Cutaneous Papules Research Articles

Multicentric reticulohistiocytosis associated with recurrent breast carcinoma

Multicentric reticulohistiocytosis (MRH) is a rare systemic disorder that most often affects women in the fourth to fifth decades of life and is characterized by widespread cutaneous papules and nodules, often associated with a destructive arthritis. The characteristic histologic feature of the skin lesions, and of other affected organs, is the presence of histiocytes and multinucleated giant cells containing abundant eosinophilic cytoplasm with a “ground glass appearance.” Approximately 30% of the patients have an underlying malignancy suggesting MRH may be a paraneoplastic phenomenon. We describe a case of MRH associated with recurrent, metastatic breast carcinoma. (J Am Acad Dermatol 1998;39:864-6.)

Juvenile xanthogranuloma: three cases

Juvenile xanthogranuloma (JX) is a regressing fibrous histiocytoma occurring during infancy and characterized by cutaneous papules and nodules and less often by additional lesions in deep tissue and organs. It has a special place among childhood masses by presenting different outcomes and spontaneous regression. We report three cases of JX that were detected after birth and resected.

Angiolymphoid hyperplasia with eosinophilia affecting the oral mucosa: report of a case and a review of the literature

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon disorder of unknown aetiology. Clinically, it is characterized by cutaneous papules or nodules on the head and neck, and extracutaneous involvement is rare. We report a 30-year-old woman who had an asymptomatic submucosal nodule on the upper lip, which histopathologically showed features of ALHE, and review the previously described 15 cases of oral ALHE.

The histopathologic spectrum of palisaded neutrophilic and granulomatous dermatitis in patients with collagen vascular disease

<h3>Background and Design:</h3> Patients with lupus erythematosus, rheumatoid arthritis, and other diseases in which circulating immune complexes occur can develop a papular eruption on the extremities. Terms including<i>ChurgStrauss granuloma, cutaneous extravascular necrotizing granuloma, rheumatoid papules, superficial ulcerating rheumatoid necrobiosis, and interstitial granulomatous dermatitis with arthritis</i>have been given to this entity. We evaluated the clinical and histopathologic features of six patients with systemic lupus erythematosus, two patients with rheumatoid arthritis, and one patient with an incompletely characterized collagen vascular disease who developed cutaneous papules. <h3>Results:</h3> The lesions were located largely on the extremities and were symmetrically distributed in most of the patients. They ranged from a few to many and from skin colored to erythematous, and they had smooth, ulcerated, or umbilicated surfaces. Histopathologic examination showed a spectrum of changes that, we believe, reflect the evolution of lesions, ie, leukocytoclastic vasculitis with dense neutrophilic infiltrates and degenerated collagen in early lesions; palisaded granulomas surrounding leukocytoclastic debris, fibrin, and altered collagen in fully developed lesions; and, as the process wanes, palisaded granulomas with dermal fibrosis and scant neutrophilic debris. Each stage of development elicits a different differential diagnosis, which can be resolved by the application of histopathologic criteria. <h3>Conclusion:</h3> The histopathologic findings are consonant with the evolution of an immune complexmediated disease. The diverse histopathologic, and sometimes clinical, appearances account for the variety of names given to this condition. We propose the name<i>palisaded neutrophilic and granulomatous dermatitis of immune complex disease</i>to reflect the histopathologic evolution and clinical aspects of this condition. (Arch Dermatol. 1994;130:1278-1283)

The Histopathologic Spectrum of Palisaded Neutrophilic and Granulomatous Dermatitis in Patients With Collagen Vascular Disease

