[Plastic reconstructive correction of persistent orofacial swelling and swelling of the eyelids in Melkersson-Rosenthal syndrome]. / Plastisch rekonstruktive Korrektur eines persistierenden Lidödems bei Melkersson-Rosenthal-Syndrom.

BACKGROUND: The Melkersson-Rosenthal syndrome complex is characterised by asymmetric orofacial oedema, facial palsy, furrowed tongue (lingua plicata) and additional neurological symptoms. PATIENT: A 21-year old man presented with a bilateral asymmetric oedema of the eyelids which increased during the follow-up period of 12 years. Intermittent swelling of the auditory channel occurred and resulted in hearing loss. Furthermore, the patient complained unspecific neurological symptoms as headache, tinnitus and subjective visual defects. The lid oedema did not only result in cosmetic problems but seriously restricted visual fields. RESULTS: Our patient showed the typical symptoms of the Melkersson-Rosenthal complex, lacking however facial palsy which is often caused by mechanical compression along the course of the facial nerve. The differential diagnosis of recurrent swelling of the lids was discussed on an interdisciplinary plane. The inflammatory activity could be reduced and the frequency of swelling episodes could be diminished by a treatment with hydroxychloroquine. The initially relapsing, then persistent swelling had led to severe restriction of the visual field and resulted in a severe social stigmatisation and psychic stress for the patient. The surgical resection of the granulomatous tissue was carried out under steroid treatment to prevent recurrence. Histochemical analysis showed inflammation of the lymphoid plasmacellular type with mucipolysaccharidoid deposition typical of Melkersson-Rosenthal syndrome. Blepharoplasty resulted in an improved cosmetic appearance. The patient remained symptom-free with an excellent functional result. CONCLUSIONS: After persistence and constancy of the findings of a granulomatous blepharitis, surgical correction is a good opportunity to minimise the subjective complaints and neurological symptoms.

[An unusual case of pyoderma gangrenosum with necrotizing granulomatous dermatitis]. / Ein ungewöhnlicher Fall eines Pyoderma gangraenosum mit nekrotisierender granulomatöser Dermatitis.

Four distinctive clinical and histological variants of pyoderma gangrenosum have been proposed: (1) ulcerative, (2) pustular, (3) bullous, and (4) vegetative. The rate of progression and associated systemic diseases, as well as the histopathologic changes, can vary considerably between these clinical variants. A 64-year-old man presented with ulcerative pyoderma gangrenosum associated with renal cell carcinoma and IgA paraproteinemia; he responded to oral thalidomide. Histopathology revealed unusual findings with signs of leukocytoclastic vasculitis, basophilic degeneration of collagen and zones of suppuration with a palisaded histiocytic and epithelioid granulomatous infiltration throughout the dermis.