Makiko Kagaya

Localized Lymphomatoid Papulosis

A 50-year-old Japanese male visited our clinic in April 1999 with a 2-year history of self-healing, reddish papules on his right palm. On examination, there were grouped erythematous papules, 2–4 mm in size, which formed a relatively well-circumscribed erythematous plaque. A biopsy specimen showed a wedge-shaped, dense dermal infiltrate consisting of variously sized mononuclear lymphoid cells mixed with few large CD30-positive cells and inflammatory cells, suggesting the diagnosis of regional lymphomatoid papulosis (LyP). Analysis of the T cell receptor gene revealed a polyclonal pattern on lesional skin. Only 5 cases of LyP presenting in a regional distribution have been reported previously. Although the etiology of localized LyP remains unknown, considering that 2 of 5 reported patients developed widespread lesions regional LyP may be the initial presentation of typical LyP.

Idiopathic acquired generalized anhidrosis due to occlusion of proximal coiled ducts

Idiopathic acquired generalized anhidrosis is a very rare disease of unknown pathogenesis. We report a 25‐year‐old man with acquired generalized anhidrosis due to occlusion of the coiled ducts. He did not have sweat secretion over the entire surface of the body, including the palms and soles. Sweat‐inducing stimuli provoked tingling pain on the skin. Pilocarpine iontophoresis on the forearm did not induce sweat secretion. Neurological examination did not reveal any abnormality in the central or peripheral nervous system. Skin biopsy showed that the coiled ducts were occluded by an amorphous eosinophilic substance. This amorphous eosinophilic substance was positive with periodic acid–Schiff (PAS) staining and was resistant to digestion by diastase. Electron microscopy demonstrated that the coiled ducts were completely occluded by an amorphous substance. The substance occluding the coiled ducts contained fibrous structures. These findings suggested that the acquired generalized anhidrosis in this patient was caused by occlusion of the coiled ducts by a PAS‐positive substance probably derived from dark cell granules.

Successful Treatment of a Patient with Lymphomatoid Papulosis by Methotrexate

We report a case of lymphomatoid papulosis (LyP) that occurred in a 44‐year‐old Japanese male patient. Reddish papules with a small number of pustules and nodules were observed on the extremities, chest and upper back. Most lesions were also associated with central necrosis, ulceration and crusting, and regressed spontaneously within 4 to 6 weeks. Histopathological examination revealed wedge‐shaped dense cellular infiltrate in the dermis, which was mixed with large atypical lymphoid cells, small lymphocytes, eosinophils and neutrophils. These large atypical cells expressed CD30 on their cell membrane and cytoplasm. Rearrangement of the T‐cell receptor (TcR) β‐chain gene was detected in the skin lesion. Lymphadenopathy with histopathologic change similar to the skin lesions, but without TcR gene rearrangement, was found at the left inguinal area. Systemic administration of methotrexate (7.5–15.0 mg/week) was found to be dramatically effective in resolution of skin lesions and prevention of their recurrence.

UVB Photosensitivity due to Ranitidine

We describe a 62-year-old male with photosensitivity due to ranitidine. An oral challenge test after taking ranitidine with UVB irradiation was positive. Ranitidine-induced UVB photosensitivity was persistent even after cessation of the medication.

A Case of Type I Cryoglobulinemia Associated with a Monoclonal Gammopathy of Undetermined Significance (MGUS)

A 79‐year‐old Japanese male who had been suffering from renal insufficiency of unknown causes visited us with reddish papules and purpura on both forearms of a months duration. A skin biopsy was performed from the right forearm, and the histopathology showed an obstruction of superficial blood vessels with eosinophilic amorphous materials suggesting cryoglobulins. Although further investigations revealed the presence of monoclonal immunoglobulin (Ig) G‐κ type I cryoglobulin, a bone marrow biopsy demonstrated that there was 7.6% proliferation of plasmacytoid cells, and the serum level of Ig G was less than 3 g/dl. The diagnosis was type I cryoglobulinemia associated with monoclonal gammopathy of undetermined significance as the possible cause of chronic renal failure. Therefore, purpuric changes on extremities of the elderly, especially those complaining of renal failure should, be taken seriously and not regarded simply as an age‐related phenomenon.

CD30+ large cell transformation of mycosis fungoides after psoralen plus ultraviolet A photochemotherapy

Psoralen plus ultraviolet A (PUVA) photochemotherapy is widely used for the therapy of mycosis fungoides (MF). Clinical progression of MF is often associated with an increase in the size of tumour cells known as transformation. We report two patients with CD30+ large cell transformation that appeared after low‐dose PUVA therapy for MF. Clinical data, histopathology, immunohistopathology and T‐cell receptor gene rearrangement were studied. Nodules consisted of atypical large cells that expressed CD30. Monoclonal rearrangement of T‐cell receptors was observed in one case. Low‐dose PUVA therapy may be associated with CD30+ large cell transformation in patients with MF.