Unusual Skin Lesions Research Articles

Unusual skin and testicular lesions in a patient with CMML

Chronic myelomonocytic leukemia (CMML) is a clonal disorder of hematopoetic stem cells with myelodysplastic and myeloproliferative features. Skin infiltration by leukemic cells is rare in CMML. A case is presented with severe dermatological involvement and concomitant testicular infiltration. An unusual feature was the evidence that the skin was the primary site of an acute leukemic transformation.

Putrefaction and Wound Dehiscence

The decomposed body of a 49-year-old man was found at his home address. At autopsy, 3 incised wounds of the lower abdomen and groins were identified, raising the possibility of some form of inflicted injury. Further dissection revealed that the wounds were healing surgical incisions that had been forced open by putrefactive tissue breakdown, swelling, and gas formation. Death was due to ischemic heart disease. Putrefaction is a common problem encountered in forensic practice that may result in considerable distortion and modification of tissues. Unusual skin lesions caused by the disruption and dehiscence of healing surgical wounds may be created by decomposition. This possibility should be considered when symmetrical, cleanly incised wounds are identified.

Papular-purpuric gloves and socks syndrome: A case report

Papular-purpuric gloves and socks syndrome is a rare manifestation associated with acute seronegative arthritis and skin lesions. It is triggered by a viral infection, most commonly by Human parvovirus B19 or Coxsackie group of viruses, but precise viral diagnosis is not obtained in all cases. A 54-year old male patient experienced sudden, intense pain and swelling in the carpal area of his left hand. He also presented with unusual skin lesions, and grouped small purpuric-papules on the dorsal side of the left hand. During the next few days, both feet were similarly affected. In the next four weeks, a transient liver damage occurred, with a moderate increase in liver enzymes. Apart from elevated erythrocyte sedimentation rate and fibrinogen levels, other findings were within normal limits (complete blood and reticulocyte count, parameters of rheumatic and autoimmune diseases, abdominal ultrasound scan, radiographs of the chest and of affected hand and feet joints, urethral smears for isolation of trichomonas, yeasts and chlamydia trachomatis, and Syphilis, Borrelia, Toxoplasmosis, and Mycoplasma pneumoniae serology). Apart from positive tests for HPvB19, other viral serology tests were all negative (Coxsackievirus, Adenovirus, Influenza, Hepatitis B and C virus, HIV). The skin lesions resolved in about three weeks, the intensity of joint pain decreased, but lasted for the next 6 months. The diagnosis of papular-purpuric gloves and socks syndrome was made on the basis of the characteristic clinical picture, and confirmed by virus serology.

Moral Priorities in a Teaching Hospital

a thirty-five-year-old woman in the hospital for treatment of pain, fatigue, and unusual skin lesions, wakens suddenly as the medical team enters her room during their morning rounds. Good morning, JC, announces A, the attending physician. have a whole team of people to see you today. How are you feeling? JC shifts in bed and lifts herself a bit. She asks softly, Who are all these people? members of the team, replies A. they all have to be here? Can't I just talk to you and K, the one who admitted me? A explains. Dr. K was the doctor you saw in the ER. You won't see her up These folks are students and new doctors learning internal medicine; they're here to learn. They're all members of the team taking care of you. JC pauses, turns her head away from the students and residents toward the window; she appears exasperated and near tears. She pleads shakily with the A, It's really not comfortable to have all these people in here hovering over me. I mean, is my condition rare or something? I don't see why all these people need to be here. She flops back onto her pillows, bites her lower lip, and tries unsuccessfully to keep from crying. A walks toward the edge of JC's bed and touches the edge of the sheet that is clumped in her grip. sorry, JC. We caught you a little off guard You rest a bit, and we'll come back a little later. He motions for the resident closest to the door to exit the room. Two other residents, two medical students, and A follow. In the hall, the students and residents exchange sheepish glances. That was awkward. Yeah, she feels really bombarded. What should we do? A considers. Let's give her some time to calm down. Later I'll talk to her and see if she might be interested in getting some Xanax; that should help with the anxiety and stress. In the meantime, I'll get a chaplain up I think she could use someone to talk to. A third-year medical student asks, Didn't she know that this is a teaching hospital? Isn't someone supposed to explain that to her? There does appear to be some confusion about that, A responds. Well, let's move on for now. One of the chaplains visits JC that afternoon. JC also calls the number for the hospital's ethics consultation service and requests an ethics consultation. She tells the assistant who fields her call: It just can't be right that all these people get to crowd in here and observe me like I'm a guinea pig. Should patients be allowed to refuse to make themselves available for pedagogical purposes? If health care professionals in training are entitled to learn on or from patients, what is the scope of such entitlement? How should we look upon trainees' needs to learn from patients in light of--or in spite of--patients' vulnerabilities? commentary By Christy A. Rentmeester If the Hospital Ethics Committee gets involved in this case, the most helpful thing it could do might be to initiate a discussion of the influences that resident and student training can have on patients. This case draws attention to questions that probe this issue: Which features of patients' experiences of illness should prompt caregiver-teachers to carefully consider whether and when it is appropriate to use a particular patient to facilitate trainees' learning? Do caregivers in teaching hospitals have duties to articulate, to patients or their proxies, the special hazards of teaching hospitals--that procedures might be performed by less experienced personnel, that patients might need to repeat information to multiple students, residents, or attending caregivers, and that caregivers might be accompanied by entourages of trainees who might also listen, observe, and touch? If a patient cannot consent to being used to facilitate trainees' learning--in emergencies or because they do not have decision-making capacity, for example--might they be exposed to greater than minimal risk? …

