DOCSLIB.ORG

Urticaria Pigmentosa: a Case Report

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Urticaria Pigmentosa: a Case Report Vol. 13, No. 1, 2015 Case Report Urticaria pigmentosa: A case report Keen MA1 1Dermatologist, Jammu and Kashmir Health Services, India Abstract Mastocytosis is a heterogenous group of diseases characterized by abnormal infiltration of mast cells in the skin and other organs. Urticaria Pigmentosa is the most common variant of cutaneous mastocytosis. We herein report a case of urticaria pigmentosa in a three year old boy. Keywords: Mastocytosis, Mast cells, Urticaria pigmentosa Address for correspondence Dr. Mohammad Abid Keen Jammu and Kashmir Health Services. Iqbal Abad, KP Road , Ananntnag, Jammu and Kashmir Pin : 192101 Cell no: 9419963692 E mail: [email protected] Introduction of two year duration. There was also a history of Mastocytosis is a hematopoietic disorder which generalized urticarial flushing with occasional is usually seen sporadically and characterized by bulla formation. He had been delivered with an increased number and the accumulation of cesarean section at full term without any mast cells in one or more organs.1 It can be divided complication. The general health, growth and into cutaneous mastocytosis and systemic development of the child was unaffected. There mastocytosis.2,3 was no family history of similar disease or any other dermatological or autoimmune disease. There are four clinical subtypes of cutaneous Therapy with topical corticosteroids prescribed mastocytosis: urticaria pigmentosa, mastocytoma, by the general practitioner had shown no effect. diffuse cutaneous mastocytosis and telangiectasia Physical examination of the patient was normal. macularis eruptiva perstans (TMEP). The most Systemic examination of the patient also revealed common type of cutaneous mastocytosis in no abnormality. There was no hepatomegaly, children is urticaria pigmentosa. The disease is splenomegaly or lymphadenopathy. Cutaneous clinically characterized by multiple erythematous examination of the child revealed multiple, sharply and pigmented macules, papules and plaques and defined, red brown maculopapules and plaques localized blistering that may vary in size. Dariers on the trunk and limbs (Figure 1). sign is positive. Histopathologial examination is the gold stand for the diagnosis. Symptomatic treatment with antihistaminics, mast cell stabilizers and topical steroids is effective. A typical case of this dermatosis is being presented. Case report A 3 year old male child was presented by his Figure 1: Multiple sharply defined red brown parents with generalized eruption of multiple maculopapules and plaques on the trunk and brown maculopapular lesions on trunk and limbs limbs NJDVL - 57 Vol. 13, No. 1, 2015 Case Report No nodules or bullae were seen at the time of the mastocytosis.2,3 Cutaneous mastocytosis usually examination. On stroking the individual lesions, affects the patients in early childhood and the there was formation of wheal and flare (Darier disease often regresses spontaneously.6 However, sign - Positive). A provisional diagnosis of urticaria systemic mastocytosis frequently occurs in adult pigmentosa was made in this child. A complete patients and tends to resist permanently.7 Mast blood count, liver and renal function tests, urine cells that arise in the bone marrow are and stool analysis, chest X ray and pluripotential precursor cells. They differentiate ultrasonography of the abdomen were normal. A in the skin and other peripheral organs under the 4mm skin punch biopsy specimen was obtained influence of an array of growth factors, c-kit with due precautions and sent for histopathological ligand or mast cell growth factor. Mast cells examination. It revealed increased number of produce inflammatory mediators including mast cells in dermis (Figure 2). histamine, trysptase, TNF- a, leukotrienes, prostaglandins, platelet activating factor, heparin, IL-8.3,4 These mediators are responsible for local and systemic symptoms such as flushing, bullae, pruritus, dyspnea, exacerbation of asthma, low blood pressure, gastroesophageal reflux, peptic ulcer and diarrhea. The most important mediator causing all of these symptoms is histamine.8 The etiology of mastocytosis is unclear. Recent observations have shown a soluble form of stem cell factor in the skin and suggest an etiological role for derangement of this growth factor and its receptor.