Mid Dermis Research Articles

Reparative Perineural Hyperplasia: A Series of 10 Cases

Healing wounds are commonly examined by pathologists at the time of reexcision of skin tumors to ensure complete removal of the lesion. In addition to searching for residual tumor, possible perineural invasion must be assessed. During routine examination of reexcision specimens, 10 cases of prominent perineural proliferation were seen associated with fine nerves in the mid or deep dermis. The process showed a concentric cellular proliferation with no, or only limited, nuclear hyperchromasia or pleomorphism. In a number of cases, immunohistochemistry was essential to exclude the possibility of malignant perineural invasion or other mimics of this process such as reexcision perineural invasion. The term reparative perineural hyperplasia is proposed for this entity, which is important for pathologists to be aware of to avoid misdiagnosis.

ARNDT gottron scleromyxedema: Successful response to treatment with steroid minipulse and methotrexate

Sir, Scleromyxedema is a rare disorder of unknown cause, with fewer than 150 reported cases in literature. It usually affects middle-aged adults of 30–50 years and is clinically characterized by a widespread symmetric eruption of 2–3 mm firm, waxy, dome-shaped papules, commonly over the hands, forearms, head and neck region, upper trunk, and thighs. The characteristic is the arrangement of papules in a striking linear array with the surrounding skin being sclerodermoid.[1] We report a case of scleromyxedema who considerably improved with a trial of steroid oral minipulse and methotrexate. A 43-year-old male farmer presented with a skin colored eruption on his body of 6 months duration. Initially, the lesions began as grouped skin-colored papules on his hands associated with mild pruritus and subsequently spread to the face, neck, shoulders, upper extremities, and the abdomen. There was no history of any systemic symptoms and his past medical and family history was noncontributory. On examination, facial and ear lobe infiltration was noted with prominent forehead creases. The skin of the forehead, neck, upper trunk, and arms was bound down and exhibited numerous 1–2 mm grouped skin colored papules [Figures 1 and 2]. Our patient was thoroughly investigated and results of his complete blood cell count, urinalysis, liver and renal parameters, thyroid profile, ECG, chest X-ray, ultrasound abdomen, and sugars were normal. ELISA for HIV was negative. Immunoglobulin profile showed elevated serum IgG 1797 (mg/dl) (normal 1200–1480 mg/dl), whereas IgA, IgM were normal. Biopsy features from the papule on the forearm showed features that are characteristic of scleromyxedema with special stains demonstrating mucin in the dermis. Figure 1 Infiltrated forehead skin with papules in a linear array Figure 2 Skin-colored papules on the neck and back In the literature, the terms lichen myxedematosus(LM), papular mucinosis, and scleromyxedema have often been used indiscriminately as synonyms, but most reported cases of LM without indication of the subtype appear infact to be cases of scleromyxedema. Actually, LM includes three distinct clinicopathologic subsets: A generalized papular and sclerodermoid form called as scleromyxedema with systemic even lethal manifestations, a localized papular form that does not run a disabling course and an atypical or intermediate form (not meeting the criteria for either scleromyxedema or the localised papular forms).[2] Patients of scleromyxedema may have a number of extracutaneous manifestations pertaining to the cardiovascular, pulmonary, gastrointestinal, rheumatologic, and central nervous systems. A paraproteinemia typically an IgGλ is observed in more than 80% patients. Microscopically, scleromyxedema is characterized by a triad of a diffuse deposit of mucin in the upper and mid reticular dermis, an increase in collagen deposition, and a marked proliferation of irregular arranged fibroblasts. The exact pathophysiologic mechanism that triggers excess mucin production by fibroblasts in scleromyxedema is unclear. Although this can be attributed to paraproteins according to previous studies, all patients of papular mucinosis do not have an identifiable paraprotein, which suggest the role of other cytokines as proliferative signals.[3] The treatment of scleromyxedema remains a therapeutic challenge to the treating physician and despite anecdotal reports of success with various agents, no satisfactory treatments are currently available.[4] Our patient was diagnosed as scleromyxedema clinically on the basis of a generalized papular and sclerodermoid eruption and histologically by the presence of mucin deposition fibrosis and fibroblast proliferation with a normal thyroid function. Extensive screening did not reveal any internal malignancy and although our patient had increased gamma globulin levels, we could not investigate this further due to financial constraints to support our diagnosis further. He was commenced on minipulse therapy with oral betamethasone six tablets to be taken twice weekly with weekly methotrexate 10 mgs. After 3 months of regular treatment, patient reported 75% reduction in the cutaneous induration. There were no other systemic complaints. Patient is currently on regular treatment and is doing well.

