Balneophotochemotherapy with 8-methoxypsoralen in lichen sclerosis et atrophicus

A 66-year-old woman with longstanding lichen sclerosus et atrophicus improved strikingly with PUVA bath photochemotherapy over a period of 6 weeks. The cumulative UVA dose was 31.7 J/cm2; the single UVA dose ranged from 0.3 to 2.3 J/cm2. After 16 treatment sessions, the sclerotic lesions had softened greatly, while after 24 treatments, the skin lesions were almost completely cleared and pruritus was diminished. Histopathological analysis of biopsy specimens from previously affected sites as well as 20 MHz ultrasound examinations showed almost no residual sclerosis. Although long-term results are not yet available, PUVA bath photochemotherapy seems to be a promising and effective new treatment modality without systemic side effects for patients with disseminated lichen sclerosus et atrophicus.     

Infantile perianal pyramidal protrusion

BACKGROUND: The number of reported cases of infantile perianal eruption has been increasing. However, infantile pyramidal protrusion located in the midline anterior to the anus has apparently been reported mainly as acrochordons or skinfolds. OBSERVATIONS: Fifteen infants (14 girls and 1 boy) had a pyramidal protrusion located only in the midline anterior to the anus. Histological examination revealed acanthosis in the epidermis, marked edema in the upper dermis, and mild infiltrates in the dermis. The patients had been brought to the hospital because of swelling of the protrusion. However, all protrusions showed reduction without any treatment. CONCLUSION: Infantile pyramidal protrusion located only in the midline anterior to the anus should be distinguished from acrochordons or skinfolds based on the characteristic clinical features.     

Unilateral linear lichen sclerosus et atrophicus

We report on a 23-year-old woman with unilateral lichen sclerosus et atrophicus (LSA) who exhibited extensive eruptions on the right extremities and right aspect of the abdomen. The lesions on the right extremities appeared to exhibit a linear pattern along Blaschko's lines, while the right abdominal skin lesion was a whitish, parchment-like plaque. Histological findings for both lesions showed characteristic features of LSA. LSA should be differentiated from the linear type and the macular type of localized scleroderma.     

Lichen sclerosus et atrophicus in an eleven year old girl. Report of a case

An elevent year old girl who presented with constipation of two years' duration was found to have atrophy and depigmentation of the anogenital area. Results of a skin biopsy confirmed the diagnosis of lichen sclerosus et atrophicus, which is a rare disease in children. Treatment of the disease is non-specific, and it usually disappears in half of the patients around the time of menarche. Long-term follow-up of persistent anogenital lesions is necessary.     

Rare case of lichen sclerosus et atrophicus associated with morphea

A rare case of lichen sclerosus et atrophicus coexisting with morphea in the foot was presented. Orthotic therapy was required to alleviate an antalgic gait.     

Atypical lymphoid infiltrates arising in cutaneous lesions of connective tissue disease

Atypical lymphoid infiltrates occurring in the setting of connective-tissue disease (CTD) comprise malignant neoplasms of B-cell or T-cell phenotypes and various reactive lymphoid hyperplasias, such as myoepithelial sialadenitis, lymphocytic thyroiditis, and lymphocytic interstitial pneumonitis. We describe 17 patients with atypical lymphoid infiltrates arising in cutaneous lesions of CTD, the spectrum of which included lupus erythematosus, dermatomyositis, relapsing polychondritis, and lichen sclerosus et atrophicus. There were two principal categories, pseudolymphoma and malignant lymphoma, the former representing 15 of the 17 cases. The clinical and histologic features and possible pathogenetic mechanisms are discussed.     

No influence of echinocytic shape transformation of erythrocytes on phagocytosis by autologous monocytes in vitro

Lichen sclerosus is a chronic skin condition with a predilection for the genital area. In the present study, 35 male patients with lichen sclerosus were interviewed and examined. Blood screens were performed and histology was requested if not already performed. The findings indicate that lichen sclerosus in males exists as a spectrum of disease, ranging from a mild form with white plaques and few symptoms to a severe form with inflammation, atrophy and scarring with possible urological consequences. In many areas it differs from the condition in females; the association with autoimmune disease is weaker and there is less perianal and extragenital involvement. The association with malignancy in males is of lesser significance than initially believed.     

