|Year : 2019 | Volume
| Issue : 1 | Page : 25-29
A clinicopathological study of facial granulomatous dermatoses: A hospital-based study
Mahesh Mathur, Alina Karki, Prakash Acharya, Ayush Jha
Department of Dermatology, College of Medical Sciences, Bharatpur, Nepal
|Date of Web Publication||13-Jun-2019|
Dr. Alina Karki
Department of Dermatology, College of Medical Sciences, P. O. Box 23, Bharatpur
Source of Support: None, Conflict of Interest: None
Background: Granulomatous dermatoses of various types, etiologies, and clinical features frequently present with similar clinicopathological features which create a dilemma and diagnostic challenge for experts. Early diagnosis of facial lesions is of prime concern due to potential social stigma and psychological stress because of cosmetic disfigurement. Objective: To study the epidemiological and clinicopathological features of various granulomatous facial skin lesions and their concordance rate. Materials and Methods: A retrospective, hospital-based study was carried out at the Department of Dermatology, College of Medical Sciences, Bharatpur, over a period of 5 years (January 2013–January 2018). All clinically suspected cases of facial granulomatous dermatoses were included, their clinical and histological findings were recorded and analyzed according to the standard protocol, and a clinicopathological correlation was ascertained. Results: Among 850 skin biopsies performed during the study, 30 cases were clinically suspected as facial granulomatous dermatoses. There was female predominance (n = 22, 73.33%), and the mean age of patients was 41.7 years. Majority of cases (n = 29, 96.66%) were of infectious origin with leprosy as predominant cause (n = 25, 83.33%), followed by cutaneous tuberculosis (n = 4, 13.33%). Histologically, epithelioid cell granulomas (n = 26, 86.67%) and histiocytic granuloma (n = 3, 10%) were observed. Fite Faraco stain was positive in 6 (20%) cases of leprosy. An overall concordance rate of 80% was observed in facial granulomatous dermatoses in our study. Conclusion: In our study, infectious etiology is the most common cause of facial granulomatous dermatoses. Among infections, leprosy is the most common.
Keywords: Face, granuloma, granulomatous dermatoses, leprosy, lupus vulgaris
|How to cite this article:|
Mathur M, Karki A, Acharya P, Jha A. A clinicopathological study of facial granulomatous dermatoses: A hospital-based study. Indian J Dermatopathol Diagn Dermatol 2019;6:25-9
|How to cite this URL:|
Mathur M, Karki A, Acharya P, Jha A. A clinicopathological study of facial granulomatous dermatoses: A hospital-based study. Indian J Dermatopathol Diagn Dermatol [serial online] 2019 [cited 2019 Jul 16];6:25-9. Available from: http://www.ijdpdd.com/text.asp?2019/6/1/25/260189
| Introduction|| |
Granulomatous dermatoses are a group of dermatoses with a characteristic histological pattern of granuloma formation. A granuloma is a principal, packed collection of inflammatory cells, predominately mononuclear cells formed as a result of complex interplay of inciting agents, macrophage activity, T helper 1 cell response, and overactivity of B cells. Histologically, the granuloma can be classified as epithelioid granuloma (sarcoidal and tuberculoid), necrobiotic, histiocytic, suppurative, and foreign body granulomas.
Histological examination remains a time-proven tool for diagnosing various dermatoses; however, similar clinical and histological features of granulomatous dermatoses create a diagnostic dilemma even to experts., The pathological process is chronic and involves dermis; hence, there are chances of scarring.
The skin lesions can occur anywhere over the body, including face. Early diagnosis of facial granulomatous lesions is of prime concern because of potential social stigma, leading to psychological stress and risk of cosmetic disfigurement associated with them. The present hospital-based study aims to determine the epidemiological and clinicopathological features of various granulomatous facial skin lesions and evaluate the clinicopathological concordance rate to arrive at the correct diagnosis.
| Materials and Methods|| |
A retrospective, hospital-based study was carried out at the Department of Dermatology, College of Medical Sciences, Bharatpur, Nepal, over a period of 5 years (January 2013–January 2018).
Ethical clearance was taken from the institutional ethics committee, and written consent was obtained from all patients under inclusion in the study.
