Yuka Hanami

Pyoderma gangrenosum following surgical procedures

C3. Although biopsies were not carried out from either the palms or soles, the palmoplantar lesions were diagnosed as palmoplantar pustulosis. Topical corticosteroid ointment has been applied to both lesions. Rheumatoid neutrophilic dermatosis occurs in patients with active and severe rheumatoid arthritis, and clinically presents with symmetric erythematous papules, nodules, plaques, urticaria-like erythemas, and rarely vesicles, over the trunk and extremities. Bullous-type rheumatoid neutrophilic dermatosis is rarely reported, which predominantly involves the lower extremities. The bullae are tense, and scattered or grouped. Patients including our case are three women and one man (Table 2). All cases have severe disabling seropositive rheumatoid arthritis for long periods; however, the mechanism of vesicle formation is uncertain. Effective treatments include oral dapson and steroids. The clinical manifestations of palmoplantar pustulosis resemble psoriasis; namely, thin, scaly erythemas, or rarely pustules occasionally appear on the extra-palmoplantar areas such as extremities and trunk. Because bullous lesions were the main skin condition on the lower legs in the presented case, the lesions were diagnosed as rheumatoid neutrophilic dermatosis, but not extra-palmoplantar lesions of palmoplantar pustulosis. Both cutaneous lesions in this case are speculated to be caused by activated neutrophils, but other immunological, such as T-cell mediated, mechanisms may also be involved.

Pustular pyoderma gangrenosum: Report of two cases.

Dear Editor, In our case 1, a 23-year-old woman, who was otherwise healthy, was admitted to our hospital, complaining of multiple ulcers on the lower legs with a sudden onset 1 month prior to presentation. Physical examination revealed deep, coalesced ulcers with small satellite ulcerations and pustules on the bilateral lower legs (Fig. 1a,b). Laboratory examination showed an increased number of leukocytes (10 900/mm with 77% neutrophils), C-reactive protein (CRP; 14.55 mg/dL) and erythrocyte sedimentation rate (44 mm/h), but other data including liver and renal function were normal. The pustule was sterile. A biopsy specimen from the edge of the ulcers revealed prominent red blood cells and diffuse neutrophil infiltration in the upper to mid-dermis. Another biopsy specimen from the small pustule showed neutrophilic abscess in the epidermis and neutrophil infiltration in the upper to mid-dermis (Fig. 1c). Detail investigation did not reveal systemic underlying disorders. She was successfully treated with systemic prednisolone (30 mg/ day). In our case 2, a 52-year-old man suffered from stomachache, diarrhea and bloody stools for some months prior to presentation, and was diagnosed with ulcerative colitis (UC). Almost simultaneously, he developed ulcerative lesions on the lower legs, and also multiple, small pustular lesions on the scalp (Fig. 1d,e). Laboratory examination showed increased levels of CRP (3.12 mg/dL) and erythrocyte sedimentation rate (53 mm/h), and a white blood cell count (8900/mm) with 70% neutrophils. The pustule was sterile. Histological examination from the edge of the ulcer showed subepidermal edema and neutrophil and mononuclear cell infiltration in the midto lower dermis. After admission, systemic prednisolone (30 mg/day) was administrated for intestinal lesions, which also improved skin lesions. Pustular pyoderma gangrenosum (PG) is rare and usually seen in association with other types of PG. In both of our cases, ulcerative PG was also seen on the lower limbs. Usually, pustules do not develop into ulceration, however, evolution from pustule to ulcer may occur. Regarding the sites of pustular PG, the extremities are the most frequently involved followed by the upper trunk, and additionally, pustules can be rarely seen on the scalp and penis as well. In case 2, multiple sterile pustules were on the scalp. Ulcerative PG occasionally associates with several systemic diseases including rheumatoid arthritis, inflammatory bowel disease, hematological malignancy and Takayasu’s arteritis, whereas pustular PG frequently associates with inflammatory bowel disease, and bullous PG mostly associates with hematological malignancies. Pediatric cases of pustular PG were reported associated with UC. Case 2 had UC, whereas case 1 had no accompanying systemic disorders. In both cases, development of pustular lesions was related to the activity of PG, and responded well to oral prednisolone. Although systemic prednisolone is the first choice, there are also cases successfully treated with topical betamethasone or tacrolimus.

