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  • Open Access Icon
  • Research Article
  • Cite Count Icon 12
  • 10.3315/jdcr.2015.1205
Cryptococcal panniculitis in a renal transplant recipient: case report and review of literature.
  • Sep 30, 2015
  • Journal of dermatological case reports
  • Sunil K Kothiwala + 3 more

Cryptococcosis is a deep fungal infection caused by Cryptococcus neoformans. The infection usually involves the lungs, the central nervous system as well as the skin, the bones and the urinary tract. Immunocompromised individuals, including solid organ transplant recipients, are at higher risk for cryptococcal infections. We present a 40-year-old renal transplant recipient who developed a slightly painful, erythematous, indurated plaque on his thigh several years after a kidney transplant. Histopathology revealed cryptococcal panniculitis and cryptococcus neoformans subsequently grew from the tissue culture. There was no other systemic involvement. The primary cutaneous form of cryptococcosis is extremely rare and early diagnosis and treatment is essential in view of possible dissemination and variable nonspecific clinical manifestations.

  • Open Access Icon
  • Research Article
  • Cite Count Icon 5
  • 10.3315/jdcr.2015.1212
Pityriasis rosea-like cutaneous eruption as the presenting symptom of Hodgkin lymphoma. Case report and review of the literature.
  • Sep 30, 2015
  • Journal of Dermatological Case Reports
  • Elena Vrotsos + 3 more

Cutaneous involvement by Hodgkin lymphoma is extremely rare and usually follows extensive involvement of the lymph nodes. Cutaneous manifestations of Hodgkin lymphoma may be divided into specific and non-specific. Generalized pruritus is one of the most common non-specific presentations of Hodgkin lymphoma as is cutaneous granulomas. Such signs and symptoms should prompt thorough physical exam, including evaluation of lymph nodes, especially in a young patient. We report a case of a 22-year-old man who presented with night sweats, weight loss, dry cough, and generalized maculopapular eruption that started with a large patch in the center of the chest and spread to the extremities. Biopsy of the rash revealed pityriasis rosea-like findings. A computerized tomography scan of the chest revealed a mediastinal mass. Biopsy of the mediastinal mass revealed Reed-Sternberg cells in a fibrotic background, diagnostic of Hodgkin lymphoma, nodular sclerosis type. In conclusion, the presentation of Hodgkin lymphoma as a pityriasis rosea-like cutaneous eruption is rare and clinicians should be aware of this presentation. In this paper we review the non-specific cutaneous manifestations of Hodgkin lymphoma in an effort to raise awareness of the diversity of early cutaneous signs of Hodgkin lymphoma.

  • Open Access Icon
  • Research Article
  • Cite Count Icon 14
  • 10.3315/jdcr.2015.1206
Disseminated refractory pyoderma gangraenosum during an ulcerative colitis flare. Treatment with infliximab.
  • Sep 30, 2015
  • Journal of Dermatological Case Reports
  • Vasiiki Zampeli + 6 more

Pyoderma gangraenosum is an immune-mediated, inflammatory, neutrophilic dermatosis of unknown etiology, which represents one of the extraintestinal manifestations of inflammatory bowel disease. It is a rare disease that occurs in less than 1% of patients with inflammatory bowel disease and with the same ratio in patients with Crohn's disease and ulcerative colitis. A 36-year-old woman was diagnosed with ulcerative colitis 6 years before admission to our dermatology department with an acute disseminated pyoderma gangraenosum with mucosal involvement, during a flare of ulcerative colitis. Disease progression was interrupted by intravenous administration of the tumor necrosis factor-α inhibitor infliximab at 5 mg/kg at weeks 0, 2, and 6 (1st cycle) and every 8 weeks thereafter. Improvement of intestinal, skin and oral manifestations was evident already after the 1st cycle of treatment and has been maintained since (at least 16 months). This case report is one of very few on disseminated pyoderma gangraenosum with oral involvement complicating ulcerative colitis, where infliximab was shown to have a rapid efficacy on skin, mucosal and bowel symptoms.

