A Case of Linear Type of Lichen Sclerosus et Atrophicus?

We report a case of the linear type of lichen sclerosus et atrophicus (LSA). The patient had linear atrophic lesions from the left upper back to the left hand. Histological findings obtained from the upper back were those of typical LSA. Histology of the forearm showed mild changes suggestive of LSA. There were no histological features suggesting any association with localized scleroderma in either specimen. The relation between LSA and localized scleroderma is unclear at present. This case suggests that there is a linear form in LSA as already recognized in localized scleroderma.     

Morphea of the Eyelids

Morphea, or localized scleroderma, of the eyelids is an uncommon disease. Morphea usually involves the thorax, trunk, lower and upper extremities, face, and genitalia. In the present report a patient with a biopsy-proven morphea of both upper eyelids is described. The salient histopathologic features included thinning of the epidermis with thickening and sclerosis of the collagen fibers in both the papillary and reticular dermis. There was a marked decrease in the fibrocytes. The eccrine sweat glands were entrapped by sclerotic collagen fibers. The pilosebaceous units were markedly decreased in number. There was a moderate lymphocytic infiltration in the dermis and a prominent lymphocytic perivasculitis. The clinical and histopathologic features of morphea are compared with those of lichen sclerosus et atrophicus.     

Immune globulin intravenous replacement after plasma exchange

The infusion of high-dose (275 mg/kg body weight) immune globulin intravenous (IGIV) after 466 plasma exchanges in 64 patients with autoimmune disease was studied. Side effects occurred during 15% of IGIV infusions. For the most part they were transient and mild, and could be controlled by slowing the infusion rate. Two percent of infusions had to be terminated because of more persistent or severe side effects. Chills were the most common side effect, followed by nausea, flushing, anxiety, and nausea. Serum IgG levels were immediately restored into the normal range by IGIV infusions, and they were much more effective in restoring IgG levels after plasma exchange than intramuscular injection of 9.9 g of immune serum globulin (ISG). Up to 15 weekly high-dose IGIV infusions were well tolerated without unusual side effects. These patients did not have any major bacterial infections, but were not protected from developing Herpes zoster at the dosages used. In patients with myasthenia gravis, a short term impact of a single IGIV infusion on titers of antibody to acetylcholine receptor could not be demonstrated. This study showed IGIV to be a safe and effective preparation for the replacement of normal IgG removed during plasma exchange.     

Incidental Epidermolytic Hyperkeratosis in Progressive Systemic Sclerosis

Incidental epidermolytic hyperkeratosis (EH) has been reported in a variety of lesions. We describe here incidental EH in a patient with progressive systemic sclerosis (PSS). We reviewed 108 skin biopsies from our cases of PSS and localized scleroderma, but this finding was seen only in the present case. Focal acantholytic dyskeratosis is analogous to EH, but we believe that this case is most consonant with EH. Since there has been no other report of EH in PSS, this association may be coincidental.     

Effect of severe gastroesophageal reflux on sleep stage in patients with aperistaltic esophagus

Polysomnography and esophageal pH studies were conducted in 13 patients with an aperistaltic esophagus; seven of these had scleroderma and six were patients treated for achalasia. The percentage total time of pH <4.0 when recumbent exceeded 30% for both groups. There was a total of 51 reflux events for both groups. There were 22 reflux events recorded for both groups that were less than 5 min in length and 29 events greater than 5 min. In 26 of 32 (81%) instances, patients either began awake and went to sleep during a reflux event or did not awake during a reflux event. Only six of 32 (19%) reflux events caused sleep disruption. We conclude that even the severe reflux demonstrated in this subset of patients does not always disrupt sleep. Patients may have severe prolonged reflux and not arouse.This paper was presented in part at the AGA meeting, San Diego, California, 1995.     

Corneal and Other Ocular Changes in Progressive Systemic Scleroderma

The ocular and systemic complications are reported of two patients having progressive systemic scleroderma. Although ocular complications and particularly those affecting the cornea are said to be uncommon one patient had corneal marginal furrowing with neovascularisation and the other a bilateral diffuse opacification of the anterior corneal stroma. The ocular and systemic complications of scleroderma are reviewed.     

