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Dermatology Online Journal is an open-access, refereed publication intended to meet reference and education needs of the international dermatology community since 1995. Dermatology Online Journal is supported by the Department of Dermatology UC Davis, and by the Northern California Veterans Administration.

Volume 21, Issue 3, 2015

Special Review

Maintenance of certification and the financial status of the medical boards

Abstract

Background: The Maintenance of Certification (MOC) program proposed by the American Board of Medical Specialties (ABMS) gained approval in 2006. The implementation of this program will impact all physicians who are board certified. Therefore, The Financial Status of the Medical Boards is an increasingly relevant topic of discussion amongst all physicians and those interested in medical education and certification. With this study we aim to bring greater attention to the already publicly available financial status of the Medical Boards (MB) so that it can become part of the ongoing discussion of MOC.

Method: We analyzed the yearly revenue, expenses, net gain or loss and end of year balance for the ABMS and its 24 MB, additional member boards, as well as the Accreditation Council for Graduate Medical Education (ACGME), American Osteopathic Association (AOA) and Accreditation Council for Continuing Medical Education (ACCME) organizations.

Results: We have commented on notable trends based on the available IRS Form 990s spanning from 1997 to 2012. When comparing the most recently available reported end of year balance to the first available reported end of year balance, 87% (27/31) of the member boards have reported an increase. All three of the additional organizations studied, ACGME, AOA and ACCME reported an increase as well.

Conclusions: It is clear from the data and analysis that the majority of MB have financially benefited from the MOC program. It remains to be proven whether or not this economic benefit will translate into an improvement in physician education and patient care.

Review

Successful use of the excimer laser for generalized psoriasis in an ustekinumab non-responder

Effective treatments for moderate to severe psoriasis are phototherapy and biologics. These treatments are similar in that they both decrease cutaneous immune system hyperactivity yet do so via different mechanisms. Our patient, a 63 year old Asian male had a rapid response to treatment with the high dose excimer laser, having previously failed treatment with 28 weeks of ustekinumab therapy. A pre-treatment biopsy of a psoriatic plaque was found to contain relatively low levels of IFN-γ (Th1) and IL-17 (Th17) secreting T cells. Following treatment with the excimer laser, the patient had a quick improvement in PASI that was reflected by a 3-fold reduction in the number of live T cells found in the post-treatment biopsy. Although ustekinumab and the excimer laser both result in decreased levels of these cytokines, the excimer laser directly causes apoptosis of T cells and induces DNA damage in antigen presenting cells. Thus, the broader effects of phototherapy on immune cells compared to the targeted inhibition of IL-12 and IL-23 by ustekinumab likely account for the superior response observed.

 

Case Report

Scleromyxedema with histology resembling granuloma annulare

Scleromyxedema is a generalized and progressive fibromucinous disorder associated with substantial cutaneous and systemic morbidity.  The diagnosis is often challenging, as is management. We present here a patient with scleromyxedema with atypical, granuloma annulare-like histology, which contributed to delayed diagnosis and management, including a delayed workup for multiple myeloma.  Ultimately, the patient did well with appropriate therapy, but his presentation illustrates the importance of more widespread familiarity among dermatologists and dermatopathologists with this variant of scleromyxedema.

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Histiocytoid giant cellulitis-like Sweet’s syndrome: case report and review of the literature

Background: Histiocytoid Sweet syndrome is an uncommon variant in which the dermal infiltrate is composed of mononuclear cells with a histiocytic appearance that represent immature myeloid cells. Giant cellulitis-like Sweet syndrome is a recently described variant characterized by relapsing widespread giant lesions.

Purpose: We report a unique patient with histiocytoid giant cellulitis-like Sweet syndrome and review the current literature on histiocytoid Sweet syndrome and giant cellulitis-like Sweet syndrome.

Material and Methods: We reviewed PubMed for the following terms and have reviewed the literature: histiocytoid, giant cellulitis-like, and Sweet syndrome.

Results: Six individuals, including our patient, have been reported with giant cellulitis-like Sweet syndrome; four had obesity, two had a hematologic malignancy, and one had breast cancer. Histiocytoid Sweet syndrome has been reported in association with autoimmune diseases, infection or inflammation, inflammatory bowel disease, malignancies, medications, and other conditions.

Conclusions: Histiocytoid Sweet syndrome is a rare variant of Sweet syndrome, often associated with malignancy. Giant cellulitis-like Sweet syndrome has been reported in six individuals; four of the patients were obese and three of the patients had an associated cancer. Our patient had histiocytoid giant cellulitis-like Sweet syndrome-associated myelodysplastic syndrome/myeloproliferative disorder. The diagnosis of histiocytoid Sweet syndrome or giant cellulitis-like Sweet syndrome should prompt the clinician to consider additional evaluation for a Sweet syndrome-associated malignancy.

