Year: 2017 I Volume: 1 I Issue: 1 I Page: 48-52
Clinicopathological Correlation in Erythroderma
Anshul Maheshwari 1, Uma Shankar Agarwal2, Ram Singh Meena2, Saroj Purohit2, Surendra Kumar Thalore3
2Professor, Department of dermatology, SMS Medical College & Hospital, Jaipur.
3Assistant Professor, Department of dermatology, SMS Medical College & Hospital, Jaipur.
Dr. Uma Shankar Agarwal
397, Shree Gopal Nagar, Gopalpura Byapss, Jaipur
Background: Erythroderma, or generalized exfoliative dermatitis, is a disease characterized by erythema and scaling involving more than 90% of the body's surface. Diagnosing erythroderma is easy but finding its cause is difficult. There is a paucity of Indian studies over the etiology, clinical profile and its histopathological correlation.
Aims and objectives: To assess the demographic profile, clinical features and histopathological correlation in erythroderma patients.
Material and Methods: We registered all patients of erythroderma consecutively from January 2013 to December 2014. After a thorough history and clinical examination, a provisional clinical diagnosis was made. We performed biopsy from two representative sites of patient and it was sent for histopathological examination. The slides were examined by three independent observers without any relevant clinical information. The clinical diagnosis was matched with the blinded microscopical diagnosis.
Results: A total of 66 patients were enrolled in this study. The mean age of the study group was 53.7±16.56 years (Range: 14 to 86 years) with male outnumbering female in a ratio of 3.4:1. Most common cause of erythroderma noted in the study was eczema of various types (53.03%), followed by psoriasis (30.30%), drug induced (12.12%), lymphoma (1.515%), mycosis fungoides (1.515%) and idiopathic (1.515%). Clinico-pathological correlation occured in about 67% (range: 63.6% to 68.2%) of patients (k value 0.495 to 0.572).
Conclusion: Most of the clinical features of erythroderma are overlapping. Specific and diagnostic features of diseases are seen only in a few patients. Clinico-pathological correlation should be done for better diagnosis of patient. Repeated evaluations, close follow-up and multiple skin biopsies are recommended for a better clinical diagnosis and patient care.
Key words: erythroderma, clinicopathological correlation, histopathology
How to cite this article:
Maheshwari A, Agarwal US, Meena RS, Purohit S, Thalore SK. Clinicopathological correlation in erythroderma. Indian Journal of Clinical Dermatology. 2017;1:48-52.
Erythroderma or exfoliative dermatitis is an inflammatory disorder in which erythema and scaling occur in a generalized distribution involving more than 90% of the body surface 1. Because most patients are elderly and skin involvement is widespread, the disease implies an important risk to the life of the patient 2. Hasan and Jansen estimated the annual incidence of erythroderma to be 1 to 2 per 100,000 patients 3. This disorder may represent a variety of cutaneous and systemic diseases, and therefore a thorough workup is essential which include detailed history of triggering factors like drugs, occupation, sunlight exposure, pre-existing dermatoses, infections, malignancies etc. It should be followed by a meticulous clinical examination for specific diagnostic clues to rule out its etiology. Histopathology can help in identifying the cause of erythroderma in up to 50% of cases, particularly by multiple skin biopsies 4.
Indian studies showed a higher prevalence of erythroderma than other studies. Sehgal and Srivastava recorded the incidence of erythroderma from the Indian subcontinent as 35 per 100,000 dermatologic outpatients. But there are conflicting views over role of histopathology as some studies were unrewarding 5.
The study was performed to find out the causes of erythroderma in north-west part of India, to find out the epidemiological, clinical profile of these patients and histopathological correlation.
Material and Methods:
The study was conducted from January 2013 to December 2014. All cases of erythroderma attending skin outpatient department were included in the study. A thorough history followed by a meticulous general, physical and dermatological examination was to form a clinical diagnosis. Laboratory investigations including complete hemogram, blood glucose, blood urea, serum creatinine, liver function test, serum electrolytes and chest radiograph were done in all cases. Other relevant investigations including abdominal ultrasound, peripheral blood smear, fine needle aspiration cytology (FNAC) of lymph nodes and CT scan were done wherever needed. A four millimeter skin punch biopsy was performed in all patients from two representative sites. The slides were independently analysed by three different observers without relevant clinical information. Histopathological diagnosis was correlated with clinical diagnosis to make final diagnosis.
A total of 66 patients were enrolled in this study. The mean age of the study group was 53.7±16.56 in years (range : 14 to 86 Years). Males outnumbered females in a ratio of 3.4:1. The total duration of disease ranged from 10 days to 20 years with an average duration of 3.9 years. The exacerbation of disease was from 7 days to 1 year with a mean of 1.9 months. Majority of male patients were involved in outdoor activities and were farmers (39.4%) and laborers (16.67%). Majority of female patients were housewives (80%).