Effects of incubation temperature on the energetics of embryonic development and hatchling morphology in the Brisbane river turtle Emydura signata

AIMS: To investigate the malignant potential of lichen sclerosus, a study using the cell proliferation marker Ki67 comparing lichen sclerosus with and without associated squamous cell carcinoma was performed. METHODS AND RESULTS: Formalin-fixed, paraffin-embedded slides of 13 cases of lichen sclerosus with associated carcinoma, and 31 cases without associated carcinoma, including 16 random cases, seven with epidermal thickening and eight with epidermal thinning, were examined by the immunoperoxidase technique for Ki67, a cell proliferation marker. Ki67 reactivity was mostly seen in the basal and parabasal cells in both groups of lichen sclerosus and this pattern was similar to normal skin, squamous cell hyperplasia and analogous to that of one form of squamous cell carcinoma. There was a mean of 50 Ki67 positive cells per 100 basal cells in lichen sclerosus with associated squamous cell carcinoma; however, in squamous cell hyperplasia adjacent to carcinoma this rose to 90 Ki67 positive cells per 100 basal cells. In lichen sclerosus without associated carcinoma, the random cases had a count of 53 per 100 basal cells, those with epidermal thickening 53 and those with thinning 42. Non-genital normal skin had a count of 71 per 100 basal cells. CONCLUSION: The lack of qualitative differences of Ki67 expression in normal skin, in lichen sclerosus with and without carcinoma, in squamous cell hyperplasia and in one form of squamous cell carcinoma indicates that these conditions share a common localized pattern of cell proliferation and does not support or deny the malignant potential of lichen sclerosus. The higher Ki67 count in squamous cell hyperplasia adjacent to carcinoma could indicate premalignancy or a reaction to the carcinoma. In patients without carcinoma, the higher Ki67 count in thickened lichen sclerosus compared to thinned suggests that some or all of the cases of thickened lichen sclerosus were lichen sclerosus with squamous cell hyperplasia or that lichen simplex chronicus superimposed on lichen sclerosus has a higher Ki67 expression or that the distinction between squamous cell hyperplasia and lichen simplex chronicus is only one of terminology.     

Histopathological and immunological studies in a cohort of balanitis xerotica obliterans

Balanitis xerotica obliterans consisting of 52 cases had been studied in the present series. This disease entity is an insidious sclerosing disease of unknown aetiology, affecting mainly the skin and mucous membrane of glans, prepuce and sometimes the fossa navicularis urethrae or even terminal urethra. Atrophic white patches on external genitalia and obstructive uropathy are two common presenting features. It has been classified as local form of lichen sclerosus et atrophicus. Poor local hygiene, circumcision and association of auto-immune diseases like vitiligo are responsible as suggested in this study. Routine tests detected associated cystitis in 8 cases, haematuria in 4 cases, non-reactive VDRL in all cases and impaired glucose tolerance in 5 cases. Immunoglobulin profile (39 cases) showed altered pattern, suggesting some chronic antigenic stimulation. Out of 33 cases where biopsy done, histologically proved typical balanitis xerotica obliterans was found in 19 cases. Malignant change was noted in one case only. Circumcision and dilatation offered temporary relief.     

Lichen sclerosus et atrophicus in a band-like arrangement

We report a case of lichen sclerosus et atrophicus (LSA) in a band-like arrangement. The patient had a white atrophic plaque on the left breast which showed the typical histological features of LSA. One month later, the patient had developed multiple, brown-colored plaques from the right breast to the right upper arm following the distribution of the peripheral nerve. These plaques showed the mild histological changes indicative of LSA. This band-like arrangement of LSA appears to be unique.     

Testicular teratoma with a unique mode of spread and spontaneous maturation

A 52-year-old woman who developed a pale sclerotic second left toe with loss of the nail plate is described. Biopsy showed changes of lichen sclerosus. There were no other skin or genital lesions present.     

Lyme disease in children and adolescents

148 children and adolescents with Lyme borreliosis and tick bite or suspection on tick bite were examined. The examined patients were aged from 14 months to 24 years and divided into four age groups. Skin lesions were discovered in 25 percent of patients with tick bite. Erythema migrans occurred in 91 percent, Lymphocytoma in 3 percent and sclerodermatous lesions (Lichen sclerosus et atrophicus and Morphea) in 6 percent of patients with Lyme disease. Serologic tests on the presence of antibodies to Borreliae burgdorferi were performed in 96 percent of cases with tick bite. Antibody titer 1:80 or higher in 8 percent of patients with tick bite, was discovered. We found positive serologic test results in 5 (29 percent) of 29 persons with Erythema migrans, in 4 (4 percent) of 110 patients with tick bite (without skin lesions), as well as, in 1 patient with Lymphocytoma. Antibiotic therapy was applied in all cases with Erythema migrans, in person with Lymphocytoma, as well as, in patients with asymptomatic infections (patients without skin lesion recalling a tick bite and with antibodies against Borrelia burgdorferi). A general sensitivity, to infection with Borrelia burgdorferi, is stressed, a fact based on appearance by Lyme borreliosis in all age groups even in the newborn children.     