 

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Marginal vitiligo: an unusual depigmenting disorder

Background: Marginal vitiligo, or inflammatory vitiligo with raised borders is a unique subset of vitiligo vulgaris presenting with scattered depigmented, pruritic patches surrounded by a raised, erythematous border. Alternative diagnoses include discoid lupus erythematosus and cutaneous T-cell lymphoma.

Purpose: To properly guide treatment, it is important to exclude other conditions that present with a similar pattern of depigmentation. This requires the ability to identify specific cutaneous and histologic clues that support this diagnosis.

Material and Methods: A 22 year-old man presented with a history of depigmented, pruritic patches and surrounding raised, erythematous borders. Several areas of depigmentation on the scalp were associated with alopecia. Punch biopsy of the rim of a patch was performed and sent for routine hematoxylin and eosin staining and direct immunofluorescence.

Results and Conclusion: Histopathology revealed a spongiotic dermatitis with superficial dermal lymphocytic infiltrate and eosinophils; DIF demonstrated no immunoreactants. A diagnosis of inflammatory vitiligo with raised borders was thus made based on consistent clinical and histopathologic features. The symptoms improved with topical clobetasol 0.05% cream twice daily.

 

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Infantile perianal protrusion

Infantile perianal protrusion is characterized by a skin fold located in the perianal area. It is a relatively recent reported condition and affects both infants and prepubertal children with a clear female predominance. Three types are recognized: constitutional/congenital, acquired, and associated with lichen sclerosus et atrophicus. We report eleven new cases, three of whom have the defect in locations that have been reported only once before. We would like to increase the awareness of this condition to avoid erroneous diagnostic and therapeutic procedures.

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Reed syndrome: an atypical presentation of a rare disease

Reed syndrome, also known as Multiple Uterine and Cutaneous Leiomyomas (MCUL), is an autosomal dominant defect in the fumurate hydrase gene, leading to a predisposition of leiomyomas of the skin and uterus. Patients with Reed syndrome may present with cutaneous leiomyomas, uterine leiomyomas and/or leiomyosarcomas. A 37-year-old woman presented to the dermatology clinic with several subcutaneous nodules. Punch biopsy was performed and the diagnosis of angioleiomyosarcoma with supervening degenerative changes was made. Medical history was positive for uterine leiomyomas. These concomitant findings led to the diagnosis of Reed syndrome. At the present time, genetic counseling is a suggested screening parameter for patients with multiple cutaneous leiomyomas. The superficial nature of these lesions and the low staging made surgical excision the preferred and actual treatment method. Adjunct radiation and chemotherapy have not been shown to provide clear benefit of survival. Owing to an association with renal cell carcinoma, a referral for nephrology consultation is also recommended.

Case Presentation

Lues maligna praecox: an important consideration in HIV-positive patients with ulceronodular skin lesions

Syphilis is commonly known as “the great imitator” owing to its varied clinical manifestations. Secondary syphilis has a variety of presentations, with the most common manifesting as a diffuse papulosquamous eruption on the palms and soles. Lues maligna praecox is a rare form of secondary syphilis, with severe constitutional symptoms, seen primarily in HIV-positive individuals. We report an atypical case of suspected lues maligna in a 45-year-old male. The patient was HIV-positive with a CD4 count of 441. He presented to our clinic with large painful gummatous ulcers in the groin and lower back. He also reported daily fevers, night sweats, and weight loss consistent with secondary syphilis. Prior to this episode the patient had a history of acute active syphilis (RPR 1:128) in 2012 treated at that time with a single dose of 2.4 million units intramuscular benzathine penicillin; he had no reported exposures since that time. The patient was treated with three weekly doses of benzathine penicillin, 2.4 million units, given intramuscularly. This case demonstrates the importance of recognizing the varied clinical presentation of secondary syphilis and keeping lues maligna in consideration for ulceronodular skin lesions in patients who are HIV-positive.

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Cutaneous and subcutaneous leiomyosarcoma: report of two cases.

Cutaneous leiomyosarcomas are uncommon malignant tumors of smooth muscle. We report herein two cases of cutaneous leiomyosarcoma, one of dermal and other of subcutaneous vascular origin, confirmed by histology and immunohistochemistry. Both cases were treated with wide surgical excision, one with local recurrence. No metastases were detected at follow-up. Leiomyosarcomas should be considered in lesions with histologic features of spindle cell malignancy. It is necessary in most of the cases a histological differentiation with specific immunohistochemical markers. The treatment of choice is radical excision with wide margins, achieving satisfactory results.