All clinically suspected and biopsy-sent cases of facial granulomatous dermatoses from the dermatology department in the above-mentioned period were included in the study. Clinical data were recorded in a preset pro forma. Ridley and Jopling's classification was used for subclassification in cases of leprosy. Histological findings were noted from sections stained with hematoxylin and eosin stain. Special stains, such as Ziehl–Neelsen (ZN), Gram, Fite Faraco, and Giemsa, were performed when necessary. The recorded data were analyzed using SPSS 20 (International Business Machine Corp., Armonk, NY, USA), and the clinicopathological concordance rate was ascertained.
| Results|| |
A total of 850 skin biopsies were performed during the study, of which 112 were from the face and 30 cases were clinically suspected as facial granulomatous dermatoses and comprised the study group. There was female predominance (n = 22, 73.33%), and female:male ratio of 2.7:1 was observed. The study patients were between 11 and 62 years of age and the mean age of 41.7 years was observed in our study. Majority (n = 27, 90%) of the lesions presented as discrete plaques. These were present mainly over the cheek (n = 22, 73.3%). The mean duration of these lesions was 3.63 months.
Among the 30 clinically suspected cases, histologically 29 (n = 29, 96.66%) cases were confirmed as granulomatous dermatoses and 1 (n = 1, 3.33%) case was nongranulomatous. Among granulomatous dermatoses, all were of infectious etiology as shown in [Figure 1]. Leprosy was the predominant infectious cause (n = 25, 83.33%) and 44% cases had borderline tuberculoid (BT) leprosy [Figure 2]a among leprosy cases. Cutaneous tuberculosis (n = 4, 13.33%) was the next common infectious cause observed in our study and lupus vulgaris constituted all these cases [Figure 2]b. One case of nongranulomatous dermatoses on histopathology was found to be a case of discoid lupus erythematosus (DLE).
|Figure 1: Histopathological diagnosis of clinically suspected cases of facial granulomatous dermatoses|
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|Figure 2: (a) Erythematous infiltrated plaques involving the left side of the face in a case of borderline tuberculoid leprosy. (b) Erythematous plaque involving the left cheek and nose with patchy areas of erosion and crusting in a case of lupus vulgaris|
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Histologically, among various types of granuloma, only epithelioid granulomas (n = 26, 86.67%) and histiocytic granuloma (n = 3, 10%) were observed. Epithelioid granuloma was present in 22 cases of leprosy and all 4 cases of cutaneous tuberculosis. Histiocytic granuloma was present in three cases of leprosy (two cases of borderline lepromatous [BL] leprosy and one case of lepromatous leprosy [LL]). Epithelioid granulomas were surrounded by a mononuclear cell infiltrate and occasional Langhans and foreign body giant cells. These granulomas were located around neurovascular bundles and adnexa in cases of leprosy [Figure 3]a and [Figure 3]b. Grenz zone was observed toward lepromatous spectrum.
|Figure 3: (a) Dermis showing epithelioid granulomas involving the nerves and adnexa (H and E, ×10). (b) Granuloma involving the adnexa at higher magnification (H and E, ×40)|
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Fite Faraco stain was performed in all cases of leprosy and found positive in 6 (20%) cases, of which 2 cases each were of BL and BT and one each was borderline-borderline (BB) (mid borderline) and LL. Acid-fast bacilli were observed to be in clumped and globi form in BL and LL case [Figure 4] whereas single in the BT spectrum. ZN stain was done in all cases of cutaneous tuberculosis and was found negative in all cases.
|Figure 4: Fite Faraco stain of skin biopsy showing abundant acid-fast bacilli in a case of leprosy|
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The clinicopathological concordance rate of various facial granulomatous dermatoses is shown in [Table 1]. The clinicopathological concordance rate of facial granulomatous dermatoses was found to be 96.67%. However, if the subclassification of leprosy is considered, the overall clinicopathological concordance rate in our study is 80%.
|Table 1: Clinicopathological concordance rate of facial granulomatous dermatoses|
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| Discussion|| |
Granulomatous inflammation, a delayed Type IV hypersensitivity reaction to multiple inciting agents, can be diagnosed and managed early with the help of histological examination. In this study, we have studied the clinical and histological features and the concordance rate of facial granulomatous dermatoses.
Female preponderance (n = 22, 73.33%) was observed in our study, which is similar to a study done by Zafar et al. However, this is not in accordance with previous studies., Such higher prevalence may be due to a concern of facial lesions in women more than men and their early visit to the hospital for management.
In our study, infectious etiology was the most common cause of granulomatous dermatoses, in which leprosy was the most common cause followed by cutaneous tuberculosis. This is similar to a study done by Bansal et al. Similar findings were observed in the study of granulomatous dermatoses done in India, Nepal, and Sri Lanka., This may be due to the endemicity of these condition in Southeast Asian countries.
