DERMATOPATHOLOGY CASES: Self-Assessment Cases: Editor - Dr Sampurna Roy MD

Digital Images of interesting cases that will include the full spectrum of Dermatopathology, presented in the form of quiz.

The answer of the cases include related links and recent abstracts of articles.

Wednesday, July 28, 2010

Answer of Dermatopathology Case 50

Cutaneous Herpes Simplex Infection

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Histopathology of the more common viral skin infections.
Actas Dermosifiliogr.2010 Apr;101(3):201-16.
We describe the histopathological characteristics of viral skin infections. Herpes simplex virus and varicella-zoster virus produce an intraepidermal vesicle with variable degrees of epithelial necrosis. Typical findings include keratinocytes with ballooned nuclei with a ground-glass appearance and giant multinucleated keratinocytes. In the endothelial cells of the dermal blood vessels, cytomegalovirus produces large eosinophilic nuclear inclusions surrounded by a clear halo. Human herpes virus 8 is etiologically associated with Kaposi sarcoma. In its early stages, this tumor contains blood vessels with a fine endothelium passing through the dermal collagen bundles. In the plaque and nodular stages, the vessel lumens are more clearly visible and there is a progressive increase in the number of neoplastic spindle cells with a low degree of pleomorphism and atypia, and occasional mitoses. The infiltrate is made up of lymphocytes and plasma cells. Contagious ecthyma and milker's nodule give rise to an acanthotic epidermis with ballooned keratinocytes containing eosinophilic cytoplasmic viral inclusions. Molluscum contagiosum shows lobules of epithelium that open onto the epidermal surface and characteristic inclusion bodies. Acanthosis, papillomatosis, and hyperkeratosis are observed in common warts, with confluence of the epidermal ridges in the centre of the lesion and koilocytes.

Disseminated mucocutaneous herpes simplex virus infection in an immunocompetent woman. Int J STD AIDS.2010 Mar;21(3):213-4.
Disseminated mucocutaneous herpes simplex virus (HSV) infection in an immunocompetent person is quite rare. A 19-year-old healthy Japanese woman presented with painful, umbilicated vesicles and pustules on her genital region, both nipples and on the forearm 10 days after the last sexual contact with her partner who had cold sore at that time. Tzanck test and biopsy confirmed the diagnosis of disseminated mucocutaneous HSV infection. She did not have any visceral HSV disease. Skin lesions improved after treatment with acyclovir and erythromycin for seven days. We propose that like herpes gladiatorum, HSV dissemination in this case was acquired by close body contact.

A clinically unrecognised and persistent facial folliculitis: herpes folliculitis. Ned Tijdschr Geneeskd.2009;153:A285.
A 33-year-old woman presented with a 5-year history of a relapsing erythematous, indurated plaque on the left cheek. Herpes simplex virus (HSV) immunostain revealed the presence of HSV in the follicular and perifollicular keratinocytes. After oral treatment with valaciclovir for a period of 3 months the lesion disappeared without leaving a scar. At the last check-up, no recurrence had occurred. Herpes folliculitis has various clinical presentations. In rare cases it mimics a pseudolymphoma, as was the case for this patient. A viral aetiology, such as HSV or varicella-zoster virus, should be considered in patients with folliculitis, especially when the condition does not respond to antibacterial and antifungal therapy.

