Chronic periaortitis (retroperitoneal fibrosis) concurrent with recurrent cutaneous eosinophilic vasculitis.

Chronic periaortitis (CP) is usually accompanied by at least mild manifestations of systemic autoimmunity; however, skin manifestations are rare. Here, we report an 82-year-old woman presenting with a pruritic annular eosinophilic dermatosis that led to the diagnosis of recurrent cutaneous eosinophilic vasculitis (RCEV) coexisting with a latent CP. The present paper is reminder that a CP should be included as a potential differential diagnosis in the elaboration of patients with cutaneous vasculitis that is suspicious of underlying autoimmunity.

Lower urinary tract involvement in patients with newly diagnosed autoimmune bullous dermatoses: an urethrocystoscopic study.

INTRODUCTION: To date, there are no published data assessing lower urinary tract involvement in patients with autoimmune bullous dermatoses (ABD). METHODS: Fourteen of 30 consecutive patients diagnosed with ABD between October 2007 and December 2008 consented to undergo a diagnostic video-recorded urethrocystoscopy: 9 patients (7 women and 2 men) with pemphigus vulgaris, 4 patients (2 women and 2 men) with bullous pemphigoid and 1 female patient with mucous membrane pemphigoid. RESULTS: None of the 14 patients complained of lower urinary tract symptoms. Urethrocystoscopy disclosed characteristic lower urinary tract lesions in almost every patient with ABD (13 of 14 patients; 93%). Two, partly overlapped, pathologic patterns prevailed: (a) nonkeratinizing squamous metaplasia (SM) found in 64% of the patients, including 2 of 4 men; (b) mucosal inflammation of the bladder base/trigone that extended-especially among male patients-to the proximal urethra (64% of the patients). SM prevailed among patients with pemphigus vulgaris, inflammatory lesions among patients with bullous pemphigoid (P = 0.003) and involvement of proximal urethra among male patients (P = 0.004), irrespective of the particular ABD diagnosis. CONCLUSIONS: The authors present, to the best of their knowledge, for the first time in the literature, urethrocystoscopic evidence that inflammatory urothelial lesions of the bladder and proximal urethra confounded with nonkeratinizing SM of the trigone are an almost invariable finding in patients with ABD. Studies focusing on the elucidation of the pathomechanism of bladder lesions in these patients may contribute to the better understanding of both the pathophysiology of the bullous skin diseases and the pathobiology of the bladder urothelium.

Dizygotic twins with congenital malalignment of the great toenails: reappraisal of the pathogenesis.

Congenital malalignment of the great toenails (CMGTN) is a heritable disorder, in which the longitudinal axis of the nail plate is not parallel to the corresponding axis of the distal phalanx of the hallux, but laterally deviated. We describe a pair of 1(1/2)-month-old dizygotic twins with laterally deviated nail plates of the great toenails since birth. By the time the infants were 10 months of age, significant realignment was observed. Adult pedigree members also showed slight similar deviations of the nail plates. We suggest that desynchronization of growth between the nail and the adherent end-phalanx of the hallux may result in temporarily larger nail plates, which are gliding outwards, in order to fit into the underlying bony space. During postnatal life, spontaneous realignment is usually observed, probably as a result of a faster growing end-phalanx.

Role of bacterial superinfections in the pathogenesis of postzosteric neuralgia.

Varicella-zoster virus (VZV) is an alpha-herpes virus that causes varicella (chickenpox), establishes latency in dorsal root ganglia and may reactivate to cause herpes zoster (shingles). Postherpetic neuralgia is the most common debilitating complication of herpes zoster. It is currently supposed that scarring of the dorsal root ganglia and atrophy of the dorsal horn as a result of intense inflammation may play a central role in the pathogenesis of this condition. The exact pathogenesis of the inflammatory reaction leading to persistent ganglion damage is still poorly understood. However, immune suppression is a recognized risk factor for the development of postzosteric neuralgia in zoster patients (increased risk, e.g., in aged patients over 80 years or diabetes mellitus patients). There is some evidence that remote streptococcal and staphylococcal infections may induce immunologic disease mechanisms consequently affecting the central nervous system. Since streptococcal and/or staphylococcal superinfection of skin lesions is common in herpes zoster, we present a hypothesis of immunopathogenesis of postzosteric neuralgia, i.e., as the result of augmentation of local ganglion inflammation due to bacteria-driven clonal expansion of VZV-specific T-cell subsets in the affected skin. Based on the aforementioned hypothesis it is interesting: (1) to study the impact of concomitant systemic antibiotic treatment to the standard antiviral regimen on the rate and severity of both bacterial superinfection of zoster skin lesions and postzosteric neuralgia and (2) to quantify the VZV-specific T-cell response as a function of the degree of bacterial superinfection of zoster skin lesions. Challenging of the present hypothesis should provide an effective means of preventing postherpetic neuralgia by preventing and consequently treating the bacterial superinfection of zoster skin lesions.