Most common aggravating factor was seasonal variation. Seasonal exacerbation was present in about 51.51% of patients. Winter exacerbation was present in 40% of psoriasis patients and 2.8% of eczema patients. Summer exacerbation was present in 54.2% of eczema patients and 25% of psoriasis patients. History of atopy was present in 19 patients. Drugs were responsible in 8 patients.
History of preexisting skin disease was present in 30 patients (62.1 %). Other co-morbidities like hypertension were present in 26 patients (39.3%), diabetes in 4 patients (6.06%), and tuberculosis in 4 patients (6.06%). The site of onset of erythroderma was scalp and face in 28 patients (42.4%), extremities in 27 patients (40.9%), and trunk & abdomen in 11 patients (16.67%).
Most common clinical features were itching (98.48%), fever (31.8%), shivering (40.9%), arthralgia (13.63%), lymphadenopathy (62.1%), edema (51.5%), palmoplantar keratoderma (22.7%) and nail changes (57.8%) [Table1]. The clinical finding in psoriasis, dermatitis and drug induced ertythroderma have been described in detail in Table 2. Most common nail change was beau’s line followed by shiny nails, yellowish discoloration of nails, subungual hyperkeratosis, pitting, and onycholysis. In 3 patients, twenty nail dystrophy was present. Investigations revealed anemia in 33.3%, increased ESR in 37.9%, abnormal TLC in 18.1%, abnormal LFT in 15.2%, hypoalbuminaemia in 34.8% and abnormal RFT in 9.09% of cases.
|Table 1: Clinical Profile of Patients.
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|Table 2: Differences in clinical profile of erythroderma patients.
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Clinico-pathological correlation occurred in about 67% (range: 63.6% to 68.2%) of patients with a kappa score ranging from 0.495 to 0.572. It was of moderate agreement. In psoriatic erythroderma patients, we were able to elicit munromicroabscess, dilated blood vessel and suprapapillary thinning in 60%, 80% and 65% cases respectively. Presence of mitotic cells was also specific for psoriasis but it was present in only 20% of cases. The biopsies of drug induced erythroderma patients had necrotic keratinocyte, basal cell vacuolization and eosinophils in infiltrate in 62.5%, 75% and 87.5% of patients respectively. Spongiosis was present in 62.8% of patients of eczema. But it was also present in 50 % of drug induced erythroderma patients and 10% of psoriasis patients. [Table 3] In five patients, clinical findings mismatched histopathological findings. In these patients, clinical findings suggested the diagnosis of eczema but it came out psoriasis histopathologically. [Table 4]
|Table 3: Histopathological findings.
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|Table 4: Clinical findings of patients with mismatched histopathological findings.
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Most common cause of erythroderma in this study was eczema of various types (53.03%), followed by psoriasis (30.30%), drug induced (12.12%), lymphoma (1.515%), mycosis fungoides (1.515%) and idiopathic (1.515%). [Figure 1]
|Figure 1: Etiology of erythroderma.
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The approach to patients with erythroderma depends on their previous dermatologic background. Patient with a preexisting dermatoses are easy to diagnose. Otherwise, erythroderma remains a diagnostic challenge, especially in those patients without history of dermatologic diseases and who deny having recently taken any medications 6.
In this study, the mean age of diagnosis was 53.7±16.56 years (range: 14 to 86 Years) with men outnumbering women in a ratio of 3.4:1. This is in accordance with various previous studies 3, 6, 7. However, in a recent study by Hulmani et al, male to female ratio of 14:1 was noted8.
In our study, most common cause of erythroderma was air borne contact dermatitis compared to Hulmani et al where most common etiology was psoriasis 8. The different etiologies of erythroderma found in various studies has been summarized in Table 5.
|Table 5: Comparison of different etiology of erythroderma in various studies.
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Lymphadenopathy was seen in 62.1% of our cases. Previous studies have reported its prevalence varying from 19% to 55%.6, 8 – 10 Nail changes were seen in 57.8% of patients. Nail changes were beau’s lines, shinning in the nails, subungual hyperkeratosis, pitting, yellowish discoloration and onychodystrophy. Similar findings were present in other studies 8, 10.
Clinico-pathological correlation occured in about 67% (range: 63.6% to 68.2%) of patients. In a similar study by Zip et al, 4 each set of pathological diagnoses was compared with the final discharge diagnoses, a positive correlation of 86% was observed in the nonblinded (original) diagnostic group as opposed to 66% in the blinded group. The results of blinded group were in accordance to our study which is also a blinded study. In another study by Vasconcellos et al, 1 one or more skin biopsies along with clinical findings were diagnostic or suggestive of the underlying disease in 63.6% of the cases. Khaled et al 14 reported positive clinico–histological correlation in 77%, Jun Li et al 6 in 55.56% and Rym et al 11 in 74% of patients.