Patients with lupus erythematosus, rheumatoid arthritis, and other diseases in which circulating immune complexes occur can develop a papular eruption on the extremities. Terms including Churg-Strauss granuloma, cutaneous extravascular necrotizing granuloma, rheumatoid papules, superficial ulcerating rheumatoid necrobiosis, and interstitial granulomatous dermatitis with arthritis have been given to this entity. We evaluated the clinical and histopathologic features of six patients with systemic lupus erythematosus, two patients with rheumatoid arthritis, and one patient with an incompletely characterized collagen vascular disease who developed cutaneous papules. The lesions were located largely on the extremities and were symmetrically distributed in most of the patients. They ranged from a few to many and from skin colored to erythematous, and they had smooth, ulcerated, or umbilicated surfaces. Histopathologic examination showed a spectrum of changes that, we believe, reflect the evolution of lesions, ie, leukocytoclastic vasculitis with dense neutrophilic infiltrates and degenerated collagen in early lesions; palisaded granulomas surrounding leukocytoclastic debris, fibrin, and altered collagen in fully developed lesions; and, as the process wanes, palisaded granulomas with dermal fibrosis and scant neutrophilic debris. Each stage of development elicits a different differential diagnosis, which can be resolved by the application of histopathologic criteria. The histopathologic findings are consonant with the evolution of an immune complex-mediated disease. The diverse histopathologic, and sometimes clinical, appearances account for the variety of names given to this condition. We propose the name palisaded neutrophilic and granulomatous dermatitis of immune complex disease to reflect the histopathologic evolution and clinical aspects of this condition.

Value of immunohistochemistry in the diagnosis of leukemia cutis: study of 54 cases using paraffin-section markers.

A grave prognosis is usually associated with leukemic skin infiltrates (leukemia cutis). However, some leukemic skin infiltrates are clinically similar to reactive non-leukemic infiltrates in patients with leukemia; thus it is of great importance to distinguish them. Fifty-four cases which were thought clinically to be leukemia cutis underwent immunophenotyping with a panel of nine T, B, monocytic, and macrophage markers using paraffin sections. Immunohistochemistry helped identify 44 cases with leukemia cutis and 10 with reactive infiltrates. In all cases of leukemia cutis, the staining patterns of skin infiltrates were concordant with cell type in the bone marrow. Furthermore, the panel of markers was usually helpful in distinguishing reactive from leukemia infiltrates, especially in cases with chronic lymphatic leukemia. Immunohistochemistry is a valuable adjunct in histopathologic differentiation of skin infiltrates in most cases of leukemia. With formalin-fixed, paraffin-embedded biopsies, we recommend that CD45 (LCA), CD45RO (UCHL-1), CD3, CD20 (L-26), CD43 (Leu-22), CD68 (KP-1), lysozyme, and chloroacetate esterase be considered in cases of systemic leukemia with cutaneous papules and nodules that prove difficult to interpret with routine section.

Miliary tuberculosis presenting as skin lesions in a patient with acquired immunodeficiency syndrome

Acute miliary tuberculosis of the skin is an extremely rare infection that occurs in immunocompromised persons. We report an intravenous drug abuser with human immunodeficiency virus infection in whom erythematous papules developed on the trunk and proximal aspect of the extremities. Visceral lesions of unsuspected miliary tuberculosis were discovered at autopsy, and the cutaneous papules were found to contain Mycobacterium tuberculosis. This is the first reported case of this cutaneous infection in a patient with the acquired immunodeficiency syndrome.

Disseminated Dermal Dendrocytomas

A 77-year-old white woman presented with 1 1/2-year history of progressively enlarging cutaneous papules, nodules, and plaques, some of which had spontaneously regressed. Her past medical history included untreated chronic lymphocytic leukemia for 10 years' duration. Multiple skin biopsy specimens revealed a diffuse superficial and deep dermal spindle-cell infiltrate accompanied by occasional foamy round cells and multinucleated giant cells. The spindle-shaped cells were focally arranged in a storiform pattern with prominent fibrous stroma. The spindle-shaped cells stained positively for numerous macrophage markers including CD45, factor XIIIa, Leu M5, HLA-DR, CD4, and Leu M3, consistent with dermal dendrocytes. They were also positive for nonspecific esterase and acid phosphatase, which is typical of tissue macrophages. The spindle-shaped cells were negative for CD-1, S-100, and ATPase activity, thus excluding a Langerhans cell immunophenotype. Combining the clinical features, light microscopy, immunohistochemistry, and enzymatic analysis, this patient appears to represent a novel cutaneous fibrohistiocytic proliferative disorder that features large numbers of dermal dendrocytes.

