Multinucleate cell angiohistiocytoma: a fibrohistiocytic proliferation with increased mast cell numbers and vascular hyperplasia

BACKGROUND: Multinucleate cell angiohistiocytoma (MCAH) is an uncommon lesion clinically characterized by multiple papules usually located on the face and acral regions of elderly women. Histopathologically, MCAH is characterized by dermal vascular hyperplasia associated with increased number of factor XIIIa-positive fibrohistiocytic cells and multinucleate cells with scalloped borders. METHODS: We report the clinical, histopathological and immunohistochemical features of three cases of MCAH, with ulstrastructural study in one of them. The patients were a woman and two men of 56, 40 and 70 years of age, respectively. They all had multiple dull-red papules, which had appeared over several years and were located on the face, the trunk and the dorsa of the hands, respectively. RESULTS: The reticular dermis presented a fibrohistiocytic proliferation of factor XIIIa-positive cells, with abundant bizarre multinucleate cells and vascular hyperplasia. Increased mast cell numbers were seen in all cases, often in apposition to multinucleate cells. CONCLUSION: Histopathological differential diagnosis of MCAH includes mainly angiofibromas and dermatofibromas, even though vascular hyperplasia can be prominent and has led to many authors to classify MCAH among vascular tumors. Bizarre multinucleate cells can be found in reactive, neoplastic and inflammatory lesions in many sites of the body, and mast cells can play a role in their morphogenesis.     

Multinucleate cell angiohistiocytoma: a report of two cases

We report the clinical, histological and immunological features of two cases of multinucleate cell angiohistiocytoma (MCAH) in women of 32 and 53 years of age, respectively. Clinically, MCAH occurs mostly in middle-aged women and consists of crops of reddish-purple, dome-shaped papules especially on the limbs. Histologically, the reticular dermis presents an increased number of small vascular channels with plump endothelial cells embedded in a fibrohistiocitic stroma with numerous bizarre multinucleate cells. Bizarre multinucleated cells are not specific to MCAH; they can be observed in numerous other cutaneous conditions. However, MCAH presents quite distinctive clinico-pathological findings and may be easily differentiated from other cutaneous disorders.     

PAPULAR MUCINOSIS

Most patients previously reported to have papular mucinosis had the generalized lichenoid papular form (scleromyxedema) with abnormal gamma globulin. Microscopic examination of affected skin showed increased acid mucopolysaccharides in the dermis and proliferation of fibroblasts. Our patient had the discrete form of papular mucinosis without abnormal serum gamma globulin but with increased amounts of acid mucopolysaccharides in the dermis and no proliferation of fibroblasts.     

Erythema scarlatiniforme desquamativum generalisatum

Erythema scarlatiniforme desquamativum generalisatum (Féréol-Besnier disease) is a rare skin disease characterized by generalized erythematous rash with subsequent desquamation. An 86-year-old woman presented with generalized erythema followed by an extensive, scarlatiniform peeling especially of the hands and feet. This generalized episode may be followed by erythema scarlatiniforme desquamativum localisatum recidivans, which is a recurring variant of the disease, localized to the hands and feet.     

Cutaneous plasmacytosis and multinucleate cell angiohistiocytoma‐like lesion in a patient with hepatitis B: A fortuitous triad?

A 28‐year‐old woman of Chinese descent, with congenital chronic hepatitis B presented with a 7‐year history of erythematous‐brown papules and plaques on her groins, axillae, and forehead. A first skin biopsy showed findings consistent with two concomitant, yet highly uncommon cutaneous diseases. The presence of lymphoid nodules with germinal centers and clustered polyclonal plasma cells was consistent with cutaneous plasmocytosis. Second, a diffuse proliferation of non‐atypical small vessels (CD31+, CD34+, and HHV8?) in a hypercellular stroma peppered with angulated giant cells (CD163+, CD68?) was suggestive of multinucleate cell angiohistiocytoma (MCAH). Interestingly, the second biopsy of a different plaque on the forehead showed only plasmacytosis and the clinical appearance of both plaques and papules alluded to the distinct presence of both concurrent entities. We speculate the immune modulating effects of chronic hepatitis B may have led to a polyclonal plasmacytic proliferation within the dermis. Furthermore, MCAH has been reported in conjunction with other inflammatory skin diseases such as hidradenitis suppurativa and as such we propose that the MCAH lesion in our case may have arisen as a secondary, reactive process to the cutaneous plasmacytosis.     