A case of mid-dermal elastolysis after narrowband ultraviolet B phototherapy

Conflict of interest: none declared. Mid‐dermal elastolysis (MDE) is a rare disorder first described in 1977, which usually presents clinically with circumscribed areas of fine skin wrinkling and histologically with a band of elastin loss in the mid dermis.1 The pathogenesis of MDE is unknown but several theories have been proposed, including actinic damage to elastin fibres.2 We report a 32‐year‐old woman who developed mid‐dermal elastolysis after receiving narrowband ultraviolet (UV) B phototherapy for a first episode of guttate psoriasis. She had no preceding history of photosensitivity and had Fitzpatrick type II skin. She reported previous use of sun beds: 10 sessions over a period of 1 year, which took place 2 years before presentation. She had no other medical problems and was not taking any medication. She had not taken any recent holiday in a sunny climate. The patient had received 25 UVB treatments, with a cumulative dose of 14.37 J/cm2, resulting in almost complete remission of the psoriasis. Apart from mild facial erythema there were no other episodes of erythema or burning during the course. Four weeks after completion of the course, she presented with an asymptomatic arcuate line of erythema across the shoulders and chest. Fine wrinkling was evident within this line of erythema (Fig. 1).

Dapsone-responsive histiocytoid Sweet's syndrome associated with Crohn's disease

To the Editor: Histologically, Sweet's syndrome exhibits features of a neutrophilic dermatosis with papillary dermal edema and a dense infiltrate of neutrophils in the papillary and mid dermis.1 Several conditions have been associated with the development of Sweet's syndrome: infections, hematologic and visceral malignancies, inflammatory bowel disease, pregnancy, and certain medications.2

Pseudoxanthoma elasticum in a patient with sickle cell disease: case report

An 18 year female sickler (HbSS) presented with repeated history of epistaxis and bleeding gums. Features consistent with pseudoxanthoma elasticum were observed, such as hyper-extensile redundant skin folds in the neck, axilla, inguinal areas and abdomen. The skin biopsy showed swollen, clumped and fragmented elastic fibres and calcium deposits in the deep and mid reticular dermis, consistent with pseudoxanthoma elasticum. This is a well recognised complication of sickle cell disease which has not been described in Kenya.

Red-brown macules on the abdomen

Conflict of interest: none declared. A healthy 63‐year‐old man presented with progressive impairment of vision. Fundoscopy examination revealed angioid streaks and neovascularization of the retina in the right eye. No visible skin lesions in the neck, axillae, groin and oral mucosa were found. The patient had a 30‐year history of a red‐brown rash on the upper abdomen, which consisted of small round red‐brown macules in a weak reticular arrangement (Fig. 1). There was no history of cardiovascular disease. ... Skin from a macule showed a band of eosinophilic granular material mainly at the border of the mid and lower dermis (Fig. 2a). Elastic van Gieson stain showed fragmentation and clumping of elastic fibres in the mid and lower dermis (Fig. 2b), where calcification of the degenerated fibrous components was revealed by von Kossa stain. There was some surrounding inflammation in the form of a few foreign‐body giant cells scattered in the dermis. Anti‐elastin antibody stain confirmed the degenerated matrix components contained elastic fibres. A skin biopsy from the neck did not show any abnormalities.

