Dermatomyositis-like eruption after long-term hydroxyurea therapy for polycythemia vera

Hydroxyurea (HU) is an antitumor agent used to treat chronic myeloproliferative disorders. HU is usually well tolerated, but several cutaneous changes have been reported during long-term HU therapy. Although the occurrence of dermatomyositis-like changes during long-term HU therapy are well known, they are rarely described in patents with polycythemia vera. We report a 69 year-old-male with polycythemia vera who developed a dermatomyositis-like eruption on his face and dorsum of the hands following long-term HU therapy. Despite the cutaneous features, there were no clinical signs of muscular involvement, and muscle specific enzymes were within normal ranges. After interruption of HU administration, the skin lesions disappeared within two months. The improvement following withdrawal of HU implicated this drug as a possible etiological factor in the development of cutaneous features of dermatomyositis in our patient.     

Peripheral T cell lymphoma presenting as dermatomyositis-like eruption

Background: There are several conditions reported to mimic the cutaneous manifestations of dermatomyositis, including lymphoproliferative disorders. Objective: This case report presents an unusual case of peripheral T cell lymphoma mimicking dermatomyositis and discusses the clinical and pathologic features that distinguish it from dermatomyositis. Methods and Results: A 62-year-old woman presented with a two-month history of a progressive painful cutaneous eruption and interstitial infiltrates on chest x-ray. Skin biopsy revealed peripheral T cell lymphoma. The diagnosis was confirmed by lung biopsy. Conclusions: Although rare, a lymphoproliferative disorder must be included in the differential diagnosis of a cutaneous dermatomyositis-like eruption.     

Multicentric reticulohistiocytosis with elevated cytokine serum levels

Multicentric reticulohistiocytosis (MRH) is an uncommon non-Langerhans cell histiocytosis of unknown etiology. It is a multisystem disorder characterised by a papulonodular skin eruption, mainly in the extensor surfaces, and destructive polyarthritis. Histologically, either cutaneous lesions or the synovium show a dense dermal infiltrate of histiocytes and multinucleated giant cells with an eosinophilic granular material in the cytoplasm. In the immunohistochemical analysis these cells stain positively with monocyte/macrophage markers (CD68 and CD45), as well as with certain cytokines (tumor necrosis factor-α, interleukin 1β and interleukin 6). Moreover, recent reports suggest an osteoclastic nature of the infiltrating cells, as they stain strongly with osteoclast tissue lytic markers including tartrate-resistant acid phosphatase and cathepsin K. We report a case of MRH presenting with clinical features of dermatomyositis. Furthermore, the patient showed elevated cytokine serum levels that lowered after therapy.     

Dermatomiositis paraneoplásica: estudio de 12 casos

Introduction and objectivesAdult dermatomyositis presents as a paraneoplastic syndrome in up to 25% of cases, but no clinical, histologic, or laboratory markers completely specific for paraneoplastic disease in dermatomyositis have been identified to date. Furthermore, studies on adult dermatomyositis do not usually report the frequency of cutaneous features of dermatomyositis in patients with associated cancer. Our aim was to review the characteristics of paraneoplastic dermatomyositis in patients seen at our hospital.Material and methodsWe studied 12 cases of paraneoplastic dermatomyositis and recorded patient age and sex, associated cancer, time between onset of dermatomyositis and cancer, emergent cutaneous manifestations, muscle involvement, dysphagia, lung disease, and levels of creatine phosphokinase and circulating autoantibodies.ResultsThe mean age of the patients was 61 years and the 2 most common malignancies were ovarian cancer and bladder cancer. The mean time between the diagnosis of cancer and dermatomyositis was 7 months and in most cases, the cancer was diagnosed first. Seven patients had amyopathic dermatomyositis. The most common cutaneous signs were a violaceous photodistributed rash sparing the interscapular area and a heliotrope rash, followed by Gottron papules and cuticle involvement. Superficial cutaneous necrosis was observed in 3 cases. Myositis-specific autoantibodies were not detected in any of the 6 patients who underwent this test.ConclusionsParaneoplastic dermatomyositis is often amyopathic. There are no specific cutaneous markers for malignancy in dermatomyositis. Myositis-specific antibodies are not associated with paraneoplastic dermatomyositis.     

