A case of hypopigmented mycosis fungoides successfully treated with 311 nm narrowband ultraviolet B phototherapy

Hypopigmented mycosis fungoides (HMF) is a rare clinical variant of mycosis fungoides (MF) characterized by hypopigmented lesions involving most commonly trunk and proximal extremities. We report here a case of HMF in 22-year-old Korean woman successfully treated with 311 nm narrow-band ultraviolet B (NB-UVB) phototherapy. She presented with progressive asymptomatic hypopigmented patches on wholebody 1-year ago and diagnosed as HMF based on clinicopathologic findings. She was treated with NB-UVB phototherapy and showed almost complete clearance after 8-month without any side effects.     

Bi-clonal, multifocal primary cutaneous marginal zone B-cell lymphoma: report of a case and review of the literature

Bi-clonality is a rare phenomenon seen in approximately 5% of chronic B-cell lymphoproliferative disorders. Both true bi-clonality and somatic hypermutation resulting in intraclonal evolution have been described. We present the case of a 37-year-old female who developed extranodal marginal zone B-cell lymphoma with immunohistochemical studies showing monotypic immunostaining of plasma cells for immunoglobulin lambda light chain on her right arm in 2008. Three years later, she developed a second focus of extranodal marginal zone B-cell lymphoma on her left arm, but immunohistochemical studies demonstrated monotypic immunostaining of plasma cells for immunoglobulin kappa light chain confirmed after repeat analysis. Evaluation for systemic lymphoma with laboratory and imaging studies was negative. Together, the findings were consistent with bi-clonal, multifocal extranodal primary cutaneous marginal zone B-cell lymphoma. We present this case to highlight a rare phenomenon within primary cutaneous marginal zone lymphomas.     

Sézary syndrome treated with narrowband ultraviolet B: time-course measurement of serum levels of CCL17/CCL27

We describe a patient with Sézary syndrome (SS) who was successfully treated with topical steroid and narrowband UVB. Sézary cells in peripheral blood correlated with severity of skin lesions. In addition, serum levels of CCL17 and CCL27 decreased as disease activity improved. These chemokines may be important for the pathogenesis of SS.     

Primary hyperparathyroidism and cutaneous T-cell lymphoma: fortuitous association?

This is the third report of an association between T-cell cutaneous lymphoma (mycosis fungoides) and primary hyperparathyroidism (adenoma). Some studies support the concept that hyperparathyroidism may have promotional activity for the development of certain malignant tumors. A high risk for successive or concurrent neoplasms has been reported in patients with parathyroid adenomas. Primary hyperparathyroidism in a neoplastic context may be underreported. Patients with tumor-associated hypercalcemia should be evaluated for the possibility of primary hyperparathyroidism.     

Löffler syndrome caused by extensive cutaneous larva migrans: a case report and review of the literature

In rare cases, cutaneous larva migrans may be complicated by L?ffler syndrome. This syndrome is thought to result from a type I hypersensitivity reaction related to the pulmonary larval migration phase of various parasites. It is characterized by migratory pulmonary eosinophilic infiltrates and peripheral eosinophilia, with malaise, fever, and cough. Our patient was successfully treated with ivermectin, a corticosteroid cream, and inhalation medication in an early phase, which prevented complications. We present the details of this case and review the literature.     

Henoch-Schönlein purpura presenting with orchitis: a case report and review of the literature

Henoch-Sch?nlein purpura (HSP) is the most common cause of nonthrombocytopenic purpura in children. The clinical picture is classically a cutaneous purpuric eruption of the legs and buttocks and infrequently the upper torso and extremities. Arthritis, gastrointestinal tract symptoms, and nephritis are other common findings typically associated with the cutaneous findings. We present an unusual case of HSP with scrotal swelling and orchitis.     

Primary localized cutaneous amyloidosis: a sign of immune dysregulation?