An erythematous nodule on the eyelid

Conflict of interest: none declared. A 43‐year‐old white woman with autism presented with a 5‐month history of a slow‐growing asymptomatic lesion on the left lower eyelid. Her mother thought it might have arisen following a bee sting. There was no other medical history of note and the patient was otherwise well. On examination, an indurated erythematous nodule measuring 15 mm with oedema was found on the left lower eyelid (Fig. 1). A punch biopsy was performed. ... Haematoxylin and eosin stained sections revealed a dermal tumour, showing a solid growth pattern within the mid and deep dermis, composed of epithelioid and spindle‐shaped cells (Fig. 2 and 3a). The cells contained eosinophilic cytoplasm and prominent nucleoli. Within the superficial dermis, numerous irregular anastomosing vascular spaces dissecting collagen bundles were noted. These vascular spaces were lined by highly atypical endothelial cells with focal multilayering (Fig. 3b). Mitoses were present. Necrosis was not seen. The overlying epidermis was uninvolved. On immunohistochemistry, the tumour cells were strongly positive for CD31 (Fig. 3c) and CD34.

Pseudomycetoma caused by Microsporum canis in an immunosuppressed patient: a case report and review of the literature

A 65-year-old woman with a history of liver and kidney transplantation presented with a pruritic and tender rash of 2-year duration on her left knee. The patient had a physical examination, and multiple skin biopsies were performed. Examination showed a cluster of dome-shaped, violaceous papules. A skin biopsy specimen showed multiple granules containing basophilic material within the superficial to mid dermis. The grains were embedded in an eosinophilic substance and surrounded by granulomatous inflammation. A Gomori methenamine silver stain showed abundant septate hyphae, and cultures grew colonies of Microsporum canis. A diagnosis of dermatophytic granuloma, or pseudomycetoma, was made. After considering the patient's immune status, a prolonged course of fluconazole was initiated. Microsporum canis, a frequently encountered zoophilic dermatophyte associated with tinea capitis and tinea corporis, is rarely associated with a mycetoma-like presentation, grain formation, or systemic infection. The medical literature describes only six cases of M. canis causing mycetoma-like formations in immunocompromised and immunocompetent patients. This case report describes an unusual presentation of a common dermatophyte infection, and the literature review summarizes the importance of recognizing atypical presentations of dermatophyte infections. Such infections may warrant more aggressive treatment in immunocompromised patients.

Granuloma Faciale With Extrafacial Lesions

A 35-year-old man presented with a 7-year history of gradually enlarging plaques on his face and trunk. The first lesions had developed on both sides of the forehead and the left cheekbone (Figure 1). Four years later similar lesions appeared on his neck and back. He presented a histologic report of a biopsy specimen from a facial plaque performed 5 years earlier that was diagnostic for granuloma faciale. He had different treatments such as topical steroids and cryotherapy without improvement. The appearance of new lesions on his trunk and the gradual enlarging of the old lesions convinced the patient to seek further treatment. Physical examination revealed dusky, violaceous plaques and papules, 0.5 to 2 cm, well-circumscribed, slightly elevated, and located on the face and trunk, with mild pruritus (Figure 1 and Figure 2). Laboratory investigations, including complete blood cell count, VDRL test, antinuclear antibody test, biochemical parameters, and chest x-ray, did not reveal any abnormalities. A skin biopsy taken from the upper part of the back showed similar features to the facial lesion, detected 5 years before, revealing a dense, polymorphous infiltrate involving mid and deep dermis and displaying a diffuse and perivascular pattern (Figure 3A). A narrow grenz zone of normal collagen was consistently observed between dermal infiltrate and epidermis as well as around the pilosebaceous follicles (Figure 3A). The infiltrate mainly consisted of eosinophils and lymphocytes, but neutrophils (often displaying leukocytoclasis), macrophages, and plasma cells were also present (Figures 3B, 3C). Some mast cells were also identified by staining with toluidine blue (Figure 3D). Perivascular infiltrates were often seen, sometimes penetrating vessel walls and in association with leukocytoclasis. Hyalinization of vessel walls, extravasation of red blood cells around capillaries, and nuclear dust were also noted. The epidermis did not show any remarkable change except for slight acanthosis. A diagnosis of granuloma faciale with extrafacial lesions was made, and a systemic therapy with hydroxychloroquine (200 mg twice daily for 6 weeks) was recommended.(1,2).

