Shunichi Miyakawa

Coexistence of psoriasis and an unusual IgG-mediated subepidermal bullous dermatosis: identification of a novel 200-kDa lower lamina lucida target antigen

Summary Bullous pemphigoid (BP) is characterized by autoantibodies against 230‐ and 180‐kDa hemidesmosomal antigens located in the most superficial layers of the basement membrane zone (BMZ). Histologically. there is a predominance of eosinophils in the infiltrate. In a psoriatic patient, we identified an unusual autoimmune subepidermal bullous eruption which clinically resembled BP, but which was characterized by IgG autoantibodies against a novel 200‐kDa lower lamina lucida component, Histologically there was a predominance of neutrophils in the infiltrate.

A Case of Linear IgA Bullous Dermatosis of Childhood: Immunoelectron Microscopic and IgA Subclass Studies

A 2-year-old girl developed small tense bullae on the diaper area, face and extremities. Immunofluorescent studies revealed linear deposits of IgA, IgM and C3 at the basement membrane zone (BMZ). In addition, IgA2 deposits, as well as IgA1, were clearly demonstrated in the lesional skin. However, in circulation, only IgA1 anti-BMZ antibodies were found. Immunoelectron microscopic study showed IgA deposits just underneath the basilar surface of the basal cells, which seemed to be associated with hemidesmosomes. The survey of the literature suggests that the involvement of IgA2 is extremely rare in this disease.

Cefotiam-lnduced Contact Urticaria Syndrome: An Occupational Condition in Japanese Nurses

A case of occupational contact urticaria syndrome caused by cefotiam dihydrochloride (CTM) in a Japanese nurse is reported. The patient had become sensitized to CTM through the process of preparing antibiotics 8 months before she developed symptoms. A review of the literature revealed 13 similar cases, all involving Japanese nurses, reported since CTM became available in Japan.

Decreased Collagen and Hyaluronic Acid Content in Lesional Skin of Acrogeria

Biochemical analysis of involved and uninvolved skin of a 16-year-old female with acrogeria showed that hyaluronic acid and collagen contents were decreased only in involved skin. Explant cultures from both involved and uninvolved skin synthesized mainly hyaluronic acid in similar amounts. Since the glycosaminoglycans and hydroxyproline excreted in the urine were not increased, we speculate that a localized rather than a systemic abnormality may be present in acrogeria. Decreased collagen and hyaluronic acid contents in the patient are discussed in relation to Werners syndrome and type IV Ehlers-Danlos syndrome.

Hypertrophic Eccrine Glands in Eccrine Angiomatous Hamartoma Produce Gross Cystic Disease Fluid Protein 15

A 39-year-old Japanese woman presented at our clinic with a small nodule on her right small finger which she had first noticed in early childhood. Since that time the nodule had gradually enlarged with paroxysmal spontaneous pain. There were no signs or symptoms referable to the gastro-intestinal or any other system and no family history of a similar lesion. Physical examination revealed an elastic firm nodule measuring 14 ! 21 mm on the back of the right small finger. The lesion was partly bluish and excessive sweating around the area was observed (fig. 1a). No hypertrichosis was seen. All routine investigations were normal. Eccrine angiomatous hamartoma was suspected and the lesion was excised. The tumour was not well demarcated and bled freely during surgery. Histopathological examination of the excisional biopsy disclosed increased numbers of prominently hypertrophic eccrine sweat duct units and increased numbers of small thin-walled venules and capillaries in the dermis to the subcutis (fig. 1b). The hypertrophic eccrine glands (fig. 1c) were much larger than the normal control (fig. 1d). Staining for S-100 protein (fig. 1e) and CEA was positive in the dark cells of the gland. Staining for GCDFP-15 (gross cystic disease fluid protein 15) showed a diffuse and strongly positive reaction in the secretory cells, which mimics apocrine glands (fig. 1f). Myxomatous stroma was stained positively with colloidal iron. The irregular vessels were partly positive for elastica/van Gieson staining and showed apparent internal elastic laminae. However, most vessels were thinwalled venules forming a cavernous haemangioma. Eccrine angiomatous hamartoma is a rare condition first described by Lotzbeck [1] in 1859. It has also been referred as sudoriparous angioma by Domonkos and Suarez [2]. The term eccrine angiomatous hamartoma was coined by Hyman et al. [3]. We described a case with prominent hypertrophy of eccrine glands, which showed strongly positive staining for GCDFP-15. Focal s-100 protein positively and GCDFP-15 positivity have also been demonstrated in

Prurigo pigmentosa Affecting the Forehead

A little-known dermatosis, prurigo pigmentosa, is characterized by itchy reddish lesions and gross reticular pigmentation which occurs mainly on the trunk. Histologically, it is characterized by lichenoid tissue reaction. We present a 23-year-old male patient who showed typical eruption not only on the trunk but also on the forehead. Although the etiology remains to be explained, this unusual distribution of the eruption might help to know the real cause of this peculiar disorder.

Classic (non-AIDS-related) Kaposi's sarcoma in a Japanese patient, successfully treated with alpha-2b-interferon.

had multiple lesions, their family histories were negative, case 1 had a late onset, and neither patient had any apparent uterine involvement. As hair follicles are present on the areolae, it is likely that the leiomyomas on the breasts and areoiae in our patients are of pilar origin. Eight cytogenetic subgroups of uterine leiomyomas have been described,^ but their significance in cutaneous leiomyomas is still unknown.

In vitro cell-free synthesis of bullous pemphigoid polypeptide

SummaryWe investigated the genetic expression of bullous pemphigoid (BP) antigen (230 kD) synthesized by Pam cells using an in vitro cell-free translation assay followed by immunoprecipitation. Prior to the study, we showed that (1) the 230 kD polypeptide does not undergo further processing after it is produced by cells in the short pulse experiment; (2) the 230 kD polypeptide is not degraded at least within the 18 h of the chase experiment; and (3) with tunicamycin treatment the underglycosylated BP antigen still interacts specifically with BP serum. The polypeptide of molecular size slightly greater than 230 kD was identified in the translated proteins directed by ribonucleic acid (RNA) isolated from Pam cells. The size of the mRNA was estimated to be approximately 34S–40S (7.7–10.9 kilobase) using the fractionation method with sucrose density gradient ultracentrifugation of RNA. These results indicate that bullous pemphigoid antigen (230 kD) is a primarily translated product, genetically expressed by Pam cells.