[Ofuji's papulo-erythroderma: treatment with cyclosporin A].
BACKGROUND
Papuloerythroderma of Ofuji is an uncommon condition characterized by diffuse pruriginous eruptions of infiltrating papulae. The large folds are not involved. The eruptions are associated with lymphopenia and eosinophilia. Very few cases have been reported in the literature and because of their heterogeneous nature, there is some debate as to whether this is a single clinical entity or a particular presentation of erythrodermia; Immunomodulation is recommended.


METHODS
A 73 year-old man of asian origin living in France presented chronic pruriginous erythrodermia with flat purplish-brown confluent papulae which did not involve the large folds. Eosophilia and lymphopenia were present. The biopsy specimen evidenced dermal infiltration with CD4+CD45+RO+ T cells, eosinophils and DC1a+ dendritic cells. Further explorations did not reveal any sign favoring lymphoma, carcinoma or underlying infection. Dermocorticoids, PUVA-therapy and interferon-alpha were ineffective. Considerable clinical improvement was obtained with cyclosporine A.


CONCLUSIONS
We used ciclosporine A in our patient after repeated failure of other therapies reported to be effective in this dermatosis. Despite the favorable outcome, this therapeutic approach should be used with prudence.
Jullien
D
D
Clinique Dermatologique, Hôpital E. Herriot/Hospices Civils de Lyon. Julien@laennec.univ-lyonl.fr
Kanitakis
J
J
Souillet
A L
AL
Mathon
N
N
Faure
M
M
Claudy
A
A
D000368:Aged; D015496:CD4-Positive T-Lymphocytes / Q000473:pathology; D016572:Cyclosporine / Q000627:therapeutic use*; D003713:Dendritic Cells / Q000473:pathology; D003873:Dermatitis, Exfoliative / Q000188:drug therapy* / Q000473:pathology; D003879:Dermatologic Agents / Q000627:therapeutic use*; D004802:Eosinophilia / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D016898:Interferon-alpha / Q000627:therapeutic use; D007801:Langerhans Cells / Q000473:pathology; D008231:Lymphopenia / Q000473:pathology; D008297:Male; D011701:PUVA Therapy; D011536:Prurigo / Q000473:pathology; D012872:Skin Diseases, Vesiculobullous / Q000188:drug therapy* / Q000473:pathology; D013601:T-Lymphocytes / Q000473:pathology; D016896:Treatment Outcome
D003879:Dermatologic Agents; D007166:Immunosuppressive Agents; D016898:Interferon-alpha; D016572:Cyclosporine