Systemic Candidiasis

Systemic candidiasis is a disease of increasing incidence and proportions, which appears to be associated with the advances in modern medicine. It involves primarily patients with severe debilitating and malignant disease who are receiving immunosuppressive, cytotoxic, antimetabolite, and antibiotic therapy. Side effects of these otherwise major therapeutic agents predispose patients to opportunistic fungal infections, of which candidiasis is the most common. The high morbidity and mortality of disseminated candidiasis in neutropenic patients are difficult obstacles to obtaining the optimal, if not full, potential of modern chemotherapy for cancer. The inability to diagnose early invasive and systemic candidiasis is a major handicap that delays timely initiation of antifungal therapy. The paucity of highly efficacious antifungal agents with low toxicity severely limits the ability to successfully cure systemic fungal infections in cancer patients. Aggressive research into the basic biology of Candida spp. is necessary for directing the development of better diagnostic methods and improved antifungal drugs.

Congenital, Segmental Pigmented Lesions

REPORT OF A CASE A 64-year-old woman was evaluated at the University of California, San Diego, Medical Center for a lifelong history of pigmented macules involving her left arm, the left side of her chest, and the left side of her back and a 40-year history of multiple soft, asymptomatic, pigmented cutaneous papules involving the same locations. She had hypertension, which had been controlled with diuretic therapy, but she had no history of headaches, hearing loss, speech problems, or seizures. There was a family history of facial freckling, but no family members had similar skin lesions. Physical examination revealed multiple 2- to 10-mm hyperpigmented macules distributed over the left side of the upper part of her back (Fig 1), the left side of her chest, her left breast, the medial aspect of her left arm and forearm, and her left axilla (Fig 2). In the same distribution there were multiple

Molluscum contagiosum in a horse with granulomatous enteritis

Widespread cutaneous papules in a yearling Standardbred filly were attributed by light and electron microscopic examination to molluscum contagiosum. Concomitant granulomatous enteritis, suspected clinically due to protein-losing enteropathy, was verified histopathologically. An associated altered immune response is suggested as the reason for the widespread poxvirus infection.

A Pathological Basis for the Cutaneous Papules of Mucopolysaccharidosis II (The Hunter Syndrome)

Cutaneous papules of two patients with the Hunter snydrome (mucopolysaccharidosis II) were compared with their normal appearing skin by light and electron microscopic techniques. While most fibrocytes of normal and papular skin contained metachromatic cytoplasmic material and characteristic vacuoles, only the papules had extracellular accumulation of metachromatic material and evidence of coalescence and rupture of vacuoles. Alcohol fixation provides better preservation of the mucosubstances.

ERYTHEMA ELEVATUM DIUTINUM

Erythema elevatum diutinum is a disease characterized by red, pink, purple and yellow cutaneous papules, nodules, and plaques distributed mainly over extensor surfaces. Histologically, there is leukocytoclastic vasculitis with fibrinoid necrosis of the upper and mid dermal vessel walls. Two of the five patients presented had moderately severe arthralgias and, unlike previously reported cases, three of them had a long history of bacterial infections and one had a coincident IgA monoclonal gammopathy. Each of the four patients treated with dapsone responded dramatically with rapid resolution of existing lesions and marked diminution of systemic symptoms. Skin lesions and systemic complaints recurred within 12 to 48 hours after discontinuing dapsone. Four of the patients had Arthus-like reactions to SKSD skin tests prior to therapy, with a marked decrease in the response after institution of therapy. Neutrophil chemotactic responsiveness of two patients was impaired. The sera of three patients had significant C1q binding activity suggesting that they might have circulating immune complexes.

Unusual skin lesions of rheumatoid arthritis.

