Squamous cell carcinoma arising in chronic perianal pyoderma a case report and review of Japanese literature

We report a rare case of squamous cell carcinoma developing from fistules of chronic perianal pyoderma in a 49-year-old Japanese man. He first noticed an abscess and nodule on his buttocks and perianal area 21 year previously (at the age of 28); the fistules formed later. These fistules were surgically removed, and an artificial anus was constructed 14 years ago (at the age of 35) in our hospital, when a histopathological examination revealed no malignant changes. However, he was recently admitted to our hospital with arterial bleeding from the ulcer of the buttock. On admission, the histological diagnosis of the ulcer was well differentiated squamous cell carcinoma. Wide local excision of the ulcer and scar tissue, including the sacrum, was performed. The defect was covered with a left latissimus dorsi flap and skin graft. He received radiation therapy after the operation. However, he died of cachexia and pneumonia. This case indicated that the CPP would better have been treated with wide excision before the development of SCC. Therefore, we recommend careful follow-up of patients affected by CPP and repeated biopsies of the lesion, particularly when the condition is severe, longstanding, and extensive. We discussed the term "CPP" and reviewed 22 cases of SCC arising in CPP reported in the Japanese literature.     

Perianal hidradenitis suppurativa: early surgical treatment gives good results in chronic or recurrent cases

Hidradenitis suppurativa (HS) represents a chronic, recurrent, deep-seated folliculitis resulting in abscesses followed by the formation of sinus tracts and scarring. It is an uncommon disorder frequently involving the perianal region. We review the clinical and pathological features of 12 patients (10 men and two women) with perianal HS (PHS). The mean age at presentation was 43 years (range 24–65), and the mean duration of illness was 14 years (range 2–30). Two patients exhibited multiple areas of involvement, including the axilla and the nape of the neck, in addition to the perianal region. Histological examinations showed epidermis-lined sinuses surrounded by dense plasma cell infiltration in four of nine specimens. The complications related with PHS included anal fistula (seven patients), diabetes mellitus (four), malignant tumour (two) and sepsis (one). Three patients died of the complications. Eight patients underwent wide local excision. The defects were managed with a meshed split-thickness skin graft in five patients, and open treatment in the remainder. New lesions developed in untreated skin of two patients. However, no patient relapsed in the surgically treated sites. PHS is frequently complicated with anal fistula and is occasionally life-threatening as a result of the occurrence of malignant tumours or sepsis. Early surgical excision followed by meshed split-thickness skin graft can successfully cure the lesion without functional disturbance.     

Hidradenitis suppurativa

Hidradenitis suppurativa is a chronic disease characterized by recurrent, painful, deep-seated, rounded nodules and abscesses of apocrine gland-bearing skin. Subsequent suppuration, sinus tracts and hypertrophic scarring are its main features. Onset is usually after puberty, although it is most common during the third decade and may persist in old age. The disease tends to be chronic and may develop to subcutaneous extension leading to indurations, sinus, and fistula having a profound impact on the quality of life. The prevalence is 1% in several studies. Axillary and inguinal involvement is more common in females; peri-anal and buttocks localizations are prevalent in males. The exact aetiology remains unknown. The primary event is a follicular occlusion with secondary inflammation, infection and destruction of the pilo-sebaceo-apocrine apparatus and extension to the adjacent sub-cutaneous tissue. Infection is common. Smoking may be a triggering factor. Obesity aggravates the discomfort. Differential diagnostic includes Crohn's disease, nodular acne and furonculosis. The main complications are arthropathy, carcinoma. Treatment depends upon the stage of the disease. Early nodular lesions may be treated by antibiotics for acute stage; long-term antibiotics, zinc salts may be useful as maintenance treatment; anti-TNF drugs have been used in severe cases; systemic steroids, estrogens, anti-androgens, retinoids have been used as options with limited success. Surgical treatment includes incision with or without drainage for limited abscesses; limited excisions are used for locally recurring draining sinuses. Total wide excision and healing with secondary intention or flaps and grafts is the only curative procedure in case of advanced disease.

Conflicts of interest

None declared.     

Necrobiosis-lipoidica-like skin manifestation in lymphomatoid granulomatosis (Liebow)

A 58-year-old man developed 3 indurative erythematous lesions like necrobiosis lipoidica on the right lower leg. He had had similar cutaneous lesions 1.5 years previously. These had been surgically excised, and a histologic diagnosis of necrobiosis lipoidica was made at another hospital. He was diagnosed as having lymphomatoid granulomatosis by lung biopsy in our hospital. Nasal involvement was confirmed in later examinations and the skin lesions were also considered to be the cutaneous manifestation of lymphomatoid granulomatosis. This outlines that cutaneous manifestations may allow early diagnosis of lymphomatoid granulomatosis.     