A case of flagellation

Conflict of interest: none declared A 58‐year‐old man presented with a 3‐day history of a striking flagellate dermatitis on his thighs and trunk (Fig. 1a,b), which were intensely pruritic. There was no dermographism. He had taken no new medication but had been on antiretroviral therapy for human immunodeficiency virus for many years. There were no other symptoms. Full blood count, including eosinophil count, and measurement of serum creatine kinase were normal. The previous night he had eaten a Chinese meal. ... A skin biopsy was performed (Fig. 2). He was treated with antihistamines and potent topical corticosteroids. The dermatitis settled within 2 weeks, leaving no pigmentary change. ... Histological sections showed mild to moderate spongiosis with focal hyperkeratosis, mid‐parakeratosis and minimal exocytosis of lymphocytes. In the superficial and mid dermis there was a mild, perivascular, lymphohistiocytic inflammatory cell infiltrate. What is your diagnosis? Flagellate dermatitis due to shiitake mushroom ingestion.

Pleomorphic CD8+ Small/Medium Size Cutaneous T-Cell Lymphoma

Pleomorphic small/medium-sized cutaneous T-cell lymphoma is a recently recognized rare type of cutaneous T-cell lymphoma which is clinicopathologically different from mycosis fungoides and Sezary syndrome. By definition the phenotype of the neoplastic lymphocytes in pleomorphic small/medium-sized cutaneous CD3CD4CD8 but CD8 pleomorphic small/medium sized cutaneous T-cell lymphoma cases have been occasionally described. We describe a 55-year-old female with a pruritic erythematous nodule on the lateral aspect of her right foot present for 1.5 years. Histology revealed a nonepidermotropic lichenoid infiltrate in the papillary dermis and a patchy infiltrate in the mid and lower dermis composed of small to medium-sized pleomorphic lymphocytes. The immunophenotype of these lymphocytes was CD3CD4CD8TIA-1. Staining for CD20, CD30, CD56, TdT, and LMP1 were negative, and the Ki-67 proliferation index was 5% to 10%. Gene rearrangement studies demonstrated a T-cell clone. The laboratory and imaging workup did not reveal extracutaneous involvement. The lesion was treated by local irradiation but a follow-up biopsy demonstrated only partial remission. Consequently, the lesion was treated by surgical excision.

Generalized granuloma annulare associated with chronic hepatitis B virus infection

We describe a 63-year-old Chinese woman with generalized granuloma annulare (GGA) associated with chronic hepatitis B virus (HBV) infection for 10 years. She developed non-annular papule lesions after a previous infection of hepatitis B virus. Since then, her clinical course was aggressive with involvement of the trunk, extremities and neck. Histologically, granulomatous inflammations were found in the upper to mid dermis. The HBV DNA in these lesions was demonstrated by polymerase chain reaction (PCR). GGA was totally regressed after treatment of interferon-alpha for 3 months. To the best of our knowledge, GGA associated with chronic HBV infection has never been reported in literature. The prognostic significance of the association of granulomatous inflammation and virus infection is reviewed.

CD56brightCD16– NK cells accumulate in psoriatic skin in response to CXCL10 and CCL5 and exacerbate skin inflammation

Psoriasis is an immune-mediated skin disease characterized by lymphocytic infiltration and altered keratinocyte differentiation. Using immunohistochemical techniques we found that the cellular infiltrate in acute psoriatic plaques includes 5-8% CD3(-)CD56(+) natural killer (NK) cells, mostly localized in the mid and papillary dermis. NK lymphocytes isolated from punch biopsy specimens of psoriatic plaques showed a CD56(bright)CD16(-)CD158b(-) phenotype, failed to express the skin homing cutaneous lymphocyte-associated antigen and released abundant IFN-gamma upon stimulation. Supernatants from psoriatic NK cells induced MHC class II and ICAM-1 expression and release of CXCL10 and CCL5 by cultured psoriatic keratinocytes. Skin NK cells expressed high levels of the chemokines receptors CXCR3 and CCR5, intermediate amounts of CXCR1, CCR6 and CCR8, and low levels of CCR1, CCR2, CCR4, CCR7 and CX3CR1. In addition, they promptly migrated in vitro toward CXCL10, CCL5, supernatants of IFN-gamma-activated psoriatic keratinocytes and, to a lower extent, CCL20 and CCL4. In contrast, they failed to migrate toward CXCL8, CCL1, CCL2, CCL3, CCL17, CCL19 and CX3CL1. Taken together, our results implicate NK lymphocytes as newly identified protagonists in the pathogenesis of psoriasis. Their distinctive homing properties should be taken into account in the design of specific therapy aimed at blocking pathogenic cell accumulation in the skin.

