Clinical and histopathological spectrum of genital lichen sclerosus in 133 cases: Focus on the diagnosis of pre-sclerotic disease.

Background Early inflammatory lesions of lichen sclerosus are histopathologically difficult to diagnose until the hallmark of the disease i.e., papillary sclerosis becomes visible in histological sections. Pre-sclerotic and late or resolved phases of the disease have not been extensively studied. Methods We retrospectively reviewed all cases diagnosed as genital lichen sclerosus over a ten-year period from 2006 to 2016, correlating the clinical findings with the histological features. Results A total of 133 cases of genital lichen sclerosus (90 males and 43 females) were identified. Both genders demonstrated a similar histological spectrum. Fifty eight (44%) cases were identified as having pre-sclerotic lichen sclerosus, 64 (48%) as having progressive disease and 11 (8%) cases were classified as fully resolved with atrophy. Asymptomatic vitiligoid lesions were identified in 19 (14%) cases of which 12 were male. Low-grade squamous cell carcinoma was seen within the areas affected by long-standing lichen sclerosus, in four patients (3%, 2 male). Limitations We studied only haematoxylin and eosin stained sections. The presence of basement membrane thickening could have been better illustrated with the periodic acid-Schiff stain. Conclusion The pathogenesis of lichen sclerosus probably involves an immune reaction to the basement membrane at the epidermal interface and around the adnexa. The initial band of inflammation shifts gradually downwards from the epidermal interface into the dermis destroying the vascular channels and appendages, resulting in excessive deposition of altered extracellular matrix. Basilar infiltration of lymphocytes along with a grossly vacuolated or thickened basement membrane is proposed as the characteristic diagnostic feature of the pre-sclerotic stage. Greater awareness of the clinicopathological spectrum of lichen sclerosus should enable early diagnosis and treatment, thereby preventing structural damage and possible malignant transformation in chronic cases.

Acral Vitiligo and Lichen Sclerosus - Association or a Distinct Pattern?: A Clinical and Histopathological Review of 15 Cases.

BACKGROUND: Acral or acrofacial vitiligo (AFV) with bilateral lesions over the extremities and face is considered as a transitional form that may progress to generalized vitiligo. Oral and genital mucosal lesions are often integral to this pattern. Lichen sclerosus (LS) in a milder expression, results in oral and genital vitiligoid depigmentation without textural changes and thus needs to be differentiated from AFV. MATERIALS AND METHODS: We reviewed 217 cases of AFV recorded over a period of 12 years. RESULTS: One hundred and sixteen cases had associated oral/genital lesions. Among these, 15 patients demonstrated typical clinical as well as histological features of LS. DISCUSSION: Coexistence of typical LS essentially among oral and genital lesions of acral vitiligo suggests that acral vitiligo might be a distinct sub-group of NSV. Since both the diseases have an autoimmune basis, the co-existence may be explained by epitope spreading, as a result of interface dermatitis seen in vitiligo. In addition, the possibility of a common genetic predisposition needs to be explored.

Segmental and generalized vitiligo: both forms demonstrate inflammatory histopathological features and clinical mosaicism.

BACKGROUND: Segmental vitiligo (SV) and generalized vitiligo (GV) are perceived to evolve by different mechanisms, the former with unspecified neural mechanisms and the latter by melanocyte specific autoimmune mechanisms. However, the two diverse mechanisms are difficult to reconcile in cases of "mixed vitiligo". To test the possibility of a common pathogenesis, we reviewed clinical and histopathological features of SV and GV. MATERIALS AND METHODS: As part of an ongoing histopathological study on vitiligo and vitiligo like lesions, over a 10 year period from 2002 to 2011, biopsies were taken routinely from evolving or recently evolved lesions. 50 cases of SV with quasi-dermatomal distribution and 154 cases of GV were identified and the clinical and histopathological features were compared. RESULTS: Mild clinical inflammation was recorded in 33 of 154 GV cases but, none among 50 SV had such features. In addition to bilateral symmetrical involvement, mirror image lesions with unusual segmentation were observed in nine cases of GV. SV with a few bilateral lesions (4) and GV with quasi-dermatomal lesions (3), i.e., mixed vitiligo, were included in their corresponding groups for analytical purposes. Focal lichenoid inflammation of varying degrees around epidermal/adnexal melanocytes was identified as a common feature in evolving lesions of both SV (78%) and GV (70%). CONCLUSIONS: SV and GV demonstrated a similar inflammatory histopathological spectrum. "Segmentation/mosaicism", identified for the first time in GV is another unifying factor. Cutaneous mosaicism harboring fragile melanocyte populations, which are susceptible to external as well as auto-inflammatory mechanisms, is an attractive hypothesis to pursue in the causation of vitiligo.