Occult herpes simplex virus colonization of bullous dermatitides. Am J Clin Dermatol.2008;9(3):163-8.
BACKGROUND: Acantholytic disorders, including pemphigus vulgaris, chronic benign familial pemphigus (Hailey-Hailey disease, superficial pemphigus), Darier disease, and Grover transient acantholytic dermatosis, as well as other vesiculo-bullous disorders, including bullous pemphigoid, epidermolysis bullosa, and atopic dermatitis, are prone to florid infections by herpes simplex virus (HSV)-I and -II, and, more rarely, by varicella-zoster virus (VZV). As these infections are difficult to recognize clinically and histologically, their frequency remains unknown. A possible occult viral colonization has never been documented in these disorders. The manner in which the primary bullous disorders are contaminated by herpesviridae remains unclear. OBJECTIVE: To retrospectively assess the possible presence of HSV and VZV in a series of biopsies of acantholytic disorders and bullous pemphigoid. METHOD: The typical alpha-herpesviridae-related cytopathic signs were searched for by conventional microscopy in skin biopsies of patients with bullous pemphigoid (n = 20), pemphigus vulgaris (n = 19), Darier disease (n = 18), chronic benign familial pemphigus (n = 3), and Grover transient acantholytic dermatosis (n = 3). Immunohistochemistry (IHC) targeted specific HSV-I, HSV-II, and VZV antigens. Polymerase chain reaction (PCR) was used for detecting HSV- and VZV-specific DNA sequences. RESULTS: No cytopathic signs suggestive of HSV or VZV infection were detected. However, IHC revealed HSV antigens in Darier disease (1/18, HSV-I), Grover transient acantholytic dermatosis (1/3, HSV-I), pemphigus vulgaris (1/19, HSV-I), and bullous pemphigoid (2/20, HSV-I and HSV-II). In these IHC-positive cases, PCR amplified specific HSV primers in Darier disease (1/18), pemphigus vulgaris (1/19), and bullous pemphigoid (1/20). VZV antigens and nucleic acids were never identified. The HSV antigens were nearly always restricted to the upper part of the granular layer and thus differed from the usual HSV distribution during cutaneous infection. Negative and positive controls yielded consistently positive and negative results, respectively. CONCLUSION: This report shows for the first time that clinically and histologically occult HSV colonization may occur in Darier disease, Grover transient acantholytic disease, pemphigus vulgaris, and bullous pemphigoid. Given the frequent use of immunosuppressive treatments for primary bullous disorders, greater awareness of HSV colonization and infection is recommended in these patients.

Bullous eruption in a patient infected with the human immunodeficiency virus.Skinmed. 2008 Mar-Apr;7(2):98-101.
A 30-year-old man diagnosed with human immunodeficiency virus (HIV) infection 10 years earlier, presented with large tense blisters associated with minimal itching of 10 days' duration. He had no history of oral or genital erosions or ulcerations and showed no symptoms of HIV-related illnesses. Highly active antiretroviral therapy (HAART) had been started 6 weeks earlier when his CD4 count was 116/mL. He initially received nevirapine 200 mg once daily; after 2 weeks with no skin eruptions or other adverse reactions, the dose was increased to 200 mg twice daily. Other components of his HAART included lamivudine and stavudine. The patient was not taking any other prescription or alternative medicines. During the past year, he experienced 4 episodes of intensely itchy urticarial lesions that subsided with antihistamines. The present episode of bullous lesions was also preceded by urticarial lesions. On examination, he had multiple, large, tense bullae over relatively normal-looking skin involving all parts of the body (Figure 1). There were a few well-defined erosions. Nikolsky and bullae spread signs were negative, and no oral or genital erosions or ulcerations were noted. Results of a complete blood count, renal and liver function tests, and chest x-ray were within normal limits. Skin biopsy from one of the blisters showed a subepidermal bulla filled with eosinophils and polymorphonuclear leukocytes (Figure 2). The underlying dermis showed perivascular inflammatory infiltrate composed of polymorphonuclear and lymphomononuclear cells. The overall features were suggestive of bullous pemphigoid. A direct immunofluorescence test could not be done because of possible risk of cross-infection to the operator of the cryostat. Workup for herpes simplex virus and cytomegalovirus infection also could not be performed. HAART was discontinued temporarily with the suspicion that it was the causative factor. The patient was started on oral prednisolone 40 mg/d and topical clobetasol propionate (0.05%). Within 1 week of treatment, he had significant improvement with almost complete disappearance of the lesions. A few small, tense vesicles continued to appear between. Once the lesions completely disappeared, the prednisolone was gradually tapered off and all the components of HAART were resumed. The patient did well without any recurrence of lesions, thus virtually excluding HAART as the cause of the bullous pemphigoid-like eruptions. Subsequently, he did not return for follow-up.

