Philip C. Don

MALIGNANT SYPHILIS (LUES MALIGNA) AND CONCURRENT INFECTION WITH HIV

Background. During the past 2 1/2 years we observed six patients who had a reactive serology for syphilis, of which four developed widespread noduloulcerative and two vesiculonecrotic lesions. The purpose was to report the occurrence of lues maligna, a rare form of secondary syphilis, in five patients infected with the human immunodeficiency virus (HIV) and in one patient with risk factors for infection.

Cutaneous protothecosis and AIDS

Cutaneous protothecosis is an uncommon algal infection with a varied clinical presentation that occurs mainly in immunocompromised patients. We describe a 33-year-old Hispanic woman with AIDS who had a verrucous plaque on the dorsum of her hand. A biopsy specimen revealed organisms suggestive of Prototheca. Culture confirmed the organism to be Prototheca wickerhamii. To our knowledge, this is the first case of cutaneous protothecosis reported in a patient with AIDS.

Adult Onset of Inflammatory Linear Verrucous Epidermal Nevus in a Mother and Her Daughter

Inflammatory linear verrucous epidermal nevus (ILVEN) is a rather uncommon dermatosis that typically has an early age of onset, is unilateral, localized, pruritic and relatively refractory to treatment. Atypical presentations of ILVEN have also been described and include late onset in life, widespread involvement and response to treatment. We report the adult onset of an extremely pruritic systemized eruption in both mother and her daughter that clinically and histologically was most compatible with ILVEN. The eruptions were also partially responsive to therapy.

NAIL AND SKIN HYPERPIGMENTATION ASSOCIATED WITH HYDROXYUREA THERAPY FOR POLYCYTHEMIA VERA

A 63‐year‐old black woman presented with a complaint of nail and skin darkening for 5 months. Past medical history included polycythemia vera, hypertension, angina, and hi‐atal hernia. For several years the patients medications had included isosorbide dinitrate, iron sulfate, propranolol, and sucralfate. The only recent new medication was hydrox‐yurea (500 mg to 1.5 g daily), which the patient had started for polycythemia vera 8 months prior to presentation. She now complained of asymptomatic hyperpigmentation of the finger and toenails, which had begun proximally and extended distally over time. She also noted increased pig‐mentation of her face, neck, arms, and buccal mucosa.

Extensive calcinosis cutis in association with systemic lupus erythematosus.

Sir, Calcium deposits in the skin sometimes occur is association with certain connective tissue diseases, particularlyscleroderma and dermatomyositis (1). This ® nding is extremely rare in systemic lupus erythematosus (SLE) (2). In most cases of calcinosis cutis in SLE, the deposition of calcium is usually seen under the cutaneous lupus lesions, and the amounts are relatively small. We report here a 49-year-oldwoman featuring SLE with extensive subcutaneous and muscular calci® cations. Over time, the calci® cation became more diŒuse and extended into the subcutaneous and muscular layer of her pelvis and extremities, forming large plate-like calci® cations. Only 16 such cases of extensive calcinosis cutis in SLE have been reported in the literature (3± 12).

PROXIMAL WHITE SUBUNGUAL ONYCHOMYCOSIS IN THE IMMUNOCOMPETENT PATIENT : REPORT OF TWO CASES AND REVIEW OF THE LITERATURE

Sir, Proximal white subungual onychomycosis (PWSO) is the rarest subtype of onychomycosis (1). In PWSO, the infection begins with fungal invasion of the stratum corneum of the proximal nail fold, followed by infection of the deeper portions of the nail plate. This presentation of onychomycosis is most commonly caused by T. rubrum; other common causative agents include T. megninii, T. schoenleinii, T. tonsurans, T. mentagrophytes, and E. £occosum (1, 2). The majority of initial cases of PWSO reported were patients with AIDS (1 ^ 3). In one study, 55 of 62 HIV-infected patients with onychomycosis (83.7%) had PWSO (4). In more than half of these patients (58%), T. rubrum was the etiologic agent. PWSO has also been reported in patients with other immunode¢ciencies, including a renal transplant recipient on immunosuppressive therapy (5), and a patient with systemic lupus erythematosus on systemic steroids (6). In addition, Baran (7) described a case of proximal subungual candida onychomycosis as a manifestation of chronic mucocutaneous candidiasis. Recently, however, there have been several reports of immunocompetent patients developing PWSO (8 ^ 10). We now report two immunocompetent patients with proximal white subungual disease, and review the other cases described, emphasizing that not all patients with this presentation are immunode¢cient.

Treatment of tufted angioma with interferon alfa: role of bFGF.