Of the total 25 cases confirmed as leprosy in histopathology, 24 cases were clinically diagnosed correctly. In our study, BT leprosy (44%) was the most common lesion observed, which is similar to other studies., The highest concordance rate was observed in cases of tuberculoid subtype (100%) and the least with BL cases (33.33%).
In our study, tuberculoid granuloma was the most common granuloma observed, which is consistent with various other studies on granulomatous dermatoses., All tuberculoid leprosy and BT cases had granuloma similar to noncaseating granulomas of tuberculosis. These granulomas involved neurovascular bundles and adnexa, were invading epidermis, and had well-formed tubercles with collection of the epithelioid cells and few Langhans giant cells enclosed by dense infiltrates of lymphocytes in cases of tuberculoid leprosy. In cases of BT leprosy, the granulomas were ill defined with epithelioid cell assembly and occasional Langhans giant cells mixed with lymphocytes infiltrating dermal nerves and adnexa and occupied the majority of dermis with clear zone detaching it from the epidermis. The granulomas in mid borderline leprosy (BB) had immature epithelioid cells and a faint smattering of lymphocytes and macrophages, and no giant cells were present. The perineurium was infiltrated by macrophages and lymphocytes. The BL and LL cases showed histiocytic granuloma surrounding nerve bundles and adnexa. The granuloma in BL cases had many lymphocytes whereas, in LL cases, very fewer lymphocytes were seen.
Fite Faraco stain was done in all cases of leprosy for the detection of Mycobacterium leprae, a less acid-fast organism, due to its high sensitivity than ZN stain. It revealed bacilli in six cases, among which two cases each were of BL and BT, one each of BB and LL, and none of the tuberculoid leprosy. Acid-fast bacilli were clumped and in globi form in BL and LL case while single in tuberculoid leprosy. Thus, lepra bacilli were present in 24% of all the leprosy cases similar to a study done by Chakrabarti, which showed the presence in 28.03% of leprosy cases.
In our study, tuberculosis was the second most common cause of facial granulomatous dermatoses comprising 13.33% of cases. This is higher than that found by Gautam et al. (7.6%) and less than Chakrabarti (24.73%). All the cases of cutaneous tuberculosis in our study were lupus vulgaris, which is consistent with other studies in which lupus vulgaris is the most common type of cutaneous tuberculosis.,
In our study, 4 (0.47%) out of 850 cases were of cutaneous tuberculosis. The worldwide prevalence of cutaneous tuberculosis is 0.1%–1%. In our study, female-to-male ratio of 3:1 was seen in cases of cutaneous tuberculosis, supporting the female preponderance in lupus vulgaris of prior studies.,
Of the four clinically diagnosed cases of LV, three were histologically proven as LV. They showed atrophic epidermis and epithelioid granuloma in upper and mid-dermis with few Langhans giant cells. The epithelioid granuloma had intact nerves in the midst of granuloma. However, one case which was clinically suspected as LV showed epithelioid granulomas with occasional giant cells and peripheral lymphocytes which were infiltrating the nerves and arrector pili muscle on histological examination. The final diagnosis of BT was made for this case. The granulomas were present along the superficial vascular plexus, but not encroaching into the epidermis. The granuloma of sarcoidosis also may involve perineurium and requires differentiation from BT leprosy. However, in this case, there was no clinical suspicion of sarcoidosis and no “naked” granuloma with surrounding few lymphocytes along with a rim of mild dermal fibrosis seen in histopathological examination.
The presence of tubercle bacilli in the tissue section, smear, or in vitro recovery gives the definite diagnosis of cutaneous tuberculosis. However, literature has reported only 13%–15% positivity by ZN, thus requiring correlation of other criteria in diagnosis. ZN stain was negative in all cases of cutaneous tuberculosis in our study.
Among two cases in which the differential diagnoses included both DLE and LV, one case was diagnosed as LV and another as DLE after histological examination. This is in accordance with the study done by Bansal et al., which highlighted that LV on face could masquerade as DLE and histopathology is required for differentiation of these conditions. This finding was supported by Dhar and Dhar. Of the four cases of LV, three cases were correctly diagnosed; thus, the concordance rate of 75% was observed in the case of lupus vulgaris.
