Jaqueline Schönlebe

Tumor Necrosis Factor-α Inhibitor-Induced Psoriasis or Psoriasiform Exanthemata

Tumor necrosis factor-α (TNFα) inhibition is effective in the treatment of moderate-to-severe psoriasis. We report on 120 patients from the literature including six new patients (three women and three men) who developed pustular lesions during treatment with TNFα inhibitors. We identified 72 women and 36 men (several papers did not specify the gender of patients) with an age range of 13–78 years (mean 42.3 years). The primary diagnoses were rheumatoid arthritis (n = 61), ankylosing spondylitis (n = 21), psoriasis (n = 10), Crohn disease (n = 8), SAPHO (synovitis acne pustulosis hyperostosis osteitis) syndrome (n = 3), psoriatic arthritis (n = 2), and other diagnoses (n = 15). Psoriasis (except palmoplantar pustular type) was the most common adverse effect during anti-TNFα treatment (n = 73), followed by palmoplantar pustular psoriasis (n = 37) and psoriasis of the nail (n = 6), sometimes combined in the same patient. Palmoplantar pustulosis and psoriasiform exanthema was the diagnosis in ten patients each. A positive personal history of psoriasis was recorded in 25 patients. A positive family history was noted in eight patients. No data about personal (n = 7) or family history (n = 46) were available in a number of patients. Newly induced psoriasis was diagnosed in 74 patients whereas an exacerbation or aggravation of a pre-existing psoriasis was noted in another 25 patients.All three TNFα inhibitors available on the market were involved: infliximab (63 patients), etanercept (37 patients), and adalimumab (26 patients). Several patients were treated with more than a single TFNα inhibitor. The timing of cutaneous adverse effects (psoriasis and psoriasiform rash) varied considerably among patients, ranging from after a single application to a delayed response of up to 63 months after initiation of treatment. The mean time to appearance of the cutaneous adverse effect for all TNFα inhibitors was 9.5 months.

Mineral oil granuloma of the penis

We report a 30‐year‐old‐patient with penile sclerosing lipogranuloma following injections of Vaseline. He was successfully treated by complete surgical removal of the subcutaneous material and excision of atrophic skin, followed by penoplasty. Temporary impairment of wound healing was overcome by application of topical carbon dioxide gas. A brief review on international literature is given.

Merkel cell carcinoma – a retrospective analysis of 17 cases

Objective To report clinical experience with the rare neuroendocrine Merkel cell carcinoma of the skin.

Disabling Pansclerotic Morphea of Childhood Poses a High Risk of Chronic Ulceration of the Skin and Squamous Cell Carcinoma

Disabling pansclerotic morphea of childhood (DPMC) is a rare and severe variant of scleroderma. This report presents 3 cases that presented to the authors and studies 25 patients from the literature (English language only) for the presence of chronic nonhealing ulcers of skin and skin cancer. The authors identified a total of 30 patients (9 male and 21 female) aged between 1 and 37 years at time of presentation. All cases were less than 14 years old when the disease started. The majority of patients had an aggressive course with deep sclerotic lesions leading to joint contractures and immobility. Five patients suffered from chronic nonhealing leg ulcers (17%), but ulcers were present on other parts of the body (upper limbs, trunk, head) as well (n = 6). Four patients died because of complications of the disease such as sepsis or gangrene. Two patients developed a squamous cell carcinoma at the age of 16 years and 19 years, respectively (6.7%). The available treatment of DMPC-associated ulcers is unsatisfying. Only temporary improvements have been seen in a minority of patients. We report on marked improvement of chronic leg ulcers by a combination of sildenafil 3 × 20 mg/day and repeated application of a porcine small intestinal submucosal acellular matrix.

Nevus comedonicus: an updated review.

The intention of this review on nevus comedonicus (NC) is to update on clinical features, pathogenesis, and therapy. NC is a rare epidermal nevus type. It is part of the nevus comedonicus syndrome, a neurocutaneous disorder with ocular, skeletal, and central nervous symptoms. Recently, acne-related signaling pathways and somatic mutations of tyrosine kinase receptors have been identified and may play a role in NC pathogenesis. On preexistent NC secondary skin tumors can develop, which are often benign. Treatment options of NC include topical therapy, laser, and surgery.

Late recurrence (10 years or more) of malignant melanoma in south-east Germany (Saxony) A single-centre analysis of 1881 patients with a follow-up of 10 years or more

Background  Late recurrent melanoma (MM) is rare.

Psoriasis and Brooke-Spiegler syndrome with multiple malignancies.