Dermatopathology Case 50


Case 50

A 30 year old immunocompromised male patient with a few painful clear vesicles on the chin.


Monday, July 26, 2010

Answer of Dermatopathology Case 49

Ziehl-Neelsen staining revealed acid-fast bacilli


Fish-Tank Granuloma
N Engl J Med 1997; 336:1065April 10, 1997

Fish tank granuloma.
BMJ. 1990 April 21; 300(6731): 1069–1070.

Fish tank granuloma: misdiagnosed as cutaneous leishmaniasis. Int J Dermatol. 2010 Jan;49(1):53-5.
Mycobacterium marinum is an atypical mycobacterium that causes a skin infection known as fish tank granuloma or swimming pool granuloma affecting people who are exposed to aquatic environments. In general, it is managed medically with antimicrobials and variable treatment protocols. Here, we report a Saudi gentleman who acquired this infection in Thailand and was misdiagnosed as cutaneous leishmaniasis. After establishing the correct diagnosis, treatment with minocycline and trimethoprim-sulfamethoxazole resulted in rapid healing.

Mycobacterium marinum infections in transplant recipients: case report and review of the literature.Transpl Infect Dis.2008 Oct;10(5):358-63.
Infections due to Mycobacterium marinum are rarely encountered following organ and tissue transplantation. Herein, we report a case of M. marinum infection in a kidney and pancreas transplant recipient who manifested clinically with multiple locally spreading sporotrichoid-like cutaneous nodules in his left forearm. In order to provide a general overview of post-transplant M. marinum infections, we reviewed and summarized all previously reported cases of this infection that occurred after transplantation. Including our index case, all 6 cases presented with multiple cutaneous and subcutaneous nodules that had spread locally in the involved extremity. One patient had lesions located in non-contiguous body sites suggesting either systemic dissemination or multiple sites of inoculation. In all but 1 patient, the cutaneous nodules appeared in an ascending pattern and following exposure to fish tanks or after contact with the marine environment. The diagnosis of M. marinum infection was suspected on clinical grounds and confirmed by mycobacterial culture. Treatment consisted of at least 2 active antibiotics (such as rifamycins, ethambutol, tetracyclines, or macrolides) for 4-9 months, resulting in clinical cure or improvement. Relapse was observed in 1 patient despite completing 6 months of antibiotic therapy. One patient had surgical excision of the lesions. In conclusion, M. marinum should be considered as the cause of cutaneous and subcutaneous nodules in transplant recipients, particularly in the context of fish tank or marine exposure. Compared with the immunocompetent hosts, M. marinum infection may have a more aggressive clinical course after transplantation, and may require a longer duration of antibiotic treatment. Early diagnosis and treatment may prevent local spread and potential systemic dissemination.

Disseminated Mycobacterium marinum infection with extensive cutaneous eruption and bacteremia in an immunocompromised patient.Eur J Dermatol.2006 Jan-Feb;16(1):79-83.
Mycobacterium marinum can cause fish tank granuloma (or swimming pool or aquarium granuloma) in immunocompetent patients. Dissemination of Mycobacterium marinum-infection is a rare condition which occurs mainly in immunocompromised patients and can be life-threatening. We report the case of an 87-year-old woman who was treated with oral corticosteroids for polymyalgia rheumatica for many years and developed erythema nodosum-like lesions on the right forearm and arthritis of the right wrist. By increasing the steroid dosage and adding methotrexate only short-term remission was achieved. Seven months later painful erythematous nodules occurred on all extremities which became necrotic, ulcerative and suppurative. Ziehl-Neelsen staining revealed acid-fast bacilli and Mycobacterium marinum was cultured from skin biopsies, blood, and urine. The critically ill patient was treated with clarithromycin and ethambutol resulting in a dramatic improvement of the general condition. After four months, doxycycline had to be added because of new skin lesions. This case illustrates the impact of Mycobacterium marinum infection in immunocompromised patients.