The manifestations of primary localized cutaneous amyloidosis (PLCA) are usually limited to the skin. The exact etiopathogenesis of PLCA has not been clearly elucidated yet. An increasing number of reports in the literature that associate PLCA with various autoimmune/immune disorders suggest that underlying immune-mediated factors may be implicated.
We report a case of sarcoidosis and a case of IgA nephropathy in association with extensive macular amyloidosis, adding these two conditions to the list of other autoimmune/immune disorders associated with PLCA.
At least a subset of PLCA patients, especially those with extensive involvement, may have associated autoimmune/immune disorders raising the possibility of a common underlying immune-mediated mechanism.     

Nodular primary cutaneous amyloidosis associated with Sjögrens syndrome: presentation of a case

Nodular primary cutaneous amyloidosis is the least frequent clinical form of the cutaneous amyloidoses. It may be associated with myeloproliferative disorders, as well as with systemic amyloidosis. Its association with other entities, such as Sj?gren's syndrome, has recently been described. We present the case of a female patient with Sj?gren's syndrome who developed nodular primary cutaneous amyloidosis.     

Primary cutaneous aggressive epidermotropic CD8+ T cell lymphoma with a chronic and indolent course. Is this different from peripheral T cell lymphoma?

Cutaneous T cell lymphomas most commonly have a CD4+ memory T cell phenotype and exhibit a relatively indolent course, but may in rare cases present with a CD8+ cytotoxic phenotype with a strikingly more aggressive clinical behavior. Primary cutaneous aggressive epidermotropic CD8+ T cell lymphoma is an extremely rare entity with distinct clinicopatological features. The clinical features and prognosis of the recently-described CD8+ peripheral lymphoma are very different from cytotoxic CD8+ epidermotropic lymphoma, but the histological and phenotypic characteristics are very similar. We report a new case of CD8+ epidermotropic lymphoma with a chronic course and suggest the possibility of an overlap between these two types of lymphoma.     

Schnitzler's syndrome: report of a case with progression to Waldenström's macroglobulinaemia

Schnitzler's syndrome is a rare but distinct entity in which chronic urticaria is a prominent feature. Our patient presented with an urticarial rash associated with intermittent fevers, lethargy, a migratory polyarthralgia and loss of weight. Skin biopsy revealed a neutrophilic urticaria. An IgM kappa paraprotein was detected on serum protein electrophoresis at a level of 8 g/L (0.6-2.5 g/L). Bone marrow biopsy was normal. Marked improvement of the arthralgia, fever and lethargy was obtained with oral prednisolone. The urticarial rash, however, was only partly responsive. For the next 10 years the patient remained stable but corticosteroid dependent. Over a period of 10 years the IgM paraprotein had slowly risen to 47 g/L. Repeat bone aspirate and trephine revealed a diagnosis of a low grade lymphoplasmacytic lymphoma consistent with Waldenstr?m's macroglobulinaemia. To date, despite six cycles of chemotherapy with oral chlorambucil, his disease remains persistent but stable.     

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.     

Blue rubber bleb nevus syndrome associated with adult-onset cutaneous lesions and spontaneous intracranial hemorrhage: A case report and literature review

Blue rubber bleb nevus syndrome (BRBNS) is a rare disorder. Since 1860, only 200 case reports of BRBNS have been reported. BRBNS is characterized by numerous malformations of the vascular system that significantly involve the skin, gastrointestinal tract, and other internal organs. We describe a 72-year-old Taiwanese man with BRBNS. Besides typical adult-onset cutaneous lesions, he had venous malformation in the central nervous system, which caused massive intracerebral hemorrhage. He has been in a stupor ever since. Physicians should recognize cutaneous BRBNS lesions early and be aware of the possible complications that arise from internal organs involved.     