Bullous prurigo pigmentosa in a pregnant woman with hyperemesis gravidarum

Dear Editor, Prurigo pigmentosa is a relatively rare inflammatory dermatosis, mainly seen in Japanese patients, and is clinically characterized by pruritic erythematous papules and subsequent reticular pigmentation. As an atypical presentation, prurigo pigmentosa with bullous formation has been reported in several cases. We herein describe a case of prurigo pigmentosa with vesiculobullous formation occurring in a pregnant woman with severe hyperemesis gravidarum and ketonuria. A 30-year-old woman was hospitalized due to severe hyperemesis gravidarum. She was referred to the dermatology department, complaining of itchy eruptions, when she was 10 weeks pregnant. She had lost 7 kg in bodyweight compared with pre-pregnancy, and her body mass index was 18.5. Physical examination revealed erythematous plaques on the abdomen and back. Furthermore, reticular erythema with tense vesicles were scattered on the nuchal region (Fig. 1a,b). A skin biopsy revealed subcorneal bulla containing dyskeratotic cells and prominent neutrophils, and liquefaction degeneration of the basal layer (Fig. 1c). Infiltration by a number of mononuclear cells in the subepidermal layers was also observed. Laboratory examination showed that complete blood count, liver and kidney function, C-reactive protein, antinuclear antibody, anti-desmogleins and anti-BP180 were all within normal ranges, and hemoglobin A1c was 5.0%. However, urinary examination revealed ketone of 4+. Along with the improvement of her general condition by fluid therapy and bedrest, ketonuria disappeared, and the skin lesions were also improved leaving reticular pigmentation after 1 month (Fig. 1d). Although the etiology of prurigo pigmentosa is still unknown, several papers have suggested the role of ketonuria associated with diabetes mellitus, diet and fasting. By contrast, only two cases of prurigo pigmentosa in association with pregnancy have been reported. Both cases developed prurigo pigmentosa 13 weeks into their pregnancies. One of the cases suffered from severe vomiting and lost 7% of bodyweight. Examination of this patient revealed elevated urine ketones, which is very similar to our case. In the present case, inadequate oral intake due to severe hyperemesis gravidarum led to ketosis, which subsequently caused prurigo pigmentosa. To date, no similar cases have been reported. Prurigo pigmentosa is usually treated with minocycline, dapsone and sulfamethoxazole. By contrast, our case improved by treatment with rest and fluid therapy along with disappearance of ketonuria, which suggests that the development of prurigo pigmentosa is attributable to ketosis due to severe hyperemesis gravidarum. There have been several reported cases of bullous prurigo pigmentosa. Extensive inflammation with intercellular and intracellular edema, basal liquefaction and papillary dermal edema may result in vesiculobullous lesions. The pathomechanism of bullous formation is unknown, however, marked interand intracellular edema may induce bullous changes. Bullous lesion of prurigo pigmentosa may be induced by intense rubbing, which however is unlikely in our case because there was no scratch mark and bullous lesions were seen on sites where the fingers are difficult to reach. This is the first case of bullous prurigo pigmentosa in close association with ketosis. Prurigo pigmentosa should be recognized as a cutaneous disorder which occurs during pregnancy.

Cellulitis-like skin necrosis induced by postoperative local interferon-β injection for malignant melanoma on the buttock.

arthritis. IGD can be associated with autoimmune or rheumatologic diseases in 20–30% of patients. Etiology of IGD remains enigmatic, however; because of the frequent association with autoimmune diseases, some postulate that circulating autoantibodies may contribute to the pathogenesis. As circulating autoantibodies are not ubiquitous in patients with IGD, further studies are necessary. Interstitial granulomatous dermatitis is associated with internal malignancies. Cornejo et al. reported an IGD-like eruption in a myelodysplasia patient that ultimately progressed to leukemia, suggesting that IGD-like eruption may represent a paraneoplastic phenomenon in pre-leukemic patients. Moreover, Schreckenberg et al. reported a case of IGD with paraneoplastic rheumatoid arthritis, suggesting that IGD should be considered a paraneoplastic phenomenon in patients with malignancies. In conclusion, we report a first case of IGD with arthritis associated with anaplastic large cell lymphoma. IGD should remain a differential diagnosis for patients with rheumatologic condition that have concomitant skin lesions with histological features of interstitial infiltration with collagen degeneration. The association between IGD and malignancies indicates that further study is needed to determine whether IGD represents a paraneoplastic syndrome.