Fish tank granuloma--a frequently misdiagnosed infection of the upper limb. J Accid Emerg Med.1997 Nov;14(6):398-400.
Five patients attended the accident and emergency (A&E) department with fish tank granuloma caused by an infection with Mycobacterium marinum. All patients had forearm symptoms which were initially misdiagnosed. They were later recognised by the presence of superficial cutaneous lesions in a sporotrichotic distribution. Definitive diagnosis was confirmed by the histological appearances of a biopsy and or culture of the organism. All patients responded to oral minocycline and had uncomplicated recoveries once the diagnosis was established. A&E doctors need to be aware of the possible diagnosis of fish tank granuloma especially when treating forearm infections which have been resistant to antibiotics.

Dermatopathology Case 49


Case 49

A 45 year old fisherman with verrucoid nodules on the elbow and forearm.

Sunday, July 25, 2010

Answer of Dermatopathology Case 48

Immunohistochemistry: Smooth Muscle Actin


Cutaneous smooth muscle neoplasms: clinical features, histologic findings, and treatment options.J Am Acad Dermatol. 2002 Apr;46(4):477-90; quiz, 491-4.
Cutaneous smooth muscle is present in 3 separate locations: arrector pili muscles, blood vessel walls, and genital/areolar skin. Benign or malignant smooth muscle neoplasms may arise from each of these locations. This review discusses the pathogenesis, clinical manifestations, histologic findings, prognosis, treatment options, and controversial areas of cutaneous smooth muscle neoplasms. ( J Am Acad Dermatol 2002;46:477-90.) Learning objective: At the completion of this learning activity, participants should be able to discuss the pathogenesis, clinical manifestations, histologic findings, prognosis, and treatment options of cutaneous smooth muscle neoplasms.

Case for diagnosis: (unilateral multiple piloleiomyoma). An Bras Dermatol.2009 Mar-Apr;84(2):197-9.
Piloleiomyoma is a benign neoplasm arising from the erector pilorum muscle in the skin. It occurs in young adults of both genders. Lesions can be single or multiple and more frequently involve extremities. Pain may occur spontaneously or after physical stimulation. We describe a case of unilateral multiple piloleiomyoma in a young woman, complaining of itching lesions.

Bilateral segmental leiomyomas: a case report and review of the literature. Cutis.2008 Jul;82(1):33-6.
Cutaneous leiomyomas are benign tumors of smooth muscles. We report a rare case of bilateral segmental leiomyomas in an 81-year-old man. We also provide a concise review of the literature on leiomyomas, their associations, and genetic defects of multiple cutaneous and uterine leiomyomatosis (MCUL) and hereditary leiomyomatosis and renal cell cancer (HLRCC) syndromes.

Clinical and molecular genetic aspects of hereditary multiple cutaneous leiomyomatosis.Eur J Dermatol.2009 Nov-Dec;19(6):545-51. Epub 2009 Jul 10.
Multiple cutaneous and uterine leiomyomatosis syndrome (MCUL; OMIM 150800) is an autosomal dominantly inherited tumor predisposition disorder, characterized by leiomyomas of the skin and uterus. When associated with kidney cancer, this syndrome is known as hereditary leiomyomatosis and renal cell cancer (HLRCC; OMIM 605839). All disease variants result from heterozygous mutations in the fumarate hydratase (FH) gene. Cutaneous leiomyoma can easily be recognized and confirmed by histological examination. Recognition of these benign skin tumors can lead to the diagnosis of MCUL or HLRCC. Timely diagnosis is crucial for offering affected individuals and families potentially life-saving regular prophylactic screening examinations for renal tumors. Here we provide an overview of clinical and genetic features of this complex tumor syndrome and discuss patient management and current therapeutic strategies.

