Eosinophilic pustular folliculitis associated with hematological disorders: A report of two cases and review of Japanese literature

Eosinophilic pustular folliculitis (EPF) occurs in patients with hematological disorders. However, clinical information about hematological disorder‐associated EPF is scarce. We report two cases of EPF associated with mantle cell lymphoma and reviewed the available published work on Japanese cases. We identified a total of 23 Japanese cases, including the two cases reported here, who had hematological disorder‐associated EPF. Fourteen cases were associated with treatment for hematological malignancies (transplantation‐related EPF) and nine cases were associated with hematological malignancies themselves (hematological malignancy‐related EPF). Although the skin eruption was clinically indistinguishable between the two subtypes, transplantation‐related EPF occurred on the face and trunk of young and middle‐aged men and women, whereas hematological malignancy‐related EPF occurred mostly on the face of older men. Peripheral blood eosinophilia was more frequently observed in transplantation‐related EPF. These observations suggest variations among patients with EPF associated with hematological disorders.     

Eosinophilic dermatosis of hematologic malignancy

Cutaneous involvement by an eosinophil-rich process (eosinophilic dermatosis) may be encountered in the setting of various hematologic malignancies, including mantle cell lymphoma, acute monocytic leukemia, acute lymphoblastic leukemia, large cell lymphoma, myelofibrosis and chronic lymphocytic leukemia (CLL). Of the various hematologic malignancies, eosinophilic dermatosis has been most frequently described in association with CLL. Published previously as insect bite-like reaction and eosinophilic dermatosis of myeloproliferative disease, this rare dermatitis presents as a pruritic, papular and occasionally vesicular eruption associated with an eosinophil-rich infiltrate histopathologically. Although clinical and histopathologic features are similar to insect bites, affected patients frequently deny a history of insect bites. We report a case of eosinophilic dermatosis of hematologic malignancy in a patient with known history of CLL.     

Sweet's syndrome with acute leukemia

Sweet's syndrome presenting with malignancy or acute neutrophilic dermatosis is an unusual cutaneous disorder associated most commonly with acute myelogenous leukemia. Although other cancers may be linked to acute neutrophilic dermatosis, a large majority of patients have associated neoplasms of hematopoietic, plasma cell, or lymphoid nature. We report a patient with aplastic anemia who showed acute neutrophilic dermatosis and, ultimately, acute leukemia. The individual lesions responded to carbon dioxide laser surgery.     

Cutaneous histiocytoid Sweet syndrome and its relationship to hematological diseases

Sweet syndrome (SS) was described over 50 years ago as a distinctive form of neutrophilic dermatosis. It may be idiopathic, drug‐induced or paraneoplastic, and in the last of those subtypes, myeloproliferative diseases are prominently represented. A peculiar variant of SS is termed ‘histiocytoid’ SS (HSS), and early accounts of that condition asserted that it showed no linkage to hematological disorders. We herein report two additional cases of HSS – both of which were associated with myeloid dyscrasias – together with a review of the pertinent literature. Along with our observations, the latter process appears to contradict the contention that HSS has no relationship to hematopoietic diseases; between 35 and 55% of reported cases have indeed shown such an association, usually with myelogenous leukemia or myelodysplastic syndromes.     

Eosinophilic pustular folliculitis: A published work‐based comprehensive analysis of therapeutic responsiveness

Eosinophilic pustular folliculitis (EPF) is a non‐infectious inflammatory dermatosis of unknown etiology that principally affects the hair follicles. There are three variants of EPF: (i) classic EPF; (ii) immunosuppression‐associated EPF, which is subdivided into HIV‐associated (IS/HIV) and non‐HIV‐associated (IS/non‐HIV); and (iii) infancy‐associated EPF. Oral indomethacin is efficacious, especially for classic EPF. No comprehensive information on the efficacies of other medical management regimens is currently available. In this study, we surveyed regimens for EPF that were described in articles published between 1965 and 2013. In total, there were 1171 regimens; 874, 137, 45 and 115 of which were applied to classic, IS/HIV, IS/non‐HIV and infancy‐associated EPF, respectively. Classic EPF was preferentially treated with oral indomethacin with efficacy of 84% whereas topical steroids were preferred for IS/HIV, IS/non‐HIV and infancy‐associated EPF with efficacy of 47%, 73% and 82%, respectively. Other regimens such as oral Sairei‐to (a Chinese–Japanese herbal medicine), diaminodiphenyl sulfone, cyclosporin and topical tacrolimus were effective for indomethacin‐resistant cases. Although the preclusion of direct comparison among cases was one limitation, this study provides a dataset that is applicable to the construction of therapeutic algorithms for EPF.     