Hydroxyurea-induced dermatomyositis-like eruption

Hydroxyurea is a cytotoxic chemotherapeutic agent used for myelodysplasia. The adverse cutaneous effects due to hydroxyurea include leg ulcers, hyperpigmentation of the skin and nails, a lichen planus-like eruption, lupus erythematosus, and a dermatomyositis-like eruption. We present a case of hydroxyurea-induced dermatomyositis-like eruption and review the features of this entity as previously reported.     

Paraneoplastic multicentric reticulohistiocytosis: a clinicopathologic challenge

Multicentric Reticulohistiocytosis (MRH) is a rare, systemic non-Langerhans cell histiocytosis (non-LCH) with prominent joint and skin manifestations. It is mostly self limiting. However, 15-30% of the cases are associated with malignancy and carry a poor prognosis. We report the case of a 42-year-old man who presented with multiple reddish-brown papules that on biopsy showed aggregates of oncocytic histiocytes with several multinucleate giant cells. Immunostains were positive for CD 68, CD 45 and were negative for S-100, CD1a. An impression of multicentric reticulohistiocytosis (MRH) was made, with the recommendation to screen for malignancy. Electron microscopy of the skin lesions showed features consistent with non-Langerhans cell histiocytosis. The patient was later diagnosed with acute myeloid leukemia at a follow-up visit several months later. Thus, it appears prudent to screen and follow-up adults with MRH, to identify an underlying malignant condition.     

Cutaneous leukocytoclastic vasculitis in dermatomyositis suggests malignancy

BACKGROUND: Dermatomyositis (DM) is a rare connective tissue disease which has been shown to be associated with an underlying malignancy. OBJECTIVE: Evaluation of the prevalence of malignancy in DM at our clinic and search for characteristics of the paraneoplastic form of disease. METHODS: Retrospective review of patient files and histology reports over the period from 1991 to 1998. RESULTS: 23 patients (14 women and 9 men) with DM could be identified in this time period with a median age at diagnosis of 48 years. Malignancies were found in 5 (22%) cases. The skin biopsies of all patients showed features of DM; in 7 cases, a leukocytoclastic vasculitis was detected. Four of the 5 cases with an associated malignancy demonstrated histologically a vasculitis in lesional skin, compared to only 3 out of 18 cases without malignancy (p < 0.05, Fisher's exact test). CONCLUSION: Our data suggest that vasculitis in lesional skin biopsies has a predictive value for the presence of underlying malignancy.     

A case of multicentric reticulohistiocytosis responsive to azathioprine in a patient with no underlying malignancy

Multicentric reticulohistiocytosis (MRH), a rare histiocytic systemic condition characterized by mutilating arthritis and multiple cutaneous nodules, has been associated with malignancy including that of the breast, thyroid and colon. An unsubstantiated link with infectious agents such as mycobacterium tuberculosis has been described. Many treatments have been used with varying success. We describe the case of a 60-year-old man with MRH and no underlying malignancy who initially responded well to azathioprine and whose disease recurred upon cessation. A second course of azathioprine administered in conjunction with antituberculous treatment resulted in disease control. Broader associations and features of this rare disease are discussed.     

Dermatomyositis associated with angiotropic lymphoma

We describe a 64-year-old man with dermatomyositis sine myositis who presented with unusual cutaneous plaques and nodules. Initial investigations failed to reveal evidence of malignancy. Eighteen months later he became systemically unwell and repeat biopsies from the nodules confirmed angiotropic T-cell lymphoma. Cutaneous lymphoma is rarely reported with dermatomyositis and in the previously reported cases, mycosis fungoides was the variant seen. This is the first report of angiotropic T-cell lymphoma associated with dermatomyositis.     

