Generalized eruptive histiocytosis

The histiocytic disorders can be broadly categorized into histiocytosis X (Langerhans cell-derived) and non-X types. There are several variants of non-X histiocytoses that tend to occur in a generalized distribution on the body; these include xanthoma disseminatum, generalized eruptive histiocytosis (GEH), progressive nodular histiocytosis, and multicentric reticulohistiocytosis. Clinical and pathologic correlation are required for differentiating among these 4 disorders. We report a case of a middle-aged man in whom small, scattered, symmetrical lesions on the trunk and proximal extremities developed that, after correlating with biopsy specimen and laboratory results, were best classified as a non-X histiocytosis with features of GEH. GEH is a rare generalized non-X histiocytosis that occurs mainly in adults. It is characterized by multiple, scattered, symmetric lesions on the trunk and proximal extremities that are benign in nature and tend to resolve spontaneously. Recent literature has suggested that GEH may be a part of a continuous spectrum of non-X histiocytic disorders.     

Systemic xanthohistiocytoma: a variant of xanthoma disseminatum?

We present a 66-year-old man who had maculopapular pigmented lesions on the skin of the head, neck and trunk suggesting generalized eruptive histiocytoma (GEH). These lesions had a yellowish centre in a target-like pattern that has not been previously described. The patient suffered from diplopia and had a severe sensorimotor polyneuropathy causing progressive paresis of the limbs. The explorations performed disclosed the presence of specific xanthomatous infiltrates in the skin, lungs, respiratory tract, peripheral nerves and meninges, suggesting xanthoma disseminatum (XD) or juvenile xanthogranuloma. Multiple osteolytic lesions of large bones were also found. The infiltrate was CD68, MAC 387 and factor XIIIa positive and S-100 and CD1 negative. Some cells contained worm-like bodies visible by electron microscopy. Our patient presented clinical and immunohistochemical findings suggestive of GEH, juvenile xanthogranuloma or XD, supporting the idea of a wide spectrum of non-Langerhans cell histiocytosis. These specific target-like xanthomatous lesions seem to be unique for this new variant of XD.     

Generalized eruptive histiocytosis mimicking leprosy

Generalized eruptive histiocytosis (GEH) is a rare cutaneous histiocytosis that mainly affects adults and presents with multiple symmetric papules on face, trunk, and proximal extremities. GEH is included in type IIa (histiocytes involving cells of dermal dendrocyte lineage) of histiocytic disorders. Clinical and pathological correlations are required for differentiating GEH from other histiocytic disorders and from lepromatous leprosy which clinically mimic GEH and is prevalent in India. We report a case of a middle-aged woman who presented with generalized asymptomatic papules and nodules and was treated for leprosy but was finally diagnosed to have GEH after clinical, histopathological, and immunohistochemical correlation. Furthermore, the newer lesions also showed features of progressive nodular histiocytosis.     

Spontaneous regression of generalized eruptive histiocytosis: Possible involvement of apoptosis?

Generalized eruptive histiocytosis (GEH) is a rare generalized non-X histiocytosis, first described in 1963 by Winklemann and Muller. Since then more than 20 patients with GEH, mainly adults but also a few children, have been reported. We report a case of GEH in a middle-aged woman with spontaneous regression, in which the ultrastructural findings of apoptosis were observed.     

Successful treatment of generalized eruptive histiocytoma with PUVA

Generalized eruptive histiocytoma (GEH) is a rare benign skin disease mainly affecting adults which belongs to the family of non‐Langerhans‐cell histiocytoses. A 32‐year‐old Caucasian woman developed disseminated, monomorphic papules of the trunk after a common cold with sinusitis. Mucous membranes, palms and soles were spared. She also suffered from arthralgia without fever or night sweats. After one month, the patient noticed progression of the reddish papules involving the trunk, extremities and face. Clinical as well as histological examination and immunohistochemistry led to the diagnosis of GEH. The clinical examination and laboratory testing were normal, except for eosinophilia in the peripheral blood and bone marrow. No neoplastic diseases were found during thorough examinations. Systemic PUVA therapy produced rapid regression of the skin lesions. After 10 treatments the lesions began to regress leaving slight papules and multiple brown hyperpigmentations. The lesions resolved completely after 20 PUVA treatments. No relapses occurred. Systemic PUVA therapy represents a promising option for the treatment of GEH.     