 

Disseminated varicella-zoster virus in an immunocompetent adult

Varicella-zoster is the virus that causes varicella (chicken pox), herpes zoster (shingles), and rarely, severe disseminated disease including diffuse rash, encephalitis, hepatitis, and pneumonitis. Disseminated disease is most often seen in immunocompromised patients. We describe a case of disseminated zoster in an immunocompentent patient who had previously been immune to VZV. This case is also unusual in that his clinical presentation was most consistent with varicella while his laboratory data was most consistent with herpes zoster. For the purpose of rapid diagnosis and initiation of appropriate therapy, clinicians should be aware of these more atypical presentations of VZV infection.

A case of generalized red sweating

We report a case of a 70-year-old man with a 5-year history of red pigmented sweating on the axillae, groin, forearms, trunk, and and peri-nuchal sites. No identifiable cause of the pigmented sweat was identified. Biopsy revealed lipofuscin pigment leading to the diagnosis of idiopathic chromhidrosis. This case represents an unusual presentation of chromhidrosis that was not limited to the apocrine sweat gland distribution and ultimately revealed no identifiable cause. After failing multiple treatment options, the patient elected for a trial of treatment with 20% aluminum chloride solution.

Eccrine chromhidrosis secondary to hyperbilirubinemia

Background: Eccrine chromhidrosis, or colored eccrine sweating, may be caused by contamination of sweat by dyes, pigmentation from microorganisms, or more rarely, hyperbilirubinemia. Pigment usually affects the palms and soles, where abundant sweat glands are found.

Purpose, Material and Methods: We report a unique case of eccrine chromhidrosis in the setting of hyperbilirubinemia and review the current literature available on PubMed of previously reported cases.

Results: Six patients with chromhidrosis have been previously reported in the setting of significant hyperbilirubinemia, in association with fever and thickened stratum corneum.

Conclusions: Eccrine chromhidrosis secondary to hyperbilirubinemia is very rare, but can be diagnosed on the basis of classic clinical findings, dermoscopic examination, and negative tissue cultures.

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Elejalde syndrome (ES)

Silvery hair and severe dysfunction of the central nervous system (Neuroectodermal melanolysosomal disease or Elejalde Syndrome) characterize this rare autosomal recessive syndrome. The main clinical features include silver-leaden hair, bronze skin after sun exposure, and neurologic involvement. Large granules of melanin unevenly distributed in the hair shaft are observed. Abnormal melanocytes and melanosomes and abnormal inclusion bodies in fibroblasts may be present. We report a 10-year-old girl with silver-leaden (silvery) hair, bronze skin color on sun-exposed areas, generalized hypopigmentation of covered body parts, and congenital seizures. The child was the elder of two children born of a consanguineous marriage. The younger sibling, a female neonate, had the same clinical presentation.

Primary mucocutaneous histoplasmosis in an immunocompetent patient

We report a case of primary mucocutaneous histoplasmosis in an immunocompetent individual. The patient, a 61-year-old woman, presented with a non-healing ulcer on the lateral border of her tongue. Excisional biopsy of the lesion was consistent with histoplasmosis and no evidence of pulmonary or disseminated infection was found. Although mucocutaneous infection has been well-described as a manifestation of disseminated disease, especially in immunocompromised individuals, oral infections in immunocompetent patients are rare.

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Cutaneous metastasis from penile squamous cell carcinoma resembling carcinoma en cuirasse

Penile squamous cell carcinoma is a rare malignancy seen more frequently in developing nations. Metastasis occurs in a predictable manner, with superficial lymph node involvement occurring first, followed by deep lymph node involvement, and then distant spread. Brain, lung, liver, and bone are the typical sites of distant metastasis. We present the unusual case of an 81-year-old man with penile squamous cell carcinoma requiring total penectomy who developed a confluent red to violaceous, indurated suprapubic plaque with satellite papules and bulky inguinal lymphadenopathy. The shield-like clinical presentation and infiltrating strands and cords on histology resembled carcinoma en cuirasse, a rare form of cutaneous metastasis frequently associated with breast cancer but not reported with penile squamous cell carcinoma.

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Varicella zoster virus encephalitis in a patient with disseminated herpes zoster: report and review of the literature

Herpes zoster infection occurs owing to reactivation of varicella zoster virus and classically manifests as a vesicular eruption involving a single dermatome. Disseminated herpes zoster - defined as having greater than twenty vesicles outside the primary or adjacent dermatome - is uncommon and typically occurs in immunocompromised individuals. Central nervous system complications during or following a zoster outbreak are exceedingly rare. Encephalitis is reported to affect only 0.1-0.2% of patients and occurs more often in disseminated cases and in outbreaks involving those dermatomes in close proximity to the central nervous system. We present an elderly woman with disseminated herpes zoster and altered mental status who was subsequently diagnosed with varicella zoster virus encephalitis and describe the characteristics of patients with disseminated zoster who developed varicella zoster virus encephalitis.

