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.     

Treatment of genito-anal lesions in inflammatory skin diseases with PUVA cream photochemotherapy: an open pilot study in 12 patients

BACKGROUND: Localized skin lesions of the genito-anal region such as in lichen sclerosus et atrophicus or in lichen planus are a burden for many patients, and therapeutic efforts, including therapies with potentially hazardous side-effects, are often unsatisfactory. Recently, PUVA bath photochemotherapy has been proven highly effective in the treatment of various inflammatory skin diseases, including localized scleroderma. Another form of topical PUVA therapy, 8-methoxypsoralen-containing cream or gel preparations, has been proven to be as effective as PUVA bath therapy for palmoplantar dermatoses. OBJECTIVE: We evaluated the clinical effects of PUVA cream photochemotherapy in patients with genital lesions of inflammatory skin diseases. METHODS: Twelve patients with lichen sclerosus et atrophicus, lichen planus, vulvar eczema and pruritus vulvae were included in the study. PUVA cream therapy was performed up to 4 times a week. RESULTS: PUVA cream photochemotherapy induced a significant clinical improvement of genital lesions in most patients, as revealed by clinical examination. Clinical improvement (reduction in size of lesions of erythema, and/or of pruritus) was achieved in most patients after 10-20 treatments and was reduced in patients that had only received 5-15 treatments. Cumulative doses ranged between 4.5 and 180 J/cm(2); all patients tolerated PUVA cream phototherapy well without any side-effects. CONCLUSION: PUVA cream phototherapy represents a highly effective therapy that should be further investigated as an alternative treatment for patients with genital lesions of inflammatory skin diseases.     

PUVA therapy for chronic cutaneous graft-vs-host disease

Chronic graft-vs-host disease (GVHD) is an immunologic disorder frequently occurring as a late sequelae of allogeneic bone marrow transplantation and characterized in the skin with lichenoid or sclerodermoid lesions. Systemic immunosuppressive agents such as corticosteroids or cyclosporine are usually required to control the disease. Therapy with psoralen and UVA (PUVA) has recently been shown to be effective for skin and oral mucosa in a few cases of GVHD. We present our experience with PUVA in six patients, five with lichenoid and one with sclerodermoid GVHD. None of these patients had significant systemic involvement. All five patients with lichenoid GVHD showed clinical improvement after PUVA therapy. Three of these patients had complete clearance of skin lesions. Clinical clearance of the disease was accompanied by microscopic clearance. The patient with sclerodermoid GVHD did not respond to therapy. No significant complications or exacerbation of systemic disease occurred. We confirm that PUVA is an effective and safe therapy for the cutaneous manifestations of lichenoid chronic GVHD. We postulate that PUVA therapy clears chronic lichenoid GVHD by selective cytotoxicity for the activated lymphoid cells in the inflammatory infiltrate.     

PUVA-induced lichen planus pemphigoides

A 72-year-old woman had suffered from parapsoriasis en plaque (large plaque type) controlled by topically applied psoralen ultraviolet A (PUVA) therapy. The parapsoriasis lesions gradually disappeared, but numerous tiny red papules with pruritus appeared over the forearms and lower legs 120 days after starting PUVA therapy. These papules developed to form violaceous plaques. Histological findings demonstrated the characteristics of lichen planus. Two months later, tense bullae developed on the plaques and on uninvolved skin of the limbs. These were subepidermal, with linear deposits of IgG and C3 along the basement membrane zone (BMZ) in immunofluorescence of peribullous skin, and immunodeposits of type IV collagen along the floor of the bullae. We therefore, diagnosed lichen planus pemphigoides (LPP). Using systemic and topical steroid therapy, the lesions rapidly resolved and there has been no recurrence. This case suggests that the combination of basal cell injuries caused by chronic inflammation and PUVA therapy could expose BMZ components to autoreactive lymphocytes and induce LPP.     

PUVA-induced lichen planus

A 28-year-old male with histologically proven psoriasis vulgaris was administered oral 8-methoxypsoralen UVA therapy (PUVA). The plaques of psoriasis gradually responded to treatment, however numerous pruritic, violaceous papules over the right forearm and left thigh developed 5 months after starting PUVA (45 sittings with a cumulative dose of 156 J/cm(2)). Histopathologic examination of these lesions was compatible with the diagnosis of lichen planus (LP). On stopping PUVA therapy, the lesions subsided in one month. This case emphasizes the hypothesis that cell injury caused by PUVA therapy could expose some sequestered antigens to autoreactive lymphocytes and induce lichen planus.     

