Keratoacanthoma centrifugum marginatum of the lower extremity treated with Mohs micrographic surgery.
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Biomedical subjects
Publications and source records attributed to R P Kaplan.
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We report a case of drug-induced pemphigus caused by an angiotensin-converting enzyme inhibitor, captopril. The cutaneous reaction remitted after withdrawal of captopril therapy. Unique to this case, however, was the substitution of another angiotensin-converting enzyme inhibitor, enalapril, without exacerbation of the pemphigus. To the best of our knowledge, this is the first reported patient with captopril-induced pemphigus in whom no new lesions developed after subsequent treatment with enalapril. A difference in chemical structure between these two drugs, particularly of a sulfur moiety, may help explain why the drug-induced disease did not recur.
A 7-week-old infant with antecedent otitis media, upper respiratory infection, and aseptic meningitis was diagnosed as having Sweet syndrome. Although this disease usually affects adults, it has been reported in 17 children. This is the youngest reported patient with the disorder to date, and the first in whom the syndrome was associated with aseptic meningitis.
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A 77-year-old woman developed Kaposi's sarcoma (KS) during prednisone therapy for pemphigus foliaceus. There are 7 prior reports in the literature of KS occurring in association with pemphigus. There are multiple reports of KS occurring in patients without pemphigus who are immunosuppressed either by malignancy or by chemotherapy for various disorders. Although the relationship between KS and pemphigus is a real one, KS is more likely related to therapeutically induced immunosuppression in certain predisposed individuals rather than primarily to pemphigus itself.
A cutaneous ciliated structure from the lower neck of a 61-year-old man having seromucous, sebaceous and respiratory-type differentiation is reported. The finding of continuity between endodermal and ectodermal structures in the reported lesion suggests an origin from the branchial apparatus. Many lesions with similar appearance have been labeled bronchogenic though these, in fact, may have branchial origins. The term cutaneous bronchogenic cyst, implying an origin from the tracheobronchial tree, maybe misleading and should be reconsidered for those lesions with respiratory-type differentiation in the lower neck.
The case presented here involves a 32-year-old homosexual man with human immunodeficiency virus (HIV) seropositivity and unusual manifestations of secondary syphilis. The patient presented with syphilitic keratoderma and chorioretinitis, and his appearance superficially resembled that of a patient with Reiter's syndrome. Although nontreponemal and treponemal tests for syphilis showed reactivity, the patient's humoral immune response to individual polypeptides of Treponema pallidum, measured by Western blot analysis, was markedly abnormal. The possible relationship between asymptomatic HIV infection and an abnormal humoral immune response to a second pathogen, in this case T. pallidum, is discussed. Our case is one of several recent cases of active syphilis reported in individuals with HIV seropositivity.
A 27-year-old woman with active but stable myasthenia gravis developed pemphigus while she was pregnant. Therapeutic abortion performed for personal reasons was followed by clinical and serologic improvement in her immunobullous disease. Both autoimmune conditions, pemphigus and myasthenia gravis, may be adversely affected by pregnancy. As with another immunobullous disease, herpes gestationis (bullous pemphigoid of pregnancy), hormonal factors appear to induce pemphigus. Although myasthenia gravis and pemphigus have been reported during pregnancy, this is the first instance of coexisting myasthenia gravis and pemphigus in a pregnant woman.
A 0.25-mL quantity of 0.25%, 0.5%, and 1.0% polidocanol (Aethoxysclerol [France]), 0.5% sodium tetradecyl sulfate (Sotradecol injection), and 23.4% hypertonic saline was injected into the dorsal marginal rabbit ear vein; clinical and histologic thrombosis resulted that lasted between four and eight days. The lowest concentration of polidocanol (0.25%) demonstrated immediate thrombosis; however, no clinical or histologic changes occurred eight days after injection. With all other agents, histologic fibrosis of the vessel correlating with clinical disappearance occurred after eight days. However, 0.5% polidocanol and sodium tetradecyl sulfate developed recanalization through the initially sclerosed vessel between eight and 14 days, with clinical reappearance of the 0.5% polidocanol-injected vessel at 30 days, after injection. Cutaneous necrosis was noted clinically and histologically in three of ten vessels injected with 1.0% polidocanol and in two of ten vessels injected with hypertonic saline. Clinical and histologic evidence of necrosis occurred with and without extravasation of the sclerosants.
