|Year : 2000 | Volume
| Issue : 6 | Page : 304-305
Brunsting Perry Cicatricial Pemphigoid
RR Mittal, Jastinder Kullar, PS Sethi
R R Mittal
Source of Support: None, Conflict of Interest: None
Two cases of Brunsting Perry cicatricial pemphigoid are being reported for their rarity. Initially as a clearcut clinical diagnosis was not possible, both cases were labelled as a typical vesiculo-bullous disorder. Later only after repeated biopsies, dermoepidermal junction split, fibrosis and perivascular mononuclear infiltrate were seen and a diagnosis of cicatricial pemphigoid was established which was further confirmed by immunoflurescent studies. A favourable response to dapsone was observed in both cases.
Keywords: Pemphigoid, Brunsting Perry Cicatricial Pemphigoid
|How to cite this article:|
Mittal R R, Kullar J, Sethi P S. Brunsting Perry Cicatricial Pemphigoid. Indian J Dermatol Venereol Leprol 2000;66:304-5
|How to cite this URL:|
Mittal R R, Kullar J, Sethi P S. Brunsting Perry Cicatricial Pemphigoid. Indian J Dermatol Venereol Leprol [serial online] 2000 [cited 2020 Jun 5];66:304-5. Available from: http://www.ijdvl.com/text.asp?2000/66/6/304/4954
| Introduction|| |
Brunsting Perry cicatrical pemphigoid (BPCP), a variant of cicatrical pemphigoid (CP) in the elderly, is a condition where pruritic, recurrent crops of bullae on one or several, circumscribed, erythematous, plaques which heal with atrophic scars and confined mainly to head and neck are seen. Histopathologically subepidermal blisters with prominent perivascular infiltrate of lymphocytes, polymorphonuclears, eosinophils and prominent fibrosis in late stages are observed. Positive basement membrane zone immunofluorescent staining with IgG conjugates in 6 cases of BPCP was observed. However negative immunofluorescence was also reported., BPCP was thought to be a more benign, localised type of CP in the bullous pemphigoid to cicatricial pemphigoid spectrum of diseases. Exact relationship can be established only after identification of antigens. Good response to both dapsone and sulphamethoxy pyridazine is observed in CR.
| Case Report|| |
Case 1- A 74-year-old man was admitted in Dermatovenereology dept. of R.H., Patiala with persistent, moderate to intense pruritus associated with bilateral, asymmetrical, 3-6 cm in diameter, well defined, erythematous, urticarial plaques over scalp, beard area, face and upper trunk since 6 months. Crops of recurrent vesiculo bullous lesions localised to the urticarial plaques with a predilection for the periphery were observed. The vesicles persisted, enlarged for 3-4 days, turned into 1-2 cm thick walled tense bullae containing haemorrhagic fluid, later ulcerated and were covered with haemorrhagic crusts. Nikolsky's sign was negative. General physical and systemic examination was normal. Routine investigations, except for eosinophilia and ESR 54 mm in the 1st hour, were normal. X-ray chest and ultrasonography abdomen were normal. The first biopsy showed mild hyperkeratosis, normal epidermis, oedema in the upper dermis with cleavage at the dermoepidermal junction and focal mononuclear plus plasma cell infiltrate around thick blood vessels associated with proliferation of endothelial cells. Second biopsy revealed totally denuded epidermis with peripheral cleavage at the dermoepidermal junction, moderate to marked capillary proliferation, periadnexal and perivascular cuffing with monouclears, eosinophils, oedema of dermis and prominent fibrosis of the deep dermis. The third biopsy showed mild hyperkeratosis, a big split at dermoepidermal junction filled with fibrinoid material, mononuclears, plasma cells, neutrophils and occasional eosinophils. The dermis revealed enhanced capillary proliferation, perivascular mononuclear infiltrate, extravasated RBCs, increased dermal collagen and fibrosis. Direct immunofluoresence showed IgG and C3 deposition at the dermoepidermal junction.
Some ulcers and bullae healed with superficial scars after 2 weeks of therapy with 15 mg prednisolone daily but later responded better to dapsone and tetracycline combination.
Case 2. A 50-year-old woman had pruritus, and vesiculobullous eruptions since 6 months. There were urticarial erythematous plaques which were bilateral, asymmetrical, localised to the creases of neck, inframammary region and scalp. The lesions were 3-5 cm in diameter, well demarcated, erythematous, studded with thick walled tense bullae containing haemorrhagic fluid, ulcers, crusts and scars. Peripheral extension of the ulcerated areas after rupture of the bullae was observed. The scalp lesions healed with cicatricial alopecia. A few transient mucosal erosions were seen on the palate. Nikolsky's sign was negative. General physicial and systemic examination was normal. Routine investigations (except for eosinophilia and an ESR of 26 mm in 1st hour), X-ray chest and USG abdomen were normal. The first biopsy showed orthokeratosis, at a few places denuded epidermis with peripheral dermoepidermal junction cleavage, moderate to dense, perivascular and periadnexal, mononuclear and plasma cell infiltration. Second biopsy revealed denuded epidermis, a cleft in the upper most dermis filled with haemorrhagic material, increased vascularity with perivascular mononuclear cell cuffing and extravasated RBCs. The deep dermis revealed marked fibrosis. Direct immunofluorescence showed IgG deposition at the dermoepidermal junction. The patient responded favourably to dapsone 150mg daily.
| Discussion|| |
In the above cases, prolonged observation in the ward and repeated biopsies could establish the diagnosis of BPCP, which is a rare disease, characterised by crops of recurrent, tense vesicles and/or bullae localised to urticarial plaques, commonly on the head and neck.
Clinically, SLE was excluded as history of photosensitivity was negative, systemic examination was normal and both the patients revealed crops of pruritic, vesiculobullous lesions limited only to urticarial plaques. Bullae extended for 3-4 days, contained haemorrhagic fluid, ruptured with haemorrhagic crusts and healed with scars.
Histopathologically cleavage at the dermoepidermal junction and perivascular mononuclear infiltrate can occur in SLE also, but normal epidermis/ denuded epidermis, capillary proliferation, extravasation of RBC's increased dermal collagen and prominent fibrosis helped us in establishing the diagnosis of BPCP. Finally diagnosis was confirmed by direct immunofluorescence which showed IgG and C3 deposition at the dermoepidermal junction.
| References|| |
|1.||Brusting LA, Perry HO. Benign pemphigoid? Arch Dermatol 1957; 75: 489-501. |
|2.||Lever WF, Lever GS. Non infectious vesicular and Bullous diseases. In: Histopathology of the skin. 7th ed, Philadelphia: JB Lippincott Company 1990; 130-131. |
|3.||Michel B, Bean SF, Chorzelski T, et al. Cicatricial pemphigoid of Brunsting Perry. Arch Dermatol 1977; 113: 1403-1405. |
|4.||Mac Vicar DN, Graham JH. Localised chronic pemphigoid: A clinicopathologic histochemical study. Am J Pathol 1966; 52a: 48-50. |
|5.||Trepanier Y. Localised bullous dermatitis herpetiformis. Arch Dermatol 1970; 101:98-99. |
|6.||Venning VA. Fritch PA, Bron AJ. Muosal involvement in bullous and cicatricial pemphigoid. Br J Derm 1988; 118: 7-15. |
|7.||Mc Fadden JP, Leonard IN, Powles AV, et al. Sulphamethoxy pyridazine for dermatitis herpetiformis, linear IgA disease and cicatricial pemphigoid. Br J Derm 1989; 121: 759-762. |