Unusual occurrence of a melanoma with intermixed epithelial component: a true melanocarcinoma?: case report and review of epithelial differentiation in melanoma by light microscopy and immunohistochemistry

We report a case of a 27-year-old woman with a nonpigmented lesion on the right scalp. Histological examination showed a malignant nodular neoplasm with 2 distinct but intimately admixed components: a malignant melanoma with a spindle component and an unusual glandular component. Immunohistochemical studies demonstrated epithelial differentiation on the basis of cytokeratin (CAM5.2 and AE1/AE3) expression in the glandular component and melanocytic differentiation (HMB-45, PNL2, MITF, and S-100) of the spindle cell component. A single melanocytic marker (MITF) was expressed in both components, raising the possibility of dual differentiation in a single tumor, rather than the alternative considerations of a collision tumor or a reactive pseudoepitheliomatous hyperplasia with eccrine duct lumen formation within a melanoma. This unusual tumor with both melanocytic and epithelial components may represent a true melanocarcinoma, which becomes a plausible consideration, in view of melanoma plasticity and recent experimental evidence and speculation about the role of stem cells in melanoma.     

Idiopathic generalized anhidrosis with absence of sweat glands: A case report and literature review

Idiopathic generalized anhidrosis is a rare disease characterized by sweating impairment despite exposure to heat or exercise. It could be congenital or acquired. We reported a 22-year-old male with generalized anhidrosis, except axillae, forehead, palms and soles, since infancy. No other systemic abnormalities were found. Histopathology of serial sections revealed lack of sweat glands. It is the first case report of idiopathic generalized anhidrosis with absence of sweat glands in Taiwan.     

A case report of erythroderma in a patient with borderline leprosy on reversal reaction: a result of the exacerbated reaction?

Background

Erythroderma is characterized by erythema and scaling affecting more than 90% of the body surface area. Inflammatory, neoplastic and, more rarely, infectious diseases may culminate with erythroderma. Diagnosis of the underlying disorder is therefore crucial to institute the appropriate therapy. Leprosy is a chronic infectious disease that is endemic in Brazil. Here we present an unusual case of leprosy and reversal reaction causing erythroderma, and we discuss the underlying immunological mechanisms which could contribute to the generalized skin inflammation.

Case presentation

We report a case of a patient with reversal reaction (RR) in borderline borderline leprosy presenting with erythroderma and neural disabilities. Histopathology of the skin showed regular acanthosis and spongiosis in the epidermis and, in the dermis, compact epithelioid granulomas as well as grouped and isolated bacilli. This duality probably reflects the transition from an anergic/multibacillary state to a state of more effective immunity and bacillary control, typical of RR. Leprosy was successfully treated with WHO’s multidrug therapy, plus prednisone for controlling the RR; the erythroderma resolved in parallel with this treatment. Immunologic studies showed in situ predominance of IFNγ?+?over IL-4+ lymphocytes and of IL-17+ over Foxp3+ lymphocytes, suggesting an exacerbated Th-1/Th-17 immunoreactivity and poor Th-2 and regulatory T-cell responses. Circulating Tregs were also diminished. We hypothesize that the flare-up of anti-mycobacteria immunoreactivity that underlies RR may have triggered the intense inflammatory skin lesions that culminated with erythroderma.

Conclusions

This case report highlights the importance of thorough clinical examination of erythrodermic patients in search for its etiology and suggests that an intense and probably uncontrolled leprosy RR can culminate in the development of erythroderma.

     

Histiocytic sarcoma with secondary involvement of the skin and expression of CD1a: evidence of indeterminate cell differentiation?

BACKGROUND: Histiocytic sarcoma is an exceedingly rare malignant neoplasm composed of cells with a monocyte/macrophage phenotype. In the current nosology of histiocytic neoplasms, histiocytic sarcoma is separate from indeterminate cell histiocytosis, a generally benign disorder characterized by proliferation of a CD1a+ and S-100+ population of cells lacking Birbeck granules usually limited to the skin. METHODS: We present a case of histiocytic sarcoma in a 64-year-old man presenting as a peritonsillar mass and secondarily involving the skin. RESULTS: The malignant cells in the extracutaneous foci of disease expressed macrophage-associated antigens including S-100 but were CD1a-. The malignant cells in the skin coexpressed CD1a and S-100 but lacked ultrastructural features of Langerhans cells, findings indicative of indeterminate cells. CONCLUSIONS: We discuss the clinical and histopathologic differential diagnosis in association with prior reported cases of histiocytic sarcoma, particularly in cases involving the skin and cases expressing the Langerhans cell-associated antigen CD1a.