梅本 尚可

A man in his thirties was suspected of committing a sexual offense against a young girl. A video on his mobile telephone provided the only evidence. Photographs obtained from the video showed male genitalia in two views, with the penis in both views exhibiting unique pigmentation. We appraised this case with the cooperation of dermatologists, who diagnosed the pigmentation as male genital melanosis, a relatively rare disease, which matched that on the suspected perpetrator's penis. Photographs obtained from the video were thus decisive evidence of sexual offense and identified the perpetrator.

The association of Panton-Valentine leukocidin (PVL) toxin with necrotizing soft tissue infection (NSTI) caused by Staphylococcus aureus remains controversial. Here, we report the complete genome sequence of the PVL-negative S. aureus strain JMUB1273, isolated from a patient with pervasive NSTI.

Bullous pemphigoid (BP) is a common autoimmune blistering disorder with unknown etiology. Recently, increasing numbers of BP cases which developed under the medication with dipeptidyl peptidase-4 inhibitors (DPP4i), widely used antihyperglycemic drugs, have been reported in published works. Here, we report a case of DPP4i (teneligliptin)-associated BP that developed in a 70-year-old Japanese man. Interestingly, the patient had acquired reactive perforating collagenosis (ARPC), which is also known to be associated with the onset of BP. In the present case, clinical, histopathological and immunological findings suggested that DPP4i rather than ARPC was associated with the onset of BP.

BACKGROUND: Rosacea is a common skin disease and predominantly affects on the face of middle-aged women. It exceptionally occurs on the extrafacial areas such as ear, neck, axilla, and upper extremities, and has been reported as disseminated rosacea. MAIN OBSERVATION: A 40-year-old Japanese female presented with one-month history of erythematous skin eruption with burning sensation on the face, neck, and upper limbs. Physical examination showed rosacea-like eruption on the face as well as multiple papules disseminated on the neck, forearms, and hands. These extrafacial lesions demonstrated papulonecrotic appearance. Bilateral conjunctiva showed marked hyperemic which was consistent with ocular rosacea. Corneal opacity was also seen. Histology of the umbilicated papule on the neck revealed necrobiotic granulomas around the hair follicle with transepidermal elimination. Another tiny solid papule on the forearm suggesting early lesion also demonstrated necrobiosis with palisading granuloma but no transepidermal elimination. Systemic administration of minocycline and topical tacrolimus therapy promptly improved the skin lesions. Topical application of fluorometholone in temporary addition with levofloxacin improved ocular involvement 12 weeks after her 1st visit. The clinical course of the skin lesion and ocular symptoms mostly correlated. Then, the skin lesion and ocular symptoms often relapsed. Rosacea uncommonly associates with the extrafacial involvement as disseminated rosacea. The present case is characterized by the disseminated papulonecrotic lesions of the extrafacial areas histologically showing transepidermal elimination of necrobiotic granulomas. CONCLUSIONS: Dermatologists should recognize that papulonecrotic lesions of the neck and upper extremities might be extrafacial rosacea when the patient has rosacea on the face.

A 61-year-old Japanese man developed bullous skin lesions during topical therapy for psoriasis vulgaris. Physical examination demonstrated numerous tense bullae and scaly erythemas on the trunk and extremities. Histopathology of the skin biopsy demonstrated subepidermal bullae and lymphocytic infiltration with eosinophils in the dermis. Direct immunofluorescence revealed linear deposits of immunoglobulin (Ig)G, IgA and C3 along the basement membrane zone. Indirect immunofluorescence of 1 mol/L NaCl-split skin showed IgG reactivity with both epidermal and the dermal sides. IgM reactivity with both the epidermal and dermal sides was also detected. Enzyme-linked immunosorbent assays showed negative results for both BP180 and BP230. Immunoelectrophoresis of serum and bone marrow aspiration revealed underlying primary macroglobulinemia with M-proteinemia of IgM-κ type. Immunoblot analysis revealed IgG, but not IgM, antibodies to recombinant protein of BP180 C-terminal domain. We diagnosed the present case as bullous pemphigoid with IgG anti-BP180 C-terminal domain autoantibodies associated with primary macroglobulinemia and psoriasis vulgaris. Systemic administration of prednisolone 30 mg/day resulted in dramatic improvement of both bullous and psoriatic skin lesions. When the bullous and psoriatic lesions relapsed, DRC chemotherapy (dexamethasone, rituximab and cyclophosphamide) for macroglobulinemia was performed. Then, the psoriatic lesions improved and the bullous lesions disappeared. We suggested that the present case may be paraneoplastic syndrome of bullous pemphigoid associated with primary macroglobulinemia and psoriasis vulgaris.

