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Cicatricial pemphigoid is a chronic, scarring, vesiculobullous eruption, with a theoretical autoimmune cause. It is closely related to bullous pemphigoid, yet unlike bullous pemphigoid, no circulating antibodies have been found. We present here a patient with cicatricial pemphigoid with circulating antibodies to the basement membrane zone of the skin, thus giving more evidence for it being an immunologic disease and for rationale for treatment.

A comparative ultrastructural study of bullous lesions of four cases of Cicatricial Pemphigoid and three cases of Bullous Pemphigoid showed: a) in Bullous Pemphigoid the blister seems to form in the intermembrane space while the basal lamina is still visible on the floor of the blister, b) in Cicatricial Pemphigoid it is the basal lamina which appears to be directly affected, its disappearance probably being the consequence of a marked edema of the superficial dermis.

To the Editor.— Despite the unusual feature of scarring, the case reported by Trepanier 1 may well be one of localized bullous dermatitis herpetiformis. Certainly its histopathology, chronicity, pruritus, and response to sulfone therapy speak for that diagnosis. I would not, however, eliminate the diagnosis of cicatricial pemphigoid because of the absence of basement zone antibodies on indirect immunofluorescent staining. The work showing the presence of such antibodies has been done with bullous pemphigoid, which, while sharing subepidermal bulla formation, is a different condition from cicatricial pemphigoid. A point of clinical distinction that may be useful in the differential diagnosis of cases similar to the one presented by Trepanier is the occurrence of milia in the scars of cicatricial pemphigoid and their absence in the healed areas of dermatitis herpetiformis. This is exemplified by a photograph that accompanies the report 2 in the "Society Transactions" of a patient with cicatricial

To the Editor.— I was pleased to read of Dr. Sams' interest in my article that appeared in theArchivesand would like to reply to his questions about the diagnosis in this patient. I believe there is little question that this patient has cicatricial pemphigoid. She has a chronic bullous, erosive disease that has lasted nine years and resulted in involvement and scarring of skin, oral mucosa, vaginal and cervical mucosa, pharynx, larynx, and esophagus. In the differential diagnosis of oral cicatricial pemphigoid, there are five diseases 1 : (1) pemphigus vulgarus, (2) erythema multiforme, (3) lichen planus, (4) bullous pemphigoid, and (5) desquamative gingivitis. The first three are easily eliminated from consideration in this case by clinical presentation and histopathologic findings. Dr. Sams implies that this patient might have bullous pemphigoid. However, bullous pemphigoid rarely lasts nine years; rarely involves cervix, larynx, or esophagus; does not cause severe

A slowly progressive disease characterized clinically by a noninflammatory process, produced over many years unique annular atrophic disfiguring plaques of the face in two patients. The histopathological findings of many lesions revealed sclerosis and fibrosis of the upper and middle dermis, mild lymphocytic inflammation, and atrophy of the epidermis and hair follicles. Severe solar elastosis was a prominent feature in most biopsies and may be an important factor. No evidence of photosensitivity, porphyria, cicatricial pemphigoid, lichen planus, or any significant systemic disease was present. The disease did not respond to corticosteroids, antimalarials, or many other forms of therapy. Many consultants in pathology suggested various diagnoses: morphea, lichen sclerosus et atrophicus, and discoid lupus erythematosus.

The clinical and laboratory data on 81 patients with benign mucous membrane pemphigoid (BMMP) seen during the years 1950 through 1968 were reviewed. Mucous-membrane and cutaneous involvements were variable, but the oral mucosa and conjunctivae were eventually involved in approximately 75% of the cases. Scarring was a common complication of mucosal and cutaneous lesions. The most severe sequela was blindness. Of the 81 patients in this series 21 became blind, 17 in both eyes. Systemic administration of corticosteroids, used in 33 cases, was the single treatment of greatest benefit. Daily doses equivalent to 40 mg of prednisone were required initially to retard or arrest the disease. Of the various corticosteroid preparations employed, triamcinolone was the most effective. There were no deaths attributed to BMMP, but one patient died from the side effects of corticosteroid therapy.

Bullous Pemphigoid. Presented by Irving D. Ehrenfeld, MD. The patient, a 73-year-old woman, first became aware of blisters on her left breast four months ago. There are several bullae on a non-erythematous base overlying the lower half of the areola and extending to the adjacent skin of the left breast. Histopathologic examination was interpreted as showing "bullous pemphigoid." Neither topical application of 0.5% triamcinolone acetonide cream nor intralesional injection of triamcinolone has had any effect. Grenz ray therapy (200 rads) has been given five times at weekly intervals without any noticeable response. Discussion Samuel B. Frank, MD: I think that this is a case of cicatricial pemphigoid. Indirect immunofluorescent studies can help differentiate the two processes in that results of such studies usually are abnormal in bullous pemphigoid and are normal in cicatricial pemphigoid. Harvey J. Cohen, MD: There is atrophic scarring in the involved site, further supporting the diagnosis

Lever (1951, 1953) first clearly differentiated ocular pemphigus, which he renamed benign mucous membrane pemphigoid and, more recently, cicatricial pemphigoid, from pemphigus vulgaris. He pointed out the benign course, the subepidermal position of the bullae, and, most important of all, the tendency for the bullae to form scars on mucosae and skin. Church and I (1953) drew attention to the lesions on mucous membranes other than the conjunctiva and confirmed (1956) Lever's observations that there were 2 types of skin lesions. The most common is a generalized bullous eruption of short duration which is similar to the bullous eruption of pemphigoid. The second is a localized erythematous plaque, the site of recurring bullae, which may become scarred or ulcerated. Such ulceration is superficial, moist, and with little evidence of epithelialization. The localized skin lesions occur near affected mucous surfaces and are not unusual on the scalp. Lortat-Jacob (1958) stressed that

Two unusual, striking, and puzzling cases of persistent denudation of the scalp have been followed for 5 and 10 years respectively by 2 of us (A. H. S. and J. W. B.). The first patient was presented before the Chicago Dermatological Society in February, 1955, as: "A Case for Diagnosis— Pemphigus Vulgaris?" 1 This led to the comparative study of the second such extraordinary clinical manifestation. The diagnosis of pemphigus is often made only after enough time has elapsed to complete the characteristic picture. Senear 2 has pointed out the effectiveness of certain well-accepted criteria for diagnosis in certain cases. When the disorders of the pemphigoid group are added to the differential problem, however, the dilemma becomes compounded. The present report concerns 2 cases of cicatricial pemphigoid. Both patients displayed massive nonhealing erosive lesions affecting the major portions of the scalp. Report of Cases Case 1.— A 56-year-old white woman

Direct immunofluorescent staining of specimens from the conjunctiva, buccal mucosa, and skin of four patients with cicatricial pemphigoid revealed the presence of tissue-fixed, basement membrane zone antibodies similar to the basement membrane zone fluorescent staining characteristic of bullous pemphigoid. No circulating basement membrane zone antibodies could be detected in the sera of these patients. These findings suggest a relationship between cicatricial pemphigoid and bullous pemphigoid and that cicatricial pemphigoid may represent a forme fruste of bullous pemphigoid with predilection for mucous membrane and a tendency to scarring.