Published Web Locationhttps://doi.org/10.5070/D33r74n91z
From New York University Department of Dermatology
Thomas C. Gallagher
Dermatology Online Journal 8(2): 11
This 81-year-old woman complained of a problem involving her lowerabdomen, lateral and anterior thighs, inguinal folds, intergluteal cleft, and popliteal fossae. Five years ago, the patient developed an indurated plaque on the left anterior thigh. The lesion slowly enlarged, and new plaques gradually appeared. She has noted progressive difficulty walking as the plaques have spread. Approximately six months ago, she first noted the vesicles and bullae in the abdominal and thigh plaques. Previous treatments have included medium and high potentcy topical glucocorticoids with miminal improvement. UVA phototherapy will be initiated.
|Figure 1||Figure 2|
Multiple, hypopigmented, indurated plaques with scale were noted. There were several vesicles and bullae overlying the hypopigmented plaques of the abdomen and thigh.
An antinuclear antibody test was negative.
There is dermal sclerosis, a sparse perivascular infiltrate, papillary dermal edema, and a focal subepidermal blister. No adnexal structures are present. A direct immunofluorescence test shows no abnormal epidermal, basement-membrane zone, or vascular deposits of IgG, C3, IgM, IgA, or fibrin.
Bullous morphea is a rare disease, which occurs most commonly on the lower extremities and inferior abdomen. Fewer than 100 cases have been reported in the world literature.
While the etiology of bullous formation is not certain, lymphatic obstruction from the sclerodermatous process is considered the likeliest cause. Pautrier also suggested that vascular changes like arteritis and phlebosclerosis play a role in bullae formation. This hypothesis gains credence from the hemorrhagic nature of many morphea bullae. O'Leary found a correlation between local trauma and blister formation. Daoud, et al, found the eosinophil granule component major basic protein (MBP) in the base of morphea blisters. They concluded that major basic protein is responsible for blister formation in at least some cases of morphea.
Treatment of bullous morphea is unsatisfactory. A letter to Clin Exp Dermatol suggested that salazopyrin has some efficacy. Daoud, et al, found variable success with hydroxychloroquine. Broad-band low-dose UVA phototherapy and UVA1 phototherapy have efficacy in morphea. It is likely that bullous morphea would show a similar response as multiple authors have concluded that morphea and bullous morphea are distinct manifestations of the same disease.
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