Propranolol: a novel treatment for angiolymphoid hyperplasia with eosinophilia

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon, idiopathic disease that manifests as dermal or subcutaneous red or brown papules or nodules, most commonly on the head and neck. Histologically, ALHE is characterized by vascular proliferation with epithelioid endothelial cells with surrounding lymphocytic and eosinophilic infiltrate. There may be an accompanying serum eosinophilia and local lymphadenopathy. We report a case of a 32‐year‐old woman who presented with multiple erythematous nodules in the periauricular area and the vertex of her scalp. The nodules had been present for several months. Surgical removal of one of the lesions confirmed the histological diagnosis of ALHE. The patient was started on oral propranolol (40 mg once daily) in an effort to reduce the vascular component of the lesions. Within 6 weeks, the patient noted that several of the lesions had decreased in size, and all were less erythematous. Propranolol was subsequently stopped within a few months of initiating treatment. One lesion recurred over a year later, and propranolol was then restarted. No new lesions occurred after 2 years of follow‐up.     

Angiolymphoid hyperplasia with eosinophilia: Successful treatment with the Nd:YAG laser

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon idiopathic proliferation of blood vessels that manifests in adults as isolated or grouped papules, plaques, or nodules in the skin of the head and neck. We describe the case of a 31‐year‐old woman with a 3‐year history of persistent ALHE, located on the tragus of her right ear, with no sign of spontaneous regression over a period of 3–6 months and refractory to intralesional corticosteroids. We report the successful use of the Nd:YAG laser for this condition, which offered excellent symptomatic and cosmetic results and suggests the consideration of this modality in the treatment of ALHE.     

Köbnerization in a woman with generalized lichen sclerosus

A 52‐year‐old woman presented with an 18‐month history of genital and extragenital lichen sclerosus. In addition to the classical genital findings, lesions of lichen sclerosus were present over her back, chest and the medial aspect of her right thigh and leg. On her right thigh and extending to her right leg, lesions of lichen sclerosus displaying the Köbner response were noted over the course of a varicosed long saphenous vein. There were no features of varicose dermatitis in the region displaying the Köbner response. It is proposed that the ambulatory venous pressure within the vein acted as a stimulus for the Köbner response.     

Angiolymphoid hyperplasia with eosinophilia affecting the scrotum: A rare case report with molecular evidence of T‐cell clonality

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare, benign entity of unknown pathogenesis. It often presents as painful or pruritic intradermal or subcutaneous red to brown papules or nodules on the head and neck of young adults. A 38‐year‐old man had a gradually enlarging and mild pruritic plaque on the scrotum for half a year. Pathological findings showed dermal proliferation of anomalous blood vessels lined by plump endothelium with a significant perivascular inflammatory infiltrate composed of lymphocytes, histiocytes, scattered plasma cells and many eosinophils. They were consistent with the diagnosis of ALHE. In addition, the inflammatory infiltrate was analyzed by immunohistochemistry and T‐cell receptor (TCR) gene rearrangement. They were mostly CD3⁺ T cells and a monoclonal T‐cell population. To the best of our knowledge, this is the first case of ALHE affecting the scrotum to be reported in the published work. We present this case to expand the anatomical distribution of this rare tumor. The molecular study of our case supports that ALHE might be a low‐grade T‐cell lymphoproliferative disorder.     

Localized soft tissue angiomatosis with subsequent development of angiolymphoid hyperplasia with eosinophilia

We report an unusual case of arteriovenous (AV) malformation (localized form of soft tissue angiomatosis): a reaction of angiolymphoid hyperplasia with eosinophilia (ALHE) developed over the lesion of this pre-existing AV malformation. To the best of our knowledge, there is no other report of ALHE in association with a pre-existing bona fide AV malformation. A 71-year-old woman consulted our clinic because a reddish, dome-shaped tumor on her preauricular region, present as a slightly elevated, skin-colored tumor since her childhood, had rapidly enlarged and become more elevated with color change during the preceding two months. The histological features of most of the lesion corresponded to those of soft tissue angiomatosis (localized form). The proliferative vessels underlying the soft tissue angiomatosis streamed out and welled into the dermis with an accompanying prominent inflammatory infiltrate composed of lymphoid cells and eosinophils: these histological features were characteristic of ALHE. The recent abrupt clinical changes in the tumor were considered to represent a reaction to the angiomatosis with development of ALHE.     

