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[Evaluation of the past history of chilblain in cases of systemic lupus erythematosus (SLE) and its similar diseases].

We sent out a questionnaire to 47 patients of SLE and its similar diseases as to their past histories of chilblain. The results of the patients were compared to those of 141 cases of control. Although the percentage of cases who had revealed chilblain frequently (40.0%) and the age of chilblain onset (mean: 10.8 years old) in SLE group were not significantly different from those of control (28.4% and 12.4 years old, respectively), the chilblain which SLE patients developed had some characteristics compared to that of control, (1) higher incidence of chilblain episodes, (2) longer duration until cure, (3) more liability that chilblain leads to erosion or ulceration and (4) frequent occurrence of chilblain in the other seasons than winter. Especially SLE patients with the characteristic of (4) had higher association rates of Raynaud's phenomena, chilblain LE and livedo, suggesting disorder of peripheral circulation. It was also revealed that females are generally more liable to develop chilblain than males (females: 40.0%, males: 13.1%). Those results suggest some important relationship between chilblain and LE lesions. It is supposed that chilblain with the characteristics described above may possibly be transformed into LE lesions.

Adolescent↗

Clinical and histopathologic features and immunologic variables in patients with severe chilblains. A study of the relationship to lupus erythematosus.

We investigated 33 patients affected with chilblain lesions following a persisting course of more than 1 month. We focused on the incidence of an underlying connective tissue disease, mostly lupus erythematosus (LE), and we analyzed features of idiopathic chilblains compared with those of chilblain lesions associated with connective tissue disease. We also carried out a prospective follow-up of patients. Eleven patients included in the study were free of any clinical and/or laboratory abnormality suggestive of connective tissue disease, while 22 of 33 patients showed 1 or several abnormalities raising suspicion for connective tissue disease, and among them 8 had a diagnosis of systemic lupus erythematosus (SLE) established at initial evaluation based on the American College of Rheumatology revised criteria. The comparative analysis of patients with idiopathic chilblains and patients with chilblains associated with LE showed that female sex and persistence of lesions beyond cold seasons were significantly associated with chilblain LE. Histopathologic studies of chilblain lesions did not reveal features typical of LE in any case, but revealed a higher incidence of a deep perisudoral infiltrate in idiopathic chilblains. In patients showing signs of connective tissue disease, positive cutaneous immunofluorescence was correlated with the presence of circulating antinuclear antibodies. Two patients had an ascertained diagnosis of SLE with severe manifestations during prospective follow-up, requiring treatment with oral steroids in both cases. Chilblains following a chronic course may reveal connective tissue disease, and patients affected with chilblains associated with autoimmune abnormalities may develop severe SLE. Accordingly, long-term follow-up of these patients is warranted.

Adolescent↗

A histologic and immunohistochemical study of chilblains.

BACKGROUND: The histopathologic diagnosis of chilblains is controversial and the histologic changes are often considered nonspecific, mainly because they are poorly documented. Although a dermal inflammation in chilblains has been noticed, the infiltrate has not yet been characterized. OBJECTIVE: Our purpose was to analyze microscopic and immunohistochemical findings in a large series of chilblains and to compare the results with those of lupus erythematosus (LE). METHODS: We included 36 cases of clinically typical chilblains of the hands, of which 17 were thoroughly investigated to rule out cryopathy or LE. Ten biopsy specimens of hand lesions from patients with proven LE were included as controls. All slides were analyzed by conventional microscopy and by immunohistochemistry with anti-CD3, anti-CD20, and anti-CD68 antibodies. RESULTS: The most characteristic finding in chilblains (47% of cases) was the association of edema and reticular dermis infiltrate that showed a perieccrine reinforcement. Such a combination of changes was not observed in LE. Epidermal changes in chilblains consisted mainly in necrotic keratinocytes in 52% of cases. The comparison of 17 idiopathic chilblains with LE showed significant differences in spongiosis (58% vs 0% respectively), vacuolation of basal layer (6% vs 60%), edema of the dermis (70% vs 20%), and deep perieccrine inflammation (76% vs 0%). Immunohistochemistry showed that the infiltrate was composed of a majority of T cells associated with macrophages and a few B lymphocytes. The same pattern was observed in both chilblains and LE. CONCLUSION: Our results show that a predominantly T-cell papillary and deep infiltrate with a perieccrine reinforcement, associated with dermal edema and necrotic keratinocytes, are the hallmarks of chilblains of the hands. These changes can help differentiate idiopathic perniosis from LE; immunohistochemistry is of no use in differentiation.