Keratoses in patients with psoriasis: a prospective study in fifty-two inpatients

Twenty-eight of 52 consecutive psoriasis inpatients admitted for therapy with the modified Goeckerman regimen had discrete, gray-white, asymptomatic, keratotic lesions approximately 4 mm in diameter at discharge from the hospital. In 18 of these 28 patients no keratoses were noted on admission. In 9 of 10 patients who had keratoses at admission, there was an increase in number at discharge. All patients with keratoses on admission had multiple, closely spaced previous admissions. No age, sex, or skin type predominance was evident in the group with keratoses compared with the remainder of the group (24 patients) who did not have keratoses. The development of keratoses was not related to the type or duration of psoriasis. Statistically significant predisposing factors were the number of UVB and PUVA treatments and the number of inpatient admissions. Fifty-two percent were located on the extremities, 33% on the trunk, and 15% on the face, head, and neck. Histologic examination of these lesions suggested that they may be a variant of seborrheic keratoses. We have not seen these lesions on patients receiving UVB, PUVA, or tar therapy for other diseases. This raises the possibility that these lesions may be unique to psoriasis and most likely are related to long-term therapy.     

Molluscoid skin lesions in histoid leprosy with pseudo‐isomorphic Koebner phenomenon

A 30‐year‐old Indian man presented with multiple asymptomatic skin lesions on the arms, buttocks, trunk, and knees of 4 months’ duration. These lesions had started over the right arm and gradually increased in number and spread to involve the other sites. There was no history of any skin lesions in the past. Family members were normal. Cutaneous examination showed multiple, skin‐colored, shiny papules abruptly arising over the surrounding normal skin of the arms, buttocks, legs, trunk, and pinnae, varying from 2 to 3 mm in size. Some of the papules over the trunk and arms showed central umbilication resembling molluscum contagiosum, and a few showed superficial erosions and/or crusting ( Fig. 1a,b ). A few similar, erythematous papules over the left buttock showed a linear distribution, suggestive of pseudo‐isomorphic Koebner phenomenon ( Fig. 2a,b ). There was hypoesthesia in the distal part of the arms and legs for thermal, touch, and pain sensation, and the bilateral lateral popliteal and ulnar nerves were thick and mildly tender. The clinical features were suggestive of histoid leprosy. Systemic and eye examination were normal. Routine hematology and liver and kidney function tests were normal, and blood venereal disease research laboratory (VDRL) test and enzyme‐linked immunosorbent assay (ELISA) for human immunodeficiency virus (HIV) 1 and 2 were negative. Histopathology from a papular lesion showed a thinned out epidermis, flattened rete ridges, a subepidermal Grenz zone, and granulomas of closely packed, spindle‐shaped histiocytes arranged in whorls, with foamy macrophages. In addition to the above histologic findings, the molluscoid lesion also showed an epidermal erosion with acid‐fast bacilli demonstrated by Ziehl–Neelsen stain ( Fig. 3a,b ). Slit skin smear examination from both the ear lobes and umbilicated lesions was highly positive, with a bacillary index (BI) of 6+ (> 1000 bacilli per oil immersion field) and a morphologic index (MI) of 80% ( Fig. 3c ). A diagnosis of histoid leprosy was made. The patient was treated with daily rifampicin and ofloxacin for 2 months, followed by standard multibacillary (MB) multidrug therapy (MDT) with daily dapsone and clofazimine with monthly rifampicin for 2 years. An excellent response to treatment was obtained with regression of all the skin lesions.

Figure 1 Open in figure viewer PowerPoint (a) Umbilicated and shiny papular skin lesions arising abruptly over normal skin. (b) Umbilicated, crusted, and eroded pigmented lesions over the arm     

Evaluation of PUVA, topical corticosteroids and the combination of both in the treatment of psoriasis

A total of 90 patients with psoriasis were treated either by photochemotherapy (PUVA) or by topical steroids under occlusion (TOC). In a third group of patients a combination of both regimens was applied. All patients received approximately 15 treatments of either type. During therapy, biopsies were taken in all treatment groups and the epidermal thickness was monitored planimetrically. After clearing of the skin the patients were left without treatment and the time of onset of new lesions was recorded. While TOC produced rapid clearing of the skin this was followed by early relapses (in 50% of the patients after 3 weeks). PUVA reduced psoriatic hyperplasia more slowly; however, 50% of the patients remained free of psoriasis for approximately 10 weeks after clearance. Psoriasis treated with steroids under occlusion together with PUVA showed a significantly faster return of the skin to normal as compared to PUVA. As in PUVA-treated patients relapses occurred after approximately 12 weeks in this group. Therefore the combination of both treatments appears to be advantageous for rapid skin clearance and comparatively long remissions.     