  • Open Access Icon
  • Research Article
  • Cite Count Icon 9
  • 10.3315/jdcr.2015.1208
Brooke-Spiegler Syndrome - an underrecognized cause of multiple familial scalp tumors: report of a new germline mutation.
  • Sep 30, 2015
  • Journal of Dermatological Case Reports
  • AndrĂ© Castro Pinho + 4 more

Brooke-Spiegler syndrome (BSS) is probably an underdiagnosed genodermatosis that predisposes for the development of cylindromas, spiradenomas and trichoepitheliomas mainly of the head and neck. Wide phenotypic variability regarding the number and type of lesions can be observed within a family. Mutations of the CYLD gene are identified in the vast majority of cases and play a key role in BSS pathogenesis. Two first degree relatives with numerous erythematous telangiectatic nodules of the scalp present for decades, with recurring tendency regardless the multiple previous excisions. Histopathological review of the lesions revealed predominantly "spiradenocylindromas" in the proband and cylindromas in her sister. The suspicion of BSS was confirmed after detection of a new nonsense germline mutation of CYLD (c.1783C>T pGln 595*) in the proband. BSS diagnosis can be challenging and is based on clinical-pathological correlation, positive familial association and identification of CYLD mutations. CYLD exerts antineoplastic effects by downregulating intracellular NF-ÎşB signalling pathways. The reported mutation affecting the ubiquitin-specific protease domain leads to a truncated and catalytically inactive enzyme. Despite the expanding list of CYLD mutations no firm genotype-phenotype correlation is known so far. Early recognition and treatment of BSS avoid disfiguring changes like "turban tumor".

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  • Research Article
  • Cite Count Icon 7
  • 10.3315/jdcr.2015.1211
Large eccrine angiomatous hamartoma: a novel clinical presentation of disease.
  • Sep 30, 2015
  • Journal of Dermatological Case Reports
  • Uffe Nygaard + 3 more

Eccrine angiomatous hamartoma is a rare benign cutaneous malformation with a diverse clinical appearance, therefore likely to be misdiagnosed and underreported. A 44-year-old man presented with a congenital erythematous hyperhidrotic plaque on the left upper back measuring 18 x 25 cm. No pain or tenderness nor hypertrichosis were observed. Histopathology was consistent with the mucinous variant of eccrine angiomatous hamartoma. Intralesional injection of botulinum toxin type A greatly reduced localized sweating, improving patient quality of life. This article describes a novel clinical presentation of eccrine angiomatous hamartoma: large, erythematous, and slightly indurated plaque localized on the upper back. It emphasizes the role of histopathology in the diagnostic process and botulinum toxin as a viable treatment option.

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  • Research Article
  • Cite Count Icon 3
  • 10.3315/jdcr.2015.1202
Photoletter to the editor: Lamellar ichthyosis and arthrogryposis in a premature neonate.
  • Jun 30, 2015
  • Journal of dermatological case reports
  • Chiara De Leonibus + 8 more

Lamellar ichthyosis is a rare congenital disorder characterized by collodion membrane at birth and facial anomalies (eclabium and ectropion). The major underlying genetic defect is in TGM1, with mutations of this gene found in 50% of patients. An early diagnosis is fundamental in view of establishing a specific treatment due to the severity of the disease. We report a case of severe lamellar ichthyosis and arthrogryposis, without the typical facial presentation, negative for TGM1 mutations. The clinical improvement was achieved only after treatment with oral retinoids, highlighting the importance of early diagnosis and prompt administration of a specific therapy.