Two patients are reported with the usual manifestations of rheumatoid arthritis but they also had widespread atypical cutaneous papules, nodules, and ulcerated lesions of the legs. The histopathologic changes in the skin in both patients showed multiple areas of necrobiosis with peripheral palisading of fibrocytes at all levels in the corium, thickening and obliteration of small blood vessels, fibrinoid degeneration, and a granulomatous vasculitis. The ulcers developed in preexisting plaques or nodules. The histopathologic features of a granulomatous vasculitis, the rising levels of macroglobulins and latex rheumatoid arthritis factor are compatible with the cutaneous lesions being due to an immunologic reaction involving the cutaneous blood vessels and extravascular connective tissue.

Cutaneous cholesterol ester deposition in Tangier disease

Dermal deposition of cholesterol esters was found in cutaneous papules or clinically normal skin in four of the five known living patients with Tangier disease (familial α- or high-density lipoprotein deficiency). Five patients with Niemann-Pick disease and three normal patients studied in like manner exhibited no evidence of cutaneous cholesterol ester deposition. These cutaneous findings will be useful in the diagnosis, follow-up, and study of this rare and interesting syndrome.

Cutaneous Cholesterol Ester Deposition in Tangier Disease

Dermal deposition of cholesterol esters was found in cutaneous papules or clinically normal skin in four of the five known living patients with Tangier disease (familial α- or high-density lipoprotein deficiency). Five patients with Niemann-Pick disease and three normal patients studied in like manner exhibited no evidence of cutaneous cholesterol ester deposition. These cutaneous findings will be useful in the diagnosis, follow-up, and study of this rare and interesting syndrome.

CUTANEOUS PAPULES HERALDING RHEUMATIC CARDITIS (ROSENBERG).

Cutaneous rheumatic papules, widely disseminated and clinically reminiscent of papular sarcoidosis, were found in a patient with acute rheumatic fever and carditis.

Lipoid dermato-arthritis: Reticulohistiocytoma of the skin and joints

1. 1. Lipoid dermato-arthritis is a recently recognized disease of lipid metabolism characterized by a destructive polyarthritis resembling rheumatoid arthritis and by cutaneous papules and nodules. 2. 2. Characteristic multinucleated giant cells are found in both the affected skin and synovial tissues. 3. 3. The giant cells contain a lipid substance which is probably a glycolipid. 4. 4. Early lesions may contain no giant cells but a polymorphous dermal infiltrate of histiocytes, eosinophils, lymphocytes and extravasated red blood cells. 5. 5. The blood lipids may be normal or slightly elevated. 6. 6. An abnormal electrophoretic lipoprotein pattern is demonstrated. 7. 7. Treatment is unsatisfactory. Administration of corticosteroid hormones may cause temporary regression of the skin nodules but have little effect on the arthritis.

Granuloma annulare in identical twins.

In the past decade, many papers on dermatoses occurring simultaneously in identical twins have been reported in the dermatological literature. No adequate theory has been advanced for this phenomenon. Sharlit 1 believes that it is merely coincidence. Granuloma annulare is not included in the long list of dermatoses in twins in the 1,381-page treatise of Gedda. 2 Recently, we observed identical twins, both with granuloma annulare (Fig. 1). REPORT OF CASES Paula R., a 9-year-old white girl, was first seen at the skin outpatient department of the Memorial Hospital, Pawtucket, R. I., in August, 1951, complaining of a lesion located on the left wrist, of one month's duration (Fig. 2). She had always been in good health. There was no incidence of tuberculosis in the family. Examination revealed cutaneous waxy papules, forming an almost complete circle, measuring 2.5 cm. in diameter, on the anterior aspect of the left wrist. The center was

Correlation of numbers of cutaneous BCG vaccination papules and subsequent tuberculin allergy and tuberculosis resistance in guinea pigs.

Correlation of numbers of cutaneous BCG vaccination papules and subsequent tuberculin allergy and tuberculosis resistance in guinea pigs.