[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.

[Erosive lichen of the scalp]. / Lichen érosif du cuir chevelu.

BACKGROUND: We report an exceptional clinical presentation of erosive lichen planus of the scalp associated with hepatitis C and idiopathic CD4 lymphocytopenia. CASE REPORT: A 90-year-old woman was hospitalized in May 1995 for erosive dermatosis of the scalp and alopecia, associated with ungual lesions. Histology of the scalp lesions demonstrated lichen and the serology tests were positive for hepatitis C. The patient also had severe CD4 lymphocytopenia (290/mm3). The lesions regressed with clobetasol and occlusion and growth of scalp hair turned. DISCUSSION: Erosive lichen planus of the scalp has, to our knowledge, not been reported previously. The causal effect of drugs taken by this patient (tetrazepam and clorazepam) was not retained. Seropositivity for hepatitis C (with no evidence of active disease) was however probably not fortuitous. The association with CD4 lymphocytopenia merits discussion.

[BCG-induced mycobacterium infection induced by alternative medicine]. / Bécégite du visage après mésothérapie.

INTRODUCTION: Mesotherapy is an alternative medical process defined by the intradermal injection of small amounts of pharmacological substances. It has been associated with the development of mycobacterial infections. CASE REPORT: A 80-year-old woman presented with a Mycobacterium bovis infection of the face following mesotherapy. Genome analysis of the mycobacterial strain isolated from a skin lesion using pulse-field gel electrophoresis demonstrated the presence of a vaccinal strain identical to the one employed by the same physician to vaccinate a child two hours before. DISCUSSION: Given the severity of mycobacterial infections following mesotherapy and given the lack of evidence about its efficacy, mesotherapy should not be performed on the face. Standardisation of aseptic measures in the daily medical practice could prevent such infectious complications.

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.

[Value of the light-test histological study in photodermatoses]. / Intérêt de l'étude histologique du phototest dans les photodermatoses.

Clinic and histologic analysis of 92 phototests of various photodermatosis (14 benign summer light eruption (BSLE), 47 polymorphous light eruption (PMLE) 20 persistent light reactor photodermatitis (PLRP), 11 drug induced photodermatitis (DIP) has been performed. Clinically, a phototoxic aspect was observed in 30 cases, it was photoallergic in 36 cases and uncertain in 26 cases. Histologically, a phototoxic picture was present in 32 cases, a photoallergic one in 48 cases, and 12 cases exhibited unclassified aspect. Only one case of BSLE was associated with photoallergic aspect, clinically and histologically. Photoallergic type was observed in 70 p. 100 of iterative exposure phototest (IEP) of PMLE; clinical and histological aspects were concordant; in contrast phototoxic aspects were constantly observed when single exposure phototest (SEP) were used. In PLRP histological aspect of IEP was constantly of photoallergic type; whereas clinical aspect was uncertain in 50 p. 100 of the cases; 7 of the 10 biopsies of SEP exhibited a photoallergic picture. In DIP histological aspect of IEP was constantly photoallergic, whereas 5 of the 6 cases exhibited uncertain clinical aspect; only one of the 5 SEP gave a photoallergic reaction both clinically and histologically. Comparison of histological features revealed a more typical photoallergic picture in PLRP than in PMLE. We conclude that: the reproducibility of clinical and histological lesions is better with the iterative exposure phototest than with single exposure phototest; histologic examination is more reliable than clinical findings in persistent light reactor photodermatitis and drug induced photodermatitis.

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.