A Case of Lichen Sclerosus et Atrophicus Accompanying Bullous Morphea

Abstract

Bullous morphea is a rare form of morphea characterized with bullae on or around atrophic morphea plaques. Whereas lichen sclerosus et atrophicus (LSA) is a disease the etiology of which is not fully known, and which is characterized with sclerosis. Coexistence of morphea and LSA has been identified in some cases. Some authors believe that these two diseases are different manifestations which are on the same spectrum. The 70-year-old patient stated herein, presented to us for 6 months with annular, atrophic plaques, ivory color in the middle, surrounded by living erythema, on the front and back of the trunk. Occasionally bulla formation on the plaques on the trunk lateral was identified. Fibrotic and atrophic plaques of ligneous hardness were present on the front side of tibia of both legs. In the histopathologic examination, the lesions were found concordant with bullous morphea and LSA. With colchicine 1.5 mg/day, pentoxifylline 1,200 mg/day, topical calcipotriol ointment and clobetasol propionate cream, the erythema in the patient's lesions faded and softening in the fibrotic plaques was observed. Concomitance of bullous morphea and LSA is a rarely seen, interesting coexistence which suggests a common, as yet unknown, underlying pathogenesis.

Skin ulcers related to chronic graft-versus-host disease: clinical findings and associated morbidity

“…In bullous morphoea, biopsy findings generally reveal thickened sclerotic deeply eosinophilic collagen bundles that extend to subcutaneous tissue or at the level of deep reticular dermis, subepidermal blisters, variable subepidermal oedema, lymphatic dilatation, and moderate inflammatory infiltration around the blood vessels. The bullae development mechanism in the sclerotic area may involve the deposition of eosinophilic granule major basic protein, lymphatic obstruction and increased hydrostatic pressure . Histology of bullous lichen planus reveals an intense lichenoid reaction with subepidermal detachment, probably secondary to the basal vacuolization with apoptotic keratinocytes …”

Morfea Ampollosa tratamiento exitoso con fototerapia UVA 1, calcipotriol y superóxido dismutasa tópico, reporte de un caso

“…Presenta una baja incidencia, en 1959 Garb y Sims reportaron que había 25 casos publicados en la literatura a nivel mundial (3,5,6). La causa de la formación de ampollas sobre la esclerosis no es clara, sin embargo se han postulado varias hipótesis, dentro de las cuales la más aceptada es la obstrucción linfática, soportado por los hallazgos histopatológicos donde se observa ectasia de vasos linfáticos en la dermis papilar, además se ha observado que las extremidades inferiores son el área más comúnmente comprometida, por lo tanto se cree que la dilatación de los canales linfáticos y el aumento de la presión venosa deben cumplir un papel importante en la patogénesis.…”

“…La causa de la formación de ampollas sobre la esclerosis no es clara, sin embargo se han postulado varias hipótesis, dentro de las cuales la más aceptada es la obstrucción linfática, soportado por los hallazgos histopatológicos donde se observa ectasia de vasos linfáticos en la dermis papilar, además se ha observado que las extremidades inferiores son el área más comúnmente comprometida, por lo tanto se cree que la dilatación de los canales linfáticos y el aumento de la presión venosa deben cumplir un papel importante en la patogénesis. Se han planteado otras hipótesis referentes a la formación de ampollas como el daño enzimático de la membrana basal secundaria a obstrucción vascular, el depósito de gránulos de la proteína básica mayor de eosinófilos y el daño en estructuras nerviosas secundario a trauma (3,(5)(6)(7)(8).…”

Extragenital bullous and hemorrhagic Lichen sclerosus in a 58-year-old woman - A case report

“…Some authors accepted that Bullous morphea, a rare type of Morphea (Scleroderma circumscripta), are a disease from the same spectrum of LS and described cases of their simultaneous presentation [7]. The association between these diseases of degeneration of connective tissue and sclerosis of the dermis motivated us to look for the presence of serology markers, characteristic for scleroderma in our patient.…”