In our study, the clinicopathological concordance rate of 96.67% was observed with facial granulomatous cases, and the overall clinicopathological concordance rate (considering leprosy subtypes) of 80% was observed, which is similar to the study by Dhar and Dhar (77.27%).
No cases of sarcoidal, necrobiotic, or foreign body granuloma were observed. Other granulomatous dermatoses such as actinomycosis, sarcoidosis, and cutaneous leishmaniasis as reported in other literature were not observed in our study. This may be due to the rarity of these conditions in our country and exclusion of granulomatous dermatoses occurring at other parts of the body in our study.,,
It may also be due to the inclusion of biopsy-sent, clinically suspected granulomatous dermatoses from the department of dermatology only. Cases who refused biopsy examination and those who did not come for follow-up after primary medical management were not included and may also be a factor for the absence of other granulomatous dermatoses in our study. Because this is a retrospective study, follow-up of these cases could not be ascertained.
| Conclusion|| |
Our study revealed infectious etiology as the most common cause of facial granulomatous dermatoses, and among infectious cause, leprosy is the most common. The histological study has immense importance in the early diagnosis of these facial granulomatous dermatoses because of potential stigmata and psychological impact on quality of life of the patient.
We would like to thank Prof. Dr. T.S. Rao and Dr. Subechhya Jaiswal, Department of Pathology, College of Medical Sciences, Bharatpur, for their support.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Weedon D. The granulomatous reaction pattern. In: Weedon D editor. Skin Pathology. 2nd
ed. Philadelphia: Churchill Livingstone; 2002. p. 193-220.
James DG. A clinicopathological classification of granulomatous disorders. Postgrad Med J 2000;76:457-65.
Zaim MT, Brodell RT, Pokorney DR. Non-neoplastic inflammatory dermatoses. A clinical pathologic correlative approach. Mod Pathol 1990;3:381-414.
Dhar S, Dhar S. Histological features of granulomatous skin diseases: An analysis of 22 skin biopsies. Indian J Dermatol 2002;47:88-90. [Full text]
Bansal C, Batra M, Sharma KL, Tulsyan S, Srivastava AN. Facial granulomatous dermatoses: Aclinico-pathological study. J Dermatol Dermatol Surg 2013;17:55-61.
Ridley DS, Jopling WH. Classification of leprosy according to immunity. A five-group system. Int J Lepr Other Mycobact Dis 1966;34:255-73.
Chakrabarti S, Pal S, Biswas BK, Bose K, Pal S, Pathak S, et al.
Clinico-pathological study of cutaneous granulomatous lesions – A 5 yr experience in a tertiary care hospital in India. Iran J Pathol 2016;11:54-60.
Zafar MN, Sadiq S, Menon MA. Morphological study of different granulomatous lesions of the skin. J Pak Assoc Dermatol 2008;18:21-8.
Gautam K, Pai RR, Bhat S. Granulomatous lesions of the skin. J Pathol Nepal 2011;1:81-6.
Bal A, Mohan H, Dhami GP. Infectious granulomatous dermatitis: A clinico-pathological study. Indian J Dermatol 2006;513:217-20.
Hirsh BC, Johnson WC. Concepts of granulomatous inflammation. Int J Dermatol 1984;23:90-100.
Adiga DS, Hippargi SB, Rao G, Saha D, Yelikar BR, Karigoudar M, et al.
Evaluation of fluorescent staining for diagnosis of leprosy and its impact on grading of the disease: Comparison with conventional staining. J Clin Diagn Res 2016;10:EC23-6.
Mohan H, Bal A, Dhami GP. Non-infectious granulomatous dermatitis: A clinicopathological study. J Cutan Pathol 2006;33:767-71.
Yasmeen N, Kanjee A. Cutaneous tuberculosis: A three year prospective study. J Pak Med Assoc 2005;55:10-2.
Khan Y, Anwar J, Iqbal P, Kumar A. Cutaneous tuberculosis: A study of ten cases. J Pak Assoc Dermatol 2001;11:6-10.
Munday WR, McNiff J, Watsky K, DiCapua D, Galan A. Perineural granulomas in cutaneous sarcoidosis may be associated with sarcoidosis small-fiber neuropathy. J Cutan Pathol 2015;42:465-70.
Marcoval J, Servitje O, Moreno A, Jucglà A, Peyrí J. Lupus vulgaris. Clinical, histopathologic, and bacteriologic study of 10 cases. J Am Acad Dermatol 1992;26:404-7.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]