To the Editors: Tufted angioma was first described in 1989 by Wilson Jones and Orkin (1) as a distinct vascular lesion that is characterized by circumscribed lobules of hypertrophied capillaries with a pericyte component. Tufted angiomas most commonly arise on the neck and upper trunk in young persons. Over half of patients develop the lesions within the first 5 years of life, and only three patients previously reported have had lesions at birth. These lesions typically undergo a growth phase, characterized by lateral extension, for 5 months to 10 years (2). Treatment of tufted angioma is usually unsatisfactory. Recently Suarez et al. (2) reported a good response to treatment with interferon alfa. Despite low initial levels of urinary bFGF, a successful trial of interferon alfa was initiated. We report a patient with a tufted angioma and elevated urinary bFGF who underwent a trial of interferon alfa. The patient demonstrated a marked decrease in urinary bFGF levels, but no clinical response. This observation indicates that other growth factors in addition to bFGF contribute to the development of tufted angiomas. A 12-year-old white girl presented for evaluation of a congenital lesion involving her right thigh. The lesion had significantly increased in size over a 2-year period. The patient had no additional past medical history. On physical examination, a 15 cm x 15 cm, nontender, purple plaque was present on the right anterior and lateral thigh and knee. Both limbs were of equal circumference. No bruits or thrills were detected at the site. A biopsy specimen of the lesion revealed focal collections of tightly packed capillaries throughout the dermis. Multiple rounded lobules of poorly canalized capillary channels were widely distributed. Baseline MRI revealed vascular proliferation limited to the subcutaneous fat without extension into the muscle layers, synovial space, or bone structure. However, the subcutaneous fat layer was enlarged as a result of increased vascular volume present in the area. The patient was referred to hematology/oncology for a trial of interferon alfa. Baseline urinary bFGF level was 4500 pg/g Cr. She received a total course of 12 weeks of daily subcutaneous injections of interferon alfa 2 to 3 Mu/m2 without significant change in the size of the lesion over a 3-month period. Two urinary bFGF levels of less than 500 pglg Cr were obtained while the patient was on treatment, which were significantly less than her baseline level. Many treatments have been used for tufted angioma, including surgical excision, x-ray therapy, dye laser, cryosurgery, and electrocautery, all of which have been associated with recurrences of the lesion (2). Treatment with topical or intralesional corticosteroids has been reported to have no effect. Interferon alfa has been shown to dramatically decrease the size of cutaneous hemangiomas in expanding lesions (3), and Suarez et al. (2) applied this therapy successfully in the treatment of tufted angioma. In addition to this case, there is one further case of successful treatment with interferon alfa (4), although one case failed to respond to subcutaneous injections (5 ) and one to intralesional injections of interferon alfa (6). Levels of urinary bFGF are often elevated in patients with significant capillary proliferation, as in early hemangioma, and tend to diminish to normal during treatment with interferon alfa. Our patient had an initial bFGF level of 4500 pg/g Cr which decreased to less than 500 pg/g Cr after treatment with interferon alfa. However, despite this decrease, our patient failed to demonstrate any resolution of the lesion. In the case of Suarez et al. (2), interferon was successful despite normal bFGF levels. Both of these cases suggest that other factors are involved in the pathogenesis and regulation of capillary proliferation in tufted angiomas and that further investigation is required to elucidate its pathophysiology. This will enable the formulation of better therapeutic options.

Cutaneous cryptococcosis mimicking basal cell carcinoma in a patient with AIDS.

Background: Cryptococcosis is an opportunistic infection caused by the encapsulated yeast Cryptococcus neoformans. This ubiquitous organism has emerged as a frequent finding in immunosuppressed patients, especially those with underlying malignancies, organ transplants, and the acquired immune deficiency syndrome (AIDS). Cutaneous manifestations of cryptococcosis occur in 10 to 15% of patients having systemic involvement. These skin lesions may simulate a variety of different disease entities. Methods: A case of crytococcosis mimicking a basal cell carcinoma is the subject of a case report presentation. Results: A case of cutaneous cryptococcosis mimicking basal cell carcinoma occurred in a patient with AIDS, who did not appear to have dissemination, but was treated aggressively to stem possible occult systemic disease. Conclusion: Cutaneous crytococcosis may mimic other dermatologic disorders.

Resolution of pyoderma gangrenosum after therapy with lyophilized bovine collagen matrix