Multinucleate cell angiohistiocytoma: an uncommon mucosal tumour

Multinucleate cell angiohistiocytoma (MCAH) is an uncommon benign soft-tissue lesion with characteristic histological and immunohistochemical features. It is unclear if it represents a benign neoplasm or a reactive/inflammatory process. The overwhelming majority of these tumors have been described on cutaneous surfaces. One case has been reported on the skin of the lip. This is the first documentation of MCAH within the oral cavity. The clinical presentation, histopathological features and immunohistochemical reactivity pattern are described. Because of the benign nature of this lesion, a conservative approach is recommended in its management. Surgical excision appears to be curative.     

Multinucleate-cell angiohistiocytoma occurring in a patient with mycosis fungoides

Multinucleate-cell angiohistiocytoma (MCAH) is a benign vascular proliferation of unknown etiology. Clinically, MCAH presents as grouped, erythematous, or violaceous papules on the extremities in older women. These lesions often resemble Kaposi's sarcoma. Histologic examination reveals characteristic bizarre-shaped, multinucleate giant cells, some of which contain three to six nuclei arranged in a ring-like or overlapping pattern, which stain positively for Factor XIIIa. In addition, there is a proliferation of dermal capillaries and venules with a mild lymphohistiocytic infiltrate. To our knowledge, this is the first reported case of MCAH occurring in a patient with mycosis fungoides.     

Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema

Lichen myxedematosus (LM) is an idiopathic cutaneous mucinosis; its classification dates back to 1953, when Montgomery and Underwood distinguished 4 types of LM: a generalized lichenoid eruption, later called scleromyxedema, a discrete papular form, a localized or generalized lichenoid plaque form, and an urticarial plaque form. In the literature, the terms LM, papular mucinosis, and scleromyxedema have been often used indiscriminately as synonyms, but most reported cases of LM or papular mucinosis without indication of the subtype appear in fact to be cases of scleromyxedema. On the basis of personal experience, the anatomoclinical manifestations of published cases of LM, papular mucinosis, and scleromyxedema are reviewed to distinguish clearly between a generalized form with systemic, even lethal, manifestations and a localized form, which does not run a disabling course. LM includes two clinicopathologic subsets: a generalized papular and sclerodermoid form (also called scleromyxedema) and a localized papular form. Diagnosis of scleromyxedema should fulfill the following criteria: (1) generalized papular and sclerodermoid eruption; (2) mucin deposition, fibroblast proliferation, and fibrosis; (3) monoclonal gammopathy; and (4) the absence of thyroid disease. The criteria for localized LM are as follows: (1) papular or nodular/plaque eruption; (2) mucin deposition with variable fibroblast proliferation; and (3) the absence of both monoclonal gammopathy and thyroid disease. The localized form is subdivided into 5 subtypes: (1) a discrete papular form involving any site; (2) acral persistent papular mucinosis involving only the extensor surface of the hands and wrists; (3) self-healing papular mucinosis, of a juvenile and an adult type; (4) papular mucinosis of infancy, a pediatric variant of the discrete form or of acral persistent papular mucinosis; and (5) nodular form. A third group of atypical or intermediate forms, not meeting the criteria for either scleromyxedema or the localized form, includes cases of (1) scleromyxedema without monoclonal gammopathy, (2) localized forms with monoclonal gammopathy and/or systemic symptoms, (3) localized forms with mixed features of the 5 subtypes, and (4) not well-specified cases.     

The sarcoidal granuloma: A unifying hypothesis for an enigmatic response

Although the cause of sarcoidosis is unknown, there is growing support for the concept that sarcoidal granulomas result from a hypersensitivity reaction producing a nonspecific response to an extrinsic or intrinsic (autoimmune) antigen in genetically susceptible individuals. The immune milieu associated with these antigens, localized in a specific cutaneous area, produces a variant of Ruocco’s “immunocompromised district.” This may explain the predilection for sarcoidal granulomas in association with foreign bodies, tattoos, herpes zoster–affected dermatomes, and scars. Similar antigenic stimulation produces sarcoidal granulomas surrounding internal tumors. Finally, systemic sarcoidosis, as manifested by hilar adenopathy, may reflect the lymphatic spread of foreign antigens.     

