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.

Scleredema of Buschke associated with lichen sclerosus: Three cases.

Scleredema adultorum of Buschke is a rare fibromucinous, scleroderma-like connective tissue disease most commonly found in a post-infectious setting or linked to hematological disorders or diabetes. Lichen sclerosus et atrophicus is an autoimmune condition only in 2.5% of cases localized exclusively at an extragenital site, occurring in up to 34% of patients in association with other autoimmune diseases such as vitiligo, thyroid disorders, alopecia areata, lichen planus, morphea, pernicious anemia and systemic lupus erythematosus. In particular, a stronger link with an autoimmune background in lichen sclerosus et atrophicus has been observed in women who showed higher prevalence for autoimmune conditions and circulating autoantibodies. Literature reveals a genetic susceptibility linked to specific HLA types. We report three patients who developed lichen sclerosus et atrophicus superimposed on skin involved by scleredema adultorum of Buschke. Although the association of lichen sclerosus et atrophicus with scleredema adultorum of Buschke could be coincidental, both diseases could be considered part of the spectrum of sclerodermoid disorders with common underlying pathogenetic mechanisms; which could explain the sequential or simultaneous occurrence of both lesions in our patients.

Atypical presentations of eosinophilic fasciitis.

Eosinophilic fasciitis is an uncommon connective tissue disease that may mimic and overlap with other sclerosing disorders such as morphea and lichen sclerosus. Herein, we report four patients (two men and two women, aged 16-64 yeas) with eosinophilic fasciitis. There was overlap with both morphea and lichen sclerosus in 2 patients and with morphoea alone in 1 patient. Magnetic resonance imaging (MRI) was used for diagnosis in three patients and for assessing treatment response in one patient. Eosinophilic fasciitis may co-exist with morhoea and lichen sclerosus. In view of the overlapping clinical and histopathological features of these disorders, MRI may be helful in delineating the conditions by detecting involvement of fascia.

Lichen sclerosus: role of occlusion of the genital skin in the pathogenesis.

Lichen sclerosus (LS) is a chronic inflammatory skin disease, which most commonly involves the anogenital region. The etiology of LS is obscure, but genetic susceptibility, autoimmune mechanisms, infective agents like human papillomavirus and spirochaetes, and Koebner phenomenon has been postulated as causative factors. We report our observation in 6 patients (3 males and 3 females) with histologically proven lichen sclerosus that showed relative sparing of the uncovered areas of the genitals, thereby suggesting that the occlusion of the genital skin may be playing a greater role in the causation of LS than is currently thought, in both sexes.

Generalized morphea, lichen sclerosis et atrophicus associated with oral submucosal fibrosis in an adult male.

Generalized morphea is a disease characterized by wide-spread sclerosis of the skin. A 39-year-old man presented with history of multiple pigmented and bound-down plaques on the body along with mucosal involvement. Dermatological examination showed multiple indurated and sclerosed plaques with follicular plugging in few of them and gross thickened eroded and glazed tongue. The constellation of these findings with histopathological correlation led us to diagnosis of this spectrum of cutaneous involvement. The coexistence of localized morphea with lichen sclerosis et atrophicus has been reported earlier but existence of these entities with submucosal fibrosis in a same patient is documented here and is the first of its kind.

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.