Atypical epidermolysis bullosa simplex with a missense keratin 14 mutation p.Arg125Cys.

Epidermolysis bullosa (EB) is a group of hereditary autosomal dominant bullous diseases. EB is divided into four major phenotypes: intraepidermal EB (or EB simplex), junctional EB, dermolytic EB and mixed EB (Kindler syndrome). EB simplex is further divided into three subtypes: localized EB simplex, Dowling-Meara EB simplex and other generalized EB simplex. We report a 28-year-old man with EB simplex with a missense keratin 14 mutation p.Arg125Cys associated with clumping of keratin filaments and acantholysis in mainly the spinous cells and basal cells. Immunohistochemistry revealed that the broader expression of keratin 5 and 14 was observed in the epidermis, while the expression of keratin 1/10 was quite normal. Dysregulated expression of keratin 5/14 may hinder some functions or roles of keratin 1/10, namely filament assembly of keratin 1/10 in spinous cell integrity, although the expression of keratin 1/10 was not affected and this has not been demonstrated before.

Allergic contact dermatitis caused by gum rosin and wood rosin in Tako-no-Suidashi ointment.

Tako-no-Suidashi ointment (TSO) is an old Japanese over-the-counter drug, used for the drainage of infectious pustular disease, such as furuncles, carbuncles and infectious atheroma, although whether it works well or not is unknown. The ingredients of the TSO compound commonly include rapeseed oil, gum rosin, wood rosin, Japanese wax, paraffin, petrolatum, copper sulfate, Peru balsam, acetic acid, salicylic acid and trace amounts of Guinea green B. We report a case of contact dermatitis in a 38-year-old Japanese woman caused by TSO. The patient presented to our hospital with pruritic erythema on her left cheek. In order to remove a subcutaneous tumor, she had applied TSO 4 days prior to presentation. Clinical examination showed a well-demarcated exudative erythematous macule with yellowish crusts and scales on her left cheek. Patch testing showed a positive reaction to TSO (++), gum rosin (++) and wood rosin (++) at 72 h. As TSO includes highly allergenic material, caution should be made in applying this topical therapy.

Concomitant occurrence of patch granuloma annulare and classical granuloma annulare.

Granuloma annulare (GA) is characterized clinically as annularly-distributed, erythematous papules on the extremities in children and adolescents. GA is recognized histologically as palisading granulomas with central degenerated collagen and mucin deposits. Here, we present a case of concomitant occurrence of patch GA (PGA), the most rare type of GA, and classical GA in a patient. A 60-year-old man was referred to our hospital for asymptomatic eruptions on the upper arms, forearms, right flank and right lateral chest. Clinical examination revealed annular erythematous plaques composed of numerous small papules on bilateral upper arms and forearms. Moreover, an indurative, exudative erythematous to violaceous plaque was present on the right lateral chest and right flank. Histopathology of the former was compatible with palisade-type GA, and the latter interstitial-type GA. This is the first report of PGA concomitant with "classical" annular papular lesions.

Unilateral eyelid angioedema with congestion of the right bulbar conjunctiva due to loxoprofen sodium.

Angioedema is a variant of urticaria that causes deep dermal and subcutaneous swelling. It frequently is a unilateral reaction and usually lasts for several hours but may persist for several days. We report 2 cases of angioedema that involved the right upper and lower eyelids and was associated with congestion of the right bulbar conjunctiva; the symptoms started approximately 1 to 2 hours after taking loxoprofen sodium. All of the symptoms subsided after oral corticosteroid therapy. In both cases, an oral challenge test with 60 mg of loxoprofen sodium (contained in a tablet) caused swelling of the right upper eyelid within several hours, followed by swelling of the right bulbar conjunctiva. We believe the drug reaction in both patients is angioedema.

Histopathologic findings in Unna's nevus suggest it is a tardive congenital nevus.