Congenital smooth-muscle hamartomas are rare, benign tumors of the skin. Since their original description in 1969, 16 case reports have appeared in the literature, with 7 cases reported within the last year. We describe the 17th patient with this lesion and have included a review of the literature. In addition, we describe three morphologic types of smooth muscle cells-the pale cells, dark cells, and intermediate cells-found on electron microscopy, and the features that support our belief that these may reflect different stages of maturity of the smooth-muscle cells. In addition to the smooth muscle, bundles of nerve fibers (both unmyelinated and myelinated) appear to be an intrinsic part of the lesion, as are the prominent vellus hairs. Congenital smooth muscle hamartoma thus appears to be an organoid nevus.
Hairy cell leukemia (HCL) is an uncommon hematologic malignancy in which simultaneous cutaneous disturbances of various types have been reported. We report a case of a 62-year-old man with HCL who developed pyoderma gangrenosum (PG) at the site of incision for splenectomy. PG has been reported previously in a patient with HCL. Since splenectomy is important in the treatment of HCL, it is important for the clinician to know that surgical wound dehiscence may not be due to poor technique nor infection, but rather to pathergy.
Linear or macular pigmentation occurs in 10-30% of patients following sclerotherapy of vessels between 0.1 and 5 mm in diameter. Its occurrence is related to solution strength, vessel fragility, injection pressure, and the type of solution used. This adverse sequela of treatment has been assumed, by some, to represent post-inflammatory hyperpigmentation (incontinence of melanin pigment), and has been said to occur in individuals with this tendency. Histologic data presented in this paper suggest that this phenomenon does not represent melanocytic alteration, but is secondary to extravasation of red blood cells into the dermis following rupture of fragile vessels with resulting deposition of hemosiderin. Therapy has included bleaching agents (hydroquinones), trichloroacetic acid, and phenolic peeling agents with variable success. Eighty percent of patients who experience this adverse sequela will clear spontaneously within 6-24 months. The remaining patients will have persistence of pigmentation for up to 5 years, with a small number of patients having pigmentation persisting 5 years after therapy.
Skin affected by a burn cancer is scarred, ulcerated, and often appears as erythema ab igne clinically in adjacent skin. The latent period in burn scar malignancy is much longer for SCC than BCC. Malignant melanoma and various sarcomas are reported to arise in burn scars, too. The other extreme on the temperature scale can less often result in enough permanent acral damage that poor wound healing may eventually result in cancer, usually SCC. About 1% of patients with chronic osteomyelitis develop cancer, usually SCC in sinus tracts. As with tumors arising in burn scars and chronic leg ulcers of varied etiology, black patients are disproportionately overrepresented in osteomyelitic malignancy. In nearly all of the patients with radiation-induced skin cancer, concomitant radiodermatitis is present. As with burn scar and osteomyelitic cancer, x-ray related cancer has a long latent period. Similar to burn scar cancer, SCC predominates in osteomyelitis and occurs on the extremities. BCC, when it arises, is more common on the face and neck in burn- and radiation-induced tumors. Multiple tumors are frequent as is recurrence in x-ray malignancy. Mortality is high: one out of three to four patients with burn scar, osteomyelitic, and radiation cancer die of dermatosis-related malignancy. Recently, radioactivity-contaminated gold rings have been implicated in causing SCC. Carcinoma tends to occur in irradiated benign dermatoses whereas sarcomas tend to complicate irradiated malignancies. Stasis ulceration and anogenital fistulae may rarely lead to cancer, SCC in the former and adenocarcinoma in the latter. SCC can rarely develop in four related conditions (acne conglobata, dissecting perifolliculitis of the scalp, hidradenitis suppurativa, and pilonidal sinus) after a lengthy latent period; prognosis is poor with a high metastatic rate. A whole host of chronic cutaneous infections can lead to malignancy occasionally; these include lupus vulgaris, lymphogranuloma veverum, granuloma inguinale, leprosy, actinomycosis, and candidiasis. BCC more than SCC is known to complicate smallpox vaccination sites. Certain erosive and/or scarring dermatoses other than those mentioned above can be unusually affected by secondary malignancy. Discoid lupus erythematosus lesions often subjected to the carcinogenic effects of sunlight can degenerate into SCC in patients with either light or dark skin. In acrodermatis chronica atrophicans, a condition not often seen in the United States, the involved skin, particularly of the lower extremities, is susceptible to SCC, lymphoma, and BCC. Epidermolysis bullosa, especially the recessive dystrophic variant, can be complicated by SCC on affected mucous membrane and acral skin.(ABSTRACT TRUNCATED AT 400 WORDS)