Linear IgA dermatosis: association with malignancy

We report the association of linear IgA dermatosis with hematologic malignancy (chronic lymphatic leukemia and plasmacytoma) in two patients. Ten documented cases of linear IgA dermatosis and internal malignancy have been reported in the literature. The possible association of malignancy and linear IgA dermatosis is discussed.     

Insect-bite-like Wells' syndrome in association with mantle-zone lymphoma

Wells' syndrome is a multifaceted dermatosis with a wide morphological spectrum, ranging from characteristic cellulitis-like erythema and wheals to an unusual presentation of vesicles and bullae. We describe a patient in whom Wells' syndrome presented as an insect-bite-like eruption and was associated with underlying mantle-cell lymphoma. We recommend meticulous investigation of patients diagnosed with Wells' syndrome manifesting as an insect-bite-like eruption.     

Transient acantholytic dermatosis associated with lymphomatous angioimmunoblastic lymphadenopathy

Transient acantholytic dermatosis is a papulovesicular cutaneous eruption first described in 1970. There have been subsequent reports of similar disorders occurring in patients with malignancy. Angioimmunoblastic lymphadenopathy with dysproteinemia is a disorder characterized by an acute onset of generalized lymphadenopathy associated with fever, malaise, pruritus, night sweats, and hepatosplenomegaly. The patient described had a papular acantholytic dermatosis associated with the development of angioimmunoblastic lymphadenopathy with dysproteinemia-like T-cell lymphoma. The cutaneous manifestations of angioimmunoblastic lymphadenopathy with dysproteinemia are discussed.     

Acute Febrile Neutrophilic Dermatosis (Sweet''s Syndrome): Case Report and Review of the Literature

An 8-month-old male infant had fever, polymorphonuclear leukocytosis, and tender, firm, elevated erythematous plaques on his face, trunk, and extremities. Histologic examination revealed a dense, perivascular, polymorphonuclear, inflammatory cell infiltrate with nuclear dust in the dermis and intrafollicular abscesses. The rash responded promptly and dramatically to oral corticosteroids. In our opinion, these features represent a distinctive hypersensitivity reaction consistent with acute febrile neutrophilic dermatosis (Sweet's syndrome). It seems that Sweet's syndrome has two age peaks: infancy and middle age. In the former, no sex predilection and no underlying malignancy have been recorded. In the latter, females are preponderantly affected and an associated underlying malignancy has been described.     

Acrokeratosis paraneoplastica—a new cutaneous marker of malignancy

The historical background and the main clinical features of a specific paraneoplastic dermatosis, acrokeratosis paraneoplastica, are reviewed. Typical features are erythematous scaly lesions on the extremities, ears and bridge of the nose, associated with a malignancy, most frequently in the laryngo-pharyngeal region.     

Acanthosis nigricans associated with primary hypogonadism: successful treatment with topical calcipotriol

Acanthosis nigricans is a mucocutaneous dermatosis characterized by hyperpigmentation and a velvety papillomatous hyperkeratosis. It is frequently associated with systemic diseases including malignancy and endocrinal disorders. We report the beneficial effect of topical calcipotriol ointment 50 mcg/g twice a day in acanthosis nigricans associated with primary hypogonadism.     

持久性隆起性红斑伴再生障碍性贫血一例并文献复习

持久性隆起性红斑(erythema elevatum diutinum,EED)是一种罕见的皮肤白细胞破碎性血管炎,其病因不明确,认为与感染、血液系统疾病、自身免疫性疾病感染的循环免疫复合物在血管壁沉积有关,因此除单纯的皮肤表现外,既往报道39.4％的EED伴发HIV、病毒性肝炎、链球菌感染、副蛋白血症、骨髓增生异常综...     

Pityriasis rubra pilaris in association with laryngeal carcinoma

Pityriasis rubra pilaris (PRP) is a rare group of hyperkeratotic, papulosquamous diseases that can be acquired or inherited. Cases of PRP associated with malignancy have been rarely reported. We report a case of 46‐year‐old man who presented with rapidly progressing PRP as a possible initial cutaneous symptom of a previously undiagnosed laryngeal carcinoma. Microlaryngoscopy was performed because of the patient’s hoarseness, and this revealed leucoplakia on the left vocal cord. Histopathological examination led to the diagnosis of squamous cell carcinoma in situ. After surgical treatment, the clinical signs of PRP began to resolve, and the patient was free of skin lesions at follow‐up. This case represents a rare coexistence of PRP with malignancy, and indicates that PRP can occur as paraneoplastic dermatosis associated with laryngeal cancer.     