Topical clobetasol propionate compared with placebo in the treatment of unretractable foreskin

OBJECTIVE: To assess whether it is the steroid alone or the gentle physical retraction combined with ointment that is responsible for the excellent results observed with topical steroid treatment of unretractable foreskin. DESIGN: Prospective, randomised, double-blind study. SETTING: University hospital, Sweden. SUBJECTS: 30 Boys randomised to be treated with clobetasol propionate (n = 15) or placebo (n = 15). INTERVENTIONS: The boys were examined 1, 2 and 6 months, respectively, after treatment. MAIN OUTCOME MEASURES: Comparison between the effects of clobetasol propionate and placebo. RESULTS: Two patients in the steroid group and one in the placebo group withdrew from the study. 10 Boys in the steroid group showed an improvement within 2 months. The remaining 3 boys had no effect and were circumcised. Histopathological examination showed lichen sclerosus et atrophicus. Seven boys in the placebo group improved. The 7 non-responders were prescribed clobetasol propionate ointment, and all 7 improved. CONCLUSION: 17 of 27 boys referred with "phimosis" were successfully treated with an ointment and gentle traction. When clobetasol propionate was given the non-responders success rate was increased to 24/27 (89%).     

Evidence of androgen receptor expression in lichen sclerosus: an immunohistochemical study

OBJECTIVE: While topical androgen administration is widely used in the treatment of lichen sclerosus of the vulva, localization and level of expression of androgen receptor (AR) have not been described previously. METHODS: Thirty-nine paraffin-embedded punch biopsies of patients with lichen sclerosus of the vulva were examined. Androgen receptor, estrogen receptor (ER), and progesterone receptor (PR) expression in lichen sclerosus and in normal vulvar skin were investigated by immunohistochemistry. RESULTS: Five tissue specimens (12.8%) of lichen sclerosus showed nuclear staining with anti-AR in the parabasal cell layers of the epidermis. Median age of patients with positive nuclear staining for AR versus women without AR expression was 71 (range, 63-78) and 66.5 (range, 38-91) years, respectively. Estrogen receptor expression was present in only one patient. Nuclear staining reaction for PR expression was absent in all cases. Four of the five AR-positive women reported no complaints and therefore received no topical testosterone therapy. CONCLUSION: Our results suggest a lack of complaints in AR-positive lichen sclerosus patients. Our findings could justify a larger study comparing symptoms of patients with and without AR expression.     

Interphase ribosomal RNA cistron silver staining in refractory anaemias with and without excess blasts

Of the 74 patients of vulvar lichen sclerosus diagnosed over a period of 29 years from 1966-1995, two cases of mixed dystrophies characterised by lichen sclerosus with squamous cell hyperplasia were studied and one of these on close follow-up, developed an invasive squamous cell carcinoma after three years. The other case has been doing well and has not shown further progression of the lesion. The remaining 72 patients of vulvar lichen sclerosus alone have shown no signification change on close follow-up for a minimum of five years. This paper highlights the fact that all cases of vulvar dystrophy especially, lichen sclerosus must be regularly followed up for development of squamous cell hyperplasia and their progression to overt vulvar carcinoma, emphasizing the need for early diagnosis and treatment of non-neoplastic vulvar dystrophies.     

Manual medicine in pain treatment

BACKGROUND: The aim of this study was the clinical evaluation of an original device produced to evaluate edema in a semi-quantitative way, the ACI Medical, Edema Tester (ET). ET1, is a soft plastic plate characterised by two parallel conic pyramidal protrusions. One side of the protrusion is rounded while the other side has an angular edge. The two protrusions are placed on the plate in inverse decreasing height. ET2 is characterised by two lines of 7 holes placed in the plate. The ET is applied at the internal perimalleolar region with the conic pyramidal protrusions in contact with the skin. A standard sphygmomanometer cuff is applied over the area and pressure is maintained at 50 mmHg for a period between 1 an 3 minutes. The cuff is then removed. ET1: skin marks are usually just visible in normal subjects without edema and disappear in a few minutes. When edema is moderate some half of each protrusion is visible as a skin mark. In limbs with severe edema the whole length of the protrusion is clearly visible. ET2: skin marks are usually just visible in normal limbs without edema and disappear in a few minutes. In limbs with edema the number of holes visible on the skin is increased and in severe edema all holes are visible. METHODS: To evaluate semi-quantitatively the level of edema the length of the two skin marks can be measured and for the ET2 the numbers of visible holes can be counted, as they are generally proportional to the degree of edema. The ET testers were evaluated in 22 normal subjects, 19 limbs with varicose veins, 22 patients with CVI and lipodermatosclerosis, 5 patients with initial primary lymphedema and in 8 subjects with severe, chronic, lymphedema and skin alterations. RESULTS: The results showed a significant difference between normal limbs an patients. CONCLUSIONS: In conclusion, the method of evaluating edema with ET can be used to supplement the clinical and noninvasive evaluation. In general practice the presence of edema measurable with ET indicate the need for treatment. The several degrees of skin marks visibility and disappearance time may be used as a general guideline to indicate the need for different types and length of treatment.     