Palmoplantar lichen nitidus: a rare cause of palmoplantar hyperkeratosis

BACKGROUND: Lichen nitidus is a rare condition, which may be a cause of palmoplantar hyperkeratosis. We report two cases. CASE REPORTS: A 53 year-old woman presented with a dry and fissured palmoplantar hyperkeratosis. Histological examination of a biopsy showed the typical features of lichen nitidus. Significant improvement was obtained with acitretin. A few months later, multiple lichen nitidus papules appeared on the limbs and the abdomen. A 67 year-old woman was referred to us for a fissured, disabling palmoplantar hyperkeratosis refractory to topical steroids. Histological examination led to the diagnosis of lichen nitidus. Local PUVA therapy resulted in the cleaning of her lesions. Later, typical papules of lichen nitidus appeared on her elbows. DISCUSSION: Nineteen cases of palmoplantar localization of lichen nitidus have been described. The features are usually tiny yellow papules but sometimes a non-specific keratoderma resembling chronic eczema. Palmoplantar involvement of lichen nitidus may be isolated or associated with cutaneous lesions on unusual sites. Oral retinoids and local PUVA are effective treatments.     

Pigmented lesions on the palms and soles during PUVA therapy

Pigmented skin lesions due to PUVA are well recognized. They include freckles, mottled skin, naevus spilus-like lesions and 'PUVA lentigines' (Kietzmann & Christophers, 1984). The PUVA lentigo appears to be common, occurring in up to 53% of patients (Rhodes, Stern & Melski, 1983). These discrete brown macules, 2–5 mm in diameter, may become very widespread, particularly in those patients who have had many PUVA treatments. They tend to spare the face and have not previously been reported on the palms or soles.
We have now seen three female patients who have developed pigmented lesions on their palms (and in one case also on the sole) whilst receiving long-term oral PUVA therapy for psoriasis. The lesions were of two types: ill-defined light grey-brown macules (histologically a freckle, with no increase in number of basal melanocytes), and discrete dark brown macules. The latter showed focal proliferation of dendritic melanocytes at the dermo-epidermal junction, with increased pigmentation throughout the epidermis and also in the superficial dermis. These lesions developed after a minimum of 70 PUVA treatments (over 18 months) in one case, but only appeared after 5 years' PUVA (330 treatments) in another.
Ultrastructural studies (Rhodes, Harrist & Momtaz, 1983) have demonstrated that the melanocytes in PUVA lentigines may show cytological atypia. PUVA lentigines may also persist for up to 2 years (and probably longer) after stopping PUVA. Patients undergoing prolonged PUVA therapy to the palms and soles should be warned about the possibility of developing pigmented lesions which may persist after cessation of therapy.     

Acral lesions of vitiligo: why are they resistant to photochemotherapy?

Background Acral lesions of vitiligo are usually resistant to conventional lines of treatment as well as surgical interventions. Objective To clarify causes underlying resistance of acral lesions to pigmentation in vitiligo by studying some of the factors associated with mechanisms of repigmentation following photochemotherapy. Methods The study included twenty patients with active vitiligo. Skin biopsies were taken from lesional and perilesional skin of areas expected to respond (trunk and proximal limb) and skin of acral areas, before and after PUVA therapy. Sections were stained with H and E, Melan‐A, MHCII, CD1a, SCF and c‐kit protein. Results Before treatment acral areas showed significantly lower hair follicle density, melanocyte density, Langerhans cell (LC) density, epidermal MHCII expression, lesional SCF expression and perilesional c‐kit expression. Following treatment with PUVA in both non‐responsive acral and repigmenting non‐acral lesions identical immunohistochemical changes in the form of significant decrease in LC density, epidermal MHC‐II and SCF expression were observed. Conclusion The surprisingly similar histochemical changes in response to PUVA in acral and non‐acral lesions did not manifest with clinical repigmentation except in non‐acral ones. Factors such as inherent lower melanocyte density, lower melanocyte stem cell reservoirs and/or lower baseline epidermal stem cell factor may be considered as possible play makers in this respect.     