Proliferating multicentric reticulohistiocytosis associated with papillary serous carcinoma of the endometrium

Multicentric reticulohistiocytosis (MRH) is a rare histocytic disease characterized by destructive arthritis in association with classic skin findings. Although MRH is not strictly a paraneoplastic disease, one quarter of cases are malignancy related. We report a case of MRH with an initial remission followed by an acute exacerbation several years later heralding the clinical presentation of endometrial carcinoma. During this flareup a skin biopsy specimen revealed a diffuse dermal infiltrate composed of histiocytes with ground-glass cytoplasm and multiple atypical mitoses. Approximately 40% of the cells stained with the proliferation marker Ki-67. Treatment of endometrial carcinoma resulted in improvement of skin and joint symptoms, and a repeat biopsy specimen no longer demonstrated mitotic figures. These findings support a reactive and proliferative cause of MRH.     

Multiple Sclerotic Fibromas in an HIV‐Positive Patient Without Evidence of Cowden''s Disease

Dermatomyositis is an inflammatory condition of the skin and muscles, and an underlying malignancy is noted in 10% or more of cases. Clinical features of dermatomyositis include increasing general fatigue and proximal (thighs and shoulders) muscle weakness accompanied by erythematous lesions of the skin. There have been several distinct types of dermatomyositis described. Here we describe a case of vesiculo‐bullous dermatomyositis, which is a rare variant of dermatomyositis. A 49‐year‐old woman was admitted to our hospital with a painful erythematous vesicular eruption of the face, trunk and extremities. In addition, edema of the face and fever were observed. Clinically, dermatomyositis was considered because of typical skin rashes (Gottron's papules, periorbital heliotrope rash and poikiloderma) and serum creatine phosphokinase level of 1,031 IU/L. A skin biopsy was performed. Microscopically, subepidermal vesiculation with marked edema was present. Lymphoplasmacytic infiltration was also observed in the upper dermis. So far only a few case reports of vesiculo‐bullous dermatomyositis have been reported in the literature. It should be kept in mind that dermatomyositis may present subepidermal vesiculation in order to avoid a misdiagnosis and unnecessary delayed treatment. Furthermore, an internal malignancy should be considered in such a variant of dermatomyositis.     

Regression of primary melanoma with metastases associated with amyopathic dermatomyositis

BACKGROUND: Dermatomyositis is a rare and serious inflammatory connective tissue disease characterized by a typical cutaneous rash and myopathy. Amyopathic dermatomyositis is a particular form of dermatomyositis involving only cutaneous signs and without myopathy present for over 2 years. PATIENTS AND METHODS: A 48 year-old woman presented with a 3-year history of cutaneous rash without myopathy characteristic of amyopathic dermatomyositis. Clinical examination revealed extensive axillary adenopathy, histological examination of which suggested secondary melanoma. The patient reported a black nevus in the axillary area that had disappeared 1 year earlier. Curettage of the lymph node was negative and the patient was treated with interferon (3M 3 times a week). Regression of the cutaneous signs was noted. DISCUSSION: The data, there have been no other reports of paraneoplastic amyopathic dermatomyositis associated with regression of primary melanoma. The literature contains few reports of dermatomyositis associated with melanoma. Amyopathic dermatomyositis may be associated with malignancy.     

Adult and juvenile dermatomyositis treatment

Introduction

Dermatomyositis is a rare autoimmune inflammatory condition affecting skin and muscles. The disease can be seen in both adults and children. It can be associated with malignancy. Considering involvement of skin in the disease, many patients consult dermatologists for its treatment. Hence, knowledge about its presentation, complications, prognosis, and treatment is necessary.

Objective

The objective of this review article is to provide comprehensive information about treatment of dermatomyositis.

Methods

In this review article, we reviewed the published literature on adult and juvenile dermatomyositis to highlight the treatment. Articles published in peer-reviewed journals including reviews, clinical trials, case series, and case reports published in electronic database (MEDLINE/PubMed) through January 2021, cross references of respective articles and trials from clinicaltrials.gov were included for qualitative analysis of the literature.