Adolescent↗

Milkers' chilblains.

AIM: To report on the clinical features, causative factors, management and preventive measures for chilblains of the hands occurring in milkers in the Waikato area of New Zealand. METHODS: A review was made of the case notes of all patients with milkers' chilblains seen in dermatological practice in Hamilton over a ten year period ending in 1991 and follow up interviews were made. RESULTS: Eleven milkers were seen with distinctive, at times disabling, seasonally-recurring chilblains of the hands. In most patients the chilblains had developed early in their milking careers. Difficulty was experienced by both the patients and their doctors in recognising the nature of their skin condition. Sunlight aggravation of the lesions was a feature. In most patients treatment of the presenting episode required time off work and in several patients significant scarring resulted. Three patients left dairying because of the chilblains. Those who continued received preventive advice but four patients had further lesions in subsequent years although these were mild. CONCLUSIONS: Milkers' chilblains are uncommon but some cases are misdiagnosed and milder cases may not present for medical attention. Morbidity can be significant. Preventive measures are helpful but their effect may be limited by the difficulty of wearing gloves for milking. The vasodilator nifedipine is suggested as a prophylactic therapy for these patients.

Adolescent↗

Chilblain lupus erythematosus (Hutchinson). A clinical and laboratory study of 17 patients.

Chilblain lupus erythematosus (LE) is a chronic unremitting form of LE ssen predominantly in women. It occurs commonly on the digits, calves and heels. Nasal lesions are rare. Chronic facial discoid LE usually appears before the chilblain form but, in most instances, resolves even though the chilblain lesions persists. Transformation to systemic LE occurs more often in those who develop both forms of cutaneous LE simultaneously and in the erythema multiforme syndrome. The chilblain lesions are the result of microvascular injury secondary to exposure to cold and possibly hyperviscosity from immunological abnormalities, Elevated serum gammaglobulins, positive latex factor and speckled pattern antinuclear factor are common. None of the usual treatments for cutaneous LE is effective for the chilblain lesions, probably because none is directed towards prevention and treatment of the microvascular stasis.

Adolescent↗

Celiac disease presenting with chilblains in an adolescent girl.

Chilblains, or pernio, are cutaneous lesions that may accompany systemic illnesses including states of malnutrition and autoimmune diseases. We report an adolescent girl in whom chilblains were the chief presenting sign of celiac disease. A gluten-free diet led to weight gain and resolution of the chilblains. We speculate that in this patient, weight loss due to celiac disease contributed to the development of chilblains.

Adolescent↗

Chilblain lupus erythematosus (lupus pernio): clinical review of the Mayo Clinic experience and proposal of diagnostic criteria.

Five cases of chilblain lupus erythematosus were retrospectively reviewed regarding their clinical, histopathologic, serologic, and immunofluorescence findings. Ages at onset of chilblain lupus erythematosus varied from 26 to 73 years, with a female-to-male ratio of 3:2. Since other entities can be confused with this disorder, we propose the following diagnostic criteria. The two major criteria are skin lesions in acral locations induced by exposure to cold or a drop in temperature, and evidence of lupus erythematosus in the skin lesions by results of histopathologic examination or direct immunofluorescence study. The three minor criteria are coexistence of systemic lupus erythematosus or other skin lesions of discoid lupus erythematosus, response to anti-lupus erythematosus therapy, and negative results of cryoglobulin and cold agglutinin studies. We conclude that chilblain lupus erythematosus can be diagnosed and treated. Discoid lupus erythematosus lesions respond more quickly to treatment than chilblain lupus erythematosus lesions. Treatment with antimalarial agents, prednisone, pentoxifylline, or dapsone was of benefit to our patients.

Adult↗

[A case of male who was taken systemic lupus erythematosus with chilblain lupus].