Cutaneous pilar leiomyoma: clinicopathologic analysis of 53 lesions in 45 patients. Am J Dermatopathol.1997 Feb;19(1):2-9.
As cutaneous pilar leiomyomas have received little attention in the recent literature, 53 lesions from 45 patients were studied to analyze their clinicopathologic features. There was an equal distribution between both sexes; most patients were adults with a wide age distribution. Both multiple (29 lesions from 21 patients) and solitary tumors (18 patients) were included. Lesions on the extremity (29 tumors) were common in both groups, whereas truncal tumors (11) were confined largely to patients with multiple lesions. In six patients the number of lesions was not specified. The tumors were painful in 17 patients. Three patients had a positive family history of similar lesions. Histologic study revealed ill-defined bundles of well-differentiated smooth muscle cells in the reticular dermis in all cases, although nine lesions had a more nodular pattern. Overlying epidermal hyperplasia was noted in 29 cases (54.7%). Immunohistochemically there appeared to be an increased number of nerve fibers within and surrounding the tumors. Mitotic activity was observed in 15 lesions (28.3%), 13 of which had <1 mitosis per 10 high power fields (HPF); the remaining two lesions had 1-2 mitoses per 10 HPF. Follow-up was available in 10 of these mitotically active tumors and ranged from 9 months to 7 years. There was no recurrence in any of them. We have concluded tentatively that leiomyomas of arrector pili origin may exhibit a low mitotic activity of <1 per 10 HPF and that this does not adversely affect the prognosis for these patients.

Dermatopathology Case 48



A 35 year old male with multiple painful , firm, reddish-brown papulonodules on the extensor surfaces of the extremities.

Answer of Dermatopathology Case 47

Porokeratosis of Mibelli

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Mibelli revisited: a case of type 2 segmental porokeratosis from 1893. J Am Acad Dermatol.2010 Jan;62(1):136-8. Epub 2009 Jul 25.

In autosomal dominant skin disorders, a pronounced mosaic involvement may sometimes be found to be superimposed on the ordinary nonsegmental lesions. Such "type 2 segmental manifestation" reflects loss of heterozygosity occurring at an early developmental stage, giving rise to a cell clone that lacks the corresponding wild-type allele. Here, this genetic concept is applied to an unusual case of plaque-type porokeratosis of Mibelli (PM) as published in 1893 by Vittorio Mibelli in the International Atlas of Rare Skin Diseases. The right forearm and hand of the 21-year-old patient showed a pronounced linear porokeratosis that had developed since the age of 2 years. Moreover, nonsegmental lesions of PM involved both hands and forearms as well as the face and the neck, having first been noticed at the age of 7 years. Two siblings and the father were likewise affected with PM. Hence, Mibelli's case from 1893 meets all of the criteria of a type 2 segmental manifestation of an autosomal dominant skin disorder. Recognizing such cases of superimposed segmental involvement may help elucidate the molecular basis of PM.

Hyperkeratotic variant of porokeratosis Mibelli with dermal amyloid deposits. J Dermatol.2010 May;37(5):475-9.

We report a case of hyperkeratotic variant of porokeratosis Mibelli with dermal amyloid deposits. A 66-year-old man presented with multiple brownish keratotic lesions on the lower extremities, a verrucous nodule on the third toe of the left foot and brownish verrucous plaques on the buttocks for several years. Histopathological examination of the hyperkeratotic plaque in the right gluteal region revealed extreme hyperkeratosis and cornoid lamella. In the papillary dermis, there were prominent eosinophilic amorphous materials which were positive to Dylon staining. Treatment with oral etretinate resulted in a remission of the skin lesions in this case.

Porokeratosis Mibelli gigantea: case report and literature review. Hautarzt. 1999; 50(6):435-8.

Porokeratos of Mibelli is a rare inherited disorder of epidermal keratinization, whose pathogenesis is not fully understood. The common clinical feature is a erythematous plaque surrounded by a hyperkeratotic border. The histopathologic hallmark is a parakeratotic cornoid lamella. An unusual case of porokeratosis gigantea, a morphological variant of classical porokeratosis of Mibelli, is reported. The pathogenesis, taking in account especially the clonal hypothesis and premalignant nature of porokeratosis, is discussed.