Lichen sclerosus of lips: a clinical and histopathologic study of 27 cases.

BACKGROUND & METHODS: Oral lichen sclerosus (LS) has been considered uncommon and involvement of lips extremely rare. We reviewed the clinical and histologic features of 72 cases of LS with oral/genital involvement, seen in our institute from 2002 to 2007. RESULTS: Lichen sclerosus was diagnosed with exclusive genital lesions in 45, exclusive lip involvement in 20, and orogenital involvement in seven cases. Fifteen of 27 histologically confirmed lip LS lesions were considered as early inflammatory or presclerotic, eight were intermediate/progressive, and four as late resolved lesions. Lip LS presented as asymptomatic vitiligoid lesions in 70% and dermal sclerosis was demonstrable in only 44%, which was limited to the papillary layer. This was in contrast with genital LS lesions which were asymptomatic in only 12% and demonstrated both papillary and reticular dermal sclerosis in 69%. CONCLUSION: Lip LS is far less symptomatic and destructive with limited dermal sclerosis compared with genital LS. Greater awareness and histologic assessment are essential for diagnosis because of the misleading vitiligoid appearance. "Vitiligoid LS" a superficial variant proposed by Borda can be aptly applied to lip LS. Dermatologists need to be aware of this rarely reported manifestation of LS as it adds to the spectrum of oral lichenoid lesions and lichenoid dysplasia, which are suspected to have a malignant potential.

Lichenoid inflammation in vitiligo--a clinical and histopathologic review of 210 cases.

BACKGROUND: '"Inflammatory vitiligo" cases with clear lichenoid infiltrates have been reported. However, the inflammatory nature of common vitiligo has not gained wide acceptance because of its benign appearance and scanty cellular infiltrates. We have observed in our patients a few lesions with mild erythema, scaling and marginal hyperpigmentation which were suspected to be inflammatory. This study was conducted to assess the histological features and prevalence of such marginally active lesions, in comparison with common vitiligo. METHODS: Two hundred and ten consecutive new cases of vitiligo seeking treatment for the first time were included in this study. Clinical lesions were carefully examined and biopsies were taken in all cases. Biopsies were also taken from pigmented skin 3 cm away from the vitiligo lesion in 20 cases and normal pigmented skin over the contralateral side in 20. RESULTS: Marginally active lesions with erythema, scaling and hyperpigmentation were identified in 27 patients (13%). Lymphocytic infiltration of dermo-epidermal interface was observed in 89% of these cases which was clearly lichenoid in 59%. Similar lichenoid infiltrates were also seen in 50% of pigmented skin samples 3 cm away from the lesion and 23% of common macular vitiligo lesions. CONCLUSIONS: Vitiligo is an inflammatory disease that with development involves a lichenoid tissue reaction.

Vitiligoid lichen sclerosus: a reappraisal.

BACKGROUND: Many case studies of lichen sclerosus (LS) have reported an association of vitiligo. However, such an association is not reported from larger case studies of vitiligo, which happens to be a common disease. Autoimmune etiology suspected in both LS and vitiligo has been considered as the reason for their association in some patients. It has also been suggested that lichenoid inflammation in LS may trigger an autoimmune reaction against melanocytes. AIMS: To test this association, we reviewed clinical and histological features of 266 cases of vitiligo and 74 cases of LS in a concurrent study of both diseases. METHODS: All outpatients seen in our department between 2003 and 2006 and who were diagnosed as having LS or vitiligo on the basis of clinical and pathologic features were included in the study. RESULTS: Vitiligoid lesions were seen along with stereotypical LS lesions in three patients but all the three lesions had histological features of LS. Oral/genital areas were affected in 57 out of the 74 LS cases and of those, 15 were initially suspected to have vitiligo. These cases with a clinical appearance of vitiligo and histological features of LS were considered as 'vitiligoid LS', a superficial variant proposed by J. M. Borda in 1968. Association of LS was not observed in the 266 cases of vitiligo. CONCLUSION: Exclusive oral/genital depigmentation is a common problem and histological evaluation is essential to differentiate vitiligoid LS from true vitiligo. The association of vitiligo with LS may have been documented due to the clinical misdiagnosis of vitiligoid LS lesions as vitiligo as histological investigations were not undertaken in any of the reported cases.