A 38-year-old male was admitted to our hospital because of high grade fever, erythema of face and extremities and oral ulcer. The laboratory examination showed leukopenia, high titer (320 dil.) of antinuclear antibody, a positive reaction of anti-Sm antibody. Especially histopathology of hand erythema showed hydrophilic degeneration consist with chilblain lupus. Because his symptoms were consistent with the criteria of American College of Rheumatology (1982), he was diagnosed as systemic lupus erythematosus (SLE). After administration of 60 mg prednisolone daily, the symptoms gradually improved. Any recrudescence of serological abnormalities or clinical symptoms was not observed until now, although chilblain lupus still persisted. Originally SLE is frequent in young female and often attended with renal disease, central nerve disease and serositis. We reported a case of middle-aged male who developed SLE with chilblain lupus which was rare skin lesion of SLE without severe organic lesion and discussed the relation among chilblain lupus, discoid lupus erythematosus (DLE) and SLE.

Adult↗

The chilblain-like eruption as a diagnostic clue to the blast crisis of chronic myelocytic leukemia.

A 70-year-old Japanese man visited our clinic with the chief complaint of chilblain-like eruptions on the toes of both feet. His toes were bluish, erythematous, and swollen. Neither oral administration of vitamin E for 2 weeks nor wearing insulated socks improved the clinical manifestations. Peripheral blood examination revealed the presence of a large number of monocytic atypical cells and myeloblasts, anemia, and thrombocytopenia. In the bone marrow, monocytic cells were elevated, and myelocytic atypical cells were observed. Chromosomal analysis demonstrated Philadelphia chromosome. We diagnosed him as having a blast crisis of chronic myelocytic leukemia (CML). A biopsy specimen of the skin from the chilblain-like eruption showed infiltration of large, atypical, mononuclear cells; most of them were positive for CD68, and some of them were positive for CD14. Therefore, we concluded that the chilblain-like eruptions on his toes were specific skin lesions of a blast crisis in CML.

Aged↗

Chilblain lupus erythematosus is associated with antibodies to SSA/Ro.

Chilblain lupus erythematosus (CL) of Hutchinson is a subtype of lupus erythematosus (LE) characterized by erythematous lesions induced by cold, damp climates. A number of patients affected by CL eventually develop features of systemic lupus erythematosus (SLE). We report here 9 patients with chilblain cutaneous lesions, 6 of them were affected by SLE and 2 by SCLE. The onset of CL preceded the diagnosis of LE, from 1 to 10 years in 3 cases, it was concurrent in one case and was subsequent in the remaining 4 cases. Raynaud's phenomenon and photosensitivity were other prominent clinical features in patients with CL. Nailfold capillaroscopy revealed pathological changes in every patient examined. ANA and anti-SSA/Ro antibodies were detected in all nine patients. Anti-SSB/La were detected in 2 cases, anti-Sm in one case, and anti-Sm and anti-RNP in a one case. Antibodies to dsDNA and complement consumption were found in the six patients with SLE. The fine specificity of anti-SSA/Ro was determined by immunoblotting: anti-60kD and anti-52 kD were detected in three sera, anti-60kD alone in 5 sera, while one serum did not blot. In conclusion, the present study suggests that chilblain LE is associated with SSA/Ro autoantibodies, as is SCLE, hypergammaglobulinemic purpura and neonatal lupus erythematosus.

Adolescent↗

[Clinical observation on 136 cases of chilblains treated by acupuncture combined with massage].

OBJECTIVE: To compare therapeutic effects of acupuncture plus massage and medicine on chilblains. METHODS: Two hundred and sixty-four cases of chilblains were randomly divided into an acupuncture group of 136 cases and a medicine group of 128 cases. The acupuncture group were treated by acupuncture at 9 acupoints such as Yamen (GV 15), Laogong (PC 8), Sanyinjiao (SP 6), etc. plus massage, and the medicine group by dong chuang Plaster. RESULTS: In the acupuncture group, 111 cases were cured and 136 cases were effective, the effective rate being 100.0%; and in the medicine group, 55 cases were cured and 98 cases were effective, the effective rate being 76.6%, with a very significant difference between the two groups in the therapeutic effect (P < 0.01). CONCLUSION: Acupuncture plus massage is superior to dong chuang plaster in therapeutic effect on chilblains.