Comparative ultrastructural study of generalized and localized granuloma annulare

The dermal changes in four cases of generalized granuloma annulare (GGA) and in four of localized granuloma annulare (LGA) were studied ultrastructurally, and the findings in the two groups were compared. The basic alterations were similar in both types, and showed varied stages of development. The cellular infiltrate was composed mostly of histiocytes. Cell debris was found in all lesions. The degenerative changes affected the collagen fibers, the elastic fibers, and the cellular infiltrate. Of special interest are the following findings in GGA: First, masses of intercellular fibrin material were seen only in the lesions of GGA. Second, thick and multilayered basal lamina around capillaries was apparently more common in the generalized form. These changes may suggest that a more pronounced immune reaction is responsible for the development of the generalized form of the disease.     

Scleromyxedema: An experience using treatment with systemic corticosteroid and review of the published work

Scleromyxedema, a rare cutaneous mucinosis of unknown cause, is a variant of generalized papular mucinosis that is also known as generalized lichen myxedematosus. It is characterized clinically by generalized papular or scleroderma-like eruptions. Histopathological examination reveals mucin deposition and a proliferation of fibroblasts in the upper dermis. We describe the case of a man with scleromyxedema treated with systemic corticosteroids whose skin lesions improved gradually within 4 weeks.     

Increased expression of tenascin in the dermis in scleroderma

The expression of tenascin, a recently discovered extracellular matrix protein, was studied by immunohistochemical techniques in scleroderma skin and compared with its distribution in normal skin. In progressive systemic sclerosis, a marked increase in tenascin content was observed in the superficial reticular dermis. In localized scleroderma, the deposition of tenascin was increased both in the superficial and deep dermis of involved skin, whereas in clinically uninvolved skin the distribution of tenascin was the same as in normal control skin, i.e. the papillary dermis and peri-appendiceal zone. The distribution of tenascin did not strictly parallel that of fibronectin. These findings and the current knowledge of tenascin biology suggest that the overproduction of tenascin in scleroderma dermis could be secondary to stimulation of fibroblasts by immune cell-derived cytokines, or could be due to abnormal fibroblasts, or a subpopulation of fibroblasts, producing high levels of this extracellular matrix protein.     

环状肉芽肿43例分析

目的 探讨局限型、泛发型环状肉芽肿诱发因素、临床、组织学改变及治疗情况。方法 回顾性分析24例局限型及19例泛发型环状肉芽肿患者临床资料。结果 两型中尤其是泛发型中部分患者病情与日晒明显相关。局限型皮损常局限于手、颈后、足背，小丘疹排列成环状，直径1～2cm，最大7cm；泛发型皮损较小，0．5～1cm，常泛发。组织学改变以栅状肉芽肿最多见，占61．9％，散在性组织细胞浸润次之，占38．1％．两者均以局限型较泛发型为多。局限型以避光、局部外用糖皮质激素、冷冻、手术切除为主．必要时口服维生素E、烟酰胺、顽固者口服小剂量氯喹可控制病情。泛发型以系统用药为主，氯喹、氨苯砜、糖皮质激素及异维A酸可取得满意疗效，但部分停药后易复发。结论日光照射可能在泛发型环状肉芽肿发病中起着一定的作用；组织学改变以栅状肉芽肿最多见；局限型以局部治疗为主，泛发型以系统治疗为主。     

Acral peeling skin syndrome

Peeling skin syndrome is a rare autosomal recessive disease characterized by widespread painless peeling of the skin in superficial sheets. We describe a 34-year-old man with a lifelong history of spontaneous asymptomatic peeling skin limited to the acral surfaces. This patient probably represents a localized variant of peeling skin syndrome, which has previously been described as a generalized condition. Light and electron microscopic studies of biopsy specimens taken before and after immersion in water were performed. It was concluded that this patient has abnormal keratohyalin granules and inadequate aggregation of keratin filaments that caused the separation of the epidermis in the stratum corneum through the clear zone. Alternatively, unknown keratin species expressed in the clear zone may also cause the abnormality. (J Am Acad Dermatol 2000;43:1112-9.).     