Lymphoplasmacytic plaque in children: a report of two new cases with review of the literature

Lymphoplasmacytic plaque in children has been proposed as a rare, emerging clinicopathologic entity characterized by solitary, extratruncal, asymptomatic papules and plaques that are typically found in healthy young Caucasian females. Biopsy of these lesions reveals a dermal lymphoplasmacytic infiltrate with or without epithelioid granulomas. Two unique patients with lymphoplasmacytic plaque in children are presented in this report, including a 26‐month‐old female with a lesion on her finger, who represents both the youngest described patient and the first documented with a finger lesion, as well as a 17‐year‐old young woman with a left thigh lesion, who represents the patient with the longest clinically and histopathologically observed lesion to date. These two additional patients corroborate the experience of lymphoplasmacytic plaque in children in the six previously reported cases and further expand the clinicopathologic spectrum of the disease. Recognition of lymphoplasmacytic plaque in children is important to facilitate distinction from potential differential considerations, including lymphoproliferative disorders and infectious conditions, particularly as the experience to date appears to suggest that lymphoplasmacytic plaque in children represent a reactive (pseudolymphomatous) condition.     

A congenital smooth muscle hamartoma masquerading as a reticulate vascular naevus

Smooth muscle hamartoma (SMH) is a benign congenital or acquired hamartomatous lesion comprising a dermal proliferation of smooth muscle bundles. We report a case of congenital SMH with an unusual clinical appearance. A 3‐year‐old girl presented with an asymptomatic atrophic linear lesion on the posterior surface of her right thigh, which had been present since birth. The striking resemblance to a vascular lesion initially led to the erroneous clinical diagnosis of atrophic reticulate vascular naevus. However, a skin biopsy showed typical features of SMH. To our knowledge, SMH with linear configuration has only been described in two previous cases, and there are no previous reports of SMH with such a marked resemblance to a vascular lesion.     

Cutaneous aneurysmal bone cyst—First report of a case and literature review

Aneurysmal bone cyst (ABC) is a benign, expansile, multiloculated and blood‐filled cystic lesion most commonly involving bones. We report herein the case of a 78‐year‐old woman with a dermal ABC located in the skin of the right foot. Histologically, the lesion displayed characteristic features of ABC, including blood‐filled cystic spaces lined by loose connective tissue, admixed with solid areas composed of spindle cells and osteoclast‐like giant cells, associated with foci of woven bone formation and matrix calcification (“blue bone” formation). Fewer than 30 cases of extraosseous ABCs have been described in soft tissues and, to the best of our knowledge, ABC primarily occurring in the skin, not associated with an underlying lesion in the bone, has not so far been reported in the literature.     

An unusual clinical presentation of lupus erythematosus tumidus localized on the thigh

A 44‐year‐old woman with seronegative polyarthritis presented with a 2‐year history of a solitary, bluish‐red, oedematous, nonscaly, annular and partially reticulated macule on her right thigh. Histopathological findings revealed perivascular and periadnexal lymphocytic infiltrate in the dermis. Alcian blue and colloidal iron stains highlighted mucinous deposit in the upper and mid dermis. Direct immunofluorescence showed a linear deposit of IgG and C3 along the basement membrane zone. Antinuclear antibody was positive at a titre of 1 : 80, with homogenous and speckled patterns. Except for its unusual localization and lack of photosensitivity, our case had the clinical and histopathological features of lupus erythematosus tumidus. These characteristics were also reminiscent of reticular erythematous mucinosis and erythema annulare centrifugum, both of which are considered to be associated with cutaneous lupus erythematosus (CLE). Hydroxychloroquine 200 mg daily led to improvement of the skin lesion. The unusual clinical presentation of our case emphasizes the heterogeneity of clinical manifestations of CLE.     