Acupuncture Points↗

A double-blind study of ultraviolet phototherapy in the prophylaxis of chilblains.

A randomized, double-blind study was carried out to assess the prophylactic value of ultraviolet irradiation in the autumn as a means of preventing the development of chilblains on the toes and fingers during the course of the winter. Placebo irradiation was achieved by means of an optical filter which absorbed all ultraviolet radiation from the lamps but allowed the visible light component to be transmitted, thus giving patients the impression that both limbs were being treated. Patients were reviewed at monthly intervals during the winter. The response between patients was variable; some patients developed chilblains whilst others remained symptom free. However, in no patient did the ultraviolet treated limb differ from the untreated limb. We conclude that the ultraviolet phototherapy is of no value in the prophylaxis of chilblains.

Chilblains↗

Chilblains (perniosis).

Unfamiliarity of physicians with chilblains (perniosis) gives rise to unnecessary hospital admissions with expensive laboratory and radiologic evaluations and, at times, hazardous therapy. Seven cases of chilblains were seen in San Francisco from November 1986 through January 1987. The patients presented with pruritic, at times painful, purple acral patches or plaques on the fingers, toes, and nose after exposure to a cool or a cold, damp environment. Histologic examination in two cases revealed a perivascular lymphocytic infiltrate with endothelial swelling of the subcutaneous fat and of the upper and lower dermal plexus.

Adult↗

Chilblains and Raynaud phenomenon are usually not a sign of hereditary protein C and S deficiencies.

Hereditary protein C and S deficiencies are risk factors for thrombosis. They are associated with purpura fulminans and coumarin-induced skin necrosis. Recently, necrotic livedo of the extremities, severe chilblains and severe frostbite have been observed in protein C or S deficient patients. Our study was designed to evaluate the prevalence of cold-induced acral manifestations in patients with protein C or S deficiency. One-hundred-and-six patients with protein C or S deficiency and controls matched for sex and age were studied by questionnaire. Data included any history of acral manifestation possibly related to cold exposure, i.e. chilblains, Raynaud phenomenon, acrocyanosis and possible associated factors. Assessment of the diagnosis by a dermatologist was recorded. No difference was found in the prevalence of acral manifestations between patients and controls. This study suggests that protein C and S deficiencies are not risk factors for cold-induced acral manifestations.

Adult↗

Chilblain lupus erythematosus: report of 15 cases.

In this retrospective study, the authors describe the clinical, histologic and laboratory features of 15 cases of chilblain or perniotic lupus. In winter, the patients (14 women, 1 man) develop chilblain-like lesions, chiefly in the toes (8 times) and fingers (11 times). Histologic features are identical to those of discoid lupus erythematosus. The damaged skin gives a positive fluorescent band test. Usually, these lesions occur in association with discoid lupus of the face. However, in 8 patients, they were the only cutaneous sign of lupus. This form of lupus can evolve to a systemic form, as was the case with 3 patients.

Adult↗

[Chilblain lupus erythematosus].

Chilblain lupus erythematosus is a chronic form of lupus erythematosus that is seen predominantly in women and first manifests itself during cold weather as painful purplish acral swellings. These symptoms and possible progression to systemic lupus erythematosus are discussed with regard to two cases. Patients with recurrent or persistent "chilblains" should be investigated histologically and serologically as well as by immunofluorescence.

Aged↗

[Chilblains].

Chilblain is a frostbite, it belongs to the vascular acrosyndromes by the mere fact of symmetrical lesions and to the dystrophic group by its organic obstructive and thrombotic microangiopathy, especially of venules. Chilblain is conditional on a dysesthesia for cold, diagnosis is easy if in the know, prognosis as usual good and treatment must be more preventive than curative.

Chilblains↗

[Chilblains].

Chilblain is an erythrocyanotic lesion of hands or feet, occurring after prolonged exposure to moderate to cold humidity. Most common in young and slim women, it can happen at every age, and relapses characteristically in autumn and winter. Clinical diversity is the rule, in frequent association with microvascular dysreactivity (mostly acrocyanosis). Chilblain is benign in most cases.

Chilblains↗