Iatrogenic Mycobacterium abscessus infection: histopathology of 71 patients

The histopathology is described of 190 skin biopsies from 71 patients who presented during an epidemic of nodules and abscesses in the periumbilical region and buttocks, secondary to the application of xylocaine injections. Positive cultures for Mycobacterium abscessus were obtained from the specimens and from the xylocaine solution. The severe inflammatory lesions involved the dermis and the hypodermis and had three main histopathological patterns: (i) granulomatous nodular or diffuse inflammation with mixed granulomas in 57 (80%) of the cases; (ii) prevailing abscesses with mild granulomatous reaction in 28 (15%) of the biopsies; and (iii) deep dermal and subcutaneous granulomatous inflammation with no neutrophil component in three (4%) of cases. Bacilli were detected in 51 (27%) of the specimens, frequently forming small clumps at the centre of clear spaces or vacuoles and which were lipid-like structures in 156 (82%) of the abscesses or granulomas. This series represents one of the largest reported outbreaks caused by atypical mycobacteria and in which the source of infection was confirmed. The results emphasize the essential role of skin biopsies in determining the histopathological substrate, in helping to detect the atypical mycobacterial origin and in encouraging the practice of cultures for the identification of micro-organisms.     

Juvenile hyaline fibromatosis: a case report

Juvenile hyaline fibromatosis ( JHF ) is a rare autosomal recessive disease characterized by papulonodular skin lesions, gingival hyperplasia, joint contractures, and bone lesions. The skin lesions may consist of multiple large tumors, commonly on the scalp and around the neck, and small pearly, pink papules and plaques on the trunk, chin, ears, and around the nostrils. Here, we report a 2-year-old boy with characteristic stiffness of the knees and elbows and pink confluent papules on the paranasal folds, and periauricular and perianal regions. He also had hard nodules all over the scalp and around the mouth, and severe gingival hyperplasia. The lesions were totally excised and clinicopathological diagnosis was JHF.     

A Pediatric Case of Atypical Mycobacterium avium Infection of the Skin

We report a case of cutaneous atypical mycobacteriosis in a 12-year-old healthy girl due to Mycobacterium avium. The cutaneous symptoms were three well-defined subcutaneous nodules on both buttocks and on the posterior surface of the left thigh. One had a fistulous opening on the skin surface. Histopathological examination revealed epithelioid cell granulomas surrounded by dense lymphocytic infiltration and acid-fast bacteria were seen with modified periodic acid-carbol fuchsin staining. Using Ogawa's medium at 37°C, acid-fast bacteria were isolated from the biopsied specimen and identified by the DNA-DNA hybridization method as Mycobacterium avium. In drug susceptibility test, these were resistant to all antituberculous drugs. Oral administration of minocycline 100 mg/day for two months had little effect on the two remaining lesions, which were therefore excised. Based upon reported cases of Mycobacterium avium complex, we considered that our pediatric patient with multiple intradermal or subcutaneous nodules on the buttocks and the thigh exhibited the characteristic symptoms of M. avium infection.     

Annular Lupus Vulgaris Mimicking Tinea Cruris

Cutaneous tuberculosis is an infrequent form of extrapulmonary tuberculosis. It is often clinically and histopathologically confused with various cutaneous disorders. A 36-year-old man attended our clinic with slowly progressive, asymptomatic, annular skin lesions on both the thighs and buttocks for 10 years. He consulted with many physicians and was improperly treated with an oral antifungal agent for several months under the diagnosis of tinea cruris, but no resolution of his condition was observed. A diagnosis of lupus vulgaris was made based on the histopathologic examination and the polymerase chain reaction assay. Anti-tuberculosis therapy was administered and the lesions started to regress.     

Subcutaneous nocardial abscesses in a patient with bullous pemphigoid during immunosuppressive therapy: report of a case and review of the Japanese literature

We describe a rare case of multiple, subcutaneous nocardial abscesses. A 67-year-old Japanese male with bullous pemphigoid was admitted to our hospital, and systemic prednisolone and double filtration plasmapheresis were administered. During the combination treatment, he noted a solitary subcutaneous nodule on the right upper arm. The lesions gradually increased in number and disseminated. The microbial diagnosis was thought to be a Nocardia species. Administration of combination chemotherapy with minocycline and diamino-diphenyl sulfone failed to eliminate the nodules completely, so he was treated with daily injection of amikacin sulfate into the nodules. The lesions dramatically responded to this therapy and resolved in four weeks.     

