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Coexistence of mucous membrane pemphigoid and vitiligo.

Aithal S, Ganguly S, Kuruvila S - Indian Dermatol Online J (2014)

Bottom Line: Mucous membrane pemphigoid describes a rare heterogeneous group of chronic, inflammatory, mucous membrane-dominated, subepithelial blistering diseases that manifest a varying constellation of oral, ocular, skin, genital, nasopharyngeal, esophageal, and laryngeal lesions.Life-threatening airway obstruction and sight-threatening ocular scarring can occur in this condition, which is rarely reported in Indian literature.Vitiligo is associated with a number of disorders also considered to be autoimmune.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Venereology and Leprosy, Pondicherry Institute of Medical Sciences, Pondicherry, India.

ABSTRACT
Mucous membrane pemphigoid describes a rare heterogeneous group of chronic, inflammatory, mucous membrane-dominated, subepithelial blistering diseases that manifest a varying constellation of oral, ocular, skin, genital, nasopharyngeal, esophageal, and laryngeal lesions. Life-threatening airway obstruction and sight-threatening ocular scarring can occur in this condition, which is rarely reported in Indian literature. Vitiligo is another acquired autoimmune disorder characterized by loss of melanocytes. Vitiligo is associated with a number of disorders also considered to be autoimmune. Here we report a very rare coexistence of MMP and vitiligo, the first such report from India.

No MeSH data available.


Related in: MedlinePlus

Subepidermal bulla with fibrosis beneath it, black arrow demonstrates the site of blistering (H and E, ×10)
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Figure 4: Subepidermal bulla with fibrosis beneath it, black arrow demonstrates the site of blistering (H and E, ×10)

Mentions: A 37-year-old male patient presented with complaints of redness and pain in his left eye since two years, painful erosions in the oral cavity since six months, and ulcers over the scrotum and right knee since two months. On ocular examination, there was congestion of the left sclera mainly on the lateral side along with a fibrotic band connecting the upper palpebral conjunctiva with the bulbar conjunctiva near the lateral canthus [Figure 1]. On examination of the oral cavity there were widespread erosions involving the inner side of lips, gingival, and labial mucosa [Figure 2]. Detailed examination revealed a single depigmented macule over the glans penis [Figure 3], which according to the patient appeared insidiously around 8 years ago and remained static. Based on the clinical features, a diagnosis of mucous membrane pemphigoid was considered. A Tzanck smear from the oral erosions, done to rule out pemphigus vulgaris, failed to show any acantholytic cells. Subsequently, the patient developed multiple tense vesicles and bullae over his left ear. In addition there was involvement of nasal mucosa, larynx, scrotal skin, perianal skin, and right knee [Figure 2]. A skin biopsy from the right ear showed subepidermal bullae and fibrosis beneath it [Figures 4 and 5]. The direct immunofluroscence of perilesional uninvolved skin showed strong linear deposits of IgG along the basement membrane zone. Detailed hematological, biochemical, and imaging studies did not reveal any evidence of malignancy. The patient was placed on a combination regimen of oral corticosteroid and daily cyclophosphamide. The long-standing depigmented macule over the glans penis did not show any evidence of atrophy, ruling out lichen sclerosus et atrophicus. Hence it was diagnosed as mucosal vitiligo and the patient was counseled regarding it.


Coexistence of mucous membrane pemphigoid and vitiligo.

Aithal S, Ganguly S, Kuruvila S - Indian Dermatol Online J (2014)

Subepidermal bulla with fibrosis beneath it, black arrow demonstrates the site of blistering (H and E, ×10)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4228648&req=5

Figure 4: Subepidermal bulla with fibrosis beneath it, black arrow demonstrates the site of blistering (H and E, ×10)
Mentions: A 37-year-old male patient presented with complaints of redness and pain in his left eye since two years, painful erosions in the oral cavity since six months, and ulcers over the scrotum and right knee since two months. On ocular examination, there was congestion of the left sclera mainly on the lateral side along with a fibrotic band connecting the upper palpebral conjunctiva with the bulbar conjunctiva near the lateral canthus [Figure 1]. On examination of the oral cavity there were widespread erosions involving the inner side of lips, gingival, and labial mucosa [Figure 2]. Detailed examination revealed a single depigmented macule over the glans penis [Figure 3], which according to the patient appeared insidiously around 8 years ago and remained static. Based on the clinical features, a diagnosis of mucous membrane pemphigoid was considered. A Tzanck smear from the oral erosions, done to rule out pemphigus vulgaris, failed to show any acantholytic cells. Subsequently, the patient developed multiple tense vesicles and bullae over his left ear. In addition there was involvement of nasal mucosa, larynx, scrotal skin, perianal skin, and right knee [Figure 2]. A skin biopsy from the right ear showed subepidermal bullae and fibrosis beneath it [Figures 4 and 5]. The direct immunofluroscence of perilesional uninvolved skin showed strong linear deposits of IgG along the basement membrane zone. Detailed hematological, biochemical, and imaging studies did not reveal any evidence of malignancy. The patient was placed on a combination regimen of oral corticosteroid and daily cyclophosphamide. The long-standing depigmented macule over the glans penis did not show any evidence of atrophy, ruling out lichen sclerosus et atrophicus. Hence it was diagnosed as mucosal vitiligo and the patient was counseled regarding it.

Bottom Line: Mucous membrane pemphigoid describes a rare heterogeneous group of chronic, inflammatory, mucous membrane-dominated, subepithelial blistering diseases that manifest a varying constellation of oral, ocular, skin, genital, nasopharyngeal, esophageal, and laryngeal lesions.Life-threatening airway obstruction and sight-threatening ocular scarring can occur in this condition, which is rarely reported in Indian literature.Vitiligo is associated with a number of disorders also considered to be autoimmune.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Venereology and Leprosy, Pondicherry Institute of Medical Sciences, Pondicherry, India.

ABSTRACT
Mucous membrane pemphigoid describes a rare heterogeneous group of chronic, inflammatory, mucous membrane-dominated, subepithelial blistering diseases that manifest a varying constellation of oral, ocular, skin, genital, nasopharyngeal, esophageal, and laryngeal lesions. Life-threatening airway obstruction and sight-threatening ocular scarring can occur in this condition, which is rarely reported in Indian literature. Vitiligo is another acquired autoimmune disorder characterized by loss of melanocytes. Vitiligo is associated with a number of disorders also considered to be autoimmune. Here we report a very rare coexistence of MMP and vitiligo, the first such report from India.

No MeSH data available.


Related in: MedlinePlus