Mary F. Farley

Birt-Hogg-Dubé syndrome: A review of the literature and the differential diagnosis of firm facial papules

Birt-Hogg-Dubé syndrome (BHDS) was originally described in 1977 as the grouping of 3 skin tumors-the fibrofolliculoma, trichodiscoma, and acrochordon-in family members with an autosomal dominant inheritance pattern. In recent years it has become clear that these 3 lesions likely represent only 1 of these tumors, the fibrofolliculoma. More important, evidence now supports a definite susceptibility to malignant renal tumors and pulmonary disease in patients with BHDS. Clinical recognition of this entity is possible in spite of the fact that several syndromes exist that are characterized by the presence of multiple firm facial papules. We will discuss the evolution of BHDS from the original description to the recent discovery of the genetic susceptibility locus, illustrate the clinical differential diagnosis, and highlight the workup needed for newly diagnosed patients and their family members.

Birt-Hogg-Dubé syndrome: two patients with neural tissue tumors ☆

We present 2 unrelated patients found to have tumors of neural tissue origin, a neurothekeoma and a meningioma, who were additionally diagnosed with the syndrome of Birt-Hogg-Dubé (BHDS). We are unaware of previous BHDS patients with neural tissue tumors. In light of recent linkage analysis studies delineating the genetic susceptibility locus for BHDS, we speculate about a possible association between BHDS and neural tissue tumors.

Surgical Pearl: Purse string suture in the management of poorly delineated melanomas

The purse string suture technique has received little attention in the surgical literature. Its application in dermatologic surgery has been mainly to reduce the size of a surgical defect to allow placement of a smaller, full-thickness skin graft.1-4 We have found the purse string suture to be effective in the management of poorly demarcated melanoma. We have used the purse string technique in 10 patients with biopsy-confirmed melanoma. For most of them, the clinical margin of the neoplasm was poorly defined. All underwent excision of the clinically detectable lesion, using recommended guidelines of 5 mm for in situ melanoma, 1 cm for invasive melanoma with Breslows depth less than 1 mm, and 2 cm for intermediate-thickness melanomas.5,6 The oval surgical defects were then managed with an intradermal circumferential purse string suture, with 3-0 or 4-0 nonabsorbable polypropylene.4 The purse string was tied, thereby either reducing the size of or in some cases completely closing the defect. The pathology reports were received in 48 to 72 hours. If a positive margin was present, the patient returned for additional surgery. The original oval defect was re-created after the removal of the purse string suture. This greatly facilitated a more precise measurement for additional margins and excision of the residual tumor. The newly created larger defect was often managed again with a purse string suture. If clear surgical margins were achieved, the purse string suture was removed after 21 days. The type of final reconstruction and its timing were primarily dependent on size and location of the lesion, as well as the degree of confidence in obtaining clear margins. We used fullthickness skin grafts, flaps, primary closure, and second-intention healing as final reconstructive options. No significant complications were observed. A biopsy-confirmed lentigo maligna on the right cheek (Fig. 1) with poorly definable clinical margins was outlined with the aid of a Woods lamp and excised with a 5 mm margin. The oval From the Dermatology Service, Walter Reed Army Medical Center. The opinions and assertions contained herein are the private ones of the authors and are not to be construed as official or reflecting the views of the Department of Defense or the United States Army. Reprints not available from the authors. J Am Acad Dermatol 1998;38:99-101. 16/74/85559 Surgical Pearl: Purse string suture in the management of poorly delineated melanomas

Anaphylaxis to topically applied bacitracin

Abstract Background: Bacitracin is widely used for the treatment and prevention of superficial skin infections. Although commonly used, anaphylaxis to topically applied bacitracin is rare, with only nine previously reported cases. Most reported reactions have occurred in middle-aged to elderly women with leg ulcers or chronic eczema. Although most reports include a challenge test to confirm the diagnosis, no recommended standardized method of skin testing for bacitracin anaphylaxis has been advocated to date. Objective: We report two patients who developed anaphylaxis to topically applied bacitracin ointment confirmed by skin prick testing. The subject of bacitracin anaphylaxis is reviewed, and a method for confirmatory skin testing is recommended. Methods: Literature was searched in the MEDLINE database using the key words anaphylaxis and bacitracin, and articles from the American Journal of Contact Dermatitis were also included. Relevant references from these reports were also reviewed. Percutaneous skin prick testing was performed on our 2 patients using the bacitracin powder dilution (10 U/mL and 1 U/mL). Normal control individuals were also tested. Results: Two patients with anaphylaxis to bacitracin are reported. One developed symptoms after a biopsy of chronic eczema was performed. The second patient had no prior history of chronic skin disease and developed symptoms after incurring an abrasion on his knee. The diagnosis was confirmed by a positive wheal and flare reaction after skin prick testing at 1 U/mL and 10 U/mL concentrations, respectively. Normal controls showed no wheal and flare reaction at 10 U/mL. Conclusions: Anaphylaxis to topical bacitracin is rare but potentially fatal. Based on our literature review and the results of our skin testing, we recommend initiating future bacitracin prick testing with 0.1 U/mL. If no reaction occurs at this dilution, subsequent prick testing should be serially performed with a 10-fold higher concentration. Each test should be separated by 15 minutes to a maximum concentration of 10 U/mL. Testing at higher concentrations or by the intradermal method may lead to a false-positive test. This is a US government work. There are no restrictions on its use.