Eosinophilic fasciitis with severe joint contracture in a patient with bladder cancer and B-cell lymphoma

has been reported. Our patient further developed hematological malignancy during the course. Dichloromethane and 1,2dichloropropane were carcinogenic, ink-removal agents used in the printing industry, that can cause cholangiocarcinoma, and we suspect that they were contained in the organic solvents used by our patient. This might have played a role in the occurrence of hematological malignancy. Eosinophilic fasciitis is sometimes associated with several autoimmune conditions, among which morphea is the most frequent but association with generalized morphea is rare. Furthermore, association with vitiligo and eosinophilic fasciitis is also rare, and only a few cases have been reported including vitiligo-like hypopigmentation. Whether hematological malignancy is associated with eosinophilic fasciitis or environmental factors is uncertain, however, our rare case demonstrates that diverse hematological as well as immunological manifestations can occur in association with eosinophilic fasciitis.

Hidradenitis suppurativa and multiple dermatofibromas in a patient with ulcerative colitis

1 Franchini M, Lippi G. Acquired factor VIII inhibitors. Blood 2008; 112: 250–255. 2 Franchini M, Gandini G, Di Paolantonio T, Mariani G. Acquired hemophilia A: a concise review. Am J Hematol 2005; 80: 55–63. 3 Merlini M, Moccia F, Delucchi M, Grillo G. Severe bleeding in a case of acquired factor VIII inhibitor associated with generalized pustular psoriasis. Ann Ital Med Int 2005; 20: 248–252. 4 Green D. Suppression of an antibody to factor VIII by a combination of factor VIII and cyclophosphamide. Blood 1971; 37: 381–387. 5 Di Minno MN, Iervolino S, Peluso R et al. Assessing joint involvement in haemophilia by clinical rheumatologic scores. A pilot study on similarities with subjects with psoriatic arthritis. Clin Rheumatol 2011; 30: 915–919.

Eruptive xanthoma with isomorphic response of Koebner in a construction worker with severe hyperlipidemia

Dear Editor, A 31-year-old man visited the dermatology clinic of Fukushima Rosai Hospital, complaining of multiple small nodules on the palms. Physical examination showed a number of yellowish asymptomatic small firm nodules on the bilateral palms and flexural aspect of the fingers, with left-sided predominance (Fig. 1a). He had been working as a construction worker for over 4 years. He was right-handed and handled a machine that vibrated minutely using his left hand. Thereafter, he noticed that the palmar nodules increased in number after he started this occupation. Similar nodules were also observed on the upper limbs. Laboratory examination revealed extremely high levels of triglyceride (2556 mg/dL; normal, <149), as well as increased levels of total cholesterol (408 mg/dL; normal, <219), blood sugar (238 mg/dL) and hemoglobin A1c (10.6%). His mother had died of subarachnoid hemorrhage at the age of 47 years, but no other family members had diabetes mellitus, hypertension or hyperlipidemia. A biopsy specimen showed a number of xanthoma cells infiltrating the dermis (Fig. 1b,c), which were immunoreactive for CD68 (Fig. 1d), CD163, CD206 and monocyte chemoattractant protein-1. Oral medication for his hyperlipidemia (pitavastatin) and diabetes (anagliptin, metformin) were started. Detailed genetic examination for familial dysbetalipoproteinemia could not be carried out. Eruptive xanthoma is frequently associated with hypertriglyceridemia (World Health Organization types I, IV and V) and also diabetes. Xanthoma lesions of eruptive type contain more triglyceride and fewer cholesteryl esters at the early phase. In xanthomas, lipids are derived from circulating plasma lipoproteins. In cases of eruptive xanthomas in association with metabolic syndrome, insulin resistance leads to acquired lipoprotein lipase deficiency, which results in impaired clearance of chylomicrons and very low-density lipoproteins. Our patient was obese, and his body mass index exceeded 30. In addition, he had hypertension and metabolic syndrome. Hyperlipidemia and diabetes were for the first time recognized as clues to establish the reason for his skin lesions. Unfortunately, the type of hyperlipidemia was not determined as he was soon transferred to another place of work. As far as we could follow, the patient’s eruptions did not resolve within the next few months. Koebner phenomenon is occasionally observed in histiocytoses, such as eruptive xanthoma and multicentric reticulohistiocytosis. Nodular xanthoma frequently occurs on the elbow and Achilles tendon, which may be due to Koebner phenomenon. To date, only some cases of eruptive xanthoma showing Koebner phenomenon have been reported. In previous cases, xanthoma lesions developed in the scar due to

