Incidence of cutaneous tuberculosis in patients with organ tuberculosis

BACKGROUND: Tuberculosis continues to be a health problem in some countries. The development of resistance to antituberculitic drugs and the increase in diseases and conditions associated with immunodeficiency such as AIDS and chemotherapy have caused tuberculosis to increase recently. As a result, the incidence of cutaneous tuberculosis has been increasing as well. AIM: To detect cutaneous tuberculosis in patients with organ tuberculosis and to establish some characteristics of the relation between organ and cutaneous TB. MATERIAL AND METHODS: A total of 370 patients (145 females and 225 males), aged 2-76 years (mean age 27.5), enrolled for this screening study. These patients were hospitalized patients who already had pulmonary or extrapulmonary tuberculosis diagnosed before admission. All patients underwent a general skin examination, and, if needed, cutaneous biopsies were taken from involved skin areas. RESULTS: Three hundred and forty-seven (93.78%) out of 370 patients had pulmonary tuberculosis only or in association with one of other organ tuberculoses. Twenty-three patients had extrapulmonary TB: nine were TB adenitis, six were TB peritonitis, three were bone tuberculosis, and five were TB meningitides. Of 370 patients, only 13 (3.51%) had cutaneous TB: seven scrofuloderma (SCD; 2.16%), four lupus vulgaris (LV; 1.35%), one LV and SCD, and one Bacille Calmette-Guerin (BCG) adenitis (0.027%). Cutaneous tuberculosis was observed in seven out of 260 patients with parenchymal tuberculosis (2.96%). Four out of nine patients with TB adenitis (44.4%), one out of 12 pulmopleuretic (8.3%), and one out of 67 pleuresic patients (1.40%) had cutaneous TB as well. Mean age of the 13 patients was 32.46 years: mean age of SCD and LV was 24.8 and 48 years, respectively. The one patient with BCG adenitis was 7 months old. Five (62.5%) out of eight patients with SCD, and only one (20%) out of five patients with LV were new cases. Four patients with SCD had a positive family history, while LV patients did not. CONCLUSIONS: Organ tuberculosis is rarely associated with cutaneous tuberculosis. Scrofuloderma and LV are the most frequent forms of skin TB associated with organ TB in this population. Tuberculosis adenitis is the organ TB that causes cutaneous TB most often among other organ tuberculoses. More than one form of cutaneous TB affected only one patient with pulmonary TB; therefore, it is very rare. Tuberculids were not observed in any of the patients.     

近25年结节性红斑患者中结核感染特点的Meta分析

目的：明确1993-2018年结节性红斑(EN)患者中结核(TB)感染特点。方法：2位研究者独立检索1993年1月至2018年9月PubMed、Embase、MEDLINE、CNKI、CBM、万方数据库筛选出符合纳入标准的文献，采用STATA 12.0软件通过Meta分析方法进行单个率的合并分析。结果：共纳入27篇文献，包括国内11篇（1019例患者，其中结核性结节性红斑患者277例，感染率25.4%），国外16篇（995例，其中结核性结节性红斑患者52例，感染率6%）。国内亚组分析显示东部、中部及西部地区EN患者中结核感染率分别为16.0%、27.9%和32.5%，Meta回归显示地区因素而非年份因素可解释异质性来源(P=0.02)。结节性红斑患者结核病灶为肺结核、淋巴结核、其他类型结核（如：结核性胸膜炎、卵巢结核等）、仅PPD强阳性，分别占44.4%、11.1%、7.1%及38.2%。结论：大多数EN患者没有明显的结核症状，因此应尽量寻找潜在的结核病感染病灶。     

AIDS in Africans living in London.

