Acrokeratosis neoplastica (Bazex syndrome): Report of two cases and literature review.

The skin often reflects the presence of internal diseases. Acrokeratosis neoplastica (Bazex syndrome) is a unique skin manifestation characterized by its erythematous hyperkeratosis with yellowish, adherent scales on the palm, sole, or other acral locations. There is a potentially high association between Bazex syndrome and malignant pathology, especially squamous cell carcinomas (SCC). To date, various skin conditions have been recognized as diagnostic indicators of insidious malignancies. The recognition of paraneoplastic dermatoses has a strong potential for prompt cancer detection and early therapeutic intervention. Here we describe clinical and forensic cases of Bazex syndrome that are associated with SCC of the glottis and lung. Bazex syndrome has been reported to be associated with a variety of cancers in addition to SCC. We review the clinical manifestations of Bazex syndrome and include updated knowledge on disease pathogenesis.

Dupilumab Alters Both the Bacterial and Fungal Skin Microbiomes of Patients with Atopic Dermatitis.

The skin microbiome at lesion sites in patients with atopic dermatitis (AD) is characterized by dysbiosis. Although the administration of dupilumab, an IL-4Rα inhibitor, improves dysbiosis in the bacterial microbiome, information regarding the fungal microbiome remains limited. This study administered dupilumab to 30 patients with moderate-to-severe AD and analyzed changes in both fungal and bacterial skin microbiomes over a 12-week period. Malassezia restricta and M. globosa dominated the fungal microbiome, whereas non-Malassezia yeast species increased in abundance, leading to greater microbial diversity. A qPCR analysis revealed a decrease in Malassezia colonization following administration, with a higher reduction rate observed where the pretreatment degree of colonization was higher. A correlation was found between the group classified by the Eczema Area and Severity Index, the group categorized by the concentration of Thymus and activation-regulated chemokine, and the degree of skin colonization by Malassezia. Furthermore, an analysis of the bacterial microbiome also confirmed a decrease in the degree of skin colonization by the exacerbating factor Staphylococcus aureus and an increase in the microbial diversity of the bacterial microbiome. Our study is the first to show that dupilumab changes the community structure of the bacterial microbiome and affects the fungal microbiome in patients with AD.

Deck-chair sign as a diagnostic clue for insidious T-cell lymphoma: An autopsy case of mycosis fungoides.

Skin reflects the presence of systemic diseases, leading to an unexpected diagnosis of insidious diseases. Deck-chair sign is a unique skin eruption characterized by widespread erythematous papules that become erythrodermic with spare skin folds. An association between the deck-chair sign and malignancies, especially hematological neoplasms, has been suggested. We report a forensic case of mycosis fungoides unexpectedly diagnosed in the presence of a deck-chair sign. Mycosis fungoides is representative of cutaneous T-cell lymphomas. Here, we successfully demonstrated the feasibility of analyzing mycosis fungoides in a forensic autopsy case using basic histopathology and serology. We emphasize that the underlying malignancy should be primarily considered in cadavers with a positive deck-chair sign and review current reports about this characteristic skin manifestation.

Malignancy-related Hypercalcemia Caused by Metameric Cutaneous Metastasis of Parathyroid Hormone-related Protein-producing Bladder Carcinoma with Squamous Cell Differentiation: An Autopsy Case of Cobb Syndrome.

A 74-year-old woman was admitted with hypercalcemia and prolonged disturbance of consciousness. The left buttock to the anterior aspect of the left thigh was swollen and erythematous, with a collection of 1.0-cm large, firm, elastic nodules distributed in a zosteriform pattern in the L1-L4 region. Based on autopsy findings, a very rare case of Cobb syndrome was diagnosed due to a spinal vascular malformation at the Th12-L4 level and L5 vertebral hemangioma. Cobb syndrome-associated cutaneous metastasis extending along the same metamere was complicated by immunohistochemically proven parathyroid hormone-related protein-producing advanced bladder carcinoma in this case.

Symmetrical Skin Lesions on the Gluteal Region in a Patient with Anti-Laminin-332 Mucous Membrane Pemphigoid.