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Pemphigus vulgaris presenting with multiple lesion morphologies

Pemphigus vulgaris is an uncommon intraepidermal blistering disorder that typically presents with flaccid bullae or erosions. We report a patient with pemphigus vulgaris who presented with several unusual clinical features: tense bullae with dependently layered pus, true target lesions coalescing into annular configurations, and diffuse desquamation that initially raised concern for toxic epidermal necrolysis. We discuss the differential diagnosis and implications of these morphological findings.

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Blastic plasmacytoid dendritic cell neoplasm of the skin associated with myelodysplastic syndrome

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a recently described, rare hematologic malignancy with prominent skin involvement. The prognosis of this disease is poor in most cases, with rapid progression despite chemotherapy administration. The first reported case of this disease was in 1994, and less than 200 cases worldwide have been described in the literature to date. Moreover coexistence of BPDCN and MDS is extremely rare. In this study, we describe a typical patient with BPDCN in China who presented with cutaneous involvement as the first manifestation associated with MDS; a brief review of literature is also given.

Commentary

Cutaneous manifestations of the ebola virus

The current Ebola outbreak has drawn attention to the virus in the medical community. Zaire ebolavirus, more commonly known as ‘the Ebola virus,’ is a level 4-security agent in the Filoviridae family. The main cutaneous finding of Ebola is a nonspecific maculopapular rash that appears between day four and six of disease. Patients have “ghost-like” features, and the rash initially presents on the upper arms, flexor forearms, and upper legs, sometimes in a centripetal fashion. Skin biopsy, immunohistochemistry, ELISA, and electron microscopy can help provide early diagnosis and conceivably prevent spread if proper precautionary measures are in place.

Photo Vignette

Lipoid Proteinosis: a case report in two siblings

Lipoid proteinosis was first reported by Urbach and Wiethe in 1929. It is also known as hyalinosis cutis et mucosae or Urbach-Wiethe disease. It is a rare autosomal recessive disorder and characterized by the infiltration of hyaline material in the skin, oral cavity, larynx, and internal organs. Lipid proteinosis presents early in life. Hoarseness develops in infancy. The classic sign is beaded eyelid papules along the lid margin, also known as ‘Monilform Blepherosis’. In India about 30 cases have been reported to date. We report the following case because of its rarity in the Indian literature.

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Extensive cutaneous sarcoidosis and coexistant Crohn disease with dual response to infliximab: case report and review of the literature.

Sarcoidosis and Crohn disease (CD) are granulomatous disorders of unknown etiology that are rarely seen together in one patient. We describe a woman in her 40s with well-established diagnoses of pulmonary and cutaneous sarcoidosis and CD involving the terminal ileum, whose skin and gastrointestinal symptoms improved dramatically with infliximab treatment (5mg/kg on weeks 0, 2, 6, then every 8 weeks). The concurrence of sarcoidosis and CD has only been reported in a handful of cases and a review of the literature reveals that the two diseases share many clinical and immunological features, suggesting the presence of an underlying connection. Further studies of patients with overlap syndromes may provide deeper insight into the clinical spectrum, and possibly the pathogenesis, of idiopathic granulomatous diseases.

Otophyma: a rare benign clinical entity mimicking leprosy

Otophyma is a rare condition characterized by edematous deformation of the ear that is considered to be the end-stage of an inflammatory process such as rosacea and eczema. This report illustrates a case in an elderly male, originally thought to have leprosy. Biopsy revealed a nodular infiltration of inflammatory cells around adnexal structures and an intraepidermal cyst. No acid-fast organisms were identified. We present a patient who is of a different ethnic group than usually seen with this disease and provide a review of the clinical presentation, histopathological features, and management of this rare condition.

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Letter

Dermatitis medicamentosa: severe rebound erythema secondary to topical brimonidine in rosacea

Background: Rebound erythema secondary to use of topical brimonidine in the setting of rosacea is an important, possibly significantly distressing potential side effect that may be under-reported; there is little photo-documentation in the literature to date. This article documents such a case.

Observations: A 28-year-old woman (Fitzpatrick II) with a long-standing history of untreated rosacea presented for initiation of treatment of what was noted to be primarily erythematotelangiectatic rosacea and was offered Mirvaso for daily use. Initial improvement was followed by progressive worsening of baseline erythema several hours following treatment, only improved with subsequent applications of additional Mirvaso. The patient's symptoms were improved upon discontinuing use of Mirvaso.

Conclusions: There are few cases documented of rebound erythema secondary to use of Mirvaso to date. Further reporting might prompt further long term investigation for this topical medication for further delineation of its role in treatment of rosacea. What is proposed is a phenomenon similar to that of rhinitis medicamentosa with upregulation of alph-adrenergic receptors, suggesting the name "dermatitis medicamentosa" for this phenomenon.

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