- [Rare presentations of infantile hemangiomas: 4 cases]. [Journal Article]
- ADAnn Dermatol Venereol 2018 Nov 08
- CONCLUSIONS: We report 4 rare forms of infantile hemangioma resulting in initial diagnostic error. The atypical nature of some IHs may direct the clinician and the radiologist toward other diagnoses that in some cases have no vascular contingent. It is important for the dermatologist to be aware of these rare forms of IH in order to reduce the time to diagnosis and allow early initiation of appropriate management.
- Familial Angiofibrohistiocytic Hamartoma Syndrome. [Journal Article]
- SSkinmed 2018; 16(5):347-349
- A 27-year-old Hispanic man presented with multiple papules and nodules measuring up to 10 cm in diameter. These lesions were widespread (Figure 1), but not on mucosal epithelium. At birth, the patien...
A 27-year-old Hispanic man presented with multiple papules and nodules measuring up to 10 cm in diameter. These lesions were widespread (Figure 1), but not on mucosal epithelium. At birth, the patient had had multiple hypopigmented macules that had progressed to papules and nodules over time. Dermatoscopic examination of these papules and nodules showed prominent capillaries. New lesions were constantly developing but were slow-growing. Other family members had similar lesions, including the patient's three brothers, father, paternal aunt, and a 3-year-old niece (daughter of the 2nd eldest brother), although not as extensively as in this patient. The paternal grandparents were not affected (Figure 2). As the patient had been raised in rural Mexico with limited financial resources, access to health care was limited, so the condition had been left undiagnosed and untreated for most of the patient's life.
- A case of primary cutaneous peripheral T-cell lymphoma, not otherwise specified with cytotoxic phenotype showing multiple ulcers on the entire body. [Case Reports]
- JCJ Cutan Pathol 2018 Nov 08
- Primary cutaneous peripheral T cell lymphoma, not otherwise specified (pcPTCL-NOS) is a rare, aggressive, fatal type of cutaneous T-cell lymphoma. The clinical presentation of pcPTCL-NOS is character...
Primary cutaneous peripheral T cell lymphoma, not otherwise specified (pcPTCL-NOS) is a rare, aggressive, fatal type of cutaneous T-cell lymphoma. The clinical presentation of pcPTCL-NOS is characterized by generalized plaques, nodules or tumours but ulcers are uncommon. We report an atypical case of pcPTCL-NOS with cytotoxic protein expression, presenting as multiple ulcers on the entire body. A 48-year-old man first presented with pruritic papules on the trunk. The papules gradually increased in number and became ulcerated. We finally diagnosed with pcPTCL-NOS because of diffuse dermal infiltration of medium- to large-sized pleomorphic CD4 positive lymphoid cells. Ulceration suggests infiltration of lymphoid cells expressing cytotoxic proteins which can induce apoptosis in the epidermis and dermis. Our cases died of bacterial sepsis that invaded from the uncontrollable ulcers. A suspicion of pcPTCL-NOS is needed when encountering clinical pictures of refractory multiple ulcers and a biopsy should always be performed, because treatment delay may lead a very poor prognosis. This article is protected by copyright. All rights reserved.
- Long-term Follow-up of a Case of Lymphomatoid Papulosis with a Benign Course. [Journal Article]
- ADActa Dermatovenerol Croat 2018; 26(3):264-266
- Dear Editor,We present the case of a 40-year old male patient with lymphomatoid papulosis of a waxing and waning course on whom three biopsies were performed during a 14-year period with no change in...
Dear Editor,We present the case of a 40-year old male patient with lymphomatoid papulosis of a waxing and waning course on whom three biopsies were performed during a 14-year period with no change in histopathological or immunophenotypical characteristics. Lymphomatoid papulosis (LP) is a chronic, recurrent, self-healing papulonodular skin eruption with the histopathologic features of a cutaneous T-cell lymphoma but an often benign and indolent clinical course (1). It is designated as a primary, cutaneous, CD30+ lymphoproliferative disorder. The histopathologic features of LP are variable, with five main types (A-E) and several other variants (2). In most cases, LP presents with a generalized eruption of reddish-brown papules or nodules usually smaller than one cm on the trunk and limbs. Rarely, large, rapidly growing nodules may be the first manifestation of the disease (3). Patients with LP have an excellent prognosis even though they are at increased risk of developing secondary cutaneous or nodal lymphomas such as mycosis fungoides, primary cutaneous anaplastic large cell lymphoma (PC-ALCL), or Hodgkin lymphoma (4). LP-associated lymphomas develop in between 10% and, as recently reported, 52% percent of patients and may occur before, concurrent with, or after the onset of LP (4,5). Our patient was diagnosed with "conventional" type An LP 14 years earlier based on the clinicopathologic correlation. The diagnosis was confirmed a year later after excision of a rapid growing ulcerated nodule on the forearm measuring 17 mm in diameter, which was clinically suspected to be anaplastic large cell lymphoma. During these 14 years, there were only a few worrisome recurrences of the disease, which resolved spontaneously or were successfully controlled with local steroids. During a recent exacerbation, when the third biopsy was performed, the patient presented with a large number of generalized reddish-brown pruritic papules and nodules on the trunk, extremities, neck, and face, predominantly up to one cm, some among which were necrotic and excoriated (Figure 1). There were three sites of clustered papules on the trunk, groin, and neck that resembled large, infiltrated plaques larger than two cm, at a glance mimicking cutaneous lymphoma (Figure 1, b, c). There were also older residual hyper- and hypopigmentations on the skin with prominent scarring. Excisional skin biopsy of one larger papule from the abdominal plaque was performed and was not morphologically or immunophenotypically different from the previous ones (Figure 2). Immunohistochemistry showed expression of CD 30 and the phenotypic markers of T-helper lymphocytes (CD 3+/-, CD4+) by neoplastic cells (Figure 2, c, d). Associated systemic malignant lymphoma was excluded based on examination findings, normal laboratory tests, the absence of palpably enlarged lymph nodes, hepatosplenomegaly, and systemic symptoms followed with MSCT. Serology for HIV and EBV was performed and was positive for EBV EBNA, VCA IgG, and IgM, which could be associated with the exacerbation of LP. Topical corticosteroids and phototherapy were administered when needed in this 14-year period, and methotrexate in a lower dose was prescribed during the extensive generalized eruptions. All of the applied therapeutic modalities led to a partial response. LP is a self-limiting disease for which many patients do not require specific treatment. Therapy should be directed at controlling symptoms in generalized eruptions or minimizing the frequency of recurrences, but none of the available treatment options disrupt the natural history of LP or reduce the risk of developing an associated lymphoma (6). Low-dose methotrexate is the initial therapy of choice in patients with the extensive or symptomatic disease or disease involving cosmetically sensitive areas like the face or hands, which were the affected areas in our patient (6,7). There are no markers that can help predict the course of the disease in a given patient, although some indicators have been suggested (8,9). Because of this lack of markers that can help predict the course of the disease and occurrence of malignant lymphoma, patients should remain in monitoring for the rest of their life.