[4] In addition, a somatic mutation of the proto-oncogene c-kit that could be responsible Figure 2: Histopathological image revealing for mast cell proliferation has been detected.9 c- increased number of mast cells in dermis kit mutation analyses are important for the therapy and prognosis of mastocytosis. The child was treated with antihistaminics and topical steroids for 4 week which led to the In 2001, four distinct clinical variants of cutaneous regression of his lesions.The parents were advised mastocytosis were published by WHO. These regarding avoidance of excessive scrubbing and were: massage of the skin. At the end of six weeks, the 1. Urticaria pigmentosa child had only residual pigmentation left at the 2. Isolated mastocytoma (solitary lesional sites with no new lesion formation. mastocytoma) 3. Diffuse Cutaneous mastocytoma Discussion 4. Telangiectasia macularis eruptiva perstans Mastocytosis represents a spectrum of clinical (TMEP) disorders with clinical features determined by infiltration of various organs and skin with mast In 2008, WHO updated the classification of cells. The cutaneous and systemic symptoms such cutanean mastocytosis as:- as pruritus, urticaria, wheezing, diarrhea and 1. Maculopapular cutaneous mastocytosis hypotension are caused by the release of mediators (Urticaria pigmentosa) by non-allegic mechanisms.4 Despite the fact that a. Special form: Plaque form most common location is the skin, it may also b. Special form: Nodular form occur in the liver, spleen, bone narrow, lymph c. Special form: TMEP nodes, lungs and gastrointestinal tract.5 It can be 2. Diffuse cutaneous mastocytosis divided into cutaneous mastocytosis and systemic 3. Solitary mastocytosis NJDVL - 58 Vol. 13, No. 1, 2015 Case Report Urticaria pigmentosa also known as esterase.16 Careful technique when taking the skin maculopapular mastocytosis, was first reported biopsy, to minimize traumatic degranulation, is by Nettleship and Tay in 1869.10 Their patient important. Injecting local anaesthetic around the was a female child of 2 years, and their paper lesion to be sampled and avoiding adrenaline was entitled chronic Urticaria leaving Brown containing local anaesthetics may yield a higher stains of Nearly two years Duration. Unna later number of stainable mast cells. The diagnostic demonstrated mast cells in increased numbers in work-up of patients includes complete blood urticarias of the Nettleship type. Urticaria counts, routine biochemistry tests, liver function pigmentosa is the most common form of tests, and basal serum tryptase levels. In case the cutaneous mastocytosis (70-90%), within an patient is an infant or a child and, has an abnormal approximate Incidence of 1/1000-8000. There is blood count, enlarged liver, spleen or no sex predilection and occurs more often in lymphadenopathy and elevated serum tryptase infants and children than the adults. Clinical levels (>20ng/ml), all necessary tests such as features appear within the first two years of life. abdominal ultrasound and CT, gastrointestinal The clinical features are multiple oval or round system endoscopy, bone radiographs, scans and hyperpigmented macules, papules or patches, even bone narrow biopsy should be done.17 brown-red-yellow and 2-4mm in diameter.11 These However, if the patient is an adult, bone narrow lesions urticate by manipulation (e.g, rubbing) or examination must be done absolutely.18 Treatment spontaneously. This reaction is known as Darier of urticaria pigmentosa is mainly symptomatic sign. Dariers sign is not always demonstrable, with avoidance of all known mast all degranulation especially in those with a long history of this stimuli (physical triggers especially rubbing, disorder, and is not 100% specific for alcohol, morphine, codeine, NSAIDS, mastocytosis, since it has been described rarely radiocontrast media, scopolamine, d-Tc). in Juvenile xanthogranuloma and acute Antihistaminics such as H1 or H2 antihistamines lymphoblastic leukemia of neonate.12,13 The sites or a combination of both, are the first step of predilection are chest and dorsal areas of the medications among systemic treatment options. body, while the palms, soles and face are usually Doxepin may also be used for its antihistamine unaffected.14 Lesions may blister in infancy or properties. The mast cell stabilizers like Sodium childhood and may be the presenting feature, but cromoglycate and ketotifen are also used in heal without scarring. Systemic manifestations patients with urticaria pigmentosa. Topical include flushing, vomiting, diarrhea, tachycardia, Cortisosteroids can be used and also intralesional headache, weight loss and wheezing.15 triamcinolone acetonide injection is available for Histologically there is mild to moderate localized lesions.6 Systemic PUVA therapy is an perivascular infiltrate with dendritic mast cells alternative modality of treatment for patients not in the papillary dermis; a band like infiltrate or responding to standard treatments. Patients with sometimes even nodular infiltrates extending to chronic and widespread involvement should keep the subcutis may be seen, especially with special adrenaline pen for self-administration and a stains like toluidine blue and chloroacetate
