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BACKGROUND: The gene deletion responsible for the type I human complement C2 deficiency was reported in 1992. The purpose of our study is to evaluate clinical and immunological characteristics of 11 patients with lupus erythematosus and type I C2 deficiency. OBSERVATIONS: We observed 5 patients with a homozygous C2 deficiency and 6 with a heterozygous C2 deficiency. Eight patients had systemic lupus erythematosus, 2 had subacute cutaneous lupus erythematosus, and 1 had chronic lupus erythematosus. Photosensitivity was present in 73% of the patients, and 64% tested positive for anti-Ro (SSA) antibodies. Renal involvement that required immunosuppressive therapy was present in 54% of the patients. Ninety percent of the patients tested positive for antinuclear antibodies, and 54% tested positive for anti-double-stranded DNA antibodies. Phenotyping of the fourth component of the complement was performed in 82% of the patients and showed a C4A4B2 phenotype, which is suggestive for the type I C2 deficiency. CONCLUSIONS: Most patients with lupus erythematosus associated with C2 type I deficiency are photosensitive, and this is probably related to the presence of anti-Ro (SSA) autoantibodies. The prognosis for those patients is not better than that for patients with lupus erythematosus in general.

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OBJECTIVE: The aim of this study was to analyze the temporal evolution of melanoma incidence in the department of the Bas-Rhin, France, and to study the evolution of tumor thickness. DESIGN: Retrospective study including all histologically proven melanomas recorded at the cancer registry of the department of the Bas-Rhin between January 1980 and December 1992 and at the Cutaneous Histopathology Department of the University Hospitals, Strasbourg, between January 1980 and December 1997. SETTING: Population-based cancer registry and academic cutaneous histopathology department. PATIENTS: A total of 1254 patients with histologically proven melanomas. INTERVENTION: None. MAIN OUTCOME MEASURES: Temporal evolution of melanoma incidence and tumor thickness. RESULTS: The mean (SD) and median tumor thicknesses were 1.48 (1.59) mm and 0.87 mm, respectively, and they decreased during the study period. The increase in the number of melanomas was mainly related to an increase of superficial spreading melanomas in both sexes. The number of intermediate melanomas (1-2 mm) in both sexes and the number of melanomas with a Breslow index between 2 and 4 mm in women increased only slightly. The number of melanomas with a Breslow index greater than 2 mm in men and greater than 4 mm in women remained stable during the reference period. CONCLUSION: A striking increase in incidence of thin melanomas contrasts with a stable incidence of thick melanomas.

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Congenital and/or nevoid skin disorders following the lines of Blaschko may have a delayed onset after birth. They have to be differentiated from acquired dermatoses exhibiting the same linear pattern. In common dermatoses, such as psoriasis or lichen planus, lesions in a blaschkolinear distribution most often occur together with scattered lesions, but occasionally they may be isolated. Less common self-limited dermatoses such as lichen striatus and adult blaschkitis always present in a blaschkolinear fashion. In these diseases, or some other conditions occasionally distributed along these lines (chronic graft versus host reaction, fixed drug eruption, lupus erythematosus, atopic dermatitis, etc.), the cause of the disease may lead to the unmasking of tolerance to an abnormal keratinocyte clone that remained hidden in these lines. In addition to epithelial cells, other cells may be involved in the occurrence of acquired blaschkolinear dermatoses. In linear atrophoderma and linear fibromatosis, the histogenesis seems to involve hypothetic dermal clones. The extension of an acquired dermatosis on a preexisting linear nevoid disorder is an argument in favor of an early embryonic somatic mutation of a skin cell line.

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The arylpropionic acid derivatives (APADs) ketoprofen and tiaprofenic acid can provoke photoallergic dermatitis. Possible cross-reactivity between APADs is of importance in patients using nonsteroidal anti-inflammatory drugs. Because of the similarities in chemical structures, we investigated patients with photoallergy to ketoprofen or tiaprofenic acid, in order to study cross-reactivity between APADs and a possible pattern of cross-reactivity between benzophenone-containing molecules, so as to determine the molecular basis of photoallergy to ketoprofen or tiaprofenic acid. 10 patients with photoallergy to topical ketoprofen, 2 with photoallergy to oral tiaprofenic acid, and 15 control subjects with no history of contact dermatitis from APADs, nor from benzophenone-containing molecules, were photopatch tested in triplicate with ketoprofen, tiaprofenic acid, other APADs (alminoprofen, fenoprofen, flurbiprofen, ibuprofen and naproxen), benzophenone-containing molecules (fenofibrate, oxybenzone, sulisobenzone), and unsubstituted benzophenone. 1 set was irradiated with UVA light, 1 with solar-simulated irradiation and 1 dark control. Tests were read at 2, 3 and 4 days. Patients reacted to both ketoprofen and tiaprofenic acid (12/12), fenofibrate (8/12), oxybenzone (3/12) and unsubstituted benzophenone (11/12), but not to other APADs, nor to sulisobenzone. Tests remained negative in control patients. Photoallergy is due to the benzophenone moiety of ketoprofen, or to the very similar thiophene-phenylketone of tiaprofenic acid, but not to their arylpropionic function. This induces cross-reactivity to fenofibrate and oxybenzone but not to APADs without a benzophenone moiety, which may therefore probably be used in such patients. Unsubstituted benzophenone should be added to standard phototesting series.

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In French Guayana, climatic and geographic conditions enhance development of numerous fungal, parasitic and microbial skin diseases. The most particular infectious diseases of this French department are lobomycosis, chromomycosis (Phialophora pedrosoi), creeping disease, cutaneous leishmaniasis, tungiasis, mycobacterial ulcus (Mycobacterium ulcerans) and papular dermatitis caused by nettling hairs of lepidoptera (Hylesia urticans). The occurence of these skin diseases is demonstrative of pathological ecology of the Amazonian country.

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