表现为发疹性瘙痒性丘疹的汗孔角化病

报告1例表现为发疹性瘙痒性丘疹的汗孔角化病.患者男,50岁.臀部丘疹伴剧烈瘙痒7年,逐渐融合成斑块,丘疹突然增多并累及双下肢1年.皮损组织病理检查符合汗孔角化病.给予角质剥脱剂外用及阿维A口服治疗4周,皮损暂无明显改善.     

Trichophyton rubrum abscesses in immunocompromised patients. A case report

A 72-year-old immunocompromised man with myelodysplastic syndrome who developed multiple erythematous, scaly abscesses like lesions on his left foot and lower leg is described. He also had dry scaly lesions on his soles and lesions on several toe nails. A punch biopsy showed abscesses with fungal elements and Trichophyton rubrum was cultured from skin scales and the biopsy. A diagnosis of T. rubrum abscesses should be suspected in all immunocompromised patients with signs of superficial dermatophyte infection.     

Keratosis lichenoides chronica in an Indian child following erythroderma

A 4-year-old boy presented with mildly itchy, linear, skin lesions over the trunk, arms, and face of 3 months' duration. He had previously been admitted to a private hospital for generalized exfoliation of the skin following drug intake for fever and throat pain. The nature of the drugs was not known. The exfoliative dermatitis was treated with oral prednisolone, 10 mg daily, tapered over 3 weeks. No further topical or oral medication was given. The present skin lesions started 1 month after the cessation of the steroids. There was no family history of skin lesions, voice changes, or systemic complaints. Cutaneous examination showed multiple violaceous, linear, reticulate ridges with adherent scaling over the chest, back, and neck. There were scaly, flat-topped papules over the extensor aspects of both upper arms and the buttocks, and scaly plaques over the cheeks (Figs 1a-d and 2a,b). The scalp showed diffuse greasy scaling. There were no oral, genital, axillary, or eye lesions. The nails were normal. Systemic examination did not reveal any abnormal finding. Routine hematologic investigations, liver and kidney function tests, tests for hepatitis B and C, and enzyme-linked immunosorbent assay (ELISA) for HIV were normal. Histopathology from skin lesions on the back revealed hyperkeratosis, patchy parakeratosis, follicular plugging, alternating irregular acanthosis and epidermal thinning, basal cell degeneration, and a band-like inflammatory infiltrate of lymphocytes, histiocytes, and a few plasma cells (Fig. 3). Based on the classical clinical features and histopathology, keratosis lichenoides chronica was diagnosed, and topical 1% hydrocortisone acetate cream, twice daily, was prescribed. There was slight relief of pruritus at a follow-up visit after 3 weeks; however, the patient was subsequently lost to follow-up.     

Disseminated blastomycosis

A 26-year-old veiled Saudi-Arabian woman presented with hemoptysis, and multiple nodules and abscesses. A skin biopsy specimen revealed yeast forms consistent with Blastomyces dermatitidis. Fungal cultures from bronchoscopy and skin specimens also grew B dermatitidis. She was treated with oral itraconazole (200 mg twice a day). Both lung and skin lesions showed improvement within 6 weeks.     

Penicillamine induced pseudoxanthoma elasticum with elastosis perforans serpiginosa

Long term D-penicillamine therapy, especially when used to treat Wilson's disease has been shown to cause elastosis perforans serpiginosa, pseudoxanthoma elasticum perforans and other degenerative dermatoses. We report a 23-year-old male patient who presented with multiple firm papules, nodules over the neck, axillae, front of elbows for five years. He was a known case of Wilson's disease on long-term treatment with penicillamine for the past 12 years. The papulonodular lesions were non-tender and some were discrete while others were arranged in a circinate pattern. There was central scarring of the skin within the circinate lesions. In addition, there were several small yellowish papules on both sides of the neck which eventually became confluent to form plaques. Histopathology confirmed the diagnosis of elastosis perforans serpiginosa and pseudoxanthoma elasticum. He was treated with cryotherapy (using liquid nitrogen through cryojet) for former lesions. The lesions showed remarkable improvement after five sittings. Now the patient is under trientine hydrochloride (750 mg twice daily) for Wilson's disease.     

Nokardioseinfektion einer Gefäßendoprothese

A 61-year-old woman presented with purple-red subcutaneous nodules on her right leg. She had experienced fever up to 40 degrees C for the past 10 days and felt generally weak over the last two months. Four months earlier, a vascular graft had been implanted in her right femoral artery. Based on the diagnosis of skin infection due to implantation of an infected prosthesis, she was hospitalized and treated with an antibiotic regime. During the initial antibiotic treatment, the symptoms deteriorated, and she developed joint, hepatic and CNS abscesses. Finally, a microbiologic culture with an extended incubation time revealed the diagnosis of an infection with the slowly growing bacterium Nocardia ssp. Hereupon the patient was treated purposefully with Meropenem over six months. This treatment resulted in complete recovery.     

