[Lichen planus in children: 12 cases]. / Lichen plan de l'enfant. 12 cas.

OBJECTIVE: Lichen planus is in children uncommon and poorly understood. The classical description is comparable to lichen planus in adults. We conducted a retrospective analysis of 12 cases in children. PATIENTS AND METHODS: Twelve children with lichen planus consulted the Saint-Louis or Robert-Debré hospitals between February 1994 and March 1996. Data collected included: age, sex, ethnic origin, drug use, anti-hepatitis vaccination status, disease history, physical examination, skin histology, liver tests, hepatitis B and C serology, treatment and outcome. Histological proof was obtained in all cases but one (a child with isolated ungueal involvement whose sister had histologically proven ungueal lichen planus). RESULTS AND DISCUSSION: The clinical features classically described in adults were atypical in all our childhood cases. A rapidly extensive eruption was the main sign in 6 cases. The localizations were unusual with lesions involving all four limbs and the trunk as well as the face in 5 cases and the scalp in 1. Mucosal involvement, observed in 65 p. 100 of adult cases was only found in one of our children. Unguel involvement also appears to be uncommon in children. The etiological pattern was also unusual since we did not observe a single case related to drugs or hepatitis B or C infection. Three children developed a lichen eruption after anti-hepatitis B infection. Four other cases of lichen planus after anti-hepatitis B vaccination have been reported in the literature. Mean delay between the booster vaccination and onset of eruption is reported to be 40 days. The increased incidence of childhood lichen planus in tropical zones suggests ethnic, genetic and climatic factors may be involved. Prognosis is poorly defined in the literature. Certain authors emphasize the long duration of the disease and resistance to treatment in cases of childhood lichen planus. Currently, there is no consensus on treatment. Dermocorticoids in combination with antihistaminics are usually prescribed. General corticosteroid therapy would appear to be warranted in extensive progressive forms with important functional and esthetic impact (scalp involvement with cicatricial alopecia, pigmentation sequellae). The role of other drugs, particularly retinoids, remains to be defined. This retrospective series was not statistically significant. Data in the literature are rather discordant, emphasizing the need for a prospective analysis to acquire a better understanding of the real incidence of childhood lichen planus and better define the therapeutic strategy.

Chronic urticaria and macroglobulinemia (Schnitzler's syndrome): report of two cases.

Two cases of chronic urticaria associated with macroglobulinemia are reported, and the characteristics of 13 other cases are reviewed. This entity was described by Schnitzler in 1974 and has the following characteristics: chronic nonpruritic urticaria with leukocytoclastic vasculitis, bone pains with hyperostosis, intermittent fever, and a monoclonal IgM gammopathy. Liver, lymph node, and spleen enlargement may occur. Criteria for the diagnosis of Waldenström's disease are lacking (IgM level less than 10 gm/L, no overt lymphoid proliferation in bone marrow). Other immunologic findings (complement, C1 inhibitor, cryoglobulin, rheumatoid factor, antinuclear antibodies) are negative or normal. Evolution is long-term with a long follow-up period. In one case a lymphoplasmocytic lymphoma developed. No adequate treatment has yet been found. Pathogenesis is unclear but seems to be caused by skin deposits of the IgM paraprotein, as attested to by the direct cutaneous immunofluorescent findings in some cases.

Malignant acanthosis nigricans associated with non-Hodgkin's lymphoma. Report of 2 cases.

Two cases of malignant acanthosis nigricans associated with non-Hodgkin's lymphoma are presented. A 61-year-old negro male with a diffuse large cell non-Hodgkin's lymphoma presented extensive acanthosis nigricans, pachydermatoglyphy and florid cutaneous papillomatosis. No viral particles were observed by electron microscopic studies of the wart-like lesions. Acanthosis nigricans disappeared under chemotherapy. The second case was a 21-year-old caucasian male with non T non B diffuse large cell non-Hodgkin's lymphoma. Very few cases of acanthosis nigricans associated with non-Hodgkin's lymphoma have been reported in the literature, since to our knowledge these two are only the fourth and fifth. Pachydermatoglyphy and florid cutaneous papillomatosis are markers of malignant acanthosis nigricans.