Extracellular signal‐regulated kinase activation of self‐healing Langerhans cell histiocytosis: A case report

A 3‐month‐old boy developed small papules on his trunk. After the papules increased in number, the patient was diagnosed with Langerhans cell histiocytosis based on the pathological findings. He was referred to our department for further examination. Upon initial examination, the papules and nodules were scattered on his back, abdomen and lumbar region. Because he did not present with any organ involvement except the skin, he was diagnosed with single‐system and skin‐limited Langerhans cell histiocytosis. Skin rashes were treated with a topical steroid and started regressing 3 months after onset. All papules disappeared 6 months after onset. In this boy, the Langerhans cell histiocytosis tumor cells expressed phosphorylated extracellular signal‐regulated kinases. In Langerhans cell histiocytosis, BRAF V600E and other genes are known to mutate to act as driver mutations in stem cells of the myeloid dendritic cell lineage. Consequently, extracellular signal‐regulated kinases are continuously activated, which contributes to Langerhans cell histiocytosis carcinogenesis.     

Papuloerythroderma Ofuji: Bade-PUVA-Behandlung

Papuloerythroderma of Ofuji is a rare skin disorder described primarily in Japanese patients. It occurs primarily in elderly men. The initial lesions are diffuse red papules, sparing the face, palms and soles. Later the papules coalesce into an erythroderma, with typical sparing of the skin folds and creases (the deck chair sign). Pruritus is usually intense. Lymphadenopathy, peripheral blood eosinophilia and elevated IgE levels all are common. Both systemic corticosteroids and systemic PUVA therapy have been recommended. We describe a German male who fulfilled the diagnostic criteria for papuloerythroderma of Ofuji and responded well to PUVA bath therapy with both improvement in skin findings and reduction in pruritus.     

Langerhans cell histiocytosis in an adult: good response of cutaneous lesions to acitretin

A 57‐year‐old man presented with a 2‐year history of bilateral erosive lesions on the inguinal region, and erythematous, brown and crusted papules over the trunk. Histological examination of one lesion in conjunction with immunohistochemical study and electron microscopy led to the diagnosis of Langerhans’ cell histiocytosis. After a thorough examination, the only associated findings were retroperitoneal fibrosis and hypergonadotrophic hypogonadism with a granulomatous testicular infiltrate. The patient was treated with oral acitretin for 1 year (with a topical corticosteroid for the inguinal lesions), resulting in clearing of the cutaneous lesions. He underwent placement of bilateral double‐J ureteral catheters and was started on hormone replacement therapy. At follow‐up 1 year after treatment with acitretin ceased, the patient remained free of cutaneous lesions and his overall condition, including the retroperitoneal fibrosis, had improved. This case had an uncommon combination of features, with a good response to acitretin.     

Therapeutic evaluation of UVB‐targeted phototherapy in vitiligo that affects less than 10% of the body surface area*

Background Current treatments for vitiligo include different therapeutic modalities, such as corticosteroids, immunomodulators, pseudocatalase, skin grafts, diverse types of phototherapy [ultraviolet B (UVB), psoralen plus UVA (PUVA), narrow‐band UVB (NB‐UVB)], and, recently, targeted phototherapy. After a literature search, we found only two studies using different targeted broad‐band UVB units for the treatment of vitiligo. Objective To evaluate the repigmentation response induced with broad‐band, UVB‐targeted phototherapy used as monotherapy in patients with vitiligo affecting less than 10% of the skin surface. Methods Twelve patients were recruited for treatment with 30 sessions of UVB‐targeted phototherapy administered twice weekly. The assessment of repigmentation was made from a comparison of baseline photographs with those after 30 sessions by two independent investigators. Morphometric analysis was performed using a computer program. Results Repigmentation with an average of 66.25% was obtained on lesions of the face, and of 31.5% on the neck, trunk, and genitalia. On the extremities, there was no repigmentation. Itching, a burning sensation, erythema, desquamation, and transitory hyperpigmentation were observed in some patients. Minimal blistering and ulceration were observed in one patient. Conclusion Targeted UVB phototherapy seems to be effective for the repigmentation of vitiligo in lesions located on the face, to a lesser degree on the trunk, and with no response in acral lesions; there were minimal adverse effects that did not require discontinuation of treatment.     