According to A. Bernard Ackerman's clinical histopathologic classification of nevi, the essential histopathologic finding of an Unna's nevus is localization of melanocytic nevus cells to a markedly thickened papillary dermis of an exophytic lesion. In this study, we examined 94 completely resected Unna's nevi in which several sections of the same lesion could be examined. We examined that melanocytic nevus cells were not just localized to the exophytic portion but were also commonly distributed below it (81 lesions, 86.2%). Melanocytic nevus cells were found in a periadnexal distribution in most of the lesions in which they extended below the exophytic part and were most frequently noted around hair follicles (60 lesions, 63.8%), then around eccrine ducts (43 lesions, 45.7%), and least frequently around sebaceous glands or ducts (36 lesions, 38.3%). Nests of melanocytic nevus cells were present in follicular epithelium in 7 lesions, sebaceous ducts in 1 lesion, and eccrine ducts in 1 lesion. Moreover, type A nevus cells containing melanin granules were observed in 16 lesions (17.0%) when they were distributed around hair follicles, in 8 lesions (8.5%) when distributed around sebaceous glands or ducts, and in 1 lesion (1.1%) when distributed around eccrine ducts. Although Unna's nevus is clinically an acquired nevus, it has many histopathologic characteristics of a congenital melanocytic nevus and is therefore likely a tardive congenital lesion.

Case of cheilitis granulomatosa associated with apical periodontitis.

The etiology of cheilitis granulomatosa is unknown. In some cases, rapid improvement and/or complete elimination of swelling of the lips after dental treatment has been reported. Here, we describe another case of improvement following dental treatment. A 57-year-old woman had developed asymptomatic swelling of the lower lip 2 months previously. Histological examination revealed non-caseous giant cell granulomas. Neither facial nerve palsy nor fissuring of the tongue was present. Patch testing for metal allergy revealed only mild irritation to zinc ion. Although topical corticosteroid ointment and oral tranilast for 4 months were ineffective, rapid and remarkable improvement of the swelling was noted soon after treatment of two lesions of apical periodontitis. Thorough examination for foci of infection is necessary when treating a patient with cheilitis granulomatosa.

Reticulated acanthoma with sebaceous differentiation.

Reticulated acanthoma with sebaceous differentiation (RASD) is characterized by the reticulated proliferation of spinous cells with aggregates of mature sebocytes in the bases of the strands of the keratinocytes, often linking rete ridges. Here, we report the first case reported as RASD. A 55-year-old woman presented with a 15-year history of a slightly pruritic patch on the upper back, and the histology was typical for RASD. She had been bothered by a yellow discharge from the tumor. Skin tumors with sebaceous differentiation are occasionally associated with Muir-Torre syndrome. However, immunohistochemical staining for mutS homolog 2 (MSH-2) and mutL homolog 1 (MLH-1) showed positive staining within the nuclei of sebaceous cells and cells in the dermis. Therefore, it is most likely that the present case is not associated with Muir-Torre syndrome.

Contact dermatitis around a tracheostoma due to salbutamol sulfate and Aldecin.

Contact dermatitis around a tracheostoma is quite rare. So far, there have been only 2 reports about this in medical literature. We, in this study, report herewith contact dermatitis in a 61-year-old Japanese man around a tracheostoma due to salbutamol sulfate and Aldecin. The patient used inhaled Sultanol and Aldecin for the treatment for allergic asthma. On examination, it was found that there was lichenified, exudative erythema with pigmentation around the tracheostoma. Patch testing with 1% aq. Sultanol and Aldecin revealed a positive reaction. Furthermore, patch testing for salbutamol sulfate 1% pet. also showed positive reaction. Although the contact allergen of our patient has not been fully determined (beclomethasone or other ingredients), this must be the first reported case of double contact dermatitis around a tracheostoma from salbutamol and Aldecin.

Drug hypersensitivity syndrome caused by minocycline.

BACKGROUND: Minocycline is a commonly prescribed drug for the treatment of acne. Its use is generally not associated with systemic side effects. OBJECTIVE: To describe a case of minocycline-induced drug hypersensitivity syndrome in a 20-year-old Japanese woman. METHODS AND RESULTS: Following 2 months of minocycline treatment, the patient developed skin lesions composed of exudative maculopapules, purpuratous macules, and target-like, erythema multiforme-like plaques over most of her body. In addition, she had fever, abnormal liver function tests, eosinophilia, and atypical lymphocytosis. Laboratory tests indicated no elevation of antibody titers against cytomegalovirus, Epstein-Barr virus, and human herpesvirus 6. Her ongoing exposure to minocycline was stopped, and treatment with oral prednisolone was begun. Her signs, symptoms, and laboratory abnormalities then began to resolve. Subsequently, the syndrome was observed to return briefly in response to an oral challenge with minocycline. CONCLUSIONS: Minocycline is able to elicit a drug hypersensitivity syndrome that can resemble infectious mononucleosis. This drug reaction can be treated effectively by cessation of exposure to this drug and steroid therapy.