Load more

Recommended publications
  • Dumitras, Cu, MC; Popa, A.; Petca, A.; Miulescu, R.-G. Cutaneous Mastocytosis in Childhood—Update from the Literature
    Journal of Clinical Medicine Review Cutaneous Mastocytosis in Childhood—Update from the Literature 1,2 1,3 1,4 1,5, Florica Sandru ,Răzvan-Cosmin Petca , Monica Costescu , Mihai Cristian Dumitras, cu *, Adelina Popa 2, Aida Petca 1,6,* and Raluca-Gabriela Miulescu 1,7 1 “Carol Davila” University of Medicine and Pharmacy, 030167 Bucharest, Romania; fl[email protected] (F.S.); [email protected] (R.-C.P.); [email protected] (M.C.); [email protected] (R.-G.M.) 2 Department of Dermatology, Elias University Emergency Hospital, 0611461 Bucharest, Romania; [email protected] 3 Department of Urology, “Prof. Dr. Theodor Burghele” Clinical Hospital, 061344 Bucharest, Romania 4 Department of Dermatology, “Dr. Victor Babes” Clinical Hospital of Infectious and Tropical Diseases, 030303 Bucharest, Romania 5 Department of Obstetrics & Gynecology, University Emergency Hospital, 050098 Bucharest, Romania 6 Department of Obstetrics & Gynecology, Elias University Emergency Hospital, 0611461 Bucharest, Romania 7 Department of Dermatology, Vălenii de Munte Hospital, 106400 Prahova, Romania * Correspondence: [email protected] (M.C.D.); [email protected] (A.P.); Tel.: +40-722-223223 (M.C.D.); +40-745-787448 (A.P.) Abstract: Mastocytosis (M) represents a systemic pathology characterized by increased accumulation and clonal proliferation of mast cells in the skin and/or different organs. Broadly, M is classified into two categories: Cutaneous mastocytosis (CM) and systemic mastocytosis (SM). In children, CM is Citation: Sandru, F.; Petca, R.-C.; the most frequent form. Unfortunately, pathogenesis is still unclear. It is thought that genetic factors Costescu, M.; Dumitras, cu, M.C.; are involved, but further studies are necessary. As for features of CM, the lesions differ in clinical Popa, A.; Petca, A.; Miulescu, R.-G.
    [Show full text]
  • Urticaria Pigmentosa: a Case Report and Review of Current Standards in the Diagnosis of Systemic Mastocytosis
    Urticaria Pigmentosa: A Case Report and Review of Current Standards in the Diagnosis of Systemic Mastocytosis Riddhi J. Shah, DO,* Mark A. Kuriata, DO, FAOCD** *Dermatology Resident, 2nd year, MSUCOM/Lakeland Regional Medical Center, St. Joseph, MI **Dermatology Residency Program Director, MSUCOM/Lakeland Regional Medical Center, St. Joseph, MI Abstract Mastocytosis is a group of diseases that is characterized by mast-cell infiltration of the skin. The cutaneous forms of the disease are most identifiable, yet it is important to recognize the progression to systemic disease due to the eaffect on morbidity and mortality. We Our goal is to describe a case of cutaneous mastocytosis as well as review the current standards in diagnosis and management of systemic mastocytosis. Introduction patient had several bouts of loose stools. This and spleen did not reveal any organomegaly. Mast-cell disease is a rare disorder, primarily was accompanied by bloating and indigestion, Muscle strength and tone appeared to be within of childhood, that is usually self-resolving. which occurred 30 minutes after a meal. A normal limits. There were no palpable lymph Approximately two-thirds of cases are limited to colonoscopy was performed one year prior at an nodes in the neck, axillae or groin regions. outside facility, and it was within normal limits. the skin. The most common forms of cutaneous Our differential diagnosis included systemic However, no biopsies were performed to look mastocytosis include: mastocytoma, urticaria mastocytosis as well as carcinoid syndrome, for mast-cell infiltration of the digestive tract. pigmentosa, telangiectasia macularis eruptiva pheochromocytoma, inflammatory bowel disease, Our patient also had sharp, intermittent, left perstans, maculopapular cutaneous mastocytosis, urticaria, and myeloproliferative disorder.