[Atypical urticaria disclosing acute basophilic leukemia. Apropos of a case associated with hyperhistaminemia]. / Urticaire atypique révélatrice d'une leucémie aiguë à basophiles. A propos d'un cas associé à une hyperhistaminémie.

Acute basophilic leukaemia (ABL) is a rare disease characterized by high fever, anaemia and haemorrhagic diathesis. Its prognosis is somber and its response to therapy mediocre. Death is mainly due to cerebral or digestive hemorrhage and coronary disease. In a very small number of cases ABL, like all diseases accompanied by an increase in basophils, may be associated with hyperhistaminemia responsible for cutaneous and gastric symptoms.

Cutaneous manifestations in chronic lymphocytic leukemia.

Cutaneous lesions arising during the course of chronic lymphocytic leukemia (CLL), generally of the B-cell type, are frequent. Three types of cutaneous manifestations must be differentiated: (1) specific lesions, (2) cutaneous manifestations closely related to the disease but without a leukemoid infiltrate and (3) associations with various dermatologic conditions. The various clinical features are discussed.

[Benign adnexal tumors of late occurrence in verrucoid-sebaceous nevus (Jadassohn). Apropos of 7 cases]. / Tumeurs annexielles bénignes de survenue tardive sur naevus verruco-sébacé (Jadassohn). A propos de 7 cas.

Occurrence of basal cell epithelioma and syringocystadenoma papilliferum on sebaceous nevi is well known. But many other adnexal tumors, such as pilar or sweat gland tumours may also be associated with this dysembryoplasia. Out of 99 cases of verruco-sebaceous nevi we find 12 associated basal cell epitheliomas, 7 syringocystadenomas papilliferum and 6 benign adnexal tumors. Our report is about these 7 cases. All of these tumors appeared in adult-hood and were clinically suggesting the diagnostic of basal cell epithelioma. But, after histopathological examination was performed, they revealed to be 2 nodular hidradenomas, 1 chondroid syringoma, 1 trichilemmoma, 1 apocrine cystadenoma, 1 follicular poroma. Similar data are given by Mehregan and Pinkus in 1965 and Wilson Jones and Heyl in 1970, respectively out of 150 and 140 cases of verruco-sebaceous nevi.

[Wells' syndrome or eosinophilic cellulitis. Apropos of 2 cases. Review of the literature]. / Le syndrome de Wells ou cellulite à éosinophiles. A propos de 2 cas. Revue de la littérature.

In 1971, four cases of a new dermatosis were described by Wells, under the name of recurrent granulomatous dermatitis with eosinophilia. In 1978, eight additional cases were reported by Wells and Smith and three authors suggested a shorter title: eosinophilic cellulitis for this syndrome. Since then, four additional cases were published in the literature. We report here two additional cases. From these eighteen upto now published cases, there is no doubt that this dermatosis, as initially described by Wells, is a distinct entity. Clinical course is characterized by sudden eruption of large infiltrated, itchy and/or painful plaques. Blisters are often associated. During the two or three weeks following the initial rash, the inflammatory aspect disappears. Lesions become indurated, and may resemble morphea. Spontaneous resolution occurs after about six weeks. Recurrences are constantly observed. Histologic features are a striking eosinophilic infiltrate associated with eosinophilic deposits constituting flame figures. Blood eosinophilia is present in most cases. Etiology of this entity remains unknown.

[Histiocytosis X with partial antehypophyseal deficiencies. 2 cases]. / Histiocytose X avec déficits antéhypophysaires partiels. Deux observations.

Diabetes insipidus is the most common endocrine disturbance associated with chronic multifocal form of histiocytosis X. Involvement of the hypothalamus can lead to growth hormone deficiency and short stature in children. Two cases with hypogonadism, growth hormone deficiency and diabetes insipidus are reported. In a 22-year-old man plasma testosterone was low and increased after administration of human chorionic gonadotrophin. Cutaneous and lytic bone lesions disappeared after treatment with Vinblastin. Partial diabetes insipidus was treated with clofibrate. A 59-year-old woman was found to have low pituitary gonadotrophins that failed to rise after administration of gonadotropin-releasing hormone and hyperprolactinemia. Fatal outcome was associated with polyuria, bone, cutaneous, gut and hypothalamic infiltration by histiocytes. There was no direct involvement of the anterior pituitary gland.