The histopathology of eythroderma differs depending on the underlying diagnosis. In our study, in psoriasis patients the findings observed were munromicroabscess(60%), dilated blood vessel (80%), suprapapillary thinning (65%) and mitotic cells (20%). In similar study by Zip et al, 4 biopsies of psoriatic erythroderma patients revealed suprapapillary thinning, dilated blood vessel and munromicroabscess in 69%, 81% and 69% of patients respectively.
The biopsies of drug induced erythroderma patients had necrotic keratinocyte, basal cell vacuolization and eosinophils in infiltrate in 62.5%, 75% and 87.5% of patients respectively. In study by Zip et al 4, necrotic keratinocyte and eosinophils in infiltrate were present in 50% of cases each. Microscopically, eosinophils in infiltrate, necrotic keratinocyte and basal cell vacuolization were the most specific findings to diagnose a case of drug induced erythroderma. In previous studies, spongiosis was one of characteristic finding to diagnose a case of erythroderma due to eczema, present in 62.8% patients. But it was also present in 50 % of drug induced erythroderma patients and 10% of psoriasis patients. Thus as spongiosis was not one of the specific findings to diagnose a case of erythroderma due to eczema we had to collaborate it with other findings for diagnosis. It is generally thought that oozing, cracking, fissuring, presence of beau’s lines, nail discoloration are useful for diagnosing eczema in erythroderma patients, but in our study we found that these changes were also present in some patients of psoriasis. Thus these changes might help in diagnosis of eczema but they are not diagnostic and a biopsy should be done in all patients to confirm diagnosis even when sure clinically.
Comparison of our etiologic diagnosis with the previous studies is compiled in table 4. In our case series, most common final diagnosis was eczema. It is quite different from other studies where it constituted a minority group.
Although clinical diagnosis is possible in most cases, histopathology is required to corroborate with clinical diagnosis and to avoid any misdiagnosis as clinical features might overlap, for example psoriasis Versus eczema. Microscopical clues that might help in diagnosis are munromicroabscess, dilated blood vessel and suprapapillary thinning for psoriasis and necrotic keratinocyte, basal cell vacuolization and eosinophils for drug induced erythroderma patients. Erythroderma still remains a challenge and requires skills of the dermatologist.
1. Vasconcellos C, Domingues PP, Aoki V, Miyake RK, Sauaia N, Martins JE. Erythroderma: Analysis of 247 cases. Rev Saude Publica. 1995;29:177–82.
2. Botella-Estrada R, Sanmartin O, Oliver V, Febrer I, Aliaga A. Erythroderma: A clinicopathological study of 56 cases. Ama Arch Derm Syphilol. 1994;130:1503–07.
3. Hasan T, Jansen CT. Erythroderma: a follow-up of fifty cases. J Am Acad Dermatol. 1983;8:836–40.
4. Walsh NM, Prokopetz R, Tron VA, Sawyer DM, Watters AK, Murray S, Zip C. Histopathology in erythroderma: review of a series of cases by multiple observers. J Cutan Pathol. 1994;21:419–23.
5. Sehgal VN, Srivastava G. Exfoliative dermatitis: A prospective study of 80 patients. Dermatologica. 1986;173:278–84.
6. Li J, Zheng HY. Erythroderma: A Clinical and Prognostic Study. Dermatology. 2012;225(2):154-62.
7. Sehgal VN, Srivastava G, Sardana K. Erythroderma/exfoliative dermatitis: a synopsis. Int J Dermatol 2004;43: 39–47.
8. Hulmani M, Nandakishore B, Bhat MR, Sukumar D, Martis J, Kamath G, et al. Indian Dermatol Online J. 2014;5(1):25-9.
9. Bandyaopadhyay D, Chowdhury S, Roy A. Seventy five cases of exfoliative dermatitis. Indian J Dermatol. 1999;44:55–7.
10. Pal S, Haroon TS. Erythroderma: A clinico-etiologic study of 90 cases. Int J Dermatol. 1998;37:104–7.
11. Rym BM, Mourad M, Bechir Z, Dalenda E, Faika C, Iadh AM, et al. Erythroderma in adults: A report of 80 cases. Int J Dermatol. 2005;44:731–5.
12. Sudho R, Hussain SB, Bellraj E, Frederick M, Mahalaxmi V, Sobhana S, et al. Clinicopathological study of exfoliative dermatitis. Indian J Dermatol Venereol Leprol. 2003;69:30–1.
13. Chaudhary A, Gupte PD. Erythroderma: A study of incidence and aetiopathogenesis. Indian J Dermatol Venereol Leprol. 1997;63:38–9.
14. Khaled A, Sellami A, Fazaa B, Kharfi M, Zeglaoui F, Kamoun MR. Acquired erythroderma in adults: a clinical and prognostic study. J Eur Acad Dermatol Venereol. 2010;24(7):781-8.