Pyoderma gangrenosum (PG) is an uncommon ulcerative skin disease of unknown etiology. Current available treatment for pyoderma gangrenosum is far from satisfactory. Only two major drugs, corticosteroids and cyclosporine, have proved effective in the treatment of this disease. However, they may be poorly tolerated and are associated with significant adverse effects. We describe a patient who experienced continued pain and disfigurement from PG, which was refractory to prior treatments. She was successfully treated with topical lyophilized bovine collagen matrix, with complete healing of all treated ulcers. A 34-year-old woman with a 3-year history of PG presented to the Dermatology Clinic. The patient experienced severe discomfort from her lesions. A previous work-up had demonstrated no systemic associations. On physical examination, the patient had violaceous ulcerations on the face, mid-back, and right and left arms. The patient was initially treated with dapsone 75 mg/day as well as intralesional kenalog 10 mg/ml to several of the lesions. The patient was followed for the next several months, with an increased dose of dapsone to 100 mg/day and continued intralesional injections. She demonstrated no significant healing of her lesions. Therapy with lyophilized bovine collagen matrix was initiated. Several of the patient’s ulcers were filled with lyophilized collagen matrix particles (Medifil, Biocore Inc., Topeka, Kans., USA) moistened with normal saline and covered with a nonstick dressing. The particles were rinsed away, and a new layer of particles and dressing was applied every other day. As the ulcerations became more superficial, the dressing was changed to a nylon mesh with an associated porous collagen membrane (SkinTemp, Biocore Inc.) and continued until the wounds had epithelialized. While treatment with the collagen dressings was carried out, the dose of dapsone was tapered gradually to 50 mg every other day. After 4 weeks, the patient had complete healing of all treated ulcerations, with the appearance of hypertrophic scars at the sites of the ulcers. Of note, there was no change in the lesions which were not treated with lyophilized bovine collagen matrix. The ulcerative form of PG is most common and typically begins as one or more sterile pustules with an inflammatory halo, which evolves rapidly into painful necrotic ulcers with undermined violaceous margins. Approximately 50% of patients with PG have an associated systemic disease, including inflammatory bowel disease, arthritis and myeloproliferative disorders [1]. PG exhibits ‘pathergy’, manifested by the localization of lesions to sites of skin damaged by trauma, surgery or venipuncture [2]. Local therapy alone may be sufficient to control early or mild lesions [3]. Measures usually include wound care with appropriate dressings, and prevention or treatment of secondary bacterial infection, as well as other therapies. These include intralesional [4, 5] and topical [6, 7] steroids, topical sodium cromoglycate [8, 9], intralesional cyclosporine [10], topical nitrogen mustard [11] and a nicotine patch [12]. Treatments with cultured allografts [13] and split-thickness skin grafts [14] have been used in combination with systemic immunosuppressive therapy. Systemic therapies commonly used in PG include prednisone, dapsone, cyclosporine, azathioprine and clofazimine [1, 15]. SkinTemp and Medifil (Biocore Inc.) are different forms of lyophilized type I bovine collagen matrix. Medifil consists of spherical hydrophilic particles of collagen 0.1–0.3 mm in diameter which can be used to fill wounds of variable depth. SkinTemp is a composite nylon mesh to which a porous collagen membrane is attached. It comes in various sizes and can be cut to fit over the surface of more superficial wounds. Bovine collagen matrix has been shown to speed secondary-intention wound healing for Mohs’ surgical wounds compared with traditional wound care [16] and for closure of facial defects [17]. In a controlled clinical trial of 72 patients, wounds of various etiologies healed significantly faster with type I bovine collagen compared to dextranomer [18]. In another study, chronic leg ulcers healed significantly faster with lyophilized type I bovine collagen compared to hydrocolloids [19]. Heterologous collagen matrices work by several mechanisms. The three-dimensional collagen network provides a scaffold for fibroblastic proliferation and production of a new collagen network [20], as well as aggregation of platelets and coagulation factors to promote hemostasis [21]. In vitro, increased fibronectin binding stimulates migration of fibroblasts, and denatured bovine collagen contains more fibronectin binding sites. Also, the degeneration of the collagen matrix may promote fibroblast migration because collagen degradation products are chemotactic factors for stromal fibroblasts [20]. The three-dimensional scaffold and chemotactic factors both probably assist more rapid migration of epithelial cells [21]. Collagen products are also a poor culture medium and do not support or enhance bacterial growth [22]. The management of PG depends on its type and severity, and usually requires aggressive local and systemic therapy. Use of a lyophilized bovine collagen dressing alone may not be sufficient for treatment of more severe variants of PG. However, given our patient’s response after failing other therapies, and the utility of bovine collagen in the treatment of various types of wounds, it would be reasonable to consider use of a bovine collagen wound dressing as either monotherapy, or as an adjunct with other local or systemic therapies.

Pedunculated malignant melanoma

Background. The pedunculated melanoma is an unusual variant of nodular melanoma that presents a challenge in staging and management. Objective. We discuss the clinical and histopathologic characteristics of a case of pedunculated melanoma and present a brief review of the literature. Methods. Routine stain with hematoxylin and eosin was performed on tissue specimens. Results. The pedunculated melanoma was excised. Sentinel lymph node dissection was performed and was negative for the presence of melanoma. Conclusions. Pedunculated melanoma is a rare type of melanoma. Conventional staging methods for melanoma may not be reliable in this type of tumor. Complete workup, possibly including sentinel lymph node dissection, should be performed in all patients with pedunculated melanomas.