DHMEQ, a novel NF-kappaB inhibitor, suppresses growth and type I collagen accumulation in keloid fibroblasts

BACKGROUND: Keloid is a benign dermal tumor characterized by proliferation of dermal fibroblasts and overproduction of extracellular matrix (ECM). Nuclear factor kappaB (NF-kappaB) plays an important role in regulation of inflammation, immune response and cell proliferation. Activation of the NF-kappaB pathway is thought to be closely linked to abnormal cell proliferation and ECM production in keloid fibroblasts. OBJECTIVE: This study was set out to investigate the effects of a novel selective NF-kappaB inhibitor, dehydroxymethylepoxyquinomicin (DHMEQ), on keloid fibroblasts. METHODS: Primary normal and keloid dermal fibroblasts were used for this study. NF-kappaB activity was assessed by DNA-binding assay and immunohistochemistry. The effect of DHMEQ was evaluated by cell viability, cell growth and type I collagen accumulation. RESULTS: Basal NF-kappaB activity was constitutively elevated in keloid fibroblasts, indicating that this pathway is involved in keloid pathogenesis. DHMEQ markedly reduced cell proliferation and type I collagen accumulation in keloid fibroblasts. CONCLUSION: The inhibition of NF-kappaB by DHMEQ may be an attractive therapeutic approach for keloids.     

Basaloid follicular hamartoma

Basaloid follicular hamartoma (BFH) is a unique benign follicular hamartoma characterized by variable clinical presentations, identical histologic features and possible associations with numerous disorders. Basaloid follicular hamartoma may be hereditary or acquired. Hereditary cases may be either generalized or unilateral nevoid. Although the generalized forms are usually associated with systemic manifestations, such as myasthenia gravis,(2) it may occasionally present without internal disorders. On the other hand, the acquired forms of BFH may present in the form of localized or solitary forms. Herein we present four cases of BFH, one of them (first case) represents a unique form of the generalized variant of BFH, showing no associated internal disorders.     

A case of multinucleate cell angiohistiocytoma in a 14‐year‐old boy showing two different clinical and histopathological findings

Multinucleate cell angiohistiocytoma (MCAH) is a rare cutaneous disease entity characterized by multiple red‐to‐brown or violaceous papules usually located on the acral regions, such as the face and the distal arms and legs. It affects elderly women more than men and rarely occurs at a young age. The exact pathogenic mechanism of MCAH is not yet clearly understood. We report an exceptionally rare case of a 14‐year‐old boy who presented with multiple asymptomatic erythematous papules and a single flat brownish plaque on the left chest. The brownish plaque lesion histologically showed proliferation of dilated small vessels in the upper‐mid dermis and numerous oddly shaped multinucleate cells intermingled with lymphocytes and macrophages. The erythematous papules also showed dilated small vessels in the upper‐mid dermis and multiple interstitial histiocytic infiltrations, but no multinucleate cells were detected. In immunohistochemistry studies, CD68 and vimentin staining were positive for both specimens. Based on the clinicopathological findings and immunohistochemistry studies, MCAH was diagnosed. To the best of our knowledge, this is the first case report of MCAH occurring in young age and showing two different clinical and histological phases at the same time.     

Recurring localized scarlatiniform scaled erythema Féréol-Besnier

Recurring scarlatiniform scaled erythema of Féréol-Besnier is a rare disease characterized by recurrent episodes of a prodromal phase with general malaise, head and muscle aches, gastrointestinal complaints and fever followed by an erythematous rash leading to extensive desquamation of the involved skin. It exists in a generalized and localized variant, the latter mainly involving the hands and feet. Its cause is unknown, although it has been speculated that a hyperergic reaction to infectious agents or medications may be etiopathologically involved. A typical case of the localized variant of this obscure disease is described and the common literature is reviewed.     

Pediatric generalized lichen nitidus treated with natural sunlight therapy

Lichen nitidus is a benign inflammatory dermatosis that typically presents in a localized distribution. We present the rare case of a 6‐year‐old boy with a 1‐year history of generalized lichen nitidus with limited access to narrowband ultraviolet B phototherapy. Over the course of a summer, he had complete and lasting resolution of generalized lichen nitidus after daily natural sunlight exposure. This case demonstrates a rare variant of lichen nitidus and a practical treatment alternative to in‐office phototherapy.