Mucous membrane pemphigoid (MMP), previously called cicatricial pemphigoid, is a rare subepidermal immunobullous disorder that primarily affects the mucous membranes (1,2). MMP is divided into two major subtypes, anti-BP180-type MMP and anti-laminin-332 (previously called laminin 5 or epiligrin) MMP. Anti-laminin-332 MMP is known to be associated with malignant tumors (3), which may cause overexpression of autoantibodies and induce autoimmunity to laminin-332 (4). MMP primarily affects the mucous membranes, and widespread skin lesions are rare. In MMP, circumscribed skin lesions have been previously reported as occurring on the head, neck, and upper trunk (5). We report a case of anti-laminin-332 MMP presenting with symmetrical skin lesions characteristic of MMP on the weight-bearing areas of the gluteal region. A 66-year-old Japanese man presented with a month-long history of multiple erosions and blisters on the mucous membranes and skin, with conjunctival hyperemia, nasal obstruction, oral pain, and hoarseness of voice. Three days before the first visit, he was diagnosed with gastric cancer with liver metastasis by gastrointestinal endoscopy and abdominal ultrasound examination for tarry stool. Physical examination demonstrated erosions and tense bullae on the conjunctivae, tongue, and lips (Figure 1, a,b), as well as erosive erythematous skin lesions on the nape, right index finger, both legs, and symmetric lesions on the gluteal region (Figure 1, c). His body weight was 86 kg. Laboratory examinations showed slight liver dysfunction and elevation of C-reactive protein levels. Histopathologic examination of the skin lesions demonstrated subepidermal blisters with lymphocytic and eosinophilic infiltrates (Figure 1, d,e). Direct immunofluorescence (IF) revealed linear deposits of IgG and C3, but not IgA, along the basement membrane zone (BMZ) (Figure 1, f,g). An IgG subclass study showed IgG1 and IgG4 deposits. Indirect IF on normal human skin revealed weak positivity for IgA anti-keratinocyte cell surface antibodies and IgG anti-BMZ antibodies, which were bound to the dermal side of 1 mol/L NaCl-split skin (Figure 1, h). IgG immunoblot analyses of both normal human epidermal and dermal extracts showed negative results (including BP230, BP180, 290 kDa type VII collagen, and 200 kDa laminin-γ1). Immunoprecipitation using radio-labeled cultured keratinocyte lysate demonstrated positive reactivity with laminin-332 (Figure 1, i). We established the diagnosis of anti-laminin-332 MMP. We started treatment with oral minocycline (200 mg/day) and niacinamide (900 mg/day) with topical corticosteroids without any effect after 2 weeks of therapy. Administration of oral prednisolone (40 mg/day) with topical corticosteroids and alprostadil ointment on the skin lesions, as well as beclometasone dipropionate powder on the oral lesions resulted in significant improvement of mucocutaneous lesions within 10 days. Although the gastric cancer and liver metastasis initially responded to chemotherapy with fluorouracil and cisplatin, the patient succumbed to multiple organ failure 9 months after the initial visit. Anti-laminin-332 antibodies were originally detected by immunoprecipitation, as in our case. Immunoblotting of purified human laminin-332 have been subsequently developed, which detects the 165/145 kDa α3, 140 kDa ß3, and 105 kDa γ2 subunits of laminin-332 in various patterns (6). Today, the ELISA system uses laminin-332 preparations as adjunct diagnostic tools in MMP (7). Occasionally, a wide spectrum of autoantibodies is detected in MMP, for example, MMP with IgG antibodies to both BP180 and laminin-332, which were considered to be developed via epitope spreading. Detection of circulating IgA autoantibodies against the skin have also been reported in MMP (8). However, the pathogenic significance and mechanisms of coexistence of IgG anti-laminin-332 antibodies and IgA anti-keratinocyte cell surface antibodies found in our case are currently unknown. It is generally considered that IgG1 antibodies activate complements and are pathogenic in MMP, while IgG4 antibodies behave as blocking antibodies and are protective. In our case, direct IF revealed IgG1 and IgG4 deposits; the same was reported in a previous case report (9). The pathogenic roles of autoantibodies with different IgG subclasses need to be analyzed in further studies. Conjunctival mucosal lesions in MMP may occur by rubbing of the eyes due to irritation. Blinking subjects the conjunctivae to repeated friction. Vocal cords vibrate during breathing and speaking. The tongue moves while eating and drinking; in particular, the tip of the tongue gets into frequent contact with the inner sides of the incisor teeth. In the present case, characteristic symmetrical skin lesions were seen on the weight-bearing areas of the gluteal region on bony prominences which receive mechanical stresses in the sitting position. These skin lesions were subjected to repeated stretch and pressure stresses, but no ischemic changes were observed, such as decubitus ulcers. Therefore, the symmetrical skin lesions in the gluteal region as well as the ocular and oral mucosal lesions seen in our patient might have resulted from the same mechanism of pathogenesis. We reported a case of anti-laminin-332 MMP presenting with symmetrical gluteal skin lesions, probably induced by mechanical stress. MMP primarily affects the mucous membranes, and widespread skin lesions are rare. Our case emphasizes that clinicians need to specifically check for the presence of skin lesions on weight-bearing parts of the body during examination of patients with suspected MMP.

Video image of genital melanosis provides strong evidence to support identification of a sexual offender.

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.

Case of pemphigoid with immunoglobulin G antibodies to BP180 C-terminal domain and laminin-γ1 (p200) developed after pneumococcal vaccination.

Pemphigoid cases have been reported in association with vaccination, including pneumococcal vaccination in infants but not in adults. There are also sporadic reports of pemphigoid diseases involving reactions to multiple autoantigens. We herein report a 75-year-old Japanese patient with pemphigoid who had immunoglobulin G antibodies to both the BP180 C-terminal domain and laminin-γ1 (p200), which developed 1 day after pneumococcal vaccination.

Management of Infected Calcific Myonecrosis: A Report of 2 Cases.

Calcific myonecrosis (CM) is a rare condition in which a large calcified mass develops after trauma. Generally, CM occurs in a lower extremity, and there have been no reports of its occurrence in the upper arm. We report 2 cases of infected CM, including a rare case of CM occurrence in the arm and a typical case in the leg. Case 1: An 84-year-old woman presented with a draining sinus and a large calcified mass in the arm and axillary region. The mass involved the neurovascular bundle; thus, complete resection was impossible. We performed surgical debridement and postoperative negative-pressure wound therapy with instillation and dwell. Case 2: A 43-year-old man presented with a large calcified mass in the right leg and 2 draining sinuses. After surgical debridement, negative-pressure wound therapy was initiated. However, the wound became infected, and we performed additional debridement, followed by a split thickness skin grafting. The infection was controlled in both patients, although complete resection was not feasible. Complete resection is generally considered the optimum treatment for infected CM, but it is difficult to achieve in some patients. Negative-pressure wound therapy with instillation and dwell appears as a good option for postoperative management if complete resection of infected CM cannot be achieved.

Complete Genome Sequence of a Panton-Valentine Leukocidin-Negative Staphylococcus aureus Strain Isolated from a Patient with Pervasive Necrotizing Soft Tissue Infection.

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.