OBJECTIVES--To investigate the presentation of HIV infection and AIDS amongst Africans diagnosed with AIDS living in London. METHODS--Identification of all AIDS cases of African origin attending four HIV specialist centres in South London--Guy's, King's, St George's and St Thomas' Hospitals--up to March 1994, by retrospective review of case notes of all HIV positive patients. RESULTS--Of 86 patients (53 women, 33 men) studied, 59 (69%) were from Uganda. The most frequent AIDS-defining diagnoses were: Pneumocystis carinii pneumonia (PCP) 21%, tuberculosis (TB) 20% (extrapulmonary TB 14%, pulmonary TB 6%), cerebral toxoplasmosis 14%, oesophageal candida 13%, cryptococcal meningitis 11%, wasting 6%, herpes simplex infection > 1 month 5%, Kaposi's sarcoma 5%, other 6%. Cytomegalovirus retinitis was diagnosed in one case. Late presentation was common; 70% were diagnosed HIV positive when admitted to hospital. The diagnosis of AIDS was coincident with a first positive HIV test result in 61%. The mean CD4 counts at both HIV and AIDS diagnoses were similar in both men and women: 87 x 10(6)/l and 74 x 10(6)/l in men and 99 x 10(6)/l and 93 x 10(6)/l in women respectively. Overall, TB 21 (24%) (extrapulmonary TB 12, pulmonary TB 9) was either the AIDS-defining diagnosis or was detected within three months of this event. Sixty-two per cent of TB cases were diagnosed within twelve months of entry to the UK compared to 34% of all other AIDS cases. The prevalence of STD was very low; genital herpes was the commonest STD: 17% of the women, 9% men; 28% of the men and 11% of the women tested had a positive TPHA test. In cases known to be HIV-positive prior to an AIDS diagnosis, 41% took prophylaxis for PCP and 45% had taken zidovudine (ZDV). Forty two of the study participants had 89 children: 59 of these children had mothers in the study. Overall, 37 (42%) of the children had lost at least one parent at the time of data assessment. CONCLUSIONS--PCP and TB were the most common initial AIDS-defining diagnoses. The majority of TB cases were diagnosed within 12 months of entry to the UK. An AIDS-defining diagnosis was the first manifestation of HIV-related illness in the majority of patients. Because of late presentation to medical services, access to treatments for HIV infection and prophylaxis against opportunistic infections was limited. Extending the role of clinics and staff into the community might facilitate both earlier presentation and access to services. Future provision of local services will need to be sensitive to the requirements of individuals from different cultures and backgrounds.     

Erythema nodosum in hemorrhagic rectocolitis and Crohn's disease

Erythema nodosum (EN) has been reported in patients with ulcerative colitis (UC) since 1909. The reported incidence varies from 0.9 p. 100 to 18.9 p. 100. A wide range of incidence of EN has also been found in Crohn's disease (CD), with figures varying from 0.7 p. 100 to 8 p. 100 (fig. 1). Different criteria for the diagnosis of EN, UC and CD probably account for the scattering of values. A review of 324 cases of inflammatory bowel diseases revealed 21 cases with one or more episodes of EN: 6 out of 195 cases of UC (3.1 p. 100) and 15 out of 129 cases of CD (11.6 p. 100). The incidence was higher in females (5.7 p. 100 in UC, 17.5 p. 100 in CD) than in males (0 in UC, 6.9 p. 100 in CD). At the time of the eruption, patients with CD were younger (mean 24.3 years) then those with UC (mean 37.5 years). The interval between the onset of the intestinal symptoms and the nodular eruption was shorter in patients with CD (2.6 years) than in those with UC (8.2 years). The inflammatory bowel disease (whether UC or CD) started earlier in patients with EN than in EN-free patients. EN antedated the onset of intestinal manifestations in one patient with UC. In the remaining 20 patients the intestinal symptoms came first. EN was often recurrent, and the 21 patients suffered 32 episodes at varying intervals. One patient had 4 and another 3 episodes; six patients had 2 and 13 had one single episode. Three patients with UC had 2 episodes and 5 patients with CD had 2 or more episodes. The morphology and distribution of the lesions was fairly classical, except in 2 patients who had only 1 and 2 nodules respectively on one leg. The eruption subsided in all cases within 2 to 5 weeks, and no atrophy, suppuration or ulceration was observed. Most episodes of EN occurred during active phases of the intestinal disease. This was not so, however, in 2 cases in which the eruption followed an acute streptococcal throat infection. EN was more often found in total UC than in the less extensive distal and rectal types (Table I). It was also more frequent in the ileocolic form than in the exclusively ileal and colonic forms (Table II).(ABSTRACT TRUNCATED AT 400 WORDS)     

Treatment of Miescher''s cheilitis granulomatosa in Melkersson–Rosenthal syndrome

Cheilitis granulomatosa is the most frequent dermatological sign in the Melkersson-Rosenthal syndrome. We reviewed 27 cases of this syndrome diagnosed and treated in the last 20 years. All the patients were surgically treated and received intralesional corticosteroids during surgery and tetracycline hydrochloride (500 mg, twice daily) after the operation to prevent recurrence.     