Porokeratosis of Mibelli. Overview and review of the literature.Acta Derm Venereol.1997 May;77(3):207-13.

Porokeratosis of Mibelli is an uncommon dermatosis, which may be associated with immunosuppression and may undergo malignant transformation. Due to the wide range of clinical presentations, numerous classifications have evolved, resulting in some confusion. This article examines the classification and presentation of porokeratosis and, in particular, reviews the association with immunosuppression.

Dermatopathology Case 47

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Case 47

A 7 year old girl, with a skin lesion (oval plaque with an atropic center and a thin elevated keratotic rim), on the left forearm.


Saturday, July 24, 2010

Answer of Dermatopathology Case 46

Hailey-Hailey Disease

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Familial benign chronic pemphigus (Hailey-Hailey disease) Dermatol Online J. 2009 Aug 15;15(8):15.

A case of eczema herpeticum with hailey-hailey disease. Ann Dermatol. 2009 Aug;21(3):311-4.

Reevaluation of the normal epidermal calcium gradient, and analysis of calcium levels and ATP receptors in Hailey-Hailey and Darier epidermis. J Invest Dermatol. 2009 Jun;129(6):1379-87. Epub 2008 Dec 4.

Electron probe microanalysis was used to analyze elemental content of human epidermis. The results revealed that the calcium content of the basal keratinocyte layer was higher than that of the lowest spinous cell layer in normal epidermis. This was surprising, as it is generally accepted that the calcium level increases with cellular differentiation from the proliferative basal layer to the stratum corneum. Hailey-Hailey disease (HHD) and Darier disease (DD) are caused by mutations in Ca(2+)-ATPases with the end result of desmosomal disruption and suprabasal acantholysis. The results demonstrated three major aberrations in HHD and DD lesions. First, in HHD and DD lesions the calcium content in the basal layer was lower than in the normal skin. Second, adenosine triphosphate (ATP) receptor P2Y2 was not localized to plasma membrane in acantholytic cells, whereas P2X7 appeared in the plasma membrane, potentially mediating apoptosis. Third, transition of keratin 14 to keratin 10 was abnormal as demonstrated by the presence of keratinocytes expressing both cytokeratins, which are usually exclusive in normal epidermis. Our results provide to our knowledge previously unreported elements for understanding how the disturbed calcium gradient is linked to the alterations in ATP receptors and keratin expression, leading to the clinical findings in HHD and DD.

A Case of Hailey-Hailey Disease in an Infant with a New ATP2C1 Gene Mutation. Pediatr Dermatol.2010 Apr 9.

Familial benign chronic pemphigus or Hailey-Hailey disease (OMIM 169600) is an autosomal-dominant blistering disease. Here we present a rare case of familial benign chronic pemphigus in a Chinese infant. The 5-month-old proband, who showed diffusely distributed skin lesions, is the youngest patient of Hailey-Hailey disease ever reported. The detection of an ATP2C1 gene mutation in this infant confirmed the diagnosis. His mother carried the same mutation, but with no history of skin lesions.

Heterogeneous mutations of the ATP2C1 gene causing Hailey-Hailey disease in Hong Kong Chinese.J Eur Acad Dermatol Venereol. 2010 Mar 4.