    [Show full text]
  • Dermatological Indications of Disease - Part II This Patient on Dialysis Is Showing: A
    “Cutaneous Manifestations of Disease” ACOI - Las Vegas FR Darrow, DO, MACOI Burrell College of Osteopathic Medicine This 56 year old man has a history of headaches, jaw claudication and recent onset of blindness in his left eye. Sed rate is 110. He has: A. Ergot poisoning. B. Cholesterol emboli. C. Temporal arteritis. D. Scleroderma. E. Mucormycosis. Varicella associated. GCA complex = Cranial arteritis; Aortic arch syndrome; Fever/wasting syndrome (FUO); Polymyalgia rheumatica. This patient missed his vaccine due at age: A. 45 B. 50 C. 55 D. 60 E. 65 He must see a (an): A. neurologist. B. opthalmologist. C. cardiologist. D. gastroenterologist. E. surgeon. Medscape This 60 y/o male patient would most likely have which of the following as a pathogen? A. Pseudomonas B. Group B streptococcus* C. Listeria D. Pneumococcus E. Staphylococcus epidermidis This skin condition, erysipelas, may rarely lead to septicemia, thrombophlebitis, septic arthritis, osteomyelitis, and endocarditis. Involves the lymphatics with scarring and chronic lymphedema. *more likely pyogenes/beta hemolytic Streptococcus This patient is susceptible to: A. psoriasis. B. rheumatic fever. C. vasculitis. D. Celiac disease E. membranoproliferative glomerulonephritis. Also susceptible to PSGN and scarlet fever and reactive arthritis. Culture if MRSA suspected. This patient has antithyroid antibodies. This is: • A. alopecia areata. • B. psoriasis. • C. tinea. • D. lichen planus. • E. syphilis. Search for Hashimoto’s or Addison’s or other B8, Q2, Q3, DRB1, DR3, DR4, DR8 diseases. This patient who works in the electronics industry presents with paresthesias, abdominal pain, fingernail changes, and the below findings. He may well have poisoning from : A. lead. B.
    [Show full text]
  • Table I. Genodermatoses with Known Gene Defects 92 Pulkkinen
    92 Pulkkinen, Ringpfeil, and Uitto JAM ACAD DERMATOL JULY 2002 Table I. Genodermatoses with known gene defects Reference Disease Mutated gene* Affected protein/function No.† Epidermal fragility disorders DEB COL7A1 Type VII collagen 6 Junctional EB LAMA3, LAMB3, ␣3, ␤3, and ␥2 chains of laminin 5, 6 LAMC2, COL17A1 type XVII collagen EB with pyloric atresia ITGA6, ITGB4 ␣6␤4 Integrin 6 EB with muscular dystrophy PLEC1 Plectin 6 EB simplex KRT5, KRT14 Keratins 5 and 14 46 Ectodermal dysplasia with skin fragility PKP1 Plakophilin 1 47 Hailey-Hailey disease ATP2C1 ATP-dependent calcium transporter 13 Keratinization disorders Epidermolytic hyperkeratosis KRT1, KRT10 Keratins 1 and 10 46 Ichthyosis hystrix KRT1 Keratin 1 48 Epidermolytic PPK KRT9 Keratin 9 46 Nonepidermolytic PPK KRT1, KRT16 Keratins 1 and 16 46 Ichthyosis bullosa of Siemens KRT2e Keratin 