Characteristic cutaneous lesions in patients with brucellosis

Among 436 cases of brucellosis included in a 12-year prospective protocol, we identified 27 patients (6%) with cutaneous lesions (13 men and 14 women; mean age, 35.1 +/- 16.5 years). Twenty-one patients had positive blood cultures for Brucella melitensis. A disseminated violet-erythematous, papulonodular eruption (20 cases) and erythema nodosum-like lesions (three cases) were the most frequent eruptions observed, appearing during the initial episode of the disease or in relapse. Histologic findings were a dermal inflammatory infiltrate of lymphocytes and histiocytes in a perivascular and periadnexal arrangement, with a focally granulomatous appearance, and occasional extension to subcutaneous fat. Skin cultures were positive for B melitensis in two of four cases. Our results suggest that there are characteristic clinical and histologic cutaneous findings in patients with brucellosis and that hematogenous spread of the microorganism can be the most important pathogenic mechanism of these lesions.     

Erythema nodosum

Erythema nodosum (EN) has been known since 1798,¹ when it was considered to be a clinical entity. In the latter half of the 19th century, “rheumatism” was regarded as the principal cause and tuberculosis as a minor causative factor of EN. During the first four decades of the present century, tuberculosis was reported to be the cause of EN in over 90% of adult patients, but in the 1940s and 50s, sarcoidosis, streptococcal, and other infections, and some other causes, were suggested.² During the last 15–20 years, a variety of factors have been found to trigger EN; however, despite thorough investigations, no evident cause for EN can be found in 10–30% of cases (Fig. 1).     

An aetiological analysis of erythema nodosum in a community hospital

Erythema nodosum (EN) is a reaction pattern in the skin characterized by septal panniculitis. Infectious diseases are most common cause of EN. This study was aimed to find out the most common causes for EN as seen in a community hospital over a period of two years. Fifteen patients with EN were subjected to detailed clinical and laboratory investigations to establish the etiology. Immuno - fluorescence and PCR for Mycobacterium tuberculosis were performed in relevant patients. Seven cases were diagnosed to be secondary to tuberculosis that was confirmed by a complete resolution of the lesions after a full course of anti TB chemotherapy. In one patient, the EN was drug induced, two were due to polyarteritis nodosa, one was due to SLE, one was due to streptococcal infection, and three were idiopathic in aetiology. Though the number of patients is small, it still establishes the fact that tuberculosis continues to be the most common cause of EN.     

The relationship between erythema nodosum and prognosis in systemic sarcoidosis: a retrospective cohort study

BackgroundErythema Nodosum (EN) is the most common skin manifestation in sarcoidosis and has often been associated with a good prognosis.ObjectivesTo compare the clinical characteristics and treatment-related features in patients with sarcoidosis according to whether or not EN was seen as a presenting symptom at the time of diagnosis.MethodsA 20-year single-center retrospective study was performed. The following two groups were identified: one group with EN as one of the presenting symptoms at the time of diagnosis of sarcoidosis (EN group) and a second group without EN as a presenting symptom at diagnosis (non-EN group). The clinical characteristics and treatment modalities were collected from the medical records.ResultsA total of 122 patients (31 in the EN group, 91 in the non-EN group) were included. Radiological stages of pulmonary disease were significantly lower in the EN group. Articular involvement was more common in the EN group (p = 0.001), whereas other systemic organ involvements (p = 0.025), especially neurological involvement (p = 0.036), were significantly more common in the non-EN group. In the EN group, a higher percentage of patients were managed without systemic therapy (71.0% vs. 54.9%) and spontaneous remission was more frequent (25.0% vs. 14.1%), however, this wasn’t statistically significant.Study limitationsRetrospective design.ConclusionsThe lower radiological stage of pulmonary sarcoidosis and lower frequency of systemic organ involvement in patients with EN augment the prognostic value of EN highlighted in the literature. However, this study couldn’t confirm that the patients with EN would need less systemic therapy in the course of their disease.     