Abstract Background Hailey-Hailey disease (HHD) is a rare autosomal dominant dermatosis. It causes suprabasilar acantholysis leading to vesicular and crusted erosions affecting the flexures. Mutation of ATP2C1 gene encoding the human secretory pathway Ca(2+)/Mn(2+)-ATPase (hSPCA1) was identified to be the cause of this entity. Objective The aim of this study was to study the mutational profile of the ATP2C1 gene in Hong Kong Chinese patients with HHD. Methods Patients with the clinical diagnosis of HHD proven by skin biopsy were included in this study. Mutation analysis was performed in 17 Hong Kong Chinese patients with HHD. Results Ten mutations in the ATP2C1 gene were found. Six of these were novel mutations. The novel mutations included a donor splice site mutation (IVS22+1G>A); a missense mutation (c.1049A>T); two deletion mutations (c.185_188delAGTT and c.923_925delAAG); an acceptor splice site mutation (IVS21-1G>C) and an insertion mutation (c.2454dupT). Conclusion The six novel mutations provide additions to the HHD mutation database. No hot-spot mutation was found and high allelic heterogeneity was demonstrated in the Hong Kong Chinese patients.

Dermatopathology Case 46




Case 46

A well demarcated bullous lesion in the axilla of a 48 year old female.

Saturday, July 17, 2010

Answer of Dermatopathology Case 45

Lymphomatoid Papulosis

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Primary cutaneous CD30+ lymphoproliferative disorders. Actas Dermosifiliogr.2010 Mar;101(2):119-28.
CD30+ lymphoproliferative disorders are the most common group of cutaneous T-cell lymphomas after mycosis fungoides and its subtypes. This group includes lymphomatoid papulosis and CD30+ anaplastic large-cell lymphoma; these 2 entities are the extremes of a spectrum with numerous intermediate varieties in which it is not possible to establish a clear diagnosis based on clinical and histopathologic criteria. CD30+ lymphoproliferative disorders must be differentiated from other lymphoproliferative diseases with CD30+ cells in the tumor infiltrates, such as mycosis fungoides or Hodgkin disease, and also from other inflammatory conditions or nonhematological neoplasms that can include this cell type, such as pityriasis lichenoides et varioliformis acuta or certain mesenchymal tumors (CD30+ pseudolymphomas). In contrast to their systemic homologues, which arise in the lymph nodes, CD30+ lymphoproliferative disorders generally have a good prognosis. It is very important to exclude the presence of a lymphoma of systemic origin with extralymphatic spread, as the prognosis and treatment are different.

Clinical, histopathologic, and immunophenotypic features of lymphomatoid papulosis with CD8 predominance in 14 pediatric patients.J Am Acad Dermatol.2009 Dec;61(6):993-1000. Epub 2009 Jul 3.
BACKGROUND: Lymphomatoid papulosis (LyP) is a cyclic papulonodular eruption that is clinically benign and histologically malignant. Association with hematologic neoplasias has been reported in 5% to 20% of all cases. OBJECTIVE: We sought to review the clinical and histopathologic features of LyP in pediatric patients. METHODS: We searched for the records of all patients with a clinical and histopathologic diagnosis of LyP seen at our clinic from January 1991 through April 2008. The cases of pediatric patients (aged < 20 years) were reviewed in detail. RESULTS: Of 123 patients with LyP identified, 14 (11%) were in the pediatric age group. Most were male (64%); mean age of onset was 12 years. Type A LyP was identified in 12 patients, one patient had type B, and none had type C (type not determined in one case). Ten cases showed CD8 predominance by immunohistochemistry. T-cell intracytoplasmic antigen staining was positive in 3 cases of CD8(+) LyP type A and the one case of LyP type B. Lesional T-cell receptor gene rearrangement studies were negative in 9 of 10 patients with LyP type A. The average follow-up time was 5.5 years. Lesions improved with treatment in most cases, and none of the cases were associated with hematologic malignancies. LIMITATIONS: This was a retrospective review. CONCLUSIONS: Among our pediatric patients, we noted a predominance of CD8(+) LyP, which does not seem to have an aggressive course. Further longitudinal studies are necessary to evaluate prognostic differences between CD4(+) and CD8(+) LyP and their biological significance.

Dermatopathology Case 45

Image4 (Immunohistochemistry: CD30)

Case 45

A 40 year old woman with recurrent crops of nodules on the left upper limb. The lesion spontaneously subsides after a few weeks. The clinical course has been extending over last 6 years.