Lipomatous neurofibromas with giant pigmented lesion

Neurofibroma is the most common benign neural tumour. Among the large number of histopathological variants reported, lipomatous neurofibroma is exceedingly infrequent, and shows the presence of intratumoral adipocytes admixed with spindle cells in the dermis. We present a case of a 37‐year‐old man with lipomatous neurofibromas associated with a giant garment‐like pigmented lesion involving his lower trunk and right thigh.     

Pseudogranulomatous Spitz nevus: a variant of Spitz nevus with heavy inflammatory infiltrate mimicking a granulomatous dermatitis

Spitz nevus is a benign melanocytic proliferation that shows relatively characteristic clinicopathologic features. Despite this, Spitz nevus is clinically confused with many other lesions, and histopathologically it is sometimes difficult to distinguish it from melanoma. However, Spitz nevus rarely causes differential diagnostic problems with granulomatous dermatitis. This article describes an 8‐year‐old girl who presented with a nodule on her right arm, a clinical appearance of a pyogenic granuloma. Histopathologically, there was a dermal lesion composed of aggregates of large epithelioid cells surrounded by a heavy inflammatory infiltrate, mimicking a sarcoid‐like granulomatous dermatitis. Immunohistochemistry showed epithelioid cells with strong nuclear and cytoplasmic staining with S‐100 protein, thus establishing the diagnosis of a melanocytic tumor. The heavy T‐cell lymphocytic infiltrate that accompanies the large epithelioid cells caused its granulomatous appearance. Molecular assessment showed H27H mutation in the HRAS gene. We suggest the term ‘pseudogranulomatous’ for this variant of Spitz nevus because it indicates that the lesion is not authentically granulomatous and simply mimics a granulomatous dermatitis.     

Lupus vulgaris–a case diagnosed more than 20 years after onset

A 47‐year‐old Turkish woman developed an erythematous scaly plaque on her thigh over 20 years. Cutaneous sarcoidosis had been diagnosed eight years previously on a skin biopsy. We confirmed Mycobacterium tuberculosis infection by polymerase chain reaction and cultivation of the causative organism from lesional skin. After several months of antituberculous treatment with isoniazid, rifampicin, ethambutol and pyrazinamide, the lesion resolved.     

Does bullous pemphigoid with negative direct immunofluorescence exist? Apropos of 3 cases]

Subepidermal autoimmune bullous dermatoses are defined by clinical, histological and immunological criteria, notably the presence of anti-basement membrane antibodies detectable in vivo by direct immunofluorescence. We report three cases where anti-basement membrane antibodies were not detectable in vivo by direct immunofluorescence but were detected in high titres by indirect immunofluorescence. This situation is extremely rare in the literature. The first case concerns a 69-year old woman seen for bullous and pruriginous lesions of the lower limbs of 2 months' duration. Histological examination found dermoepidermal bullae with, in their lumen, a serous fluid spotted with numerous polymorphonuclears. A search for anti-basement membrane antibodies was positive in significant titres (1,280; 320; 480) at indirect immunofluorescence on rabbit lip whereas five successive direct immunofluorescence test in perilesional skin and on the thigh medial surface remained negative. The second case is that of a 91-year old woman suffering from generalized pruritus associated with erythematous lesions predominant on the extension surfaces of the forearms and thighs, without any bullous lesion. Pathological examination only showed a superficial dermal lymphocytic infiltrate. Four direct immunofluorescence tests were negative, whereas a search for anti-basement membrane antibodies on rat oesophagus was positive at 1/1,280. The third case resembles the second one. It concerns a 72-year old woman who consulted for generalized pruritus of several months' duration which interfered with sleep and was incompletely relieved by emollients. There was no specific skin lesion. Pathological examination revealed nothing more than a discrete perivascular mononucleate infiltrate. Direct immunofluorescence tests performed on two occasions on the skin of the abdomen and of the medial thigh surface were negative.(ABSTRACT TRUNCATED AT 250 WORDS)     