A study on the prognostic value of clinical and surgical features of dermatofibrosarcoma protuberans in Korean patients

Background Dermatofibrosarcoma protuberans (DFSP) is a rare spindle cell tumour with locally aggressive characteristics. Only few studies on the epidemiology of DFSP in Asians have been reported. Objectives The purpose of the study was to evaluate the epidemiological and clinical characteristics of DFSP in Korean patients and determine the prognostic factors that affect disease‐free survival. Methods We conducted a retrospective review of patients diagnosed with primary or recurrent DFSP between 2000 and 2009 at three Seoul National University Hospitals. Patient, tumour and treatment factors were analysed for local recurrence‐free survival. Results We analysed data for 65 patients, of whom 36 (55.4%) were female. The mean age at disease onset was 34.4 years with 50% aged between 20 and 40 years. Involved sites were most often on the trunk (66.1%). Among the 65 patients, 60 patients underwent wide excision; seven patients (10.8%) experienced local recurrence during 3.6 years of follow‐up. The recurrence‐free survival was significantly related to the microscopic resection margins (P = 0.005), clinical presentation (P = 0.002) and frequency of recurrence (P = 0.014) in the univariate analysis. There were three cases with metastasis, two of whom died because of progression of the disease despite continuous chemotherapy with imatinib mesylate. Conclusions We report the epidemiologic and clinical characteristics of DFSP in Korea. Patients with recurrent disease at presentation and frequent recurrence have a high risk of recurrence and metastasis, and therefore close observation is needed in these patients.     

A clinicoepidemiological study of 50 cases of cutaneous tuberculosis in a tertiary care teaching hospital in pokhara, Nepal

Background:

Cutaneous tuberculosis (TB) is essentially an invasion of the skin by Mycobacterium tuberculosis, the same bacteria that causes pulmonary tuberculosis.

Aim:

This study was conducted to study the common types of cutaneous TB and to find the management pattern in a tertiary teaching hospital in Pokhara, Nepal.

Materials and Methods:

All the cases of cutaneous TB were biopsied and furthermore investigated by performing Mantoux test, sputum examination, fine needle aspiration cytology, chest X-ray and ELISA.

Results:

In this study, we found that tuberculosis verrucous cutis (48%) had a higher incidence than other types of cutaneous TB. More males were affected than were females (1.2:1). Commonly affected sites were the limb and the buttock (48%). The most commonly affected age group was 16–25 years (40%). All cases (except two) were more than 15 mm in size in the Mantoux test. The histopathological picture was typical in all except three cases. All patients were treated with antitubercular treatment as per the national guidelines.

Conclusion:

The most common type of cutaneous TB was tuberculosis verrucous cutis and the most commonly affected sites were the limb and the buttock. As cutaneous TB sometimes reflects the presence of pulmonary tuberculosis, its incidence should not be ignored.     

Cholinergic urticaria. A clinical and histologic study

We studied the natural history, the prevalence of atopy, and the frequency of systemic symptoms during attacks in 35 patients with cholinergic urticaria, the histologic condition of the eruption in seven patients (20%), and the response to intradermal injections of acetylcholine, histamine, and methacholine in 18 patients (51%). In most patients symptoms began between the ages of 10 and 30 years, persisted for many years, and caused them to modify their activities to avoid the provoking factors of exercise, emotion, and heat. The condition usually improved with time, and five patients (14%) had a spontaneous remission. Atopy, present in about 12 (34%) of the patients, was more frequent than in the general population. Systemic symptoms were uncommon during attacks, and patients had no greater responses than controls to the intradermal tests. The histologic study revealed neutrophils in and around the walls of superficial subpapillary dermal vessels.     

CUTANEOUS TUBERCULOSIS IN HONG KONG: A 10-YEAR RETROSPECTIVE STUDY

Background. Cutaneous tuberculosis was once a relatively common skin disease in Hong Kong. Tuberculosis verrucosa cutis was the commonest type. Because the last survey was carried out 25 years ago, it was thought necessary to find out the new incidence and pattern of this important disease in this locality. Methods. A 10-year (1983-1992) retrospective survey on the epidemiologic and clinicobacteriologic aspects of cutaneous tuberculosis had been done in the public sector of Hong Kong. The records of the confirmed cases were retrieved for statistical analysis. Results. A total of 176 cases are included in the final analysis. This represents 0.066% of all new skin cases seen during the 10-year period. Among these, 79.5% are erythema induratum, 6.3% lupus vulgaris, and 4.5% tuberculosis verrucosa cutis. They are further divided into true cutaneous tuberculosis (14.8%, n=26) and the tuberculide (85.2%, n=150). Among the patients with true tuberculosis, 42.3% had lupus vulgaris, 30.8% had tuberculosis verrucosa cutis, and 26.9% had scrofuloderma. Among the tuberculides, erythema induratum accounted for 93.3%. Conclusions. Cutaneous tuberculosis is now uncommon in Hong Kong. Tuberculosis verrucosa cutis is no longer the commonest type in Hong Kong as described in some textbooks. Erythema induratum is now the most common among the total cases and lupus vulgaris is the most common among the true cutaneous forms of tuberculosis.     