Generalized eruptive histiocytoma: a pediatric case

Generalized eruptive histiocytoma (GEH) is a rare, benign, papular, self-healing histiocytosis characterized by recurrent crops of small, firm, tan to reddish papules that appear in a symmetrical fashion on the face, trunk and arms, affecting mainly adults. Approximately 31 cases have been described, of which 8 were in children. A new concept unifying the confusing potpourri of non-Langerhans cell histiocytosis has recently been described in which GEH has been considered to represent an early undifferentiated stage of various histiocytic disorders. We describe a 9-year-old boy who had all the features of GEH and emphasize the importance of clinical, histologic, immunohistochemical, and ultrastructural examination in the diagnosis of histiocytic disorders. On the basis of the increasing numbers of similar reported cases showing overlap in clinical and histologic features and in accordance with the new unifying concept of non-Langerhans cell histiocytosis, we presume that this group of disorders may represent a continuous spectrum of a single disease rather than a collection of separate disorders.     

Multiple linear eccrine spiradenoma associated with multiple trichoepithelioma

A 37-year-old woman had multiple trichoepithelioma of the face which showed typical clinical and histological features, together with multiple papular lesions of 30 years' duration. The lesions were arranged in a linear fashion on her right breast and right upper arm. Microscopic examination of a biopsy specimen revealed typical eccrine spiradenomas. An ultrastructural study of the tumor on the breast revealed that it was composed of two main types of cells: one was a clear cell with low-density cytoplasm and the other was a dark cell with high-density cytoplasm. Some of the clear cells had secretory granule-like structures and/or tono-fibrils, while others had intracytoplasmic cavitations. Neither myoepithelial cells nor Langerhans cells were observed.     

An unusual presentation of human papillomavirus (HPV) infection in a black epidermodysplasia verruciformis (EV) patient

A 25-year-old black man had a 20-year history of disseminated plaques on his body. Two years before consultation he noticed several tumors on the genitalia. Physical examination revealed generalized, coalescing, hypopigmented plaques with a very defined and keratotic border that resembled actinic porokeratosis. (Fig. 1). Multiple verrucous and moist tumors were observed on the scrotum (Fig. 2). His past medical history was unremarkable, and no family member had the disease. Histology of the body lesions revealed hyperkeratosis with a horny-layer, basket-weave appearance, large and clear blue-gray keratinocytes with finely granular cytoplasm and vacuolized nuclei, and abundant keratohyaline granules located in the upper epidermal layer (Fig. 3). Scrotum biopsy showed hyperkeratosis, irregular acanthosis with papillomatosis, and koilocytic figures. The genital lesions were treated with monthly cryotherapy plus surgical excision with complete disappearance of the lesions. A decrease in ultraviolet exposure and daily sun-block were encouraged for epidermodysplasia verruciformis lesions. After genital wart clearance, follow-ups are being scheduled every 4 months, with no new lesions to date.     

皮损形态类似于光泽苔藓的朗格汉斯细胞组织细胞增生症一例

报道1例表现为光泽苔藓样皮损的朗格汉斯细胞组织细胞增生症。患儿女,19个月。因前胸、后背多发针尖大小丘疹伴多饮、多尿1个月余就诊。皮损临床表现为全身泛发性光泽苔藓样丘疹。垂体平扫+增强MRI提示：垂体后叶短T1信号消失。皮损组织病理显示表皮角化过度,真皮浅层密集组织细胞浸润,细胞核呈肾形,胞浆丰富。免疫组化：CD1a、S-100、CD68均阳性。结合临床、组织病理和免疫组化检查,诊断为朗格汉斯细胞组织细胞增生症。全身化疗后,皮损完全消退,目前仍在随访中。     

Generalized multinucleate cell angiohistiocytoma with possible origin from fibroblasts: A clinicopathological study of 15 cases

Multinucleate cell angiohistiocytoma (MCAH) is a vascular and fibrohistiocytic proliferation with unknown pathogenesis. Clinical lesions tend to be localized to an anatomical area. Exceptionally, the generalized variant is rare. This study reports three cases of generalized MCAH, and analyzes the clinicopathological features of 15 cases reviewed in the published work. Compared with the localized variant, generalized MCAH affected both sexes equally, had an earlier age of onset and a predilection for the trunk and extremities. Histopathologically, the most characteristic feature is the giant, bizarre multinucleate cells with angulated cytoplasm. Systemic diseases or abnormal immune conditions were revealed in six patients with generalized MCAH. For the first time, we found that the present cases showed increased elastic fibers in the affected areas, suggesting that the synthetic function of fibroblasts was active. This study suggested that MCAH originates from fibroblasts and is a distinct entity with potential correlation with abnormal immune states.     