Skin findings in internal malignant diseases

PURPOSE: A skin finding may be an indicator of internal malignant diseases. In this report, we investigated the skin findings of the cases who have had internal malignancy within the last 1 month. PATIENTS AND METHODS: Seven hundred cases who were diagnosed as internal malignancy and who did not have any treatment for the malignancy were enrolled in our study between February 2002 and September 2003. A form was completed for all of the cases, including name, surname, and the carcinoma type. All cases were examined in detail, and the observed skin findings or dermatosis was recorded. RESULTS: The most frequent skin findings among the cases were tinea pedis/onychomycosis, followed by xerosis and pruritus. The skin findings in terms of frequency were determined mostly in hematological malignancies (68.96%). CONCLUSION: We would like to emphasize that the skin is an indicator of the functions of internal organs and their disorders.     

Pyoderma gangrenosum: a clinical manifestation of difficult diagnosis

Pyoderma gangrenosum is an uncommon ulcerative cutaneous dermatosis associated with a variety of systemic diseases including inflammatory bowel disease, arthritis, hematological malignancies, hepatitis and acquired immunodeficiency syndrome (AIDS). The pathogenesis of pyoderma gangrenosum remains unknown. Its diagnosis is usually based on clinical evidence and confirmed through a process of elimination of the other possible causes of cutaneous ulcers. This report describes a case of pyoderma gangrenosum with extensive ulceration that responded well to treatment.     

Paraneoplastic pityriasis rubra pilaris in association with bronchogenic carcinoma

Pityriasis rubra pilaris (PRP) is an uncommon dermatosis occasionally reported in association with an underlying malignancy. We report a 61-year-old woman in whom PRP preceded the diagnosis of bronchogenic carcinoma by approximately 11 months. The clinical picture in this case included atypical cutaneous signs and mucous membrane involvement. Curative treatment of the malignancy with radiotherapy led to resolution of the mucocutaneous changes within months. A presentation of PRP, particularly if atypical, or in an older patient, should prompt consideration of an associated internal malignancy.     

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.     

Eosinophilic pustular folliculitis: A review of the Japanese published works

Eosinophilic pustular folliculitis (EPF), also known as Ofuji's disease, is an inflammatory dermatosis that was first described in Japan in 1970. More than 300 cases have been reported so far, and 113 Japanese cases have been reported in Japan since 1980. To comprehend the characteristics of Japanese EPF cases, we classified these cases into three types: classic, immunosuppression‐associated (IS‐EPF), and infancy‐associated (I‐EPF). Trends in age of onset and in distribution and characterization of eruptions differed between the types. We found 91 cases of classic EPF (mean age, 39.7 years), consisting of 66 males (73%) and 25 females (27%), in most of which eruptions primarily affected the face; 18 cases of IS‐EPF (44.2 years), consisting of 15 males (83%) and three females (17%), in which eruptions affected the face less predominantly; and four cases of I‐EPF (7.0 years), consisting of two males (50%) and two females (50%), primarily affecting the scalp. The number of IS‐EPF cases has increased since the late 1990s, reflecting the increasing number of HIV‐positive patients in Japan. Systemic non‐steroidal anti‐inflammatory drugs were effective in more than 70% of cases. Dimethyl diphenyl sulfone, antibiotics including minocycline, psoralen plus ultraviolet A therapy and ultraviolet B treatments worked in some cases. Topical steroids and tacrolimus were also effective in some cases of EPF, while topical indomethacin was less effective.     

Clinical and histopathological differential diagnosis of eosinophilic pustular folliculitis

Eosinophilic pustular folliculitis (EPF) is an inflammatory disease characterized by repeated pruritic follicular papules and pustules arranged in arcuate plaques, and folliculotropic infiltration of eosinophils. The diagnosis of EPF is occasionally difficult and problematic because EPF may share the clinical appearance and histological findings with other diseases. Moreover, EPF has several clinical subtypes, including the classical type, infantile type and immunosuppression‐associated type. Because the therapies of EPF are relatively specific as compared to eczematous disorders, accurate diagnosis is essential for the management of EPF. Clinical differential diagnoses include tinea, acne, rosacea, eczematous dermatitis, granuloma faciale, autoimmune annular erythema, infestations and pustular dermatosis. Histologically, cutaneous diseases with eosinophilic infiltrates can be differentially diagnosed. Follicular mucinosis, mycosis fungoides and other cutaneous T‐cell lymphomas are the most important differential diagnoses both clinically and histopathologically. It should be kept in mind particularly that the initial lesions of cutaneous T‐cell lymphoma resemble EPF.     

Eosinophilic cellulitis (Wells' syndrome) associated with colon carcinoma

Eosinophilic cellulitis (Wells' syndrome) is an inflammatory dermatosis characterized by marked eosinophilic infiltrates. Drugs and various infections are recognized causes of eosinophilic cellulitis. Eosinophilic cellulitis has been reported in non‐hematological malignancies in two patients with squamous cell carcinoma and one with nasopharyngeal carcinoma. We report the association of eosinophilic cellulitis with adenocarcinoma of the colon. Curative hemicolectomy led to a complete remission, suggesting that underlying malignancies can trigger eosinophilic cellulitis.