  • Open Access Icon
  • Research Article
  • Cite Count Icon 2
  • 10.3315/jdcr.2015.1200
Photoletter to the editor: An eczema-like, pruritic, nonbullous form of bullous pemphigoid.
  • Jun 30, 2015
  • Journal of dermatological case reports
  • Kui Young Park + 4 more

We report a case with an unusual manifestation of bullous pemphigoid (BP) in a 57-year-old woman. She presented with a one-month history of pruritus and multiple annular patches with central regression on her trunk and extremities and there were no signs of corresponding internal malignancy. Eosinophilic spongiosis was observed in histopathologic examination and direct immunofluorescence studies revealed a strong linear staining pattern of the basement membrane zone with C3 and IgG. This unusual case indicates that a lack of blisters does not necessarily exclude a diagnosis of BP, and supports previous reports suggesting gyrate erythema can be an initial manifestation of bullous disease.

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  • Research Article
  • Cite Count Icon 4
  • 10.3315/jdcr.2015.1201
Photoletter to the editor: Pigmented dermatofibrosarcoma protuberans in a 4-year-old girl and ultrasonographic findings.
  • Jun 30, 2015
  • Journal of Dermatological Case Reports
  • Ryo Tanaka + 4 more

Dermatofibrosarcoma protuberans (DFSP) in children is often clinically misdiagnosed as hemangioma or vascular malformation. Ultrasonography and color Doppler imaging are useful noninvasive tools for the diagnosis of skin tumors and may help distinguish DFSP from other vascular skin lesions in children.

  • Open Access Icon
  • Research Article
  • Cite Count Icon 66
  • 10.3315/jdcr.2015.1199
How to treat actinic keratosis? An update.
  • Jun 30, 2015
  • Journal of Dermatological Case Reports
  • Claudia Costa + 4 more

Actinic keratosis (AKs) is one of the most common skin lesions leading to an increased risk of developing squamous cell carcinoma and other skin malignancies. The lesions principally arise as a result of excessive ultraviolet (UV) exposure. AKs may regress spontaneously, remain stable or evolve to invasive squamous cell carcinoma. The risk of squamous cell carcinoma is significantly increased patients with more than 5 AKs. The main mechanisms involved in the formation of AK are inflammation, mutagenesis, oxidative stress, impaired apoptosis, immunosuppression, disregulation of cell growth and proliferation, and tissue remodeling. Human papilloma virus has also been correlated with the formation of some AKs. As an individual ages, his skin is exposed to increasing cumulative amounts of UV light and other environmental insults. This is especially true for the head, neck and forearms. These insults do not target only the skin where individual lesions develop, but also the surrounding area. In this area undetectable preclinical AK lesions or dysplastic cells may be present. The whole affected area is known as the 'field'. Therefore, management is divided into lesion-directed and field-directed therapies. Currently, the therapies in use are lesion-directed cryotherapy and/or excision, and field-directed topical agents: 5-fluorouracil, diclofenac, photodynamic therapy, imiquimod, and ingenol mebutate. Combining lesion- and field-directed therapies showed good results and several novel therapies are under investigation. Treatment is variable and personalized, what makes a gold standard management algorithm difficult to design. This review aims to describe the rationale behind the available treatment options for AKs based on current understanding of pathophysiology and epidemiology.

  • Research Article
  • Cite Count Icon 1
  • 10.3315/jdcr.v9i2.1059
An eczema-like, pruritic, nonbullous form of bullous pemphigoid
  • Jun 30, 2015
  • Journal of Dermatological Case Reports
  • Kui Young Park + 4 more

We report a case with an unusual manifestation of bullous pemphigoid (BP) in a 57-year-old woman. She presented with a one-month history of pruritus and multiple annular patches with central regression on her trunk and extremities and there were no signs of corresponding internal malignancy. Eosinophilic spongiosis was observed in histopathologic examination and direct immunofluorescence studies revealed a strong linear staining pattern of the basement membrane zone with C3 and IgG. This unusual case indicates that a lack of blisters does not necessarily exclude a diagnosis of BP, and supports previous reports suggesting gyrate erythema can be an initial manifestation of bullous disease. ( J Dermatol Case Rep. 2015; 9(2): 55-57)