We present clinically peculiar facial discoid lupus erythematosus (DLE) that mimicked tinea faciei. Although DLE is a chronic autoimmune dermatosis, it has a variety of rare clinical presentations, including periorbital DLE, comedonic DLE and hypertrophic DLE recently. In this case, a scaly, erythematous lesion on the eyelid and the central healed, mildly elevated, annularly distributed facial DLE mimicked tinea faciei, complicating our diagnosis.

目的 壊死性筋膜炎を早期に診断し、進展を防ぐ可能性を模索するために、当科で経験した壊死性軟部組織感染症について臨床的に検討した。対象 2001年から当科で経験した壊死性皮膚軟部組織感染症18例(壊死性筋膜炎13例、壊死性蜂窩織炎5例)で男14例、女4例である。年齢は41〜91歳で、平均61.3歳であった。結果 部位 深頸部感染症および上腕、臀部の各1例を除き、15例が下肢病変であった。基礎疾患 糖尿病11例のほか、肝硬変2例、腎不全と咽頭癌放射線治療中の発症1例、下肢の慢性リンパ浮腫、骨折の既往、直腸癌穿孔も各1例にみられた。誘因 打撲に続発したもの2例、足背のBowen病術後にsurgical site infectionとして発症した例もみられた。検出菌 溶蓮菌11例(A群6例)、黄色ブドウ球菌5例(MRSA3例)、緑膿菌1例、大腸菌1例、クロストリジウム1例、陰性2例であった。混合感染は4例のみであった。手術 壊死性筋膜炎13例のうち、7例は全身麻酔でデブリードマンを施行したが、4例は局所麻酔でデブリードマンを行い、抗生物質の全身投与により感染症を治癒ないしコントロールさせることができた。合併症 DICを起こした例は4例、肝不全3例、腎不全1例であった。腸腰筋膿瘍、縦隔膿瘍を合併した例が各1例にみられた。予後 死亡例は2例(11%)で、急速な進行と全身状態からデブリードマンができなかった。大腿部切断は1例、足切断は1例、再発例は2例で1例は下腿の切断を行い救命し得た。考察 壊死性筋膜炎において、皮膚の水疱、血疱、壊死を呈し、急速に進行して多臓器不全に陥る劇症例の診断は皮膚外科医にとって困難ではない。しかし、急性ないし亜急性に経過する非劇症例では、実際上、蜂窩織炎との鑑別がしばしば困難である。したがって、壊死性軟部組織感染症を疑ったら、試験切開、生検をためらわないことが重要である。切開時の出血の有無、滲出液や脂肪織の状態を観察し、フィンガーテストを行う必要がある。壊死性筋膜炎の可能性があれば、広範囲のデブリードマンを行わなくてはならない。また、非劇症例で範囲の狭い場合は宿主の免疫状態にもよるが、早期に診断をすれば局所麻酔下での生検、試験切開と抗生物質で炎症を鎮静化できる例もあると思われた。(著者抄録)

A 70-year-old-male had suffered from non-pruritic, erythematous eruptions on the trunk for 3 months without any general symptoms. The individual lesions lasted for several days. Laboratory investigation showed marked elevation of serum immunoglobulin A (2235 mg/dL) with monoclonal gammopathy (IgA k-type). Monoclonal gammopathy of undetermined significance was diagnosed. Histopathological examination of the eruption revealed diffuse neutrophilic infiltration with leukocytoclasia in the dermis. There was no vasculitis. Treatment with antihistamines alone was not effective. Diaphenyl sulfone (DDS) at 75 mg/day dramatically improved the skin lesions. A similar case of urticarial erythema associated with IgA myeloma has been previously reported. We suggest that neutrophilic, urticaria-like erythema associated with IgA monoclonal gammopathy may be regarded as a new entity.