Alterations of basement membrane zone in autoimmune subepidermal bullous diseases

Pemphigoid (BP), mucous membrane pemphigoid (MMP) and epidermolysis bullosa acquisita (EBA) are characterized by the presence of in vivo bound and circulating anti-basement membrane zone (BMZ) antibodies directed against different antigens localized in the upper part of the lamina lucida, lamina lucida-lamina densa border and in sublamina densa region, respectively. The alterations of BMZ lead to blister formation. The aim of the present study was to compare the alterations of various regions of the BMZ in BP, MMP and EBA using laser scanning confocal microscopy (LSCM). Biopsy specimens taken from perilesional patients' skin were cut into 40mum thick slides, followed by double immunofluorescence labeling with antibodies against different BMZ structures and anti-human IgG antibody. Three-dimensional reconstruction (3DR) of various regions of BMZ and of in vivo bound IgG was performed by computer program integrated with LSCM. In BP and MMP, LSCM studies revealed numerous invaginations of BMZ, most pronounced at the level of lamina lucida. Integrity of BMZ was preserved at the level of lamina lucida, lamina densa and sublamina densa. In EBA, continuity of lamina lucida was preserved and numerous invaginations were present. More pronounced invaginations of BMZ were observed at the level of lamina densa, whereas at the level of the sublamina densa region the staining along BMZ was discontinuous. Moreover, large clumps composed of collagen VII vertically oriented to the dermal-epidermal junction extending into the mid dermis were observed. The study showed that alterations of BMZ in BP and MMP occur mainly in the lamina lucida, and in EBA in the sublamina densa region. These alterations in various regions of BMZ in BP, MMP and EBA could be responsible for the differences in the level of blister formation and in the clinical course of the diseases.

Three-dimensional imaging reveals major changes in skin microvasculature in lipoid proteinosis and lichen sclerosus

Lipoid proteinosis is a rare autosomal recessive disorder characterized by deposition of hyaline-like material in several organs, including skin. Pathogenic mutations have been found in the extracellular matrix protein 1 gene (ECM1). Recent studies have disclosed that ECM1 is also a target antigen for autoantibodies in patients with the acquired disease, lichen sclerosus. Both conditions have been reported to show abnormalities in dermal blood vessels but these changes have not been fully assessed. The purpose of this study was to investigate the architecture of the cutaneous microvasculature in lipoid proteinosis and lichen sclerosus to better determine the role of ECM1 in the skin pathology observed in these disorders. Labeling of skin biopsies (lipoid proteinosis, lichen sclerosus and control skin) with antibodies to type IV collagen and laminin-1 and reconstruction of the dermal blood vessels using laser confocal microscopy and computer imaging. In both lipoid proteinosis and lichen sclerosus there was reduplication of the basement membranes surrounding blood vessel walls. There were enlarged vessels in the mid and deep dermis that were orientated parallel to the dermal-epidermal junction. In addition, the normal capillary loop network in the dermal papillae, as well as the subcutaneous plexus and transverse connecting vessels were lacking in both disorders. This study demonstrates that skin microvasculature is grossly altered when ECM1 is targeted by inherited mutations (lipoid proteinosis) or acquired autoantibodies (lichen sclerosus) and that this glycoprotein appears to have an important role in regulating blood vessel physiology and anatomy in the skin.