Generalized eruptive histiocytoma of childhood associated with rheumatic fever.

We describe a widespread papular eruption in a 5-year-old girl with rheumatic fever. Histological examination revealed a dense histiocytic infiltration in the dermis. On immunohistochemical studies, the cells were positive for vimentin, CD68, MAC387, alpha1-antichymotrypsin and lysozyme, but negative for CD1a and S-100 protein. Electron microscopic studies showed no Birbeck granules in their cytoplasm. A diagnosis of generalized eruptive histiocytoma of childhood was established. The skin lesions completely disappeared within 8 months.     

Follicular pustulous granuloma annulare

We report a 73-year-old woman presenting with recurrent eruptions of generalized follicular pustules. Histological examination revealed several palisading necrobiotic granulomas with mucin deposits, with a perifollicular distribution. A dense neutrophilic infiltrate in the upper portion of affected follicular structures gave rise to pustulous lesions. Scaly papules and pseudovesiculous lesions over the palms with deeper necrobiotic granulomas involving sweat glands and epidermal perforation coexisted in some of the eruptions with generalized pustules. We propose the term follicular pustulous granuloma annulare for this peculiar form of granuloma annulare, which widens the clinicopathological spectrum of presentation of this disease.     

Multiple squamous cell carcinomas in a patient with mycosis fungoides

A 51-year-old man with type IV skin presented for evaluation of a generalized rash associated with multiple ulcerated, nodular lesions on his legs. The nodular lesions occurred approximately 18 months after the initial onset of generalized rash, which had been diagnosed as plaque/patch stage mycosis fungoides. He continued to develop further nodular lesions on his trunk in the weeks following presentation. The nodular lesions were shown to be squamous-cell carcinoma on histopathology. He had received only topical hydrocortisone prior to the development of the second cutaneous malignancy and had no past exposure to carcinogens. His squamous cell carcinomas were treated with surgical excision and split-skin grafting. He received total skin electron-beam therapy to treat the mycosis fungoides. Second malignancy in mycosis fungoides is a recognized phenomenon and usually occurs after potentially carcinogenic therapy. This case demonstrates the occurrence of second malignancy in the absence of a precipitating factor, suggesting that there are innate, immune-mediated mechanisms in the development of cancer in patients with mycosis fungoides.     

A case of herpes zoster duplex with generalized eruption--study of the pathogenesis of the eruptions

A 77-year-old man who developed herpes zoster duplex with generalized eruptions is presented. Three strains of varicella-zoster virus (VZV) were isolated from 2 skin lesions of herpes zoster and one of the generalized eruptions. For strain differentiation, these 3 strains were investigated by restriction endonuclease analysis of the viral DNA. The 3 strains were identical, suggested that a single strain of VZV had been residing in a latent form in multiple spinal ganglions of this patient. We speculate that this strain of VZV in remote ganglions was reactivated by the operation of gastric cancer as a trigger, and, as a result, the 2 skin lesions of herpes zoster appeared, followed by development of generalized eruptions due to virus propagation from zoster lesions.     

Abrupt generalized pustules in patients with rheumatoid arthritis and interstitial lung disease

We report a case of a 30‐year‐old Chinese woman with rheumatoid arthritis and interstitial lung disease who abruptly developed generalized pustules and a high fever for 10 days. She had been taking oral prednisone, iguratimod and total glucosides of peony regularly for 5 months prior. In addition, she had taken metronidazole for 3 days 20 days prior which she had used before with no adverse reaction. She had no history of similar lesions and psoriasis. A biopsy of a pustule on the back showed spongiform pustule of Kogoj. She was suspected of having generalized pustular psoriasis or acute generalized exanthematous pustulosis. Finally, she was diagnosed with generalized pustular psoriasis (von Zumbusch type) considering the characteristics and clinical course of the rash. In addition to the above three drugs, systemic cyclosporin (5 mg/kg per day) was applied, and the lesions and fever resolved within the proceeding 2 months.     