Results

Treatment options for dermatomyositis include traditional immunosuppressive agents and immunomodulatory therapy. High-dose corticosteroids represent the first line of treatment while other immunosuppressive agents are also used, either in combination with or as an alternative to corticosteroids, after initial treatment failure. Some biological agents have been used for the treatment of dermatomyositis with variable responses.

Conclusion

Although several treatment options are available, several questions remain unanswered about the optimal treatment of dermatomyositis.     

Multicentric reticulohistiocytosis-mimicking dermatomyositis. Case report and review of the literature

Multicentric reticulohistiocytosis is a rare disorder of unknown etiology, characterized by skin and mucosal papulonodular eruptions and destructive polyarthritis. Histopathological study of these lesions shows a nodular infiltrate composed of histiocytes and multinucleated giant cells, with an eosinophilic, granular, 'ground-glass' cytoplasm. We report a case of multicentric reticulohistiocytosis with skin lesions mimicking dermatomyositis and we also review previously reported cases describing such a clinical situation. Our case further emphasizes that multicentric reticulohistiocytosis can mimic clinical features of dermatomyositis. A macular or patch-like erythema in a photodistributed fashion, in addition to other clinical manifestations, can be mistaken for dermatomyositis. However, skin biopsies from these areas may early differentiate both conditions with different treatment options and morbidity.     

Multicentric Reticulohistiocytosis: A Case Report of an Atypical Presentation in a 2‐Year‐Old

Multicentric reticulohistiocytosis (MRH) is a rare systemic inflammatory granulomatous disease marked by severe and often rapidly progressive polyarticular arthritis and cutaneous papulonodules. Initial clinical diagnosis may be difficult. We describe a 2‐year‐old girl presenting with pink dermal papules on the forehead, thighs, elbows, knees, and palms of the hands. Based on clinical findings and skin biopsy results, she was initially diagnosed with granuloma annulare. At 5 years of age, she developed arthritis, fatigue, and more widespread skin papules leading to the diagnosis of MRH. To our knowledge, this is the youngest individual with MRH yet described. We outline the timeline and unique features of her case and review the literature pertaining to MRH in children. Although rare, MRH can be permanently debilitating, making prompt diagnosis critical. A standardized approach to investigation and management needs to be developed.     

Basaloid squamous cell carcinoma of the nasal septum presenting as a primary cutaneous lesion

Background:Basaloid squamous cell carcinoma is a rare aggressive variant of squamous cell carcinoma, with a predilection for the head and neck region. There are only two case reports in the literature documenting a nasal cavity squamous cell carcinoma presenting as a primary cutaneous lesion.Objective:We report a rare case of nasal cavity basaloid squamous cell carcinoma presenting initially as a nasal bridge mass. Two initial biopsies revealed features consistent with basal cell carcinoma and basosquamous cell carcinoma, respectively.Result:Final surgical pathology showed extensive invasive squamous cell carcinoma with basaloid differentiation arising from the nasal septal mucosa with extension to the overlying skin. The clinicopathologic features were interpreted as basaloid squamous cell carcinoma.Conclusion:We discuss the difficulties in pathologic diagnosis of this condition given its varied phenotypical expression. As well, this case emphasizes the necessity for diagnostic vigilance when assessing a primary cutaneous lesion as it may be a rare presentation of an underlying malignancy extending to the skin.     

Lymphoma Arising From a Calcinotic Lesion in a Patient with Juvenile Dermatomyositis

Abstract:  Approximately one-third of adult patients with dermatomyositis develop malignancy, usually within 1 year of diagnosis of dermatomyositis. This phenomenon is not seen in patients with juvenile dermatomyositis. We are reporting a case of B-cell lymphoma arising from a calcinotic lesion in a 14-year-old girl who had been diagnosed with juvenile dermatomyositis 3 years prior. Our review of the literature revealed only one other case of malignancy arising from an area of calcinosis.     

