Cutaneous lymphadenoma.

Thirteen cases of a unique cutaneous tumor are presented. The lesions presented as single, nondescript, skin-color nodules. Eleven were located on the head and two were on the legs. The male/female ratio was 8:5. The age at diagnosis ranged between 21 and 55 years (mean: 39). The duration of the lesions was from several months to more than 20 years. All tumors were excised and did not recur. The tumors typically presented as well-circumscribed nodules with scant or no epidermal connections. (One example, however, had a plate-like configuration amply connected with the basal layers of the epidermis.) The proliferation consisted of multiple, rounded lobules of basaloid cells with some degree of peripheral palisading, immersed in a dense, fibrous stroma. There was an intense infiltrate of small lymphocytes within the lobules, with some spillage into the stroma. No clear adnexal differentiation was noted except for rare isolated cells showing apparent sebaceous differentiation within the tumor lobules. Areas of central keratinization were also present. Numerous cells with ample amphophilic cytoplasm, large vesicular nuclei, and prominent nucleoli were also seen. Immunohistochemistry confirmed the presence of keratin within tumor cells. Common leukocytic antigen highlighted the intense intralobular lymphocytic component. Vimentin not only highlighted the stromal fibroblastic component, but also stained scattered intralobular cells. Epithelial membrane antigen was positive within some of the large intraepithelial cells. S-100 protein was extensively positive within dendritic intralobular and stromal cells. Lymphocytic markers demonstrated a polyclonal B and T population. This unique tumor appears to represent a form of adnexal neoplasm with basaloid features, possibly immature pilosebaceous differentiation.

Cutaneous malignant melanotic neurocristic tumors arising in neurocristic hamartomas. A melanocytic tumor morphologically and biologically distinct from common melanoma.

Cutaneous neurocristic hamartomas (CNH) are pigmented lesions of neural crest origin that involve the skin and superficial soft tissue. They consist of a complex proliferation of nevomelanocytes, schwann cells, and pigmented dendritic and spindled cells. Malignancies can arise within the lesions, but few studies have dealt with this issue. We studied seven cases of CNH in which malignancy supervened. They included four congenital and three acquired lesions that involved the head and neck (five cases) or back (two cases) in patients aged from 11 to 67 (mean, 32) years. Malignant tumors developed 15 to 67 (mean, 32) years after identification of the pigmented lesion in the congenital CNH and after 1 to 6 (mean 3.5) years in the acquired CNH. The malignant tumors had a deep intradermal or subcutaneous origin and lacked a junctional component. Most were circumscribed, multinodular, melanin-containing tumors composed of bland, small, rounded to spindled cells, focally displaying a trabecular or nested growth pattern. Nuclear palisading and perivascular pseudorosettes were present in several tumors. In two examples, the neoplasm consisted predominantly of large pleomorphic epithelioid cells. Tumors contained immunoreactive S-100 protein (all of seven cases), a melanoma-associated antigen (HMB-45)( five of six cases, neuron-specific enolase (five of seven cases) and vimentin (six of six cases). The four patients with congenital lesions tended to have multiple recurrences and died of disease after 2 to 20 (mean, 9) years, three with metastases, one with direct invasion of the posterior fossa. The three patients with acquired lesions are alive after 1 to 5 years two with persistent disease. In contrast to common melanomas, these tumors have a propensity to recur as bulky nodules and to metastasize after many years or decades. Because these tumors exhibit melanocytic differentiation and arise in hamartomatous lesions composed of neural crest derivatives, we have designated them cutaneous malignant melanotic neurocristic tumors.

Sclerosing sweat duct (syringomatous) carcinoma.

This report describes 20 sclerosing adnexal carcinomas of the skin that recapitulate the sweat duct and share certain microscopic features with benign syringoma. Clinically, the neoplasms were slow-growing, indurated nodules or plaques. Sixteen were located on the face, including five on the upper lip. Three were situated in the axilla, and one involved the buttock. Twelve of the tumors (group 1) were composed primarily of cords and nests of whorled squamous cells with clear cell change and variable keratinization. Lumens consistent with ductal differentiation were few and isolated in some of these lesions, but others had more numerous and complex ducts. The remaining eight neoplasms (group 2) contained large numbers of small, uniform ducts, and exhibited little keratinization. Each neoplasm in the series infiltrated subcutaneous tissue or deeper tissues and was accompanied by sclerotic, fibrous stroma. Sixteen of the lesions (80%) exhibited perineural growth. One tumor involved an underlying lymph node, probably by direct extension. Cytologic atypicality was mild or lacking, and mitotic figures were sparse. Seven of 15 tumors (47%) with follow-up recurred, in some instances many years after primary excision. Tumor-free margins in the initial excision specimen appeared to be a favorable prognostic factor. The most aggressive lesion in the series showed increased cytologic atypicality in recurrences.

Site-dependent phenotypic heterogeneity in peripheral T-cell lymphoma.

Peripheral T-cell lymphomas constitute a heterogeneous population of postthymic T-cell malignancies. Characteristically, they present a varied phenotypic expression, which can be helpful in establishing the diagnosis. A case of a peripheral T-cell lymphoma in a 76-year-old man is described. The malignant cells in the skin and bone marrow were of the T4 (helper/inducer) phenotype, yet they did not express pan-T-cell antigens, such as T11, or functional E rosettes. In a biopsy specimen from a lymph node, however, the malignant cells had a helper/inducer phenotype and also expressed the pan-T-cell antigens T11 and Leu-5. Additionally, the malignant cells from the lymph node formed E rosettes. This study demonstrates the phenotypic heterogeneity of malignant T cells, which appears to be site-dependent.

Periocular granuloma annulare, nodular type. Occurrence in late middle age.

We describe the two oldest individuals with nodular granuloma annulare (pseudorheumatoid nodules) in the ophthalmologic literature and propose a unified classification scheme that recognizes pseudorheumatoid nodules to be granuloma annulare, nodular type. All lesions in both cases revealed so-called necrobiotic granulomas, characterized by an acellular central area containing mucin (hyaluronic acid) surrounded by palisading histiocytes (macrophages), diagnostic of granuloma annulare. These features are identical to those reported in the ophthalmologic and older general pathology literature as pseudorheumatoid nodules and the contemporary general and dermatologic pathology literature as granuloma annulare. We believe the diagnosis of nodular granuloma annulare should be employed for necrobiotic lesions displaying distinctive clinicopathologic features to unite the ophthalmologic, general, and dermatologic pathology literature. Granuloma annulare, nodular type, must also be considered in the differential diagnosis of ocular and periocular lesions at any age.

Differentiating neoplasms of hair germ.

Differentiating neoplasms of hair germ are benign epithelial-mesenchymal tumours of skin in which hair follicle development may be partly or completely recapitulated. The epithelial component is equivalent to the hair germ. The mesenchymal component is equivalent to the dermal papilla. Epithelial-mesenchymal interaction results in the morphogenesis of hair follicles. In neoplasms showing stromal induction, there is centrifugal organizations: hair bulbs are found at the periphery of tumour lobules and hairs are projected centrally to lie within small keratinizing cysts. Neoplasms of hair germ without advanced morpho-differentiation are termed ;trichoblastomas', and those neoplasms in which hair follicle development is advanced are called ;trichogenic trichoblastomas'.

Anaerobic bacteria in routine urine culture.

One hundred and fifty-eight anaerobic organisms from 147 patients were isolated from 15,250 consecutive clean mid-stream or catheter urine specimens. The pathogenicity of the anaerobic genera commonly isolated from urine is reviewed and discussed. Failure to establish anaerobic isolates as pathogens and a paucity of reported cases proving anaerobic bacteria as significant causes of urinary tract infection permitted discontinuation of anaerobic culture as part of the routine screening procedure for investigation of urinary tract infections.

Telogen effluvium. New concepts and review.

BACKGROUND: Telogen effluvium is the result of a perturbation of the hair cycle that is manifest by increased loss of normal club hairs. Although diverse causes for telogen effluvium have been proposed, this article suggests several diverse mechanisms for the first time. OBSERVATIONS: Five different functional types of telogen effluvia are proposed based on changes in different phases of the follicular cycle. These are immediate anagen release, delayed anagen release, short anagen syndrome, immediate telogen release, and delayed telogen release. Diverse causes are confirmed and drug-related telogen effluvia are reviewed. CONCLUSIONS: The five diverse mechanisms proposed for telogen effluvia are generally confirmed and supported by clinical findings.

Transverse microscopic anatomy of the human scalp. A basis for a morphometric approach to disorders of the hair follicle.

Transverse sections of cylindrical scalp biopsy specimens can provide excellent samples for histologic, quantitative morphometric analyses of the follicles and hair. This study describes and illustrates the morphologic details of the normal transverse anatomy of follicular structures, including the various phases of the normal hair cycle.

Psammomatous melanotic schwannoma. A new cutaneous marker for Carney's complex.

BACKGROUND: Noncutaneous psammomatous melanotic schwannoma has recently been reported as an unusual component of Carney's complex (myxomas, spotty pigmentation, and endocrinopathy). The most common locations for this lesion include peripheral nerve roots and the gastrointestinal tract. OBSERVATIONS: A cutaneous psammomatous melanotic schwannoma is reported in a patient with known Carney's complex. This neoplasm was pseudoencapsulated, with epithelioid to spindle-shaped cells and no nuclear atypia. Immunostaining was positive for S100 protein and vimentin, as well for HMB-45 antibody. Electron microscopy showed melanosomes in cytoplasmic processes of cells that were ensheathed by layers of reduplicated basal lamina. CONCLUSIONS: Location in the superficial soft tissues is extremely unusual for psammomatous melanotic schwannomas. Recognition of this new cutaneous marker as a part of this complex may aid in identification of individuals at risk for cardiac myxomas. It is also important that this lesion is not mistaken for melanoma, given the strong HMB-45 positivity.

Primary granulomatous dermatitis caused by Rhodochrous. Evidence for a pathogenic role in humans.

Bacteria belonging to the Rhodochrous complex are of uncertain taxonomic status. Currently excluded from the genus Mycobacterium, these organisms are more closely allied to Nocardia. Organisms of the Rhodochrous complex have only rarely been implicated as human pathogens. An 81-year-old man had a plaquelike cutaneous granuloma from which Rhodochrous was both cultured and demonstrated in tissue section. A pathogenic role for Rhodochrous causing a primary cutaneous infection is suggested. Specific antimicrobial treatment with doxycycline hydrochloride was successful and there has been no recurrence of the infection after three years.

Familial focal alopecia. A new disorder of hair growth clinically resembling pseudopelade.

A 14-year-old girl was evaluated for patchy hair loss present from early childhood. Her mother was found to have a similar condition. When studied in transverse section, biopsy specimens from both women showed marked anagen-telogen transformation that circumstantially appears to be irreversible. Preservation of telogen epithelium with absence of inflammation and scarring readily separate focal familial alopecia from the pseudopelade state and from localized alopecia areata. To our knowledge, this is the first description of a new familial disorder causing focal alopecia.

Purpura fulminans. A cutaneous manifestation of severe protein C deficiency.

Protein C, when activated, is a vitamin K-dependent serine protease that has anticoagulant and profibrinolytic activities. An increased risk of thrombotic disease is associated with deficiency of this enzyme. A neonate developing purpura fulminans with internal thrombotic complications and congenital absence of protein C is described. Family studies showed partial protein C deficiency in the subject's symptom-free parents and two of three siblings. Clinically and histopathologically similar lesions are also seen in two acquired conditions in which there is a thrombotic tendency: coumarin necrosis and purpura fulminans of childhood, both of which are probably the result of transient protein C deficiency. We conclude that purpura fulminans can represent a cutaneous marker of acquired or congenital protein C deficiency.

Fibroblastic rheumatism.

BACKGROUND: Fibroblastic rheumatism was first described in the French literature in 1980. Since that time, 11 other patients with this disorder have been identified in the literature, mostly from France. This is a unique syndrome characterized by the sudden onset of symmetric polyarthritis and cutaneous nodules ranging from 5 to 20 mm in diameter, with predilection for the upper and lower extremities. While the cutaneous findings resolve spontaneously after several months, permanent joint sequelae are common. OBSERVATIONS: We present the first two patients with fibroblastic rheumatism reported in the United States. The clinical features and histologic findings were identical to those of patients described in the European literature. CONCLUSIONS: Fibroblastic rheumatism is a relatively rare syndrome characterized by the association of multiple cutaneous nodules with polyarthritis. The clustering of cases in France may, in part, be attributable to increased recognition by clinicians owing to prevalence of reports in the European literature. Perhaps, as clinicians in the United States become familiar with this disorder, further cases will be diagnosed, and the pathogenesis of the disorder will be elucidated.

Epithelioid sarcoma. Enzymatic histochemical and electron microscopic evidence of histiocytic differentiation.

A 63-year-old man had epithelioid sarcoma. Light microscopy showed the tumor to resemble granuloma annulare. Enzymatic histocytochemical and ultrastructural studies demonstrated the tumor's histiocytic features. Literature review of epithelioid sarcoma and granuloma annulare showed them to have a similar anatomic distribution.

Cutaneous myxomas. A major component of the complex of myxomas, spotty pigmentation, and endocrine overactivity.

Cutaneous myxoma(s) occurred in 22 (54%) of 41 patients with the complex of myxomas, spotty pigmentation, and endocrine overactivity. Of the 16 patients who had cardiac myxoma(s), the cutaneous tumor(s) was (were) detected in 13 (81%) of them prior to diagnosis of the cardiac neoplasm. Thus, the cutaneous tumor may herald a potentially fatal cardiac neoplasm (and other important conditions as well). Clinical features of the lesion were as follows: early appearance (mean age, 18 years); multicentricity (71% of patients); small size (usually less than 1 cm in diameter); widespread distribution but with predilection for certain sites (eyelids, ears, nipples); and tendency for recurrence. Pathologic features included the following: location in dermis, subcutis, or both; sharp circumscription (sometimes encapsulation); hypocellularity; abundant myxoid stroma; prominent capillaries; lobulation (larger lesions); and occasional presence of an epithelial component.

Primary adenoid cystic carcinoma of skin.

Two primary adenoid cystic carcinomas of skin were studied histologically and histochemically. Histologically, they closely resemble adenoid cystic carcinoma found in other tissues but in the skin must be distinguished from aggressive basal cell carcinomas. The natural history of these tumors and of those collected from the literature suggest a long indolent and progressive course. Local excision with meticulous histologic control of the surgical margins appears to be an appropriate therapeutic strategy. Thus far lymph node metastases have not been observed.

Aggressive-growth basal cell carcinoma in young adults.

Aggressive-growth basal cell carcinoma (AG-BCC) defines a group of basal cell cancers that are histologically and clinically aggressive. This group includes morpheaform, infiltrating, and recurrent BCCs. Because of the clinical observation that the incidence of AG-BCC may be increased in patients under 35 years of age, compared with those older, we performed a retrospective study. We reviewed the pathologic findings of 3381 patients diagnosed with BCC, including 102 patients with BCC referred for Mohs surgery to determine whether AG-BCC occurs with increased frequency in patients younger than than 35 years of age. Among patients under 35 years of age, 38% of women had AG-BCC compared with 9% of women in the older age group. Similarly, 25% of men under 35 years of age had AG-BCC compared with 11% among men in the older age group. Aggressive-growth BCC is more frequently noted in patients under 35 years of age than in those older. Failure to diagnose this type of BCC, which may be clinically subtle, may lead to incomplete or inadequate treatment. Because of the tendency of these tumors to recur, greater long-term morbidity may result.

Topical treatment of alopecia areata.

It has been shown previously that alopecia areata can be treated with dinitrochlorobenzene (DNCB) and other contact allergens. Whether these agents work by inducing immunologic stimulation or simply a nonspecific inflammatory reaction has not been definitively demonstrated. To test the relative importance of these two mechanisms, we have randomly studied 22 patients with alopecia areata to whom either DNCB or croton oil was applied topically. Sixty-three percent of patients without spontaneous regrowth of hair regrew hair after DNCB application. None of those treated with croton oil regrew hair when treated later with DNCB. Therefore, a proved contact allergen was shown to be required for therapeutic success. Patient acceptance of the induced contact dermatitis was excellent. In light of recent data on the mutagenicity of DNCB to bacteria, other contact allergens for topical immunotherapy are being sought.

T-cell receptor gene rearrangement in regressing atypical histiocytosis.

A case of regressing atypical histiocytosis having characteristic clinical and light microscopic findings was studied immunologically for immunoglobulin and T-cell receptor gene rearrangement and for DNA ploidy analysis. Immunologic phenotyping and rearrangement of T-cell receptor Beta- and gamma-chain genes indicated that this primary cutaneous neoplasm, previously considered "histiocytic" in origin, is most probably of T-cell lineage.