Muneo Tanita

Cholinergic Urticaria, a New Pathogenic Concept: Hypohidrosis due to Interference with the Delivery of Sweat to the Skin Surface

Background: Acetylcholine has been suspected to be a pathogenetic factor for cholinergic urticaria (CU), without definite evidence. In contrast, there are scattered reports of CU associated with acquired generalized hypohidrosis. We have recently examined 2 patients with CU in both of whom we noticed the presence of extensive hypohidrosis that occurred only in winter. Objective: In these 2 patients, acquired hypohidrosis due to superficial obstruction of the acrosyringium was suspected as the cause. Both case 1, a 22-year-old Japanese man, and case 2, a 21-year-old Japanese man, began to have anhidrosis and numerous red macules on their body whenever they felt hot in winter. These symptoms ceased to appear in summer. Methods: We studied histologically their lesional skin in addition to provocation tests for CU. Results: The diagnosis of CU was confirmed by the provocation of typical wheals after physical exercise in both cases. Histological study revealed findings suggestive of the presence of occlusion of the superficial acrosyringium. Conclusion: We think that such a hypohidrosis due to occlusion of superficial sweat ducts may also play a role in many other patients with CU of unknown etiology that becomes exacerbated in winter when sweating is not a frequent event.

A novel mutation of the tyrosinase gene causing oculocutaneous albinism type 1 (OCA1)

Tyrosinase is a rate-limiting enzyme in the melanin biosynthetic pathway and a complete defect of the enzyme activity caused by homozygous mutations of the tyrosinase gene is well known to result in tyrosinase-negative oculocutaneous albinism (OCA1A) patients who never develop any melanin pigment in the skin, hair and eyes throughout life. In this paper, we report a novel missense substitution, R239W(CGG --> TGG) of the tyrosinase gene in a patient with tyrosinase-negative OCA.

Resolution of a leg ulcer after hysterectomy for huge uterine myoma.

Venous ulcers are the most common type of leg ulcers, accounting for 80% to 90% of cases. We report a large, therapy‐resistant ulcer present for three months on the right leg of a 44‐year‐old woman who also had a huge uterine myoma. Without any other treatment, the leg ulcer regressed spontaneously three months after a hysterectomy for the uterine myoma that had been demonstrated in a CT image to be compressing the right common iliac vein in the pelvis. Uterine myoma can become the cause of venous insufficiency of the leg, when it is big enough to disturb the blood circulation in the pelvis in individuals who have incompetent perforating veins.

Release of monocyte chemoattractants by polymorphonuclear leukocytes stimulated by their adhesion to stratum corneum opsonized via complement activation, measured with a human acute monocytic leukemic cell line, THP-1

Abstract: Stratum corneum (SC) exposed to living tissues, induces inflammation characterized by the formation of mixed cell granulomas consisting of infiltrative polymorphonuclear leukocytes (PMNs) and monocytes/macrophages. In this study, to clarify the mechanism for the later monocyte accumulation in SC‐induced granulomas, we evaluated monocyte chemotactic activity induced by PMNs treated with serum‐opsonized SC by using a human acute monocytic leukemic cell line, THP‐1. When the supernatant was obtained from a PMN suspension cultured with opsonized plantar SC, higher THP‐1 chemotactic activity was detected as compared with that cultured with non‐opsonized SC. Although some concentrations of the chemokines, MIP‐1α and MIP‐1β, were detected in supernatants obtained from the PMN suspensions cultured with plantar SC than in the control suspensions of PMN alone, their production by PMN was not influenced by the opsonization procedure. In contrast, MCP‐1 was found to be secreted from PMN suspensions constitutively, showing no correlation to this THP‐1 chemotactic activity. Moreover, HPLC analysis of PMN suspensions indicated that factors with far higher molecular weight values than these chemokines are involved in the chemotaxis of THP‐1 cells.

A case of pustular vasculopathy. An atypical variant of Sweet's syndrome?

in ten cases, followed by LLEP in seven cases including our case. Other manifestations were linear tumidus LE, linear subacute cutaneous LE and linear bullous LE, each in one case. Several characteristics of patients with LLEP have been described: childhood or adolescent onset, distribution along the lines of Blaschko, a low rate of progression to systemic disease, and a good response to steroids, without leaving depressed lipoatrophic areas. Our case displayed these features. However, since Marzano reported two cases of LLEP with sclerosis of collagen fibers in the dermis, which progressed to systemic LE after several years, as ‘‘sclerodermic linear lupus panniculitis,’’ we intend to keep our case under careful observation. In addition, a notable finding in our case is exacerbation of skin lesions induced by cold, which suggests that poor local blood circulation induced by cold may be an important exogenous aggravating factor in LLEP. The mechanism of distribution of such lesions along Blaschko’s lines is still unknown. Although Blaschko’s lines are considered to be the expression of epidermal rather than dermal mosaicism, in LLEP there may be cellular mosaicism of components of deep skin such as fibroblasts or lipocytes, and this may result in a loss of immune tolerance to these cells followed by local LE inflammation.