A clinical study on severe hydroa vacciniforme.
OBJECTIVE
To describe a severe type of hydroa vacciniforme (HV) in order to provide basis for treatment and prevention of this disease.


METHODS
Nine cases of HV were collected in our outpatient department, and clinical and laboratory examinations were performed.


RESULTS
All the cases had erythemas, papules, bullae, erosions or ulcers on exposed sites, with recurrences, gradually resulting in deformation. Five patients suffered from hand deformation manifesting rigidity, flexor tetanus, or malposition of the first, second and third interphalangeal joints of hands. Two of the above mentioned patients manifested partial bone absorption of fingers. Five patients showed partial defect of auricle. Two patients showed saddle nose, and partial absorption of nasal cartilage. One patient displayed cicatricial contracture of lower lip, with incisor extrusion. Four patients showed opacity of cornea. Through laboratory examinations, porphyrinopathies were excluded. Phototests showed increased sensitivity to ultraviolet-A radiation (UVA).


CONCLUSIONS
Based on the clinical features and laboratory examinations, these patients were diagnosed as having severe HV. It is believed that this condition belongs to a disease spectrum induced by ultraviolet light. Our analysis suggests that for children with severe type of HV, preventive measures and therapeutic agents should be taken as soon as possible in order to avoid deterioration of the disease and malformations.
Gu
H
H
Institute of Dermatology, Chinese Academy of Medical Sciences, Peking Union Medical College, Nanjing.
Chang
B
B
Qian
H
H
Li
G
G
D000293:Adolescent; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D004365:Drugs, Chinese Herbal / Q000627:therapeutic use; D005260:Female; D006801:Humans; D006837:Hydroa Vacciniforme / Q000188:drug therapy* / Q000517:prevention & control*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D011241:Prednisone / Q000627:therapeutic use; D013792:Thalidomide / Q000627:therapeutic use; D029757:Tripterygium
D000893:Anti-Inflammatory Agents; D004365:Drugs, Chinese Herbal; D007166:Immunosuppressive Agents; D013792:Thalidomide; D011241:Prednisone