2e 46 Pachyonychia congenita, types 1 and 2 KRT6a, KRT6b, KRT16, Keratins 6a, 6b, 16, and 17 46 KRT17 White sponge naevus KRT4, KRT13 Keratins 4 and 13 46 X-linked recessive ichthyosis STS Steroid sulfatase 49 Lamellar ichthyosis TGM1 Transglutaminase 1 50 Mutilating keratoderma with ichthyosis LOR Loricrin 10 Vohwinkel’s syndrome GJB2 Connexin 26 12 PPK with deafness GJB2 Connexin 26 12 Erythrokeratodermia variabilis GJB3, GJB4 Connexins 31 and 30.3 12 Darier disease ATP2A2 ATP-dependent calcium 14 transporter Striate PPK DSP, DSG1 Desmoplakin, desmoglein 1 51, 52 Conradi-Hu¨nermann-Happle syndrome EBP Delta 8-delta 7 sterol isomerase 53 (emopamil binding protein) Mal de Meleda ARS SLURP-1
    [Show full text]
  • Adult-Onset Mastocytosis in the Skin Is Highly Suggestive of Systemic
    Modern Pathology (2014) 27, 19–29 & 2014 USCAP, Inc. All rights reserved 0893-3952/14 $32.00 19 Adult-onset mastocytosis in the skin is highly suggestive of systemic mastocytosis Sabina Berezowska1, Michael J Flaig2, Franziska Rue¨ff2, Christoph Walz1, Torsten Haferlach3, Manuela Krokowski4, Roswitha Kerler1, Karina Petat-Dutter1, Hans-Peter Horny3 and Karl Sotlar1 1Institute of Pathology, University of Munich, Munich, Germany; 2Department of Dermatology and Allergology, University of Munich, Munich, Germany; 3MLL Munich Leukemia Laboratory, Munich, Germany and 4Institute of Pathology, University of Schleswig Holstein, Lu¨beck, Germany Adult-onset urticaria pigmentosa/mastocytosis in the skin almost always persists throughout life. The prevalence of systemic mastocytosis in such patients is not precisely known. Bone marrow biopsies from 59 patients with mastocytosis in the skin and all available skin biopsies (n ¼ 27) were subjected to a meticulous cytological, histological, immunohistochemical, and molecular analysis for the presence of WHO-defined diagnostic criteria for systemic mastocytosis: compact mast cell infiltrates (major criterion); atypical mast cell morphology, KIT D816V, abnormal expression of CD25 by mast cells, and serum tryptase levels 420 ng/ml (minor criteria). Systemic mastocytosis is diagnosed when the major diagnostic criterion plus one minor criterion or at least three minor criteria are fulfilled. Systemic mastocytosis was confirmed in 57 patients (97%) by the diagnosis of compact mast cell infiltrates plus at least one minor diagnostic criterion (n ¼ 42, 71%) or at least three minor diagnostic criteria (n ¼ 15, 25%). In two patients, only two minor diagnostic criteria were detectable, insufficient for the diagnosis of systemic mastocytosis. By the use of highly sensitive molecular methods, including the analysis of microdissected mast cells, KIT D816V was found in all 58 bone marrow biopsies investigated for it but only in 74% (20/27) of the skin biopsies.