Compound Heterozygeous RAG-1 Mutations in 2 Patients Presenting with Chronic Disfiguring Granulomatous Skin Lesions.

IntroductionMutations of the recombinase activating genes (RAG) cause a severe combined immunodeficiency (SCID) characterized by absent or low T and B cells due to defective T/B cell receptor rearrangement and failure of lymphocyte maturation. Presenting clinical features commonly are failure to thrive and opportunistic infections in early infancy. We observed 2 patients with RAG mutations where partial T- and B-cell immunity is preserved. These patients did not suffer from severe infections but presented with unusual symptoms and signs, e.g. unexplained lymphogranulomatous skin lesions mimicking malignant lymphoma and lupus vulgaris.Case presentationBoth patients, who are offsprings of unrelated German parents, underwent genetic screening because of unclear primary immunodeficiencies and were found to have compound heterozygeous RAG-1 mutations (Patient 1: R507W, R737H; R314W and patient 2: R778Q; R975W). Both have no evidence of thymic tissue on ultrasound and have persistant lymphopenia with low T-cell numbers, but are capable of mounting positive antibody responses to tetanus toxoid. In both patients unusual skin lesions were leading clinical abnormalities.Patient 1, a 6 year-old girl, has been suffering from progressive papulonodular skin lesions of extremities and face since the age of 1 year. Biopsy showed highly monomorphous T-cell infiltrates leading initially to a diagnosis of cutaneous T-cell lymphoma treated with chemotherapy. As progression occurred chemotherapy was stopped and the lesions were reinterpreted as poorly organised sarcoid-like granulomas of unknown etiology. At 5 years of age the girl developed an EBV lymphoma of her right tonsil treated with rituximab. The patient is undergoing at present hematopoetic stem cell transplantation (HSCT).Patient 2 is an 8 year-old girl, who at the age of 2 years developed persistant ulcerative skin lesions on her face and extremities. Granulomatous epitheloid tissue was found in skin biopsies, and later also in lung and tongue biopsies. No pathogens including mycobacteria were isolated. No improvement was seen on immunosuppression. Skin grafting was necessary in her lower extremity twice after partial destruction of the Achilles tendon by progressive ulceration. HSCT is planned in the near future.ConclusionRAG mutations may be associated with a combined immunodeficiency where uncontrolled granuloma formations of the skin, but not opportunistic infections, are leading clinical findings. It is unclear whether these disfiguring lesions are caused by pathogens such as mycobacteria which are difficult to culture or whether they represent reactive sarcoid-like granulomas as seen in other primary IDs, e.g. common variable immunodeficiency.

Metastatic Crohn's disease in a child

Metastatic Crohn's disease is a rare complication of Crohn's disease that has not been frequently reported in the literature. Although Crohn's disease is common in children, cutaneous manifestations are rarely a presenting sign. We report the case of a young boy who initially presented with unusual skin lesions and whose condition was diagnosed later as Crohn's disease.

