Isolated, localised extragenital bullous lichen sclerosus et atrophicus: a rare entity.

Lichen sclerosus et atrophicus (LSA) is a rare, chronic, mucocutaneous disease of unknown cause. Onset can occur in subjects of any age but more prevalent in adult females around the time of menopause. In both the sexes anogenital involvement is more common. Extragenital cases are rare, and common localizations are neck and shoulders, axilla, upper arms, flexor aspects of wrists and around the umbilicus. Bullous LSA is an unusual manifestation of the disease. Isolated extragenital bullous LSA is a distinctly rare event with very few cases reported till date.

What was known?

Anogenital involvement is most common in LSA in both the sexes.

Blister formation in the lesion of LSA occurs due to basal cell degeneration resulting in dermal-epidermal separation and marked edema in upper dermis.

Introduction

Lichen sclerosus et atrophicus (LSA) is a rare inflammatory disease of unknown etiology. It mainly affects post-menopausal women in the genital area.[1] In 15-20% of cases extragenital involvement is associated with classic genital LSA and only in 2.5% cases it is found exclusively at an extragenital site.[2] We describe a rare case of isolated nongenital bullous LSA in a male.

Case Report

A 38 year old male patient, engineer by an occupation, presented with minimally pruritic white colored lesions over anterior aspect of neck since 3 months. Lesions were gradually increasing in size as well as number. There was associated mild stinging sensation and minimal watery discharge from the pre-existing lesions since last two weeks. There was no history suggestive of involvement of oral or genital mucosa. The lesions were untreated till date. There were no systemic co morbidities or concurrent medication.

Dermatological examination revealed multiple atrophic papules with follicular plugging [Figure 1]. Closer examination revealed few lesions having a superficial wrinkled appearance which on palpation appeared to be due to the presence of atrophic epidermis overlying a collection of fluid which was confirmed after puncturing the lesion with needle [Figure 2]. At certain areas atrophic papules were coalescing to form plaques and few papules were studded with vesicles at their margins. Mucosae, palms, soles, nails, scalp were normal. Hematological investigations including the complete blood count, liver, renal and thyroid profiles were within normal limits. Biopsy findings from a representative lesion revealed presence of an atrophic epidermis with hydropic degeneration of basal cell layer forming a subepidermal split with pronounced dermal edema and homogenization of collagen in upper dermis and dense lymphocytic periappendageal infiltrate in the mid dermis [Figure 3]. A diagnosis of Bullous variant of LSA was thus established. Considering bullous nature of lesions a short course of oral and then topical superpotent steroids (clobetasol dipropionate 0.05% cream) with tacrolimus ointment (0.1%) was administered. There was clearance of lesions without any recurrence till 6 months follow up. Lesions healed with post-inflammatory hypopigmentation and scarring [Figure 4].

Figure 1

Multiple atrophic papules

Figure 2

Superficial wrinkled appearance of atrophic epidermis overlying a collection of fluid which was confirmed after puncturing the lesion

Figure 3

H and E, ×10-Atrophic epidermis with hydropic degeneration of basal cell layer forming a subepidermal split with pronounced dermal edema and homogenization of collagen in upper dermis and dense lymphocytic periappendageal infiltrate in the mid dermis

Figure 4

Healing of lesions with post-inflammatory scarring and hypopigmentation 6 months post-treatment

Discussion

LSA is an uncommon inflammatory condition. Hallopeau is credited with the first clinical description of LSA in 1887 and Darrier first reported histopathological changes in 1892.[3] Disease predominates in females and may occur at any age. However, the maximum incidence occurs between the 5th and 6th decade of life, perimenopausal group, and another peak in girls between the ages 8 and 13 years, prepubertal group.[4] In both the sexes anogenital involvement is common. Extragenital involvement is associated with classic genital LSA in 15-20% of cases and only 2.5% cases show exclusive involvement of extragenital site[2] as in our case. The underlying cause is unknown, but there seems to be genetic susceptibility and a link with autoimmune mechanism.[3] Extragenital LSA is most common on upper trunk involving neck and shoulders, axilla, upper arms, flexor aspects of wrists, around the umbilicus and on forehead and very rarely on scalp,[5] palms,[6] soles and in the oral cavity. Subclinical trauma caused by friction with the collar could be responsible for the lesions on the shoulder, possibly attributable to the Isomorphic phenomenon of Koebner. Lesions may follow lines of Blaschko's.[7] Exact prevalence of extragenital LSA may be underestimated because of its asymptomatic nature. Pruritus occurs occasionally. The lesions are small, ivory or porcelain-white, shiny, round macules or papules, coalescing to form sclerotic plaques. Individual lesions are usually slightly raised, or level with the surface of the skin and their surface shows prominent dilated pilosebaceous or sweat duct orifices, which often contain yellow or brown horny plugs. Lichenification may occur secondarily as a result of rubbing. Occasionally hemorrhagic transformation or blister formation occurs due to basal cell degeneration resulting in dermal-epidermal separation and marked edema in upper dermis. Bullous LSA has been described in a localized[5] and generalized[89] distribution, and often the vesicles and bullae have a hemorrhagic component. Telangiectases and purpura may occur. In the later stages, atrophy occurs, and the surface of the lesions becomes wrinkled, and may actually be depressed. Bullous lesions may sometimes heal with milia formation. In a similar case described by Hallel–Halevy et al., widespread hemorraghic bullae were described in a patient with extragenital LSA,[1] our case in contrast shows presence of localized involvement and non-hemorrhagic lesions. Similar localized bullous lesions of LSA over back have been described by Ballester Irene et al. and lesions resolved completely with topical potent steroid cream.[3]

Histologically LSA has a characteristic pattern. Histologically well-developed lesion of LSA is characterized by marked orthohyperkeratosis, keratotic plugging of the hair follicles and dermal appendages, atrophy of stratum malpighii with flattening and loss of rete ridges. The basal layer shows liquefactive degeneration. Beneath the epidermis there is broad zone of pronounced edema and homogenization of the collagen. Within this zone collagen fibers are swollen, homogenous and contain very few nuclei. The blood and lymphatic vessels are dilated, and there may be areas of hemorrhage. Edema in the upper part of the dermis may be enough to result in clinically apparent vesiculation. The lower dermis is characterized by the presence of dense lymphocytic infiltrate in the mid dermis which drifts from its sub epidermal position to mid dermis as the lesion develops on account of the edema and homogenization of collagen in the upper dermis.

As opposed to case of genital lesions, no increased risk of malignant transformation has been found in extragenital LSA.[3]

There is no definitive treatment for LSA, therapy for extragenital LSA is warranted only for extensive lesions, bullous and haemorrhagic lesions, symptomatic and cosmetically disfiguring lesions. Numerous therapies have been used including topical and systemic corticosteroids, topical estrogen and testosterone containing ointments, retinoids, tacrolimus and Psoralen Ultraviolet A therapy (PUVA) therapy.[3] Marked improvement in generalized bullous and haemorrhagic LSA has been reported with Adrenocorticotropic hormone in one patient.[8] The bullous lesions are usually transient and heal before typical plaques of the disease,[9] which are more resistant to treatment.

Conclusion

Isolated extragenital bullous LSA is a rare entity. It is important to bear in mind this form of presentation of LSA in order to facilitate clinical diagnosis and prompt treatment.

What is new?

Appearance of bullous lesions of LSA over an extragenital area in the absence of lesions elsewhere on the body is a rare presentation.