    [Show full text]
  • Scabies Masquerading As Bullous Pemphigoid: Scabies Surrepticius
    Journal name: Clinical, Cosmetic and Investigational Dermatology Article Designation: CASE REPORT Year: 2017 Volume: 10 Clinical, Cosmetic and Investigational Dermatology Dovepress Running head verso: Cohen Running head recto: Scabies surrepticius open access to scientific and medical research DOI: http://dx.doi.org/10.2147/CCID.S145494 Open Access Full Text Article CASE REPORT Scabies masquerading as bullous pemphigoid: scabies surrepticius Philip R Cohen Abstract: Scabies, a parasitic infestation caused by the mite Sarcoptes scabiei, is diagnosed by observing either the mite, its ova, or its excrement. The mite tracts, known as burrows and a Department of Dermatology, University of California San characteristic presentation of the pruritic condition, are typically found on the web spaces between Diego, La Jolla, CA, USA the fingers. Other cutaneous lesions include excoriated papules, pustules, and vesicles. However, atypical clinical variants of scabies, such as bullous, crusted, hidden, incognito, nodular, and scalp forms of the parasitic infestation, mimic the morphologic features of other non-parasitic dermatoses. A 76-year-old man presented with pruritic blisters and urticarial plaques that dem- onstrated not only pathology changes, but direct immunofluorescence also showed findings of bullous pemphigoid. His condition improved, but did not resolve, with topical corticosteroid cream for the management of the primary autoimmune blistering disorder. When other fam- ily members subsequently developed scabies, the correct diagnosis for his condition, bullous scabies, was established by demonstrating mites, ova, and scybala on a mineral oil preparation from a skin scraping of a newly appearing burrow. Bullous scabies can masquerade not only clinically, but also both pathologically and immunologically as bullous pemphigoid.
    [Show full text]
  • Journal of the American Osteopathic College of Dermatology Journal of the American Osteopathic College of Dermatology
    Journal of the American Osteopathic College of Dermatology Journal of the American Osteopathic College of Dermatology Editors Jay S. Gottlieb, D.O., F.O.C.O.O. Stanley E. Skopit, D.O., F.A.O.C.D. Associate Editor James Q. Del Rosso, D.O., F.A.O.C.D. Editorial Review Board Earl U. Bachenberg, D.O. Richard Miller, D.O. Lloyd Cleaver, D.O. Ronald Miller, D.O. Eugene Conte, D.O. Evangelos Poulos, M.D. Monte Fox, D.O. Stephen Purcell, D.O. Sandy Goldman, D.O. Darrel Rigel, M.D. Gene Graff, D.O. Robert Schwarze, D.O. Andrew Hanly, M.D. Michael Scott, D.O. Cindy Hoffman, D.O. Eric Seiger, D..O David Horowitz, D.O. Brooks Walker, D.O Charles Hughes, D.O. Bill Way, D.O. Daniel Hurd, D.O. Schield Wikas, D.O. Mark Lebwohl, M.D. Edward Yob, D.O. Jere Mammino, D.O. 2003-2004 OFFICERS President: Stanley E. Skopit, DO President-Elect: Ronald C. Miller, DO First Vice-President: Richard A. Miller, DO Second Vice-President: Bill V. Way, DO Third Vice-President: Jay S. Gottlieb, DO Secretary-Treasurer: James D. Bernard, DO Assistant Secretary-Treasurer: Jere J. Mammino, DO Immediate Past President: Robert F. Schwarze, DO Trustees: Daniel S. Hurd, DO Jeffrey N. Martin, DO Brian S. Portnoy, DO Donald K. Tillman, DO Executive Director: Rebecca Mansfield, MA AOCD 1501 E Illinois Kirksville, MO 63501 800-449-2623 FAX: 660-627-2623 www.aocd.org COPYRIGHT AND PERMISSION: written permission must be obtained from the Journal of the American Osteopathic College of Der- matology for copying or reprinting text of more than half page, tables or figures.
    [Show full text]
  • Computer Diagnosis of Skin Disease
    COMPUTERS IN FAMILY PRACTICE Computer Diagnosis of Skin Disease Brian Potter, MD, and Salve G. Ronan, MD Michigan City, Indiana, and Chicago, Illinois A transferable computer program for the differential diagnosis of diseases of the skin, CLINDERM, has been produced for use by physicians on standard IBM and compat­ ible personal microcomputers. This program lists the differential diagnosis and defini­ tive diagnosis of any presented disease of the skin, except single tumors. The physi­ cian operator indicates the distribution and detailed description of lesions, which the interactive system integrates with a comprehensive knowledge base. The computer diagnosis in 129 cases was compared with independent interpreta­ tion of the same information by an academic dermatologist. Results were synony­ mous in 66.7% of all diseases and similar in an additional 4.7%. