PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “Pemphigus, Benign Familial”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 19 recordsLinked to original sources
PubMed2002

Photodynamic therapy with 5-aminolevulinic acid for recalcitrant familial benign pemphigus (Hailey-Hailey disease).

Familial benign pemphigus is a chronic, recurrent, autosomal dominant blistering disease that may significantly affect quality of life. Surgical methods are often needed to control flares in difficult cases. We describe the use of photodynamic therapy (PDT) with topical 5-aminolevulinic acid (ALA) followed by irradiation with incoherent light (ALA-PDT) in 2 patients with chronic, recalcitrant familial benign pemphigus.

Aminolevulinic Acid↗
PubMed2002

Benign familial pemphigus: little benefit from superficial radiotherapy.

Benign familial pemphigus (Hailey-Hailey disease) is a rare relapsing-remitting epidermal blistering disease palliated by a multitude of medical and surgical treatments. There are limited reports of benefit from low-penetration X-rays. We describe two resistant cases that appeared to respond initially to superficial radiotherapy, a 66-year-old man who had multiple courses to the groins (4 x 2.00 Gy/5 x 3.00 Gy) and to the axillae and low back (10 x 2.00 Gy) and a 53-year-old man treated twice to the groins and perineum (5 x 3.00 Gy/10 x 2.00 Gy). However, on longer follow up, benefit was found to be questionable. We conclude that while superficial radiotherapy may offer temporary relief, there is little evidence that it alters the natural history of this disease.

Aged↗
PubMed1988

Chronic benign familial pemphigus.

Ten patients with benign familial chronic pemphigus (BFCP) (Hailey-Hailey disease) were evaluated; semiologic and localization differences were described, and special attention was given to solitary or atypical forms. In all the cases, the diagnosis was confirmed by histopathology; some histopathologic differences and the results achieved by direct immunofluorescence of skin are discussed. Most of the patients responded to the treatment with corticosteroids and antibiotics.

Adult↗
PubMed1998

Benign familial pemphigus (Hailey-Hailey disease). Treatment with the pulsed carbon dioxide laser.

BACKGROUND: Benign familial pemphigus (BFP) is a chronic blistering disease with significant morbidity. Surgical methods are often needed to control flares in difficult cases. OBJECTIVE: To describe the response of BFP to vaporization with a pulsed carbon dioxide (CO2) laser. METHODS: A 38-year-old woman with chest and axillary involvement unresponsive to conventional therapy was treated with the UltraPulse 5000 Laser (Coherent Medical Group, Palo Alto, CA). After active sites of BFP showed good response to treatment, we treated uninvolved skin of the left axilla to assess the efficacy of prophylactic therapy. RESULTS: Treatment of affected areas, except biopsy sites, resulted in clearing of active lesions after 1-2 weeks. We noted striking sparing of the treated areas from developing subsequent disease. The region that was later treated prophylactically has shown minor, asymptomatic recurrence of BFP in less than 5% of the area treated over an 18-month follow-up period. CONCLUSION: The pulsed carbon dioxide laser is a useful modality in treatment of BFP. In our patient, prophylactic treatment led to near complete eradication of disease in the treated area. A controlled, larger study is needed to confirm our results, and to determine optimal laser parameters. Long-term effects and duration of remission remain to be determined.

Adult↗
PubMed1985

[Chronic benign familial pemphigus. Clinical, histological and immunological study of 7 cases].

A clinical, histopathological and immunological study was carried on a series of seven patients of Familial Benign Chronic Pemphigus (FBCP). This condition is characterized by recurrent small blisters, mainly, on intertriginous areas and on the sides of the neck, that become wet and crusted rapidly. They are generally sharply marginated and Nikolsky's sign is often positive. The lesions appear spontaneously and may be precipitated by warm, humid environment, mechanical trauma, radiations, bacterial or mycotic infection. Healing occurs with residual non scarring hyperpigmentation. Histopathologically, the epidermal alteration respond to a primary acantholytic mechanism. Ultramicroscopic studies have suggested an alteration on the desmosome-tonofilament complex. Comparatively with Pemphigus, another acantholytic disease in which immunological pathogenesis is strongly suspected, only few reports are referred to immunological studies in FBCP. In the present paper, a direct immunofluorescent study on spontaneous and provoked blisters was made in order to investigate deposits of immunoglobulins and complement. Indirect IF was performed with sera of teh patients for detection of circulating antibodies. Two cases were also sensitized with erythrocytic antigen. The immunological response to this substance was evaluated. The clinical and histopathological findings of the present series, are similar with previous descriptions. The immunofluorescent studies do not provide evidence of antibodies to epidermal intercellular space, like Pemphigus.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