Neurotropic T‐cell lymphocytosis: a cutaneous expression of CLIPPERS

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disease of the central nervous system that predominantly involves the pons and cerebellum and that improves with immunosuppressive treatment. Only recently described, the etiology is unknown, diagnosis is difficult and long‐term neurological sequelae may occur without aggressive treatment. Herein, we describe a 59‐year‐old woman who presented with subcutaneous nodules affecting her face, trunk, limbs and an indurated annular erythematous lesion on her forearm. This was associated with marked dysesthesia of her skin, refractory to treatment. There was a 4‐year history of dysequilibrium, vertigo, truncal and gait ataxia with progressive neurological symptoms. Skin biopsy of the annular nodular lesion showed a lymphohistiocytic infiltrate in dermis and subcutis with a striking lymphocyte‐dominant infiltrate that was perineural and formed a nodular collection extending along a prominent subcutaneous nerve. Immunophenotyping indicated a marked predominance of T cells that were CD3 positive with a 2 : 1 CD4 : CD8 ratio. Scattered histiocytes were present but no well‐formed granulomas or vasculitis. Magnetic resonance imaging studies showed changes in the pontine, brain stem and cerebellar region, which subsequently were defined as characteristic for CLIPPERS, but no brain biopsy was pursued. The marked neural skin symptoms and the cutaneous histopathological findings indicate that the skin may be an additional target organ in CLIPPERS, and the immune response may be directed against a common neural antigen. In radiologically typical CLIPPERS, identification of clinical skin lesions particularly subcutaneous nodules and biopsy may potentially form a basis for tissue diagnosis in this syndrome.     

Buschke-Ollendorff syndrome

Background Buschke–Ollendorff syndrome is a rare autosomal dominant disease featuring osteopoikilosis and skin lesions. It is caused by genetic mutations in a protein deeply involved in bone and connective tissue morphogenesis. Methods We describe a 39‐year‐old woman with Buschke–Ollendorff syndrome. Results After a minor trauma, radiologic examination of the left ankle of a 39‐year‐old woman revealed features of osteopoikilosis. Physical examination of the patient showed multiple asymptomatic nodules on both thighs, present since the age of 20 years, which had increased in size and number. Recently, a linear, string‐like lesion had appeared on the right thigh. Conclusion The correct diagnosis of Buschke–Ollendorff syndrome may require a high index of suspicion.     

Progressing dermatofibromas following surgery

A 39-year-old woman, had a lesion on her right thigh that first developed when she was 6 weeks old. This lesion was said to have resembled "spider bites," and was initially treated with cryotherapy. It continued to enlarge and was biopsied in May 1969, when she was 13 years old. The patient was told she had "neurofibroma." In 1971, the lesion was rebiopsied and a question of a sarcoma was raised in the pathology report. She underwent extensive surgery to remove the lesion, requiring a skin graft at the operation sife. Multiple lesions subsequently developed around her graft. Biopsies of several lesions were interpreted as dermatofibroma, and she was referred to the Indiana University, Department of Dermatology. When seen in 1995, she had a well-healed 30 cmx14 cm hypopigmenfed split-thickness skin graft present on the lateral right thigh. The margin of the graft was outlined by multiple red-brown firm papules and plaques. Some of the papules and plaques extended beyond the margin of the graft by several centimeters (Fig. 1). Histopathology of the satellite lesions showed epidermal hyperplasia and diffuse spindle cell proliferation in the upper and lower dermis (Fig. 2). The majority of the spindle cells stained positively for Factor Xllla (a marker for dermal dendrocytes). They did not stain with CD34 (a marker for dermatofibrosarcoma protuberans).     

Primary localized cutaneous nodular amyloidosis in a patient with Sjögren's syndrome: a review of the literature

We report a 53‐year‐old Japanese woman with multiple, red, and elastic soft nodules on the left waist, left thigh, and right lower leg. She had had polyclonal hyperglobulinemia for one year, rheumatoid arthritis for 13 years, and Sjögren's syndrome (SjS) for 18 years. Histochemical examination of the nodule on the left thigh revealed a deposition of amyloid by Congo red staining. It was also positively stained with both anti‐κ and ‐λ light chain antibodies. Moreover, the cytoplasm of the infiltrating plasma cells also positively reacted to both antibodies. The major amyloid proteins of primary localized cutaneous nodular amyloidosis (PLCNA) generally consist of monoclonal immunoglobulin light chains. A review of literature demonstrates 13 cases of PLCNA with SjS, in which immunoglobulin light chains were demonstrated in the amyloid in 5 cases. Amyloid in the 3 cases was composed of a single class immunoglobulin light chain and that in the 2 cases was composed of both κ and λ light chains. Polyclonal immunoglobulin amyloid has been reported only in PLCNA with SjS, which may be related to the fact that a certain population of SjS develops polyclonal B cell proliferation and hyperglobulinemia.     