Cutaneous and systemic plasmacytosis: a Chinese case

Cutaneous and systemic plasmacytosis (CSP) is an exceedingly rare condition arising primarily in patients of Japanese descent. Herein, we describe a patient of mainland Chinese origin suffering CSP. A 49-year-old Chinese male had asymptomatic brownish-red plaques and papules of the face and trunk for 6 years. Physical examination revealed innumerable symmetric red-brownish macules on face and trunk with fewer red-brownish papules scattered among the macules. Chemical analysis revealed hypergammaglobulinemia. Computerized tomography scan discovered some lymphadenopathy in the axillary, paratracheal and pulmonary regions. Histological examination showed focal perivascular and periadnexal infiltrate of mainly plasma cells in the superficial and deep dermis. Immunohistochemical study showed that a great number of the infiltrating cells were CD20-positive. The infiltrated polyclonal plasma cells expressed both kappa and lambda light chains. Topical therapy with tacrolimus 0.1% ointment for 2 months reduced the thickness and pigmentation of the facial skin lesions. The lesions resumed the original appearance 3 weeks after discontinuing the therapy. To the best of our knowledge, this is the first case of CSP from mainland China.     

Successful treatment of lichen amyloidosis with combined bath PUVA photochemotherapy and oral acitretin

Lichen amyloidosis (LA) is a chronic, pruritic skin disorder characterized by brownish-grey papules on extensor surfaces of legs and rarely on the trunk. Thioflavin T-positive amyloid deposits are found in the papillary dermis of affected skin, which is the only organ involved. A variety of therapeutic regimens for lichen amyloidosis have been described; however, in many cases with only limited effect. We report on two patients with lichen amyloidosis with typical clinical symptoms not responding to local treatment. A combined regimen with bath psoralen ultraviolet A (PUVA) and oral acitretin was initiated, resulting in nearly complete resolution of the papules and impressive relief from the severe pruritus. The beneficial response has persisted for 8 months. The suggested combined therapy with bath PUVA photochemotherapy and oral acitretin represents an efficacious and practical treatment modality for lichen amyloidosis with long-lasting effects.     

【开放获取】度普利尤单抗治疗泛发性苔藓状淀粉样变1例国内首报

【摘要】 国内报道首例度普利尤单抗治疗泛发性苔藓状皮肤淀粉样变。患者男,70岁,躯干、四肢广泛丘疹伴瘙痒23年。皮肤科检查：躯干、右胫前、双上臂外侧见弥漫性粟粒至绿豆大小褐色半球形丘疹,质硬。血常规嗜酸性粒细胞、血清IgE未见异常。下肢皮损组织病理：表皮角化过度,真皮乳头可见均一红染的团块状物质。免疫组化刚果红染色阳性。瘙痒数字量表评分10分。诊断：泛发性苔藓状皮肤淀粉样变。治疗：皮下注射度普利尤单抗,首次600 mg,之后每2周注射300 mg。治疗第2周时,瘙痒即明显缓解,14周时皮损开始明显消退,18周时前胸、腹部皮疹基本消退,腰背部、四肢皮疹明显消退。未见明显不良反应。     

Systemic photochemotherapy decreases the expression of IFN-γ, IL-12p40 and IL-23p19 in psoriatic plaques

Psoriasis vulgaris (PV) is a chronic skin disease with unclear pathogenesis. In the present study we investigated the effect of systemic photochemotherapy (PUVA therapy- psoralen and UVA therapy) on the expression of IFN-γ, IL-12p40 and IL-23p19 in lesional psoriatic skin. Fifteen patients with chronic plaque type psoriasis selected to be treated with PUVA therapy were recruited for this study. Expression of IFN-γ, IL-12p40 and IL-23p19 in psoriatic lesions before and after twenty PUVA treatments was established by using immunohistochemistry (IHC). A significant decrease in expression (p?相似文献   

Clinical and histological spectrum of elastotic changes induced by penicillamine

A 79-year-old-man with cystinuria requiring long-term penicillamine therapy presented with a 6-month history of itchy annular lesions in both axillae. Clinical examination revealed lesions consisting of crusted keratotic papules coalescing in an annular distribution. Associated findings included generalized skin laxity accentuated on the upper trunk and arms, as well as small yellowish papules on the neck. Histological evaluation revealed short, thick, eosinophilic elastic fibres with nodular protrusions. Transepidermal elimination of abnormal elastic fibres was also evident. We discuss the histological and clinical spectrum of penicillamine-induced elastotic changes and compare these changes to those seen in primary elastotic disorders.