Anitis, vulvar edema and macrocheilitis disclosing Crohn disease in a child: value of metronidazole

BACKGROUND: We report a case of oro-genital and perianal Crohn's disease which progressed for 4 years with no digestive involvement in a 12-year-old girl. CASE REPORT: At the age of 8 years, a young girl developed recurrent anal fissures. Voluminous vulvar edema developed at 12 years with fissurar macrocheilitis. There were no digestive signs and the diagnosis of Crohn's disease was obtained on the basis of granulomatous epithelioid infiltration of biopsy specimens (Bauhin valve, anus, vulva). Metronidazole given per os at the dose of 25 mg/kg/day for 6 months led to partial significant symptomatic remission. DISCUSSION: Unilateral or bilateral vulvar edema is highly suggestive of Crohn's disease even if the classical digestive inflammatory signs are absent. Demonstration of perianal lesions (erythema, pseudocondylomatous formations) must not mislead the diagnosis (sexual abuse). Simultaneous granulomatous lesions in the genital and labial regions is exceptional in Crohn's disease. Ileocolonoscopy is indicated in such cases and alone can demonstrate latent digestive inflammatory processes. Different agents have been proposed for treatment of local Crohn's disease skin lesions. It is difficult to evaluate their efficacy due to the spontaneous variability of disease expression.     

Familial pyoderma gangrenosum presenting in infancy

Pyoderma gangrenosum (PG) is a rare, poorly understood skin disease that occurs in all age groups. Less than 0.4% of patients are infants and represent a diagnostic challenge as early lesions may resemble other skin disorders. Here we report for the first time three siblings affected with PG all presenting during infancy. Unlike the older age group, the ulcers spared the legs but involved the buttocks, thighs and perianal area in all the infants. Conclusion: This is the first reported family with PG affecting three siblings suggesting autosomal recessive inheritance. The diagnosis may be more difficult in infants due to absence of underlying associated disorders and the tendency of the lesions to appear in areas where infants frequently have other dermatoses. PG characteristically involves the buttocks, thighs and perianal area and spares the legs.     

Psychological distress in women with nonneoplastic epithelial disorders of the vulva

The aim of this study was to evaluate psychological distress in 44 women with vulvar squamous cell hyperplasia and 21 with vulvar lichen sclerosus in order to examine the presence of psychological factors in these dermatologic disorders. Two psychometric tests were used to evaluate depressive status and various aspects of anger. No significant depressive status was diagnosed with the former test either in patients with vulvar squamous cell hyperplasia or in patients with vulvar lichen sclerosus. Patients with squamous cell hyperplasia had two components of anger (state and internal anger) that were significantly higher and three components (trait anger, exteriorization and control of anger) significantly lower than did the controls. In patients with lichen sclerosus all the components of anger were within the normal range. These findings suggest that psychological factors may be associated with vulvar conditions, such as squamous cell hyperplasia, and may have some therapeutic implications in cases resistant to standard treatment.     

Tuberculosis of the skin

Tuberculosis of the skin is now becoming one of the rare diseases. However, we can still see it in the patients with immunodeficiency or treated with immunosuppressants for a long time. Diagnosis of this disease is made by the characteristic clinical findings and the results of laboratory findings. This disease is divided into two types: true cutaneous tuberculosis and tuberculid. The former is produced by the proliferation of the tuberculous bacilli in the skin. Lupus vulgaris, tuberculosis verrucosa cutis, cutaneous primary tuberculosis, tuberculosis cutis orificialis and cutaneous miliary tuberculosis are included. The latter type is the skin manifestation of allergic reaction against tuberculous bacilli in the tuberculous lesions of other organs. Culture of tuberculous bacilli from the skin lesions shows usually negative. Erythema induratum Bazin, papulonecrotic tuberculid, penis tuberculid and lichen scrofulosorum are included.