Isolated Myelosarcoma in Children--Update and Review

Primary myelosarcomas, also called leukemia cutis, granulocytic sarcomas or chloromas, are rare extramedullary manifestations of acute myeloid leukemia (AML) which precede bone marrow involvement. Skin infiltration was the most frequent localization associated with a myelomonocytic differentiation. Although first remission was achieved by most children, risk of relapse seemed to be increased. This might be caused by the specific biology of myelosarcomas, but also may be the result of delayed or reduced treatment. During the AML-BFM-studies 87/93/98 (11/1987-7/2000) 37 children with isolated myelosarcomas were diagnosed. Eighteen of the 37 patients survived with a 5-year overall survival estimation of 0.54 ± 0.09 compared to 0.59 ± 0.02; p log rank =0.94. However, reduced or delayed treatment in 17 children led to an increased relapse rate of 71% compared to 35% in children treated soon after diagnosis. The 5-year overall survival in these patients was 0.41 ± 0.11. According to our experience and review of the literature, an early diagnostic workup is needed in children with unusual skin lesions or tumors, considering myelosarcoma as primary manifestation of AML. Intensive AML-specific chemotherapy is generally recommended soon after diagnosis.

Hansen's disease in a native-born, United States resident, after a brief stay in an endemic area abroad

Despite the overall decline in number of leprosy cases in the United States, small numbers of patients with the disease continue to be reported, predominantly among immigrant populations. Occasional cases occur among native-born American residents, predominantly from the southern United States. The source of the reservoir and transmission among indigenous HD cases remains unexplained, although armadillos in the state of Texas and Louisiana have been implicated. Since most patients among the indigenous cases occur in older age groups, the possibility of reactivation of the disease through immunosenescence has been raised. In most patients, unfamiliarity with the clinical picture of HD among physicians in the United States accounts for delayed or incorrect diagnosis. High index of suspicion in a patient with unusual skin lesions, particularly with sensory loss, should be followed by a biopsy looking for the characteristic histologic changes found in various forms of leprosy. Reversal reactions and erythema nodosum leprosum are relatively frequent complications of treatment. Treatment and periodic follow-up of these patients should be done, preferably by physicians with experience with the disease entity.

Primary myelosarcomas are associated with a high rate of relapse: report on 34 children from the acute myeloid leukaemia-Berlin-Frankfurt-Münster studies.

Primary myelosarcomas are rare manifestations of acute myeloid leukaemia (AML) that precede bone marrow involvement. Out of 744 children observed during the AML-Berlin-Frankfurt-Münster (BFM) studies 87 and 93, 34 children presented with extramedullar myelosarcomas and no blasts (n = 21; 2.8%), or a low blast count (n = 13; 1.7%) in the bone marrow. Owing to the initially mild and variable symptoms, in some children (n = 12) diagnostic procedures were delayed and treatment intensity was reduced. At 0.65 +/- 0.13, the cumulative incidence of relapse was significantly higher than in other AML patients (0.28 +/- 0.02). The 5-year event-free survival was 0.19 +/- 0.08 (compared with 0.48 +/- 0.02 in AML-BFM studies 87/93; P(log rank) < 0.03). Overall, 18 out of 34 patients died from disease (estimated 5 year survival 0.44 +/- 0.09 compared with 0.55 +/- 0.02 in the AML-BFM-studies 87/93; P(log rank) = 0.35, n.s.). An early diagnostic workup is needed in children with unusual skin lesions or tumours, considering myelosarcoma as a primary manifestation of AML. Intensive AML-specific chemotherapy is recommended soon after diagnosis.

Cutaneous schistosomiasis: Report of a case and review of the literature

Cutaneous disease is a previously reported but unusual presentation for schistosomiasis. We report a case of Schistosoma haematobium infection that appeared 3 years after exposure, with skin lesions as the sole manifestation. The diagnosis was made on the basis of a routine skin biopsy and the patient did well after therapy with praziquantel. Dermatologists should be aware of this presentation of schistosomiasis when evaluating patients with unusual skin lesions who have traveled in areas where schistosomiasis is endemic. (J Am Acad Dermatol 2000;42:678-80.)

Cutaneous schistosomiasis: Report of a case and review of the literature

Cutaneous disease is a previously reported but unusual presentation for schistosomiasis. We report a case of Schistosoma haematobium infection that appeared 3 years after exposure, with skin lesions as the sole manifestation. The diagnosis was made on the basis of a routine skin biopsy and the patient did well after therapy with praziquantel. Dermatologists should be aware of this presentation of schistosomiasis when evaluating patients with unusual skin lesions who have traveled in areas where schistosomiasis is endemic. (J Am Acad Dermatol 2000;42:678-80.)

Nodular tertiary syphilis mimicking granuloma annulare

We describe a 47-year-old man with annular plaques on the arms and torso that were treated as granuloma annulare, based on clinical and histopathologic findings. Exacerbation of the lesions during treatment with topical corticosteroids prompted a search for an infectious cause, which proved to be syphilis in the tertiary stage. The clinician should maintain a high index of suspicion for syphilis in the differential diagnosis of unusual annular skin lesions in a patient with noncaseating granulomas seen on skin biopsy. (J Am Acad Dermatol 2000;42:378-80.)

Experts and gold standards in dermatopathology: qualitative and quantitative analysis of the self-assessment slide seminar at the 17th colloquium of the International Society of Dermatopathology.

The diagnosis of lymphoproliferative and melanocytic skin lesions is one of the most vexing problems in dermatopathology, a problem that is compounded by the far-reaching therapeutic and psychosocial consequences of the diagnosis for both patient and physician. On the occasion of a self-assessment slide seminar held during a dermatopathology meeting, 30 unusual lymphoproliferative and melanocytic lesions, each provided with four differential diagnoses, were evaluated by "expert pathologists" and other participants ("nonexperts") of the slide seminar. The final diagnosis was pinpointed by the majority of the experts in 16 of 30 cases (56%). The group of experts returned an unanimous decision on the diagnosis in only 2 of the 30 cases (7%). In contrast to the expert group, the preferred diagnoses given by the nonexperts showed a wider range. In 20 of 30 cases (66%), the final diagnosis could only be established after consideration of clinical, histologic, immunophenotypic, and molecular features. Our findings agree with the results of recent studies indicating quite a high degree of discordance among expert pathologists. The discordance between experts and, to a higher extent, nonexperts may have some crucial consequences for dermatopathology. Full agreement on diagnosis, particularly in unusual skin lesions, cannot be achieved only by an accumulation of expertises. Instead of relying on one single finding or diagnostic procedure ("gold standard") as the main criterion upon which to base a diagnosis, the diagnoses become more reliable if based on the integration of several factors including an evaluation of clinical and histomorphologic features and immunophenotypic and molecular findings ("diagnostic elements"), particularly in the field of lymphoproliferative and melanocytic lesions. In addition, a continuous retrospective work-up of difficult or unusual cases is recommended to ensure a long-term improvement in diagnostic reliability.

The cutaneous manifestations of mycobacterial diseases

.LI)~ohartrriitrn trd~crrulosis and certain atypical niycobacteria may cause a spectrum of cutaneous disease and m,iy sometimes pose difficult diagnostic problems (Table 1). This is especially true in irrimunocompromised patients, where even niycobacteria not previously thought to c u s e disease niay d o so. A high index of suspicion is required and unusual skin lesions presenting in these circ~unst~inces should always be biopsied and sent for mycobacterial culture and staining and PCR where possible.

Lymphedema as a Postulated Cause of Cutis Verticis Gyrata in Turner Syndrome

Abstract: Unusual skin lesions were present at birth in four infants with Turner syndrome. The skin changes in these patients appear to have resulted either from in utero entrapment or pinching of edematous skin or from redundant skin remaining after in utero resolution of lymphedema. Distention by lymphedema is thought to cause several of the phenotypic characteristics seen in patients with Turner syndrome, including nuchal webbing and nail changes. In three of these patients the clinical appearance of the skin changes was similar to cutis verticis gyrata, marked by fixed thickened plaques in folds.

Cutaneous manifestations of chronic granulomatous disease: A report of four cases and review of the literature

Chronic granulomatous disease represents a group of genetic disorders in which impaired intracellular microbial killing by phagocytes leads to recurrent bacterial and fungal infections and granuloma formation. Cutaneous disease occurs in 60% to 70% of cases. The characteristic histologic finding of pigmented lipid macrophages in visceral granulomas has not been described previously in the skin. Our purpose was to review our experience of skin disorders in chronic granulomatous disease. We studied the clinical and histologic findings in four patients with chronic granulomatous disease and unusual skin lesions. We reviewed the skin disorders seen in five additional patients with chronic granulomatous disease referred to the pediatric dermatology clinic. The literature was reviewed for previously reported cutaneous manifestations of chronic granulomatous disease. A teenage boy with chronic granulomatous colitis had nonulcerating cutaneous granulomas from which no organisms were isolated. Histologic examination of both skin and bowel revealed the characteristic golden-yellow granular pigment in macrophages. A second boy had cutaneous aspergillosis involving the left foot; histologic examination revealed macrophages containing yellow-brown pigment at the periphery of the granulomatous inflammation. Two children had vesicular skin lesions. These lesions were recurrent in one boy for several years. In the second child they were associated with fatal intracranial and pulmonary infection. Histologic examination in both cases revealed a subcorneal polymorphonuclear infiltrate and perivascular macrophages containing yellow-brown pigment. Cultures were either negative or revealed organisms that are normally nonpathogenic skin commensals, such as coagulase-negative staphylococci. The cutaneous manifestations of chronic granulomatous disease encompass a variety of infections and inflammatory lesions. Diagnostic and therapeutic problems may arise because of difficulty in isolating a causative organism. The characteristic pigmented macrophages of visceral granulomas can also be found in skin lesions.

Cutaneous cryptococcosis and histoplasmosis coinfection in a patient with AIDS

Patients with AIDS may have multiple infections at one time, and skin lesions resulting from simultaneous infections with more than one organism have been described. We report a case of disseminated cryptococcal and histoplasmosis infections with cutaneous lesions in a patient with AIDS. In addition, we demonstrate the first case of two coexisting fungal infections in a unique skin lesion. The cutaneous presentation of infectious disorders in HIV-in-fected patients is often nondescript and not diagnosed by clinical observation alone. Biopsies and cultures are essential for making accurate diagnoses in immunocompromised patients with unusual skin lesions.

The emergence of Mycobacterium ulcerans infection near Melbourne.

To document the emergence of new foci of Mycobacterium ulcerans infection (Bairnsdale ulcer) in Victoria. From data kept by one of us (JAH) and records from the Mycobacterium Reference Laboratory, Fairfield Hospital, we reviewed cases of M. ulcerans infection in Victoria between 1980 and 1995, and identified those apparently acquired outside the east Gippsland endemic region. A case was defined as a person with a lesion suggestive of M. ulcerans infection, from which the organism had been cultured or, in the absence of culture information, from which a histological specimen characteristic of M. ulcerans infection had been obtained. We identified 45 people who appeared to have acquired their infections in Victoria but outside the east Gippsland region. A new focus appeared on the northern shores of Western Port, near Melbourne, in 1982, and there was a dramatic increase in cases between 1991 and 1994 associated with foci on Phillip Island, and in the Frankston-Langwarrin area of outer suburban Melbourne. Single cases came from Crib Point, Narre Warren and Bendigo. There have been at least three new foci of M. ulcerans infection within 80 km of Melbourne since 1982. Victorian clinicians should consider the possibility of Bairnsdale ulcer when dealing with unusual skin lesions.

DERMATOLOGIC MANIFESTATIONS OF SEXUALLY TRANSMITTED DISEASES OTHER THAN HIV

This article focuses on the common skin manifestations of sexually transmitted diseases. A number of unusual skin lesions associated with these diseases are shown, and pathogenesis and treatment are briefly presented. This article will be most useful as a reference for the formulation of the differential diagnosis of genital area skin lesions.

Paederus (Nairobi Fly) Vesicular Dermatitis in Tanzania

Four cases of dermatitis following contact with insects of the genus Paederus are described. The insects are known in East Africa as Nairobi fly and cause an acute, painful toxic epidermolysis when crushed on skin. Local clinicians are familiar with the condition, but it should also be borne in mind as a cause of unusual skin lesions in visitors returning from East Africa.