Cutaneous Leishmaniasis in an American Sol‐dier Who Returned from Iraq – A Case Report

A 27‐year‐old male soldier who was stationed in Iraq from 3/03 to 9/03 presented to a local dermatologist in 4/04 with several red slightly raised non‐ulcerated nodules over the right wrist and ventral arm developed 2–3 months prior to the visit. The clinical impression was sporotrichosis. A punch biopsy was performed on a subcutaneous nodule and showed a granulomatous inflammatory process with scattered loosely organized clusters of epithelioid histiocytes palisading around dermal mucin, mainly involving the papillary and mid dermis. A diagnosis of granuloma annulare was suggested. Intralesional triamcinolone was used and the lesions initially improved but later recurred. However on deeper sections, there were scattered granulomas with a dominant necrotizing granuloma consisting of epithelioid histiocytes palisading around a central area of necrosis surrounded by lymphoplasmacytic and neutrophilic infiltrates. In 5/04, he presented to the Birmingham VA Medical Center with similar lesions. A repeat biopsy again showed a necrotizing granulomatous inflammation. In addition, numerous intracytoplasmic organisms with kinetoplasts were present within histiocytes. The findings were consistent with leishmaniasis due to L. major as confirmed by polymerase chain reaction at Walter Reed Hospital. As more US soldiers return from the Middle East, previously rare cutaneous leishmaniasis will be seen more commonly.

Evolution of histoid leprosy (de novo) in lepromatous (multibacillary) leprosy

A 26‐year‐old man presented with persistent redness of the face over the past 2 years and thickening of the ears for a year. The current state was preceded by three to four episodes of epistaxis, 2–3 months previously. The patient had not received any treatment. Cutaneous examination revealed indurated (infiltrated) plaques on the face and ears over an apparently normal‐looking skin, and numerous, small, ill‐defined, slightly hypopigmented, shiny macules all over the body. They were bilateral and symmetric (Fig. 1a,b). There was no variation in the cutaneous sensations of temperature, touch, and pain. The patient showed loss of the lateral eyebrows and conjunctival congestion. Examination of the nerves revealed enlargement of the ulnar, radial, posterior tibial, and right common peroneal nerves; however, there was no tenderness of the nerves. Systemic examination was within normal limits. Examination of a slit‐skin smear (under oil immersion), prepared from a representative lesion (plaque), demonstrated an abundance of solid and uniform‐staining acid‐fast bacilli, occurring either singly or in parallel clumps/globii, in an average field (6+). Furthermore, a scraping mount (10% KOH) prepared from the lesion on the back was negative.(a, b) Histoid leprosyimageHematoxylin and eosin‐stained sections prepared from a biopsy taken from a plaque revealed a conspicuous granuloma composed of peculiar spindle‐shaped histiocytes. Several of the granulomas were present in the mid and lower dermis. They were characterized by whorled, criss‐cross, or parallel patterns. Solid and uniform‐staining, slender, rod‐like (length three times that of the breadth) acid‐fast bacilli were found scattered throughout the section. A few histiocytes closely packed with acid‐fast bacilli, together with lymphocytic infiltrates, were also seen. There was a prominent eosinophilic stained clear zone just below the epidermis. It was free from acid‐fast bacilli as well as the inflammatory infiltrate (Fig. 2a,b). A definitive diagnosis of untreated lepromatous leprosy (LL) changing to histoid leprosy (de novo) was made.(a, b) Histoid leprosy depicting granuloma formed by histiocytes displaying whorl‐wind, criss‐cross or interlacing pattern, and a clear zone beneath the epidermis (H&E ×40) Solid and uniform staining acid fast bacilli, – slender, rod‐like, length 3 times that of breadth found scattered throughout the granuloma (H&E ×100)image

Azelaic acid as a new treatment for perioral dermatitis: results from an open study