Clinicopathologic, immunophenotypic, and molecular characterization of primary cutaneous follicular B-cell lymphoma

OBJECTIVE: To determine the clinicopathologic, immunophenotypic, and molecular characteristics of primary follicular cutaneous B-cell lymphoma (CBCL) as defined by the revised European-American lymphoma classification. DESIGN: A retrospective survey of the medical records, an immunohistochemical study of archival biopsy specimens. and molecular studies of preserved DNA of all patients with follicle center lymphoma-follicular (FCL-F) primary CBCL from 1987 to 1997. SETTING: A single-center outpatient specialty clinic at an academic medical center. PATIENTS: Twenty-one patients (68% of all new primary CBCL cases), including 14 men and 7 women (age range, 33-88 years; mean, 55 years). RESULTS: The head and neck region was the most frequent primary site. Following treatment, recurrences were relatively frequent, but the overall mortality rate during 1.0 to 11.3 years (mean, 6.3 years) of follow-up was 4.8%. Immunohistochemical analysis for B- and T-cell lineages was helpful in enhancing the folliclelike structures. CD10, bcl-2, and CD43 were expressed by the neoplastic cells in 9 (47%) of 19 cases, 4 (21%) of 19 cases, and 2 (13%) of 16 cases, respectively. Immunohistochemical detection of cytoplasmic immunoglobulin light chains, using steaming in EDTA as the antigen-retrieval technique, was successful in 12 (71%) of 17 cases. The Ig heavy-chain gene rearrangements, using the Southern blot technique, detected clonality in 17 (94%) of 18 cases. The bcl-2 gene rearrangements were detected in only 2 (13%) of 15 of the primary cutaneous FCL-F cases, compared with 9 (75%) of 12 of the primary nodal FCL-F cases (P =.002). CONCLUSIONS: Primary cutaneous FCL-F is a relatively common subtype of CBCL, with a relatively indolent course. It has many features in common with primary nodal FCL-F, except for low rates of bcl-2 expression and bcl-2 gene rearrangements.     

Evolution of cutaneous tuberculosis over the past 30 years in a tertiary hospital on the European Mediterranean coast

Background. There have been few studies on cutaneous tuberculosis (TB) in Europe in recent years. Objective. To retrospectively analyse the evolution of the various types of cutaneous TB over the past 30 years in an adult population in Spain. Methods. Patients with cutaneous TB diagnosed between 1981 and 2011 at Bellvitge Hospital, Barcelona, Spain, were included in the study. Chest radiography was performed for all patients, and the presence of TB elsewhere in the body was excluded when clinically suspected. Results. In total, 36 patients (15 male, 21 female, mean age 53.72 years) were diagnosed with cutaneous TB. There were 22 patients with lupus vulgaris (LV), 4 with scrofuloderma, 4 with miliary TB, 3 with tuberculous abscess/ulcer, and 1 each with orificial TB, warty TB, and an iatrogenic inoculation from underlying visceral focus. Of the 36 patients, 16 (38.88%) had TB presenting simultaneously in other organs. Mycobacterial culture from skin biopsies was positive for Mycobacterium tuberculosis complex in 17 of the 32 cases tested (53.12%), whereas stains for acid‐fast bacilli in skin samples were positive in only 3 of 36 patients (8.33%). Conclusions. Although the number of cases of cutaneous TB diagnosed yearly in our population has declined over the past 30 years, cutaneous TB still exists in Europe, and its incidence is expected to increase, owing to the increased immigration into the continent in recent years. The most common type of cutaneous TB in our adult population was LV. It should be noted that despite being considered a benign form of TB, cutaneous TB can be accompanied by TB in internal organs, and severe complications can occur, such as the development of squamous cell carcinoma in long‐lasting lesions.     