A case of necrobiotic xanthogranuloma without paraproteinemia presenting as a solitary tumor on the thigh

A 82‐year‐old Korean woman had had a 6‐month history of an asymptomatic, flat, hard, red to brown tumor on her right thigh. This lesion had been slowly enlarging with an advancing margin. She had noted gradually developing pain associated with necrosis and ulceration on the lesion. Examination revealed a solitary, 8 × 7.5 cm, yellow to dark red, telangiectatic tumor with multiple areas of punched out ulceration and a peripheral elevated yellowish margin on the right inner upper thigh ( Fig. 1 ). No clinically similar lesions on the periorbital area or other sites were seen. Histologic examination revealed a massive palisading granulomatous infiltration with several layers of extensive bands of necrobiotic zone in the entire dermis and deep subcutaneous tissue ( Fig. 2a ). In the granulomatous infiltrate in the dermis and subcutis, many various‐shaped, some bizarre, angulated, foreign‐body type multinucleated giant cells, many Touton giant cells, and a few Langhans giant cells were found to be scattered ( Fig. 2b ). There were numerous xanthomatized histiocytes. Dense infiltration of lymphoplasma cells was seen in the periphery of the granuloma and perivascularly. Conspicuous granulomatous panniculitis composed of lymphoplasma cells, polymorphonuclear cells, foam cells, and Touton and foreign‐body giant cells was also seen. However, cholesterol clefts and lymphoid follicles were not seen. Subcutaneous septae were widened by necrobiotic change and fibrosis with thrombosed large vessels. Gram, Gomeri‐methenamine silver and acid‐fast stains were negative. The necrobiotic areas were positive to alcian blue. Laboratory investigation revealed elevated white blood cell counts, anemia and elevated erythrocyte sedimentation rate. The following parameters were within the normal range: lipids, glucose, renal and liver function tests, serum complements, serum immunoglobulins, cryoglobulins and antinuclear antibodies. The findings of chest X‐ray, skull X‐ray and ectorcardiography were normal. Serum electrophoresis and serum immunoelectrophoresis revealed no abnormality. The patient was diagnosed as having necrobiotic xanthogranuloma without paraproteinemia. She was treated with oral steroid (0.5–0.6 mg/kg) and NSAIDS for 1 month with partial improvement of pain and the lesion ceased to enlarge. In the following 1 year of follow‐up, with only intermittent NSAIDS, her lesion did not progress and there were no signs of systemic involvement or new skin lesions.

Figure Figure 1  Open in figure viewer PowerPoint (a) A solitary, red to brown plaque with multiple ulcerations and a peripheral elevated yellowish margin on the inner upper thigh     

Cutaneous pseudolymphoma associated with molluscum contagiosum: a case report

We report an unusual case of molluscum contagiosum associated with pseudolymphoma in an otherwise healthy young woman. She presented with a 2-week history of a rapidly enlarging painful umbilicated nodule behind her right ear. With the clinical presentation suspicious for a tumoral lesion, we decided to remove it surgically. Histological examination showed a florid cellular infiltrate surrounding a typical lesion of molluscum contagiosum. The infiltrate was composed of small to large pleomorphic lymphocytes. However, clonal TCR rearrangement could not be demonstrated. As far as we know this is the first case where the clinical picture is shown.     

Angiolymphoid hyperplasia with eosinophilia: successful treatment with the Nd:YAG laser.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon idiopathic proliferation of blood vessels that manifests in adults as isolated or grouped papules, plaques, or nodules in the skin of the head and neck. We describe the case of a 31-year-old woman with a 3-year history of persistent ALHE, located on the tragus of her right ear, with no sign of spontaneous regression over a period of 3-6 months and refractory to intralesional corticosteroids. We report the successful use of the Nd:YAG laser for this condition, which offered excellent symptomatic and cosmetic results and suggests the consideration of this modality in the treatment of ALHE.