Sarcoid reaction associated with basal cell carcinoma

visit, and it had slowly grown in size. On the first examination, the mass was 25 mm 9 25 mm in diameter, soft and not tender on palpation. The overlying skin was intact and normally colored (Fig 1a). The patient had no remarkable medical history, except for hypertension. Sonography (AplioXG; Toshiba Medical, Tokyo, Japan) identified a 20-mm, well-circumscribed, oval, heterogeneous, hypoechoic mass in the frontalis muscle. The mass had a peripheral hyperechoic rim and echogenic triangular splitting of muscle fibers at the edge (Fig 1b). Color Doppler sonography showed blood flow only in peripheral muscles and not within the mass. Clinical differential diagnoses included epidermal cyst and lipoma; however, they were excluded by the absence of posterior echo enhancement and difference of echogenicity, respectively. Surgical removal of the tumor by simple excision with some margin of uninvolved surrounding muscle was performed. The tumor was placed in the frontalis muscle as per sonographic finding. The resected tumor was a smooth, soft, homogenous and gelatinous nodule (Fig 1c). Histopathologically, the tumor showed a paucity of round or spindle-shaped cells surrounded by an abundant mucoid basophilic matrix (Fig 1d). The mass infiltrated adjacent striated muscle in the periphery. The mucoid material stained positive with Alcian blue. From the clinical and pathological presentation, we finally diagnosed the case with intramuscular myxoma. There has been no recurrence 1 year after surgery. Enzinger first reported intramuscular myxoma as a new pathological entity in 1965. The most affected site is the thigh, and the occurrence in the head and neck is quite rare. The only described case occurring on the forehead was mentioned in a summarized-case table reported by Kindblom et al. The present case is the second described case occurring on the forehead and includes a detailed clinical, sonographic and pathological description. Although magnetic resonance imaging (MRI) is commonly used in diagnosis, the usefulness of sonography for myxoma diagnosis has been spotlighted recently. Compared with MRI, sonography is inexpensive, easily performed and minimally invasive. Color Doppler can also reveal the absence of blood flow. As the present case illustrates, the sonographic finding of echogenic triangles at the edge of the mass is called the “bright cap sign”, and the peripheral hyperechoic rim the “bright rim sign”. These signs may occur because of muscle atrophy and adjacent fatty infiltration, which are typical sonographic presentations of intramuscular myxoma with high sensitivity. A subcutaneous nodule on the forehead can be mistaken for an epidermal cyst and lipoma, and sonographic assessment could be useful for a preoperative diagnosis.

Six cases of purpuric eruptions induced by epidermal growth factor receptor inhibitors

Erlotinib (Tarceva®) and gefitinib (Iressa®) are novel oral tyrosine kinase inhibitors targeting epidermal growth factor receptor (EGF-R). A number of skin reactions have been induced, however, purpuric eruption is rare. We report six cases of purpuric lesions some of which exhibited clinically mimicking HenochSchönlein purpura that occurred after treatment with EGF-R inhibitors for lung cancers. Two were men and four were women, and the mean age was 67.8 years (range: 60 to 80 year old). Clinical presentations are shown in Fig. 1, and the summary of cases is shown in Table 1. On physical examination, all cases had many small purpuric lesions with a diameter of 1-2 mm on the lower extremities, and trunk was also involved in one case. All of the cases were palpable purpura. Five cases had pustules in the center of purpuric lesions, and xerosis was observed in all cases. Erlotinib was thought to be the causative agent in three cases, two of them were switched from gefitinib to erlotinib, whereas other three cases were due to gefitinib. The duration ranged between 2 weeks and 6 months (mean: 2.9 months) after starting EGF-R inhibitors. Histopathological examination was performed in four cases, which revealed mild infiltrate of lymphocytes around the capillaries and extravasation of red blood cells in the upper dermis (Fig. 2), but there were no finding of leukocytoclastic vasculitis. Direct immunofluorescence was performed in one case, and there was no deposition of immunoglobulins. Skin eruptions were spontaneously improved by only interruption of the causative drugs, almost within 1 week. Platelet counts and coagulation profiles were within normal ranges in all cases. Four cases showed a favorable response to EGF-R inhibitors. By contrast, Letter to the Editor

Case of late-onset focal dermal elastosis

meaning that ACA rather than ARAIII may be responsible for the characteristic clinical symptoms in this coexistence. Although ACA is reported to be associated with pulmonary hypertension (PTH), and ARAIII is often closely identified as a risk factor causing scleroderma renal crisis, all cases showed only cutaneous scleroderma and Raynaud’s phenomenon without PTH and renal dysfunction. Among three cases, only our patient had the history of malignancy. Longterm careful monitoring and larger cohorts are needed.