Sir, Until recently, the treatment of perioral (rosacea‐like) dermatitis has been considered to be difficult and frustrating.1 Many different substances have been employed, alone or in combination, locally or systemically. The choice has usually been occasioned by the physician's personal preferences, as the aetiology of the condition is still unknown. The mainstay of most treatment schemes has been oral tetracycline, which is effective in many cases but may be associated with adverse effects such as nausea, vomiting and phototoxicity. Topical treatments including antibiotics and metronidazole have been found useful but are not tolerated by many patients because of the unusually high skin sensitivity that often accompanies perioral dermatitis.2–4 The objective of this open clinical study was to investigate the efficacy and tolerability of a formulation containing 20% azelaic acid in 10 patients (nine women and one man; age range 32–65 years) with perioral dermatitis. At presentation, the condition had been present between 3 and 14 months. All patients had grouped erythematous papules, papulovesicles and/or papulopustules on a slightly scaling background with a distribution around the mouth, with a zone of sparing immediately adjacent to the lips. Telangiectases or comedones were not seen. There were no similar lesions on other parts of the face, especially in the periocular region. Six patients had previously used topical low‐ to high‐potency corticosteroids. Four patients had a history of atopy. All patients were negative when patch tested. Skin biopsy was performed in two patients, and showed a normal epidermis with a slight perivascular inflammatory infiltrate in the upper and mid dermis. The infiltrate consisted mainly of lymphocytes and histiocytes. No granuloma formation or Demodex mites could be demonstrated. The condition was not responsive to previous topical treatments, including antibiotics (five patients) and metronidazole (two patients). None of the patients had been given oral antibiotics.

Histiocytose non langerhansienne (xanthoma disseminatum) extensive traitée par vinblastine

Xanthoma disseminatum is a non-Langerhans histiocyte proliferation, described by Montgomery in 1938. This rare entity is characterized by skin and mucous membrane xanthomatosis, associated with diabetes insipidus and normal lipid metabolism. In this case report, vinblastine produced the regression of the lesions. A 51 year-old man presented in 1999 with a four-year history of progressive xanthomatous papulonodular lesions of his trunk, axillary and inguinal folds, neck and face (leonin facies). Treatment with thalidomide for 6 months was ineffective. A rapid extension of the lesions to the pharynx, larynx and trachea with dyspnea occurred and required a tracheotomy. Histopathological study showed a dense histiocytic infiltrate within the upper and mid dermis with Touton giant cells and inflammatory cells. The histiocyte cells were positive for CD68. Neither lungs nor hypophysis were involved. A treatment with 6 cures of cyclophosphamide was insufficient. Vinblastine therapy (32 cycles: 0.1 mg/kg/cycle) produced a spectacular regression of the mucous-cutaneous papulonodular lesions leaving cheloid scars. The patient required a transitory tracheotomy and a bilateral commissuroplasty. Prognosis of xanthoma disseminatum is in related to the mucous membrane manifestations (50 p. 100 of cases) and involvement of the upper respiratory tract. The response to any form of therapy in xanthoma disseminatum is unsatisfactory. Surgical excision or laser therapy can improve physical and functional aspects but the evolution is characterized by very frequent relapses. Treatment with antimitotic drugs seems to be ineffective in many cases. In our patient, vinblastine induced a spectacular regression of mucocutaneous lesions without neurologic toxicity. To our knowledge, this is the first report of vinblastine efficacy in this rare and severe disorder.

Identification of parasite antigen, correlation of parasite density and inflammation in skin lesions of post kala-azar dermal leishmaniasis.

Post kala-azar dermal leishmaniasis (PKDL) is an unusual dermatosis following kala-azar (KA). Demonstration of the amastigotes in lesions plays an important role in the diagnosis of PKDL. It was aimed to evaluate the utility of an antibody G2D10 in detecting leishmania parasite antigen, to correlate the parasite number/percentage of parasites with the inflammation, and to assess the epidemiological significance associated with the location of the parasites. The study was conducted on 50 cases. Hematoxylin and eosin (H & E) stains and immunohistochemical (IHC) stains, using G2D10 antibody, was performed on the skin biopsies. The number of parasites and density of inflammation were semiquantitatively assessed. Leishmania donovan bodies (LDBs) were identified in 50% of cases with the H & E compared to 80% positivity with the IHC. All 50 cases showed inflammation in the superficial dermis (SD). About 44% showed dense inflammation compared to 16% sparse and 40% moderate inflammation. Parasite percentage was maximum in the SD (100%) compared to 75 and 42% in the mid and deep dermis, respectively. The IHC showed a higher percentage of LDB localization (80 vs. 50%). Density of inflammation was maximum in the SD. The parasite percentage was correlated with the inflammation. Location of parasites could have an epidemiological significance.