Ominous cutaneous presentation of acute myeloid leukemia without peripheral blood involvement upon initial presentation and relapse: case report and literature review

Background

Aleukemic leukemia cutis (ALC) is an extremely rare cutaneous manifestation of an aggressive systemic hematological malignancy, associated with dermal infiltration by leukemic cells preceding peripheral blood involvement. This condition is associated with poor outcome, and dermatologists are often responsible for making the initial diagnosis in a timely fashion.

Objectives

To describe a case of ALC and review the literature with an emphasis on the clinical features, summarizing the cutaneous manifestations of this rare systemic disorder.

Materials and methods

Electronic searches were performed in PubMed and Embase for published studies and case reports in English from 1970 to 2017.

Results

The presented case is a 46-year-old male with numerous asymptomatic skin-colored papules, plaques, and subcutaneous nodules with no detectable peripheral blood involvement, who was treated and subsequently relapsed twice with no evidence of peripheral blood or bone marrow involvement. Based on the review, skin nodules were the most common physical exam finding, comprising 27 cases (62.8%). The most common anatomic locations of cutaneous findings were the trunk and extremities, occurring in 23 (53.5%) and 20 (46.5%) cases, respectively.

Conclusions

Since the cutaneous presentation of ALC is notably variable, a high degree of clinical suspicion is required. In view of this case and review of the literature, leukemia cutis should be included in the differential diagnosis of evolving, infiltrative cutaneous nodules or plaques, triggering an evaluation of the bone marrow and peripheral blood when the pathologic features raise concern for this disorder.

     

Eczematoid graft-vs-host disease: a novel form of chronic cutaneous graft-vs-host disease and its response to psoralen UV-A therapy

BACKGROUND: Chronic cutaneous graft-vs-host disease (GVHD) is generally classified by whether lesions have a lichenoid or sclerodermatous morphology. Other unusual clinical forms have been reported that exhibit the features of dermatomyositis and lupus erythematosus. Within a large population of individuals who underwent allogeneic stem cell transplantation because of hematologic malignancy, a group of patients was identified in whom severe and persistent eczema developed. OBSERVATIONS: We prospectively evaluated 10 adult patients with unexplained eczematous dermatosis after allogeneic hematopoietic stem cell transplantation. The dermatosis developed between 2 and 18 months (mean, 7.5 months) after receipt of the transplant, exhibited the typical clinical features of dermatitis, and became erythrodermic in each case. The patient group had strong risk factors for chronic cutaneous GVHD: 8 had received a transplant from an unrelated donor, 7 had evidence of extracutaneous GVHD, and 7 had a history of acute cutaneous GVHD. Sampling of lesional skin revealed the histologic features of GVHD coexisting with the changes of dermatitis. The patients were treated with topical corticosteroid and systemic immunosuppressive agents. Six patients also received psoralen-UV-A. Four patients achieved prolonged remission. Six patients died, 5 of infective complications and 1 of relapsed leukemia. CONCLUSIONS: The eczematous dermatosis observed represents a novel form of chronic cutaneous GVHD that we named eczematoid GVHD. Eczematoid GVHD is an aggressive, chronic dermatosis that requires substantial immunosuppression therapy to achieve control. It is associated with a poor prognosis. Although atopy can be transmitted to an individual from a hematopoietic stem cell transplant, none of the donors in this series gave a history of an atopic disorder. Therefore, other factors must be implicated in provoking the expression of an eczematous phenotype in individuals with underlying chronic graft-vs-host activity.     

A Case of Erythrodermic Dermatomyositis Associated with Gastric Cancer

Erythroderma is an unusual cutaneous finding associated with dermatomyositis. There are only five cases of erythrodermic dermatomyositis reported in the English literature. We treated a case of erythrodermic dermatomyositis associated with a Bormann type 1 gastric cancer. The patient had a generalized, erythematous scaly eruption consistent with erythroderma and Gottron''s papules as well as a heliotrope rash; these are the hallmark skin manifestations of dermatomyositis.