A 58-year-old Japanese male visited us with painful lesions on the lower lip, oral mucosa and genital region of an 8-month duration. Histological features of the genital lesion were almost consistent with lichenoid tissue reaction. A few intraepidermal acantholytic keratinocytes were also seen in the suprabasal clefts. Direct immunofluorescence exhibited cell surface immunoglobulin (Ig)G deposition and linear deposition of fibrinogen at the dermoepidermal junction. IgG anti-desmoglein (Dsg)3 antibody, but not anti-Dsg1 antibody, was detected in the patient's serum by enzyme-linked immunosorbent assay. Immunoblotting using normal human epidermal extract detected the 210-kD envoplakin, 190-kD periplakin and 130-kD Dsg3. The diagnosis of paraneoplastic pemphigus (PNP) was made. Subsequent investigation revealed a large space-occupying lesion in the liver. Histological findings from liver biopsy specimen were consistent with hepatocellular carcinoma. The patient has been alive 38 months after the diagnosis of PNP was made, although the liver mass has slowly enlarged. Our case is clinically and histologically similar to erosive mucosal lichen planus. Immunological studies confirmed the diagnosis of PNP. The results of negative Dsg1 and positive Dsg3 were consistent with clinical features showing severe mucosal involvement without cutaneous erosion. In PNP, the association with non-hematological solid tumor is extremely rare.

Familial occurrence of sporotrichosis is rare. We report two patients, a husband and wife, with sporotrichosis. A 67-year-old man and a 63-year-old woman, who were farmers, had erythematous plaques and nodules on their faces and his forearm. They had noticed their cutaneous lesions during nearly the same period without any traumatic history. Fragments of biopsy specimens from both of them were submitted for mycological cultures and yielded pure cultures of Sporothrix schenckii (S. schenckii). Restriction fragment length polymorphisms in the mitochondrial DNA of the S. schenckii isolated from the biopsy specimens of their lesions were investigated. The isolates were identified as type 5, which is comparatively abundant in the Kanto area in Japan. The husband was treated with potassium iodide and itraconazole. His wife was treated with itraconazole alone. We failed to isolate a causative fungus from the soil. Our case is the second case in the literature of sporotrichosis in a husband and wife during the same period.

A 75-year-old Japanese male visited us with bullous eruptions on the extremities. Physical examination revealed large bullae on the hands, lower legs and feet. The oral mucosa was also involved. Histology disclosed subepidermal blister with inflammatory cell infiltrates in the dermis. Direct immunofluorescence showed deposits of IgG and IgA at the cutaneous basement membrane zone. Indirect immunofluorescence on 1 M NaCl-split human skin sections demonstrated that the patient's IgG antibodies reacted with the dermal side of the split, while IgA antibodies reacted with the epidermal side. Immunoblotting showed that the patient's serum reacted with the NC1 domain of type VII collagen (290-kDa epidermolysis bullosa acquisita antigen) as well as the 120-kDa linear IgA bullous dermatosis antigen, LAD-1. Systemic prednisolone resulted in a favorable response. From the clinicopathological findings, the present case is not consistent with either epidermolysis bullosa acquisita or IgA bullous dermatosis. Therefore, we regarded the case as mixed bullous disease of epidermolysis bullosa acquisita and linear IgA bullous dermatosis. Such a case has not been previously reported.

Vasoactive intestinal peptide (VIP) has been suggested to play some roles in atopic dermatitis. Tissue of VIP levels has been reported to increase in chronic lichenified lesions of atopic dermatitis (AD). To analyze whether serum levels of VIP in AD patients are elevated compared with normal controls and correlated with the disease severity, we measured serum levels of VIP using enzyme-linked immunosorbent assay in 53 patients with AD and 21 healthy individuals. The results showed that serum levels of VIP in AD patients (345.8+/-71.5 microg/ml) were significantly higher than those in healthy individuals (307.1+/-42.6 microg/ml). However, a correlation was not found between serum VIP levels and disease severity, other markers including serum LDH levels, total serum IgE levels, and peripheral blood eosinophil counts in patients with AD. This indicates that VIP levels in AD patients were elevated not only in the skin but also in the serum, suggesting that increased serum VIP levels in the patients with AD might be involved in its pathogenesis.

A 35-year-old Japanese woman had recurrent, pruritic, vesicular lesions on the face, neck and upper back as well as erosive lesions of the oral cavity and genitalia. The skin and mucosal lesions healed without scarring upon the systemic administration of corticosteroid and azathioprine. Direct immunofluorescence revealed linear deposits of IgG, IgA and C3 at the cutaneous basement membrane zone. Indirect immunofluorescence on 1 M NaCl-split human skin sections demonstrated that the patient's IgG antibodies reacted with the dermal side of the split, while IgA antibodies weakly reacted with the epidermal side. By immunoblot analyses, the patient's serum reacted with the NC1 domain of type VII collagen as well as both the alpha3- and beta3-subunits of laminin 5. We regarded our case as a nonscarring subepidermal blistering disease with autoantibodies to both type VII collagen and two different subunits of laminin 5. Such a case has not been previously reported.