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A patient with recurrent adenoid cystic carcinoma of the skin managed by Mohs surgery is reported. The histopathologic features and differential diagnosis of this tumor are reviewed. Mohs surgery appears to be an ideal way to manage this neoplasm since recurrences after routine excision are common and metastases are uncommon.

The prognosis of localized malignant melanoma is related to several histologic features of the primary lesion. Growth pattern, level of invasion, and tumor thickness are currently most widely used in clinical practice, but other features, including ulceration, mitotic rate, density of the inflammatory response, evidence of partial regression, angioinvasion, cell type, cross-sectional profile, and amelanosis have been accorded prognostic significance in single factor analyses. Although stringently controlled prospective studies have yet to demonstrate the validity of these factors for the determination of optimal surgical treatment in individual cases, newer statistical methods of multivariate analysis have made possible assessment of the relative importance of each of these histologic characteristics. The most important and reproducible factor for predicting survival is maximum tumor thickness. Consensus also supports ulceration as another important, independent prognostic indicator, whereas growth pattern and level of invasion derive most of their prognostic value from a secondary correlation with tumor thickness. Mitotic rate may influence survival in the subgroup of patients with high-risk, thick melanomas.

We report, in two men, the occurrence of a tiny, dome-shaped, blue-gray cystic nodule of the face. The nodule in both men clinically simulated an eccrine or apocrine hidrocystoma. Histologic examination revealed that both were cystic basal cell epitheliomas. These cases are reported in order to bring this unusual clinical presentation of basal cell epithelioma to the attention of clinicians.

It has been known for a long time that melanoma can have its origin in congenital or acquired melanocytic nevi. In regard to congenital nevi, there is sufficient evidence to state that large lesions (those greater than 20 cm in diameter) have a significant risk factor that is several-fold greater than for common acquired nevi. Prophylactic excision of such lesions should be strongly considered when it is feasible, but individual circumstances must be taken into account. The risk factors for small and medium-sized congenital nevi have not been accurately determined; therefore, no uniform recommendation can be made regarding their management. There is no objective evidence to indicate that common acquired nevi in any particular anatomic sites, such as volar or genital skin, are at greater risk for the development of melanoma than are any others. Patients with familial dysplastic nevus syndrome must be identified and followed carefully in order to recognize and eradicate evolving and early melanomas. The concept of the sporadic dysplastic nevus syndrome is intriguing and deserves careful study to further define the clinical and histologic diagnostic criteria that will enable accurate determination of its prevalence and risk factors.

Forty-five patients with chronic urticaria were studied to determine: (1) the histolgic incidence of leukocytoclastic vasculitis and (2) the clinical, laboratory and immunopathologic parameters that characterized this patient group. By histopathologic examination a spectrum of changes were noted as 9 patients showed leukocytoclastic vasculitis, 15 a dense perivascular infiltrate of lymphocytes and eosinphils, and 21 only a trate of lymphocytic perivascular infiltrate. Both the vasculitis and the dense infiltrate groups had an increased incidence of circulating immune complexes, as detected by Clq binding and monoclonal rheumatoid factor inhibition radioassays. Direct immonofluorrescence showed blood vessel deposition of immunoglobulins, complement, and/or fibrin in 33% of the vasculitis group, 13% of the dense infiltrate group, and 9% of the sparse infiltrate group. These studies suggest that a meaningful number of patients with chronic urticaria have histologic and immunopathologic findings of vasculitis.

A malignant melanoma demonstrating palisades of spindled melanocytes strikingly similar to Verocay bodies is reported. Immunohistochemical staining for S-100 protein and neuron-specific enolase revealed uniform positive staining of the neoplastic cells consistent with malignant melanoma. Immunohistochemical staining for myelin basic protein, a marker specific for Schwann cells, was negative in neoplastic cells. Ultrastructural examination revealed numerous melanosomes, confirming the melanocytic nature of this neoplasm. The differential diagnosis and possible histogenesis of this neoplasm are discussed.

A 42-year-old black woman, gravida V, para IV, developed a recurrence of pemphigus vulgaris in the third trimester of pregnancy. She delivered a 2,056-gm stillborn female fetus in the seventh month, 2 days after ultrasonography had demonstrated absence of fetal cardiac activity and other evidence of intrauterine fetal death. The macerated fetus had peeling of its skin and an intact bulla on the foot. Fetal cardiac blood, obtained after delivery, showed the presence of pemphigus antibodies at a titer of 20. Direct immunofluorescence of fetal skin demonstrated IgG in the epidermal intercellular spaces. Autolytic changes of the skin, however, precluded definitive histologic documentation of acantholytic disease.

A case of bronchial mucoepidermoid carcinoma is reported, the presentation of which was as cutaneous metastases. Histologic, histochemical, and ultrastructural features of the neoplasm are described, and the literature pertaining to bronchial mucoepidermoid carcinoma is reviewed. This case illustrates the potential for aggressive behavior in a mucoepidermoid neoplasm, the histologic features of which are considered low grade by some authors. Because such metastatic lesions may be morphologically identical to tumors that have been described as primary cutaneous mucoepidermoid carcinomas, this differential must be considered by the histopathologist confronted by such a neoplasm.

The pathologic specimens from forty-seven patients with fifty-one recurrent basal cell carcinomas referred for Mohs' surgery were studied. Specimens from the original tumor and from each subsequent recurrence were reviewed. Of the original tumors, 65% demonstrated an aggressive* histologic picture characterized by poor palisading and an infiltrating and/or micronodular pattern. In twelve cases the recurrent tumor developed a more aggressive histologic picture, but in seven cases the histologic picture became more benign. Electrodesiccation and curettage was the initial treatment for forty-one basal cell carcinomas. Ten tumors had been excised previously, with routine pathologic examination indicating tumor-free margins in six of these lesions. It can be concluded that many recurrent basal cell carcinomas are aggressive from the beginning, and this often can be predicted from the histologic picture. Lesions with an aggressive pathologic picture should not be managed by blind technics such as electrodesiccation and curettage and may defy routine pathologic examination of conventional excision specimens. Mohs' surgery appears to be the treatment of choice for such lesions.

Epidermolysis bullosa acquisita (EBA) is an acquired mechanobullous disorder characterized by traumaticlly induced bullae in skin subjected to frictional injury, i.e., acral extensor surfaces or any point of sufficient mechanical distress. Two new patients with EBA are reported. Histopathologic findings were subepidermal bullae with sparse superficial perivascular mononuclear cell inflammatory infiltrates. The periodic acid-Schiff (PAS)—positive basement membrane zone (BMZ) was split between the floor and roof of the blisters with most attached to the blister roof. Electron microscopy of normal skin from one patient revealed a bandlike zone of granular material within the dermis immediately beneath the basal lamina. Nonbranching filaments were embedded in the granular material. Electron microscopy of normal skin from the second patient revealed a markedly diminished density of anchoring fibrils but no granular material. Immunologic findings in skin biopsies were C′3, IgG, and other immunoglobulins in varying combinations in both cases and in all reported cases. Comparisons of direct immunofluorescent (IF) findings in nine reported cases with 223 cases of bullous pemphigoid (BP) suggest that BMZ deposits of IgG plus IgA and/or IgM occur more frequently in EBA than in BP. Indirect IF studies of patients' sera revealed antibodies to the BMZ in two samples of one of our two cases and in one of seven cases reported by others. While both EBA and BP characteristically yield positive IF findings in the BMZ, clinical, electron microscopic, and some immunopathologic findings indicate that the two diseases are distinct.

The papillary eccrine adenoma is a rare benign sweat gland neoplasm characterized by a potentially locally aggressive clinical course. The light microscopic findings in two cases of this unusual neoplasm are discussed, with emphasis on the differential diagnosis of this tumor from other benign and malignant sweat gland neoplasms. Immunohistochemistry studies directed against various epithelial antigens were also performed. Immunoperoxidase stains positive for carcinoembryonic antigen, S-100 protein, and epithelial membrane antigen strongly support the hypothesis that this unusual neoplasm differentiates toward the eccrine secretory coil.

Udgivelsesdato: 2007-Oct Multiple self-healing squamous epithelioma (MSSE), also known as Ferguson-Smith Disease, is a rare cancer-associated genodermatosis with an autosomal dominant inheritance. Affected patients suffer from recurrent skin lesions, which clinically and histologically resemble keratoacanthomas or well-differentiated squamous cell carcinomas, but which, if left, undergo spontaneous regression, leaving pronounced scarring. The majority of MSSE cases previously described were of Scottish ancestry and all shared the same at-risk haplotype, suggesting that this disorder was caused by a founder mutation. The candidate locus for MSSE lies in a region of

Keratoacanthoma centrifugum marginatum (KCM) is a rare variant of keratoacanthoma (KA) possessing several characteristics distinguishing it from typical KAs. Clinical presentation deviates from common KAs with lesions that continually expand peripherally.1, 2, 3 KCM also deviates from the usual histologic findings with a lack of some classic KA features. This is probably attributable to sampling issues given the size of the lesions and the limited capacity of a biopsy. Because of its rarity and lack of distinctive histopathologic features, KCM poses a difficult diagnostic challenge to clinicians and pathologists. Excellent communication and collaboration between the clinician and the pathologist is crucial for the correct and timely diagnosis, allowing for quick and proper treatment of the patient.

Three skin biopsies (proximal nailfold, extensor forearm and buttock) and serum samples were studied by direct and indirect immunofluorescence, immunodiffusion and radioimmunoassay techniques in 6 patients with dermatomyositis. A variety of seroligic and immunopathologic abnormalities was observed in the patients. Three of ft patients had antinuclear antibodies (ANA) of a speckled pattern mixed with a peculiar dot pattern. Epidermal intercellular deposits of immunoglobulins were seen in the nailfold biopsies of 2 patients; one of them also had IgG deposits in the cytoplasm of epidermal cells. Forearm and buttock biopsies were negative.

A patient with lichen planus pemphigoides is reported. Initial pathologic and immunopathologic evaluation was consistent with coexisting bullous pemphigoid and lichen planus. However, further immunologic evaluation suggested that the circulating antibody was not directed toward bullous pemphigoid antigen but another basement membrane zone antigen. This finding indicates a need for immunologic re-evaluation of lichen planus pemphigoides.

Agminated blue nevi are rare lesions that may be congenital or acquired. A case is reported in which they developed in an adult. This exceptional presentation of blue nevi merits recognition by physicians because of possible confusion with satellite metastases from a malignant melanoma.

The findings from immunofluorescent techniques have made a profound contribution to our knowledge of bullous diseases. The techniques, once considered investigational, are now routinely employed in dermatologic diagnostic tests. Direct immunofluorescent methods applied to specimens of perilesional skin are extremely reliable to demonstrate characteristic deposition of various immunoreactants in pemphigus vulgaris, bullous pemphigoid, and dermatitis herpetiformis. Autoantibodies in serum revealed by indirect immunofluorescent techniques, are demonstrable in most cases of pemphigus vulgaris and bullous pemphigoid. The disappearance of these antibodies from the skin and serum of patients so afflicted appears to herald remission of their diseases. Thus, detection of these antibodies may be employed to regulate therapy for maximum effect.

Pyostomatitis vegetans is a rare and unusual disorder of the oral cavity, characterized by erythema and edema of the mucosa and numerous small, superficial yellow pustules. Its significance lies in its association with inflammatory disease of the bowel, either ulcerative colitis or Crohn's disease. We report here a patient with pyostomatitis vegetans who, upon medical workup, was discovered to have asymptomatic Crohn's disease. The clinical features, histopathology, and treatment of pyostomatitis vegetans, and its purported relationship to pemphigus vegetans of Neumann are discussed.

The Tsk mouse is a genetically transmitted example of cutaneous fibrosis which has been compared with human scleroderma. During a systematic histopathological study of the Tsk mouse, both an increased number and an increased proportion of degranulated mast cells were observed. The consistent association of mast cells and fibrosis in scleroderma, graft-vs-host reactions (GVHR), and now the Tsk mouse raises the question of a pathogenetic role for mast cells in fibrotic disorders in general.

• Herpes gestationis is a pruritic, blistering eruption of pregnancy and the puerperium. In three patients with immunologically verified disease, the clinical presentation consisted of widespread erythematous, edematous papules and plaques, grouped vesicles on erythematous bases, and tense bullae. Histologically, these lesions showed a moderately dense, mixed-inflammatory cell infiltrate around superficial and deep dermal blood vessels, and focal necrosis of epidermal basal cells. Papillary dermal edema, subepidermal bullae, and spongiosis were prominent. Eosinophils were frequently present in the subepidermal and intraepidermal vesicles. Differentiation of herpes gestationis from other blistering diseases and other dermatitides of pregnancy may be difficult. ( Arch Dermatol 112:1543-1548, 1976)

Sixty-seven patients with histologic and immunologic features of bullous pemphigold (BP) were evaluated. Eleven patients had a localized blistering disease that was predominantly confined to one area of the body, most commonly the lower extremities. Two patients displayed a dapsone-responsive blistering disease that was characterized by a flexural distribution of ten to 20 1-cm or less, intensely pruitic, subepidermal bullae and linear IgA basement membrane zone deposition. Two patients had a chronic recalcitrant generalized scarring, hyperkeratotic, subepidermal blistering eruption that demonstrated serologic and direct immunofluorescence (IF) findings of BP. One patient displayed grouped small vesicles surmounted on an erythematous base; the clinical diagnosis was dermatitis herpetiformis, but direct IF examination of the biopsy specimen showed features of BP. One patient with epidermolysis bullosa acquisita had serologic and direct IF features suggestive of BP.

• Various forms of true pemphigus have been reported to occur with myasthenia gravis, with and without thymoma, more frequently than can be ascribed to chance. A 57-year-old woman with myasthenia gravis in remission developed pemphigus foliaceus. No roentgenologic evidence of a thymoma was present. Antinuclear antibodies caused interference with indirect immunofluorescent determination of pemphigus antibody. Indirect immunofluorescent studies showed no evidence for cross-reactivity between intercellular substance antibody and thymic components, as is found in the case of antiskeletal muscle antibodies with thymic myoid cells. The concurrence of these two diseases may involve the failure of a subgroup of thymic-dependent lymphocytes to suppress underlying autoimmune phenomena. (Arch Dermatol111:1334-1339, 1975)

An 87-year-old woman had xanthoma disseminatum. Laboratory studies showed underlying multiple myeloma. This association may be coincidental, but it parallels the well-known relationship of generalized plane xanthoma with myelomatosis. As occurs in the latter case, this patient's myeloma appears to be following a benign course.

Kyrle's disease is an uncommon dermatosis of hyperkeratotic papules, with a distinctive histologic picture characterized by the penetration of a keratotic plug into the dermis. We report a case of Kyrle's disease in association with type II hyperlipoproteinemia. Report of a Case A 51-year-old man with a family history of diabetes mellitus had a personal history of proven nondietary type IIb hyperlipoproteinemia characterized by elevated plasma cholesterol and triglyceride levels; he also had known coronary artery disease and a three-year history of asymptomatic papules on both legs. Physical examination disclosed multiple well-demarcated, flesh-colored papules, with a central keratin plug, over the knees, as well as tibial tuberosities (Fig 1). The diagnosis at the time of examination was Kyrle's disease. A skin biopsy specimen of a keratotic papule showed marked invagination of the epidermis, filled with parakeratotic stratum corneum; absence of the granular layer beneath the parakeratotic horn, with marked thinning

• A healthy, 55-year-old woman developed a subcutaneous abscess and systemic symptoms of nausea, dizziness, and chills following minor trauma to her leg. Histopathologic examination of a skin biopsy specimen revealed golden-brown colored mycelial elements, and culture resulted in growth of a dematiaceous fungus identified asExserohilum rostratum.Surgical excision of the abscess and concomitant oral therapy with ketoconazole resulted in resolution of symptoms. In previously reported cases of human phaeohyphomycosis caused byExserohilumand relatedBipolarisspecies in both immunocompromised and immunocompetent hosts, treatment has varied from topical antimicrobial therapy to combined surgery and intravenous antifungal chemotherapy. Our experience leads us to believe that surgical debridement of an accessible focus of infection along with orally administered ketoconazole may provide adequate therapy in an immunocompetent host. (Arch Dermatol1987;123:1346-1350)

• A case of epidermotropic carcinoma involving the urethral meatus and the skin of the glans penis was associated with primary transitional cell carcinoma of the urinary bladder with urethral extension. The histopathologic and histochemical features were identical to those seen in extramammary Paget's disease. There was positive intracytoplasmic staining for the carcinoembryonic antigen with the use of an immunoperoxidase stain, another similarity between this neoplasm and extramammary Paget's disease. However, these staining characteristics are consistent with those of the primary bladder tumor and with staining patterns described in the literature for urothelial carcinoma. (Arch Dermatol1985;121:532-534)

To the Editor.— Metronidazole is widely used as the drug of choice in the treatment of trichomoniasis. The drug has also been used effectively in treating amebiasis, lambliasis, dracontiasis, and the aucte phase of Vincent's disease.1In addition, it has recently been reported to have a substantial therapeutic effect in rosacea.2The most common side effect of metronidazole therapy is gastrointestinal distress. The more common cutaneous and mucosal reactions include furry tongue, glossitis, stomatitis, and occasional exacerbation of moniliasis; urticaria, flushing, and pruritus may also occur.1 We wish to report a pityriasis rosea-like eruption associated with the administration of metronidazole. Report of a Case.— A 26-year-old married woman was well and in good health until she received metronidazole, 250 mg orally, three times daily, for suspected trichomonal vaginitis. After six days of therapy, six slightly scaling, erythematous oval plaques whose long axes were aligned with the skin

To the Editor.— The recent publication by Wilkin and Kirkendall entitled "Pityriasis Rosea—like Rash From Captopril" in the March 1982Archives(118:186-187) was a worthwhile addition to the dermatologic literature. The authors summarized "pityriasis rosea—like" drug eruptions and stated that "Eight pharmacologic agents have been incriminated in the genesis of a pityriasis rosea—like eruption...." It is unfortunate that the authors failed to include metronidazole, for metronidazole has been reported in this journal as a cause of a pityriasis rosea—like drug eruption. 1

In Reply.— Reactions to drugs may clinically simulate almost every dermatitis and may elicit histological patterns similar to that of almost every inflammatory reaction in the skin. Among the most common are the lichenoid, acneiform, and urticarial. Furthermore, drugs are among the most common causes of bullous dermatitides, such as erythema multiforme and toxic epidermal necrolysis, and of allergic vasculitis. The fixed drug eruption is evoked by drugs. In our experience, however, a drug has yet to provoke histological changes such as those of herpes gestationis and for that reason that condition was not included among our differential diagnoses. Oral contraceptives, however, may exacerbate herpes gestationis as in case 1. We think that, apart from bullous pemphigoid, the histological features of herpes gestationis are rather specific and not imitated by any drug eruption.

The relationship between cutaneous carcinoma and visceral malignant neoplasms has long been a subject of considerable interest as well as controversy. Moller et al 1 and Andersen and colleagues 2 at the Finsen Institute in Denmark have recently refocused attention on this problem. They have found no appreciable deviation from the expected incidence of visceral neoplasms in patients with either Bowen disease or multiple basal cell carcinomas. Their conclusion in the case of Bowen disease differs considerably from that of US authors. Bowen disease is a relatively uncommon type of in situ carcinoma. Graham and Helwig 3 studied 155 patients with Bowen disease; in more than one quarter of their patients, a primary extracutaneous malignant neoplasm developed on an average of five to six years after onset of the cutaneous tumor. Treatment of the Bowen disease had no effect on the subsequent development of systemic malignant neoplasm. Several other investigators

The development of malignant neoplasms in renal transplant recipients is a problem of considerable practical importance as well as biological interest. There is not, however, a simple, acrossthe-board increase in the incidence of all types of neoplasms, as would be expected if this was due solely to the suppression of host defense mechanisms against carcinomas. Skin cancers (39%) and lymphomas (27%) account for approximately two thirds of all cases recorded in the Denver Transplant Tumor Registry. 1 One explanation that has been offered for the increased incidence of lymphomas is that chronic antigenic stimulation of the host lymphoreticular system by the grafted tissue, which bears "nonself" histocompatibility antigens, may lead to the emergence of a neoplastic clone of lymphoid cells. 2 This would not account for the problem of cutaneous carcinomas. Here are other explanations for the increased incidence of neoplasms 3-5 : (1) immunosuppressive therapy impairs the "surveillance" function of

A 9-year-old mentally retarded girl had a systematized leukoderma in the configuration of whorls and streaks that began during the first year of life. No other family members were affected. Histologic examination of a hypopigmented area revealed decreased amount and patchy distribution of melanin in the basal layer of the epidermis. There was no loss of pigment into the underlying dermis. The clinical and histologic features of this case correspond to those previously reported in the Japanese and European literature as incontinentia pigmenti achromians (Ito). Although certain clinical features of this rare syndrome are similar to those found in incontinentia pigmenti (Bloch-Sulzberger), the relationship between the two remains unproved. At present it seems best to consider incontinentia pigmenti achromians as a variant of hypochromic nevus even as Ito did in describing the original case.

Four patients had a distinct variant of exophthalmic goiter (Graves' disease) characterized by pretibial myxedema and nonthyrotoxic thyroid disease. A review of literature revealed 19 cases with similar findings. All were adults and two thirds were women. Exophthalmos and acropachy were present. One third were hypothyroid and the rest were euthyroid at the time the pretibial myxedema, exophthalmos, and acropachy developed. None had ever had signs or symptoms of hyperthyroidism. Long-acting thyroid stimulator (LATS) was present in all cases in which it was sought. Even though a hypermetabolic state was not present in these patients, the occurrence of dermopathy, opthalmopathy, and LATS furnished three of the four components of Graves' disease. Present evidence suggests that the thyroid disease in the patients, whether it be primary hypothyroidism or thyroiditis, is etiologically closely related to the diffuse toxic goiter of Graves' disease.