W.P. Daniel Su

Pyoderma gangrenosum: Classification and management

Pyoderma gangrenosum (PG) has four distinctive clinical and histologic variants. Some have morphologic and histologic overlapping features with other reactive neutrophilic skin conditions. PG often occurs in association with a systemic disease, and the specific clinical features of the skin lesion may provide a clue to the associated disease. Management of PG depends on its type and severity and usually requires aggressive local and systemic treatment.

Classification of Morphea (Localized Scleroderma)

OBJECTIVE To classify and describe morphea (localized scleroderma). DESIGN A review of morphea and its subtypes is presented. RESULTS The current classification of morphea is incomplete and confusing. As knowledge of the spectrum of disease continues to evolve, the controversy and confusing nature of its multiple subtypes present a challenge for the physician who encounters a patient with this condition. Thus, we propose that morphea be classified into the following five groups: plaque, generalized, bullous, linear, and deep. This classification, based on clinical morphologic findings, will simplify the diagnostic and therapeutic approach. CONCLUSION Morphea represents a wide variety of clinical entities that seen to be on the opposite end of the scleroderma spectrum from systemic sclerosis. The cutaneous lesions eventually evolve from a sclerotic stage to a nonindurated stage, and residual hypopigmentation or hyperpigmentation follows. The histologic pattern in patients with morphea is similar to that in patients with progressive systemic sclerosis. Although treatment is nonstandardized, hydroxychloroquine sulfate may be beneficial.

Pyoderma gangrenosum: clinicopathologic correlation and proposed diagnostic criteria

Pyoderma gangrenosum is a rare but significant cause of ulcerations. It is a diagnosis of exclusion. Herein, we suggest diagnostic criteria and some historical perspectives on the diagnosis of pyoderma gangrenosum.

Sister Mary Joseph's nodule: A clinical and histologic study

The clinical and histologic findings in eighty-five cases of tumors metastatic to the umbilicus were studied. Clinically, the lesions were seen as firm, indurated nodules, sometimes with fissuring or ulceration. In twelve cases, the initial presentation of the internal primary malignancy was an umbilical nodule. Histologic material from the metastatic umbilical tumor was studied in all cases but was diagnostic of the primary carcinoma in only twenty-one. In seventeen cases, the primary site was never reliably determined, while stomach, large bowel, ovary, and pancreas were the most frequent primary sites in the other cases. Most patients died within months after the appearance of the umbilical tumors, thus emphasizing the ominous significance of this sign of metastatic, usually intra-abdominal, malignant disease.

Sweet's Syndrome: Systemic Signs and Symptoms and Associated Disorders

OBJECTIVE To characterize the findings associated with acute febrile neutrophilic dermatosis (Sweets syndrome [SS]) and the response of SS to treatment. DESIGN We retrospectively reviewed 48 cases of SS encountered at the Mayo Clinic between 1980 and 1992. MATERIAL AND METHODS Histopathologic specimens and medical records were studied to determine initial manifestations, patterns of involvement, systemic signs and symptoms (including mucosal, musculoskeletal, hematologic, pulmonary, hepatic, and renal findings), and conditions associated with SS. RESULTS In patients with SS, the typical manifestations are the acute onset of tender, erythematous or violaceous nodules or plaques in association with fever, leukocytosis, and dermal neutrophilia. In our study group, the cutaneous lesions most frequently involved the arms and legs. Of our 48 patients, 26 (54%) had a hematopoietic, plasma cell, or malignant disorder, and many of these patients had associated anemia, especially the male patients. No single laboratory finding specifically indicated an association with serious systemic disease. Most patients were treated with a tapering dose of prednisone, which yielded a good response. CONCLUSION Clinical acumen and appropriate laboratory tests are the main requirements for detection of hematologic disorders, internal malignant diseases, or other systemic conditions associated with SS.

Clinicopathologic correlations in leukemia cutis

This clinicopathologic study involved 42 cases of leukemia cutis: 3 of acute lymphocytic leukemia (ALL), 16 of chronic lymphocytic leukemia (CLL), 12 of acute granulocytic leukemia (AGL), 3 of chronic granulocytic leukemia (CGL), 5 of acute monocytic leukemia (AML), and 3 of acute myelomonocytic leukemia (AMML). The clinical appearance of leukemia cutis included papules, macules, plaques, nodules, ecchymoses, palpable purpura, and ulcerative lesions, and these were seen in all types of leukemias. Gingival hypertrophy was seen only in AML or AMML, and erythroderma and bullous lesions of leukemic infiltration were observed only in CLL. Cutaneous leukemic lesions may be concomitant with or preceding the diagnosis of systemic leukemia. Therefore, skin biopsy may be helpful in detecting the leukemia and may facilitate the work-up. Leukemia cutis probably is a dissemination of systemic leukemia to the skin, and the demonstration of leukemia in skin is associated with a very poor prognosis.