A 72-year old Caucasian man was referred to our dermatology department because of a relapse of his long-standing psoriasis, which had been treated repeatedly by the modified Ingram scheme. He had a history of multiple cylindromas and spiradenomas of the scalp and trunk. Trichoepitheliomas on the face, shoulder and trunk were surgically removed. In 1996 a malignant cylindroma of the scalp was operated on. In the same year multifocally growing basal cell adenocarcinomas of both parotid glands were diagnosed and treated by combined surgery and radiotherapy. On examination, he presented with large disseminated erythematosquamous plaques, Fitzpatrick skin type II–III. On the trunk, multiple skin-coloured to reddish papules and nodules were found. Small yellowish papules were seen in the nasolabial fold and retroauricular. An exophytic nodule (3 cm diameter) on the right flank was removed by surgery. Histological examination revealed a cystic skin tumour with adnexal differentiation. We found atypical enlarged cells with prominent nuclei, partially irregular nuclear shapes and increased ductal proliferation. Focally, there was a squamous differentiation with infiltrative growth into the hyalinized and sclerotic connective tissue (fig. 1). The diagnosis of spiradenoma with malignant transformation was made. We investigated p53 expression using pronase-digested specimen. The tumour expressed p53 as shown in fig. 2. Chest X-ray and ultrasound investigation of the abdomen and lymph nodes did not provide any evidence of metastatic spread. The combination of cylindroma(s) with trichoepithelioma(s) is characteristic for the Brooke–Spiegler syndrome. Less often, spiradenomas develop. 1,2 Malignant transformation of sweat gland tumours of skin is rare. We found only 31 cases of malignant cylindroma 2–5 and 16 cases of malignant spiradenoma 2,6–8 reported in the literature. To the best of our knowledge, malignant transformation of a spiradenoma has not been reported before in Brooke–Spiegler syndrome. The involvement of the parotid gland has occasionally been observed. 2 Basal cell adenomas are the most frequent parotid tumours in association with Brooke–Spiegler syndrome, but adenocarcinomas may also develop as in the present case. The occurrence of several malignancies in the same patient is remarkable. Based on investigative studies in skin adnexal tumours and parotid gland tumours, p53 overexpression occurs in about two-thirds of specimens. 9,10 It was assumed that UV light may play a role in the development of sweat gland carcinomas. 9 Indeed, the present patient was repeatedly exposed to UV irradiation because of his coincident psoriasis. On the other hand, we have demonstrated that the tumour expressed p53 (fig. 2). UV-therapy of psoriasis may be considered a risk factor for malignant transformation of adnexal skin tumours in Brooke–Spiegler syndrome.

Management of Severe Anogenital Acne Inversa (Hidradenitis Suppurativa)

BACKGROUND Severe anogenital acne inversa (AI) is a debilitating chronic inflammatory disease with a major negative effect on quality of life. OBJECTIVES To evaluate the role of surgery in the treatment of severe anogenital AI. METHODS We analyzed the records of patients with anogenital AI from 2000 to 2010. Assessment was done using the Hidradenitis Suppurativa Lesion, Area, and Severity Index (HS‐LASI), pain visual analogue scale, physician global assessment (PhGA), and patient global assessment (PaGA). Comorbidities and adverse events were analyzed. RESULTS Sixty‐seven patients with a Hurley score of 3 were identified. Mean follow‐up was 56.9 ± 41.3 months. A number of comorbidities were observed. After surgery, mean pain scores decreased from 6.3 ± 1.5 to 0.8 ± 0.7, PhGA improved from 6.8 ± 1.2 to 0.9 ± 0.6, PaGA improved from 7.3 ± 1.2 to 1.1 ± 0.5, and HS‐LASI decreased from 41.8 ± 21.3 to 2.4 ± 2.8. Adverse reactions were seen in 10.4%. The total relapse rate was 6% (5 patients) with only one case with healing by secondary intention (2% of 49 patients). CONCLUSIONS Surgery is the cornerstone of treatment for advanced AI. Healing by secondary intention results in significant reduction of complaints and achieves satisfying body contouring.

Deep Ulcer Shaving Combined With Split-Skin Transplantation in Distal Calciphylaxis

Calciphylaxis is a cause of painful deep ulcers. There is controversy about best wound management in this disease. A retrospective study of inpatients during the 3 years was made. Seven calciphylaxis patients were identified. All patients suffered from various associated pathologies including diabetes mellitus type II and chronic renal insufficiency. Ulcers were treated by aggressive and deep shaving combined with autologous split-skin grafting in the same session. A 30% to 90% take rate of the grafts eventually with a complete ulcer healing in 6 of 7 patients was achieved. No patient developed a deep cutaneous infection or sepsis. All patients are still alive except one. The single death was related to cardiovascular complications. In distal calciphylaxis, aggressive ulcer surgery with defect closure offers a marked improvement in quality of life and prevents early deep skin infections and sepsis as major causes of mortality.

Granulomatous tattoo reaction and erythema nodosum in a young woman: common cause or coincidence?

Tattooing has become quite popular in Western countries. With the increasing prevalence, there is also an increased risk of adverse effects. We describe a 17‐year‐old female patient with a black and red–colored tattoo, who developed immediately after red tattooing general malaise with fever, nausea, and vomiting. A bullous reaction was temporarily seen within the red part of her tattoo. The reaction later shifted to a subacute dermatitis with bacterial superinfection. Two months later, she felt ill again. She developed painful tender nodules on the anterior aspect of both lower legs identified as erythema nodosum without sarcoidosis. Is this is a unique case of adverse reaction to tattoo pigments with a type I and a type IV reaction, or is this a coincidence? The treatment was initiated with systemic and topical corticosteroids and topical antibiotics combined with compression bandages for the legs. After 3 weeks of treatment, the erythema nodosum completely resolved and did not reappear during a 1‐year follow‐up. The treatment of the local reactions, however, was unsatisfactory without complete response. There is an indispensable need for regulation of tattoo pigments and tattooing to improve consumer safety.