Juvenile ecthyma gangrenosum caused by Pseudomonas aeruginosa revealing an underlying neutropenia: case report and review of the literature.

BACKGROUND: Ecthyma gangrenosum (EG) is characterized by the occurrence of erythematous, violaceous or haemorrhagic macules and/or vesicles, often evolving into necrotic ulcers, with a central grey-black eschar. It is a rare skin condition, usually occurring in immunocompromised patients suffering from bacterial sepsis caused by Pseudomonas aeruginosa. However, seemingly healthy children have been diagnosed with this skin disease as well. OBJECTIVES: We report the workup of a case of vulvar EG caused by P. aeruginosa in a toddler, which led to a diagnosis of an underlying neutropenia. Moreover, we provide a brief literature review on those cases of EG where an underlying primary immunodeficiency, neutropenia in particular, was eventually diagnosed. METHODS: A one-and-a-half-year-old girl presented with a history of recurrent (respiratory) infections and the sporadic occurrence of purpuric, vulvar ulcers. Workup consisted of microbiological and haematological investigations, including repeated blood analyses. RESULTS: Bacterial swabs from the vulvar ulcers showed the growth of P. aeruginosa. No concomitant sepsis was present, but laboratory investigations pointed towards a cyclic neutropenia, coinciding with the occurrence of the EG lesions. Topical gentamicin ointment allowed the skin lesions to heal faster. Following the administration of granulocyte colony-stimulating factor (G-CSF), the girl experienced less infections in general and had no recurrence of EG lesions in particular. Treatment with G-CSF could eventually be stopped, and the neutropenia, ultimately transient in nature, completely resolved. CONCLUSION: Children presenting with (anogenital) EG should always alert a physician to consider a potentially underlying immunodeficiency, neutropenia in particular.

Middle ear capillary haemangioma causing vestibulocochlear symptoms: a case report.

PROBLEM: A 58-year-old man presented with transient vertigo and pulsatile tinnitus. METHODS: High-resolution computed tomography, magnetic resonance imaging, excision, and subsequent immunohistochemical assays were performed. RESULTS: Imaging showed a soft tissue mass in the epitympanum and mastoid with bone erosion of the tegmen tympani and a dural tail sign, suggesting meningioma. Subsequently, because of signs of clinical progression, a canal-wall-up attico-antromastoidectomy was performed, with near-complete removal of a granulomatous, ossifying, haemorrhagic mass. CONCLUSIONS: Radiological imaging was critical in determining the extent of the mass and excluding other pathologies. Due to the atypical clinical and radiological signs, the final diagnosis of capillary haemangioma of the middle ear and temporal bone was made only after surgical resection and histopathological examination with immunohistochemistry, which excluded meningioma. The contiguous occurrence of cutaneous capillary haemangioma of the lateral face and neck was an important clue to the diagnosis.

A controlled trial of magnesium dithiosalicylate compared with aspirin in rheumatoid arthritis.

The anti-inflammamtory effectiveness and side-effects of magnesium dithiosalicylate were compared to aspirin in a 3-month, parallel, double-blind trial in 40 patients suffering from active rheumatoid arthritis. The results showed that 3 g magnesium dithiosalicylate daily had anti-inflammatory properties similar to those of 3 g aspirin daily in rheumatoid arthritis. A statistically significant change in morning stiffness, number of tender joints, pain score and erythrocyte sedimentation rate was observed in the magnesium dithiosalicylate group. In the magnesium dithiosalicylate group, 8 patients had to be withdrawn from the trial because of serious side-effects compared to 5 in the aspirin group. Gastro-intestinal intolerance occurred as frequently in both treatment groups. Hypersensitivity to magnesium dithiosalicylate was a serious problem and the reason for withdrawal in 4 cases. The high frequency of side-effects to magnesium dithiosalicylate makes this drug unacceptable for treatment of rheumatoid arthritis at the present time. Further pharmacological studies might reveal new derivatives which are as effective but with less side-effects. The anti-inflammatory activity of magnesium dithiosalicylate resembled that observed with gold and penicillamine. The fact that all these drugs have a sulphhydril group in common is stressed.