Parry-Romberg syndrome: a case with a possible association with Lyme disease.

Parry-Romberg syndrome is an acquired slowly progressive disease characterized by an atrophy mostly involving half of the face. The pathogenesis of this disfiguring condition is still controversial. The relationship between Parry-Romberg syndrome and Lyme disease needs to be considered in depth. A 16-year-old woman from Albania presented with linear depressions of the right side of the face, clinically compatible with Parry-Romberg syndrome. She had a positive history of Lyme disease. Borrelia infection was confirmed by the positivity of PCR and the presence of IgM antibodies. The patient received intravenous penicillin and metronidazole for 14 days. After treatment and during a 2-year follow-up, the clinical disease progression was halted and the serological and microbiological tests for Borrelia burgdorferi sensu lato were negative. We cannot exclude a coincidence, however, of the bacteriological and serological evidence. Moreover, the interruption of the disease progression after the antibiotic therapy is difficult to ignore without claiming that this association is at least suggestive.

Lack of IgG antibody seropositivity to Borrelia burgdorferi in patients with Parry-Romberg syndrome and linear morphea en coup de sabre in Mexico.

BACKGROUND: Progressive hemifacial atrophy or Parry-Romberg Syndrome (PRS) is a rare, acquired, progressive dysplasia of subcutaneous tissue and bone characterized by unilateral facial involvement. Its etiology is unknown, but theories about its pathogenesis include infectious, degenerative, autoimmune, and traumatic causes among others. The causal relationship of PRS and linear morphea en coup de sabre (LMCS) with Borrelia burgdorferi infection remains controversial. Our goal was to serologically determine anti-B. burgdorferi antibodies in patients diagnosed with PRS and LMCS to establish a possible association as a causative agent. METHODS: We conducted a serology study with patients belonging to a group of 21 individuals diagnosed with PRS, six with LMCS, and 21 matched controls. Anti-Borrelia IgG antibodies were determined by ELISA. A descriptive statistical analysis and Fischer's exact test were done. RESULTS: In serological tests, only two cases had borderline values and were further analyzed by Western blot with non-confirmatory results. For both the PRS and LMCS group, the association test was not significant, suggesting a lack of association between PRS or LMCS and the presence of anti-Borrelia antibodies. CONCLUSION: In Mexico there are no previous studies on Borrelia infection and its relationship between PRS or LMCS. Our result showed a lack of association of either clinical entities with anti-Borrelia-antibodies. Former reports of this association may suggest coincidental findings without causal relationship.

Parry-Romberg syndrome: a possible association with borreliosis.

Progressive facial hemiatrophy or Parry-Romberg syndrome is a rare entity characterized by unilateral atrophy of the skin, subcutaneous tissue and the underlying bony structures. This syndrome has many features of linear scleroderma 'en coup de sabre' but is distinguished by more extensive involvement of the lower face with only slight cutaneous sclerosis. The onset typically occurs in childhood or young adult years. The aetiology of the condition is unknown. A case of progressive hemifacial atrophy occurring in a 30-year-old woman is reported, in which the aetiology is thought to be Lyme disease, a borrelial infection first recognized in 1975 that has since become the most commonly reported vector-borne disease in the world. No sure link is established between these two disease states, but their coincident occurrence in this patient is noted. We hypothesize that the aetiology of Parry-Romberg syndrome involves borreliosis.

Leoprosy- clinical aspectos of nerve involvement.

Leprosy is the cause of the commonest peripheral neuropathy. The predilection of Mycobacterium leprae for nerve tissue accounts for the clinical features that are most dreaded and most characteristic of the disease. Were it not for the progressive destruction of peripheral nerve trunks and the consequences of this, leprosy would largely remain a cutaneous condition of cosmetically unsightly hypopigmented or erythematous areas and aggregations of nodular thickenings. The neurologic damage in leprosy is confined to postganglionic changes. The central nervous system is protected, although rarely in experimental situations organisms have been reported in cerebral tissues.