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Sclerotherapy refers to the injection of a material for the purpose of obliterating a blood vessel. During this procedure a small quantity of sclerosing solution may be unintentionally injected into the tissues surrounding the vessel, either by missing the vessel or leakage of sclerosant upon withdrawal of the needle. Occasionally, the sclerosant may be intentionally injected into an extravascular site in the hope of reducing telangiectatic mats (best described as multiple, grouped, extremely fine telangiectatic vessels). The various sclerosants in use appear to vary in their potential to cause necrosis of perivascular tissues as a complication. This study examines the clinical and histologic effects of the intradermal injection of 0.1 ml of 0.25, 0.5, and 1.0% Aethoxysklerol (AES); 0.5% Sotradecol (SOT); and 23.4% hypertonic saline (HS) in rabbit skin. All three agents produced some clinical necrosis with intradermal injection. AES in all three concentrations produced the least clinical necrosis, no histologic necrosis, and resolved faster than SOT or HS.
In summary, carcinoma is the most frequent cancer that metastasizes to the skin; lung cancer in men and breast cancer in women. Clinically distinctive patterns of cutaneous metastasis of epithelial origin include alopecia neoplastica, pulsatile nodules, Sister Mary Joseph's nodules, morpheaform, and cellulitis-like lesions. Biopsying these lesions reveals adenocarcinoma, squamous cell carcinoma, or anaplastic carcinoma. The type of histologic pattern seen can be a clue to the organ of origin giving rise to the cutaneous metastasis. Skin that is damaged allows for circulating malignant cells, often of epithelial or leukemic origin, to lodge and proliferate locally (inflammatory oncotaxis). The commonest form of leukemia to affect the skin of elderly males is chronic lymphocytic leukemia. However, when leukemia involves the mucous membranes, acute myeloid leukemia (acute monocytic and acute myelomonocytic leukemia) is the most likely diagnosis. When papules, nodules, or plaques develop on the head, neck, or torso in a middle-aged male accompanied by lymphadenopathy, there must be a high index of suspicion that these lesions are metastatic lymphomatous deposits. Definitive histologic diagnosis on a skin biopsy specimen is difficult. In this situation, it is best to rely on histologic patterns seen in lymphoid tissue along with cellular marker studies. An elderly patient having bone pain, anemia, elevated blood calcium level, and renal failure along with purplish or skin-colored nodules and plaques on the trunk has a good chance of having multiple myeloma. Biopsying these lesions is most certain to reveal atypical plasma cells, and blood immunoelectrophoresis will demonstrate characteristic monoclonal gammopathy. There are two malignancies seen in children under 3 years of age that often times affect the skin in a characteristic fashion. Letterer-Siwe disease, which is distinguished from other histocytic disorders by its cell of origin, the Langerhans cell, clinically shows maculopapular and erosive lesions distributed in a seborrheic pattern. Neuroblastoma derived from cells of the neural crest demonstrates clinically widespread bluish papulonodules. Kaposi's sarcoma, a multifocal vascular malignancy, has a wide spectrum of clinical expression. Those patients who are immunocompromised secondary to concomitant disease or immunosuppressive therapy are more susceptible to a disseminated fulminant course accompanied by opportunistic infection. In conclusion, although specific signs of internal malignancy are less common than nonspecific ones, they are just as important; if the clinician managing the cancer patient is familiar with these clues to internal disease, proper patient management will ensue.(ABSTRACT TRUNCATED AT 400 WORDS)
Fat embolism syndrome is a life-threatening disease with cutaneous manifestations that occur most commonly after fractures of a long bone. The syndrome consists of a triad of dermatologic, pulmonic, and neurologic involvement. Because no major dermatologic journal has reviewed this syndrome in more than five years, we present a case and discuss the current approaches to the pathogenesis, diagnosis, and treatment of this often overlooked syndrome.