Twice weekly 5 mg dexamethasone oral pulse in the treatment of extensive alopecia areata.

Thirty patients (20 males, 10 females) with widespread alopecia areata (25 extensive alopecia areata, 5 alopecia areata) for a mean period of 4.2 years were included in the study. All patients above 12 years were administered 5 mg dexamethasone oral pulse on two consecutive days every week. Three children (< 12 years) received 2.5 mg to 3.5 mg dexamethasone oral biweekly pulse. Patients who had received treatment for a minimum period of 12 weeks were evaluated for terminal hair growth. Complete to excellent (75-95%) hair growth was observed in 16 (63.3%) patients. Growth was good (50-74%) in 2 cases and poor (< 50%) in 3 (10%) cases. Six (20%) patients has no growth of terminal hair. Complete to excellent growth of hair was obtained after a mean period of 5.35 months (range 3-10 months). Relapse occurred in one case each after three and six months but hair regrew with re-treatment. Side effects of corticosteriods were frequent, seen in 8 (26.6%) patients, but were mild. In only one case, treatment had to be discontinued. We propose that twice weekly 5 mg dexamethasone oral pulse for six months may be considered as one of the modalities in the treatment of extensive long standing alopecia areata.     

Pyoderma gangrenosum: a report of 21 cases

BACKGROUND: Pyoderma gangrenosum (PG) is an uncommon, destructive, cutaneous ulceration, belonging to the neutrophilic disease spectrum. It is associated with systemic disease in 50% of cases. METHODS: We report a retrospective study of 21 cases of PG. All cases studied fulfilled the following criteria: (i) clinical features of PG; (ii) histopathology consistent with a diagnosis of PG, and excluding other specific dermatoses. RESULTS: The average age of our patients was 41.8 years. The male to female ratio was 1.1. The typical ulcerative variant was found in 17 patients, bullous PG in two patients, and the granulomatous variant in two patients. Sixty-two per cent of our patients had lesions on their lower legs. Two patients had neutrophilic pulmonary involvement concurrent with the ulcers. An association with other internal diseases was noted in 12 patients. Histopathologic study showed vasculitis in 13 patients. Of these, 11 were leukocytoclastic and the others predominantly lymphocytic. CONCLUSIONS: PG is a rare disease, with the ulcerative variant being most frequent. The lower legs are the most commonly affected sites. The recurrence rate in our study was about 46% regardless of the treatment prescribed. Pulmonary involvement was fatal in two patients.     

Classic Kaposi's sarcoma in Greece: a clinico-epidemiological profile

Background Classic Kaposi's sarcoma (CKS) is not uncommon in Greece with a reported incidence of 0.20 per 100,000 per year. Methods Epidemiological, clinical and histological features of all CKS cases, diagnosed in‘A. Sygros’ hospital, Athens, Greece during the years 1989–1994, have been recorded and studied prospectively. Results During the five-year period studied, 66 CKS patients have been diagnosed in our hospital. Incidence among dermatologic patients was 2.11 per 10,000 patients examined, representing 1.35% of total skin malignancies. Patients' age at diagnosis ranged from 53 to 94 years (mean 72±8.8). The male to female ratio was 2.47:1. A high proportion of the patients were born in Peloponnesos (42.42%) and were residing in Athens (51.51%) or in Peloponnesos (24.24%). Nodules and/or plaques were the most frequent type of lesion, most commonly located on the feet (43.93%) or the hands (28.78%). Accompanying edema was seen in 51.51% of the patients. There were 16 stage I patients (24.24%), 40 stage II (60.60%), 0 stage III and 10 stage IV (15.15%). Involvement of visceral organs was detected in seven patients (10.60%), while 10 had lymph node involvement (15.15%) and three, involvement of the underlying bones (4.54%). Second primary malignancy was diagnosed in 6 cases (9.09%), most often of the reticuloendothelial system (83.33%). Conclusions CKS in Greece exhibits some special characteristics, including older age of onset; lower male to female ratio; endemic clustering; disseminated skin disease at diagnosis, often accompanied by lymphedema; not unusual visceral or lymph node involvement and association with second malignancies. We suggest that CKS in Greece possibly represents a distinct endemic subtype of CKS.