Odontostomatologic involvement in juvenile localised scleroderma of the face

Aim: Localised scleroderma of the face (LSF) can lead to significant aesthetical and functional abnormalities. Despite their quite frequent clinical observation, the odontostomatologic complications are not thoroughly described. Aim of the study was to describe the clinical features of the most frequent odontostomatologic abnormalities of LSF and to propose clinical and radiologic criteria for the assessment and follow‐up of these complications. Methods: We performed a cross‐sectional, multicenter study involving a multidisciplinary team formed by paediatric rheumatologists, orthodontists and radiologists. Patients with a diagnosis of LSF underwent a comprehensive rheumatologic evaluation, dental examination, conventional radiology (orthopantomography, teleradiography) and cone beam computed tomography. Results: 16 patients, nine F, seven M, aged 6.5–21.9 years, were investigated. The mean disease duration was 7.7 years, 62.5% had extracutaneous complications. All patients reported at least one odontostomatologic complication. The main alterations were: malocclusion (94%), overgrowth tendency of the anterior lower third of the face (82%), gnatologic alterations (69%), dental anomalies (63%), skeletal asymmetry (56%), bone involvement (50%) and temporomandibular joint involvement (19%). Conclusions: We found a high incidence of odontostomatologic abnormalities in LSF. Cone beam computed tomography represents a new feasible technique to evaluate and monitor soft and hard tissue changes in LSF. The observed findings highlight the importance of a multidisciplinary and standardised management of this challenging and rare condition.     

Juxtafoveolar Telangiectasis Associated with Crest Syndrome

Purpose: To report a case of CREST syndrome associated with juxtafoveolar telangiectasias (JT). Design: Case report. Methods: Observational case report. Results: A 64-year-old woman affected with CREST syndrome developed bilateral visual loss. Capillary dilatation and permeability changes in the outer retina were noticed during an angiographic study. Optical coherence tomography (OCT) showed thickening with loss of the foveal depression and inner lamellar cyst. The patient was diagnosed as stage 3, group 2A JT associated with CREST syndrome. Conclusions: Finding JT in association with CREST syndrome suggests a common pathophysiologic process.     

Scleroderma in South Australia: further epidemiological observations supporting a stochastic explanation

The aim of this study was to determine the incidence, prevalence, survival and selective demographic characteristics of scleroderma occurring in South Australia over the 10-year period 1993-2002. Analysis of the database of the South Australian Scleroderma Register: a population-based register established in 1993. Patients with scleroderma resident in South Australia (n = 353 at 2002) were ascertained from multiple sources and clinical and demographic data were obtained from mailed questionnaire and from review of computerized hospital databases, case notes or referring letters. Time-space cluster analysis was carried out according to the Knox method. Control data were obtained from the Australian Bureau of Statistics census. The mean prevalence was 21.4 per 10(5) (95% confidence interval 20.2-22.6) and the mean cumulative incidence of 1.5 per 10(5) (95% confidence interval 1.32-1.73) with no significant change in incidence over the study period (P = 0.13). Cumulative survival improved over the study period, with patients with diffuse disease having significantly reduced survival (as compared with limited disease, P < 0.001). The proportion with diffuse disease ( approximately 22%) remained steady. There was a small but significant predisposition in patients with a continental European birthplace (P < 0.001). A family history of scleroderma was noted in 1.6% with lambda1 (familial risk) of 14.3 (95% confidence interval 5.9-34.5). However, a family history of systemic autoimmunity (especially rheumatoid arthritis) was more common (6%). No socioeconomic stratification, temporal clustering nor spatio-temporal clustering was observed either at time of initial symptom or at 10 years before disease onset. Scleroderma occurs relatively infrequently in South Australia with no significant change in incidence observed over the 10-year study period. However, cumulative survival has improved. Identified risk factors include family history of scleroderma (risk approximately 14-fold), female sex (risk approximately 5-fold) and European birthplace (risk approximately 2.5-fold); however, the majority of the disease variance appears unexplained. A stochastic explanation based on genetic instability is favoured to explain this paradox.     

Connective tissue panniculitis: lupus panniculitis,dermatomyositis, morphea/scleroderma

Panniculitis is an uncommon cutaneous manifestation of connective tissue diseases. Our discussion will include panniculitis occurring in the setting of lupus erythematosus, dermatomyositis, and scleroderma/morphea. These subtypes of panniculitis are unified by an active inflammatory stage of the disease that can progress to develop scarring, atrophy, and calcifications. Treatment is most effective if initiated during the active phase of the disease and often requires systemic therapy because of the location of the inflammation. Antimalarials are the initial treatment of choice for most cases of lupus erythematosus panniculitis, whereas corticosteroids in combination with other steroid‐sparing immunosuppressive agents are the first‐line treatment for panniculitis in patients with dermatomyositis. The appropriate treatment for panniculitis in the setting of morphea/scleroderma varies based on clinical severity.