Virus-like structures in lupus erythematosus discoides.

Biopsy specimens from typical skin lesions of DLE patients were studied by means of electron microscopy. Virus-like structures were discovered in the cytoplasm of fibrocytes, endothelial cells and keratinocytes. After prolonged chloroquine treatment a decrease in the frequency and size of these structures was observed.     

朗格汉斯组织细胞增生症1例并文献复习

报告1例以皮肤损害就诊的朗格汉斯组织细胞增生症.患儿男,2岁半,因头面、躯干、下肢红斑、丘疹、脓疱一年半入院.皮肤组织病理检查:大量单个核细胞弥漫分布于真皮乳头层并向表皮侵入,单个核细胞体积较大,胞质淡染,核折叠成肾形,中央空泡化,可见线状沟,呈咖啡豆样外观.免疫组织化学法:CD1α及 S-100染色阳性.诊断为朗格汉斯组织细胞增生症.同时结合相关文献对朗格汉斯组织细胞增生症的临床表现、诊断和预后等进行分析.     

Xanthelasmoid Mastocytosis

An 8-year-old girl had small, papular vulval lesions for six years; the lesions were yellowish with numerous surface depressions. Symptoms due to the action of mastocyte mediators were observed. A biopsy specimen showed a dense monomorphous infiltrate of the deep dermis by rounded cells with granular cytoplasm and a round or oval central nucleus. The morphology of the lesions and red-purple metachromatic staining led to the diagnosis of xanthelasmoid mastocytosis. Symptoms were controlled with hydroxyzine. Annual follow-up has shown no evidence of systemic involvement to date. Surgery should be contemplated as a future therapeutic option, in view of the location of the lesions.     

Acute generalized exanthematous pustulosis with erythema multiforme-like lesions

Acute generalized exanthematous pustulosis (AGEP) resembles generalized pustular psoriasis, but may manifest targetoid lesions, purpura, and blisters in addition to pustules. We describe a case of AGEP with erythema multiforme (EM)-like features in a 35-year-old woman who presented with acute onset of high fever and a strikingly polymorphic eruption consisting of numerous tiny pustules on erythematous bases, marked facial edema, oral and genital erosions, targetoid vesicular and purpuric lesions, pustules in string-of-pearl configuration and ring-like vesicles. The histology revealed, in addition to subcorneal pustules, vacuolar interface dermatitis with involvement of eccrine glands, and microabscesses in pilosebaceous structures. Systemic corticorsteroid and antibiotics were initiated, resulting in rapid resolution without recurrence. Recognition of EM-like lesions on a background of generalized pustular eruption could facilitate the diagnosis of AGEP and the institution of appropriate treatment.     

Congenital self-healing histiocytosis (Hashimoto–Pritzker)

BACKGROUND: Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare condition, initially seen at birth or in the neonatal period, with generalized papules, vesicles, or nodules. Affected infants are otherwise well and the skin lesions tend to involute spontaneously within weeks to months. METHODS: Twelve patients with CSHLCH were seen from 1989 to 1998. RESULTS: Eight patients were girls and four were boys and all presented with lesions at birth which disappeared 1-3 months later. The lesions consisted of numerous brownish-red papules, papulovesicles, crusts, and nodules distributed on the face, limbs, palms, and soles. Two patients had oral mucosal lesions, and one had ulcerated lesions that evolved leaving hypochromic macules. Light microscopy showed a histiocytic infiltrate in the papillary dermis with epidermotrophism. Two cases were studied by electron microscopy: the Langerhans cells showed Birbeck granules and laminated corpus in their cytoplasm. Immunomarking with S100 protein was performed in all 12 patients and was positive. CD1 was also tested in four cases and was positive. CONCLUSIONS: Because CSHLCH is a rare condition, we emphasize that, although it is usually a benign, self-limited entity, careful evaluation for systemic disease must be performed and long-term follow-up must be carried out to detect evidence of relapse or progression of the disease; this is essential when treating these patients.