- [31/f with livid erythematous papules and nodules : Preparation for the specialist examination: part 6]. [Journal Article]
- HHautarzt 2018; 69(Suppl 2):100-104
- [56-year-old male with erythematous, excoriated papules and nodules on the extremities, shoulders and buttocks : Preparation for the specialist examination: part 12]. [Journal Article]
- HHautarzt 2018; 69(Suppl 2):126-131
- StatPearls [BOOK]
- BOOKStatPearls Publishing: Treasure Island (FL)
- Sporothrix schenckii, S. schenckii, is a dimorphic fungus that can cause Sporotrichosis. S. schenckii exists in either a hyphal form at temperatures less than 37 degrees Celsius or as a budding yeast...
Sporothrix schenckii, S. schenckii, is a dimorphic fungus that can cause Sporotrichosis. S. schenckii exists in either a hyphal form at temperatures less than 37 degrees Celsius or as a budding yeast at 37 degrees Celsius or greater. This characteristic morphology is important in identifying the conversion from mold to yeast. There are different strains of S. schenckii, with S. schenckii complex being most commonly found in America, Asia, and Africa. S. brasilienis has been found in Brazil and is known to be transmitted from infected cats. S. Mexicana and S. globose have also been identified throughout the world. Sporotrichosis caused by S. schenckii occurs by inoculation of soil, plants, and organic matter contaminated with the fungus. Sporotrichosis typically presents as papules or pustules that form ulcerated nodules involving local lymphatics. Sporotrichosis is classified into cutaneous, pulmonary, and disseminated, with cutaneous the most common form of the disease. Risk factors which increase the risk of disseminated disease include patients who are immunocompromised and those with chronic obstructive pulmonary disease, alcoholism, and diabetes mellitus. General prevention is to wear gloves and long sleeves when handling soil. The gold standard for sporotrichosis diagnosis is fungal culture. First-line treatment is itraconazole.
- Position Statement: Linear prurigo is a subtype of chronic prurigo. [Journal Article]
- JEJ Eur Acad Dermatol Venereol 2018 Oct 05
- CONCLUSIONS: Considering linear prurigo as belonging to the spectrum of CPG has important clinical implications, since both the diagnostic and therapeutic approach of these patients should be performed as recommended for CPG. Importantly, linear prurigo should be differentiated from self-inflicted skin lesions as factitious disorders or skin picking syndromes. In the latter, artificial manipulation rather than pruritus itself leads to the development of cutaneous lesions, which can show clinical similarities to linear prurigo.
- Primary Surgical Treatment of Erythema Elevatum Diutinum. [Journal Article]
- JHJ Hand Surg Am 2018 Sep 18
- Erythema elevatum diutinum (EED) is a rare skin disease caused an Arthrus-type immunological reaction to antigen with immune complex deposition in the cutaneous microvasculature, which leads to tissu...
Erythema elevatum diutinum (EED) is a rare skin disease caused an Arthrus-type immunological reaction to antigen with immune complex deposition in the cutaneous microvasculature, which leads to tissue damage secondary to the effects of complement and leukocytes. It presents as brown or red cutaneous nodules, papules, or plaques, often on the extensor surfaces of the hands, knees, or elbows. Onset usually occurs in the fourth to sixth decades but possibly younger in patients with human immunodeficiency virus. Medical treatment is usually successful; however, surgical treatment can be used when chemotherapy fails. We present a case of a 29-year-old man with EED treated with excision and skin grafting.
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- Familial Dyskeratotic Comedones. [Journal Article]
- SSkinmed 2018; 16(4):273-274
- A 21-year-old man presented with extensive asymptomatic comedones and inflammatory papulonodules that had been present for the previous 7 years. The lesions had first appeared on the upper part of th...
A 21-year-old man presented with extensive asymptomatic comedones and inflammatory papulonodules that had been present for the previous 7 years. The lesions had first appeared on the upper part of the trunk, subsequently spreading to the face, arms, axillae, thighs, groin, and buttocks. Physical examination revealed numerous monomorphic discrete black papules with firm central keratotic plugs. A few painful, inflamed nodules were present over the back (Figure 1). Pocklike scars were located predominantly over the face and back. His general health was otherwise normal, and he had not received any prior treatment for this condition. His father had similar lesions. Skin biopsy from the hyperkeratotic lesions revealed a crater-like invagination filled with lamellar keratinous material with foci of dyskeratosis (Figure 2).