Perifolliculitis capitis abscedens et suffodiens

Perifolliculitis capitis abscedens et suffodiens (PCAS) is rare chronic, suppurative and inflammatory scalp disease. Its aetiology and pathogenesis is not completely understood. The treatment is usually difficult and often disappointing. We report a case of 29-year-old male who presented with tender, fluctuant nodules and abscesses, with draining pus and patchy alopecia on his scalp for 3 years. A skin biopsy from scalp lesions revealed features that are characteristic of perifolliculitis. Initially, the patient was treated with periodic incision and drainage of the scalp abscesses. The answer was very poor. When admitted to our department, isotretinoin was started at daily dose of 30 mg, because initially his cholesterol and triglyceride levels were mildly increased. When dose was reduced to 10 mg the levels of cholesterol and triglyceride remained normal. A response to treatment was excellent and rapid. The treatment of PCAS represents usually difficulties and frustration for both the patient and the physician. A long course of isotretinoin can be considered as one of the most effective treatment for PCAS.     

Fibroblastic rheumatism: case report and review of the literature

A previously healthy 7-year-old boy presented with polyarthritis and skin lesions. Multiple, skin- to pink-colored, firm papules were noted on the periungual areas, hands, feet, and nose. There was swelling of the proximal interphalangeal joints, wrists, elbows, ankles, and knees. A skin biopsy specimen revealed dermal fibrosis and interspersed histiocytes and lymphocytes. These findings were consistent with fibroblastic rheumatism, a condition characterized by cutaneous nodules and a symmetric polyarthritis. He was treated with methotrexate and corticosteroids with improvement in the symptoms of his arthritis and skin lesions. This early treatment was beneficial in our patient.     

Leg ulcers and abscesses caused by Serratia marcescens

Cutaneous infections caused by S. marcescens, a gram-negative bacillus belonging to the family of Enterobacteriaceae, are uncommon but may be predisposed by immunocompromised conditions or pre-damaged skin. A 73-year-old man presented with multiple ulcers and painful nodules on the lower right leg as well as abscesses on the right malleolus lateralis. He had been treated with oral penicillin without success. Due to chronic obstructive pulmonary disease, he was receiving a systemic therapy with corticosteroids. In addition, he had a post-thrombotic syndrome of the lower right leg. Serratia marcescens was the only microorganism isolated from all cultures performed. After a microbial sensitivity test, ertapenem 1 g/day was given intravenously for 10 days, followed by oral administration of ciprofloxacin 500 mg 1-0-1 for a further 7 days. This therapy resulted in the resolution of all lesions. This rare skin infection with S. marcescens needs specific microbiological diagnosis and adapted antibiosis.     

Lupus erythematosus panniculitis with morphea-like lesions

A 22-year-old female with morphea-like lesions, deep subcutaneous nodules and lipoatrophic areas of the skin on lateral aspects of the upper arms, on the breasts and on the buttocks is described. In 1990 a biopsy specimen obtained from a subcutaneous nodule showed hyaline necrosis of fat tissue; there were no epidermal changes. Direct immunofluorescene revealed granular deposits of IgM at the dermo-epidermal junction of the skin overlying the subcutaneous nodule. In a biopsy specimen taken at the onset of the disease in 1988, hyaline sclerosis of the deep dermis, follicular hyperkeratosis and vacuolar degeneration in the epidermis were described. The was weak positivity for antinuclear antibodies. The diagnosis of lupus erythematosus panniculitis (LEP) was made. Administration of Chloroquine resulted in complete clearing of nodules in 3 months. The reported case demonstrates the difficulties in establishing the diagnosis of LEP in patients who present with subcutaneous disease, morphea-like lesions and who do not have other clinical or laboratory evidence of lupus erythematosus. The differential diagnosis of LEP and deep morphea is discussed.     

Mycosis fungoides and cutaneous Hodgkin''s disease in the same patient: a case report

We describe a case of a 34-year-old man who presented with a 10-year history of mycosis fungoides and recent onset of inguinal lymphadenopathy, fever, and cutaneous nodules on the right hip. Biopsy of the inguinal lymph node showed mixed cellularity Hodgkin's lymphoma. Biopsy of the pre-existing skin lesions showed mycosis fungoides, while that of the nodules on the hip revealed cutaneous Hodgkin's lymphoma. Immunohistochemical and molecular analysis demonstrated a null phenotype in the Reed-Sternberg cells of the Hodgkin's disease lesions, and a T-cell phenotype in the lymphoid cells of the mycosis fungoides lesions. He was treated with chemotherapy for Hodgkin's disease, which is in remission 11 years later. Topical nitrogen mustard and maintenance PUVA therapy have been used for the mycosis fungoides, which is also in remission.