Friday, July 16, 2010

Answer of Dermatopathology Case 44

Necrobiotic Xanthogranuloma

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Non-infectious granulomatous diseases of the skin and their associated systemic diseases: an evidence-based update to important clinical questions. Am J Clin Dermatol. 2010;11(3):171-81.
Non-infectious granulomatous diseases of the skin are a broad group of distinct reactive inflammatory conditions that share important similarities. As a group, they are relatively difficult to diagnose and distinguish both clinically as well as histologically. Many of these disorders have significant associations with systemic diseases that impact the patient's overall prognosis. In this update, we offer a discussion of emerging concepts and controversies in this field, as presented through evidence-based answers to seven important clinical questions regarding palisading and epithelioid granulomata. These questions offer an opportunity to review ten non-infectious granulomatous conditions that have implications for systemic disease: granuloma annulare, annular elastolytic giant cell granuloma, necrobiosis lipoidica,methotrexate-induced accelerated rheumatoid nodulosis, necrobiotic xanthogranuloma, interstitial granulomatous dermatitis, interstitial granulomatous drug reaction, palisaded neutrophilic granulomatous dermatitis, sarcoidosis, and metastatic Crohn disease. Recent clinical, epidemiologic, and laboratory studies have shed some light on these diseases, the association of these conditions with systemic disorders, and their overall prognoses.

Necrobiotic xanthogranuloma: a review of 17 cases with emphasis on clinical and pathologic correlation.Arch Dermatol. 2009 Mar;145(3):279-84.
OBJECTIVE: To identify correlations between clinical presentation, specific histopathologic findings, and subsequent disease course in patients with necrobiotic xanthogranuloma (NXG). DESIGN: Retrospective review of medical records and histopathologic examination of fixed tissue samples. SETTING: Tertiary care medical center. PATIENTS: Seventeen patients with a diagnosis of NXG established between January 1, 1994, and December 31, 2007. MAIN OUTCOME MEASURES: Description and distribution of clinical lesions, presence of monoclonal gammopathy, multiple myeloma, and correlation with microscopic patterns of skin lesions. RESULTS: Eleven patients (65%) showed involvement of the periorbital area, and the trunk was affected in 8 patients (47%). Twelve patients (71%) had a monoclonal gammopathy; of these, 3 (18%) had multiple myeloma. Histopathologic examination of 12 patients showed findings consistent with NXG, including a bandlike pattern of necrobiotic granulomatous inflammation, atypical giant cells, cholesterol clefts, and plasma cells. No correlations were identified between clinical presentation and specific histopathologic findings. Although most patients had a serum monoclonal gammopathy, staining with antibodies to CD3, CD20, kappa light chains, and lambda light chains showed polytypic lymphocytes and plasma cells in all cases. CONCLUSIONS: The association between NXG and paraproteinemia is well documented and corroborated by this study. However, the skin lesions in NXG represent reactive inflammation and are not associated with the presence of monoclonal plasma cells or multiple myeloma.

Necrobiotic xanthogranuloma with paraproteinemia; an atypical case.J Dtsch Dermatol Ges. 2008 Jan;6(1):40-3. Epub 2007 Oct 18.
Necrobiotic xanthogranuloma (NXG) is a rare marker for paraproteinemia. An 86-year-old woman had a one year history of large red-yellow to brown annular plaques involving all limbs. Biopsies showed a non-palisading granuloma with numerous multinucleated giant cells showing prominent elastophagocytosis and extensive areas of necrobiosis throughout the entire dermis. Complete loss of elastic fibers was observed in the central atrophic area of an annular plaque. Small vascular thromboses were also present. Laboratory findings revealed paraproteinemia of IgG-lambda type. Immunohistochemical staining detected the presence of roughly equal numbers of IgG-lambda-and IgG-kappa-staining plasma cells in the dermis. We diagnosed NXG with paraproteinemia with monoclonal gammopathy (IgG-lambda type) of unknown significance.

Dermatopathology Case 44

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Case 44

A 65 year old female with a nodule on the face.