Antiphospholipid syndrome and the skin.

The antiphospholipid syndrome is an acquired multisystem disorder of hypercoagulation, which may be primary or secondary to underlying diseases. Serologic markers for the syndrome are the lupus anticoagulant and anticardiolipin antibodies. Clinical features include recurrent thrombotic events (arterial or venous), repeated fetal loss, and thrombocytopenia. Cutaneous manifestations may occur as the first sign of antiphospholipid syndrome. These include livedo reticularis, necrotizing vasculitis, livedoid vasculitis, thrombophlebitis, cutaneous ulceration and necrosis, erythematous macules, purpura, ecchymoses, painful skin nodules, and subungual splinter hemorrhages. Antiphospholipid syndrome may also be associated rarely with anetoderma, discoid lupus erythematosus, cutaneous T-cell lymphoma, or disorders that closely resemble Sneddon or Degos syndromes. Noninflammatory vascular thrombosis is the most frequent histopathologic feature observed. Prophylaxis and treatment of thrombosis in patients with antiphospholipid syndrome relies principally on anticoagulant and antiplatelet agents.

Basal cell carcinoma: A population-based incidence study in Rochester, Minnesota

Population-based annual incidence rates for histologically proved basal cell carcinoma were derived from the records linkage system of the Rochester Epidemiology Program Project. Residents of Rochester, Minnesota, during the years 1976 to 1984, formed the observational cohort. A total of 657 first episodes of basal cell carcinoma were observed. Annual incidence rates per 100,000 persons in the Rochester population, standardized to the 1980 U.S. white population, were 175 for men, 124 for women, and 146 combined. Recurrent or subsequent basal cell carcinoma was observed among 30% of patients during an average of 4.5 years of follow-up; no metastatic lesions occurred. The rates of recurrence were similar after complete excision or treatment with curettage and cauterization in these observational data.

Adnexal carcinomas of the skin II. Extraocular sebaceous carcinomas

Extraocular sebaceous carcinomas are uncommonly seen neoplasms, and have been confused in the past with basal cell carcinomas showing sebaceous differentiation. In contrast to the latter tumors, however, sebaceous carcinomas have a distinct risk of aggressive behavior. This study presents clinicopathologic data on five cases of sebaceous carcinoma arising in cutaneous locations outside of the ocular adnexae. Four of five patients were men, and the average age at diagnosis was 63 years. Three tumors occurred on the face, one arose in the skin of the neck, and another occurred on the penis, an anatomic site that is extremely rare for sebaceous carcinoma. Three tumors metastasized, and two patients died of tumor or with residual tumor growth. In light of this behavior, the premise that extraocular sebaceous carcinomas rarely spread to distant sites may need reexamination.

Cutaneous manifestations of cryoglobulinemia: Clinical and histopathologic study of seventy-two patients

In the 72 cases of cryoglobulinemia reviewed, erythematous to purpuric macules or papules were present in 92%. Infarction, hemorrhagic crusts, and ulcers were present in 10% to 25% of the patients and were relatively more common in type I cryoglobulinemia than in the other types. Postinflammatory hyperpigmentation was noted in 40%. Lesions on the leg were common in all types of cryoglobulinemia; however, lesions on the head and mucosal surfaces suggested type I cryoglobulinemia. Histopathologic features were classified as vasculitis in 50%, inflammatory or noninflammatory purpura in 15%, noninflammatory hyaline thrombosis in 10%, and postinflammatory sequelae in 10%. Noninflammatory hyaline thrombosis was relatively more frequent in type I. Thus erythematous to purpuric lesions on the legs and leukocytoclastic vasculitis are the common cutaneous findings in cryoglobulinemia. Type I cryoglobulinemia is suggested by noninflammatory hyaline thrombosis, cutaneous infarction, hemorrhagic crusts, skin ulcerations, and lesions of the head and neck and of oral or nasal mucosa.