suggest that the once described as Duhring-Brocq disease in children, may refer to three distinct bullous diseases. Taking in account clinical features, photon and electron-microscopical data, investigation for basement membrane antibodies, search for intestinal villous atrophy, effectiveness of dapsone or sulfapyridine, it is indeed possible to distinguish:1) bullous pemphigoid (very rarely); 2) dermatitis herpetiformis (not frequently), those two affections being similar to adult forms; 3) benign chronic bullous dermatitis of childhood. This last child specific disease, is the most frequently observed. Its clinical features are comparable to pemphigoids, but histopathology and immunology data are different. Its response to dapsone of sulfapyridine and to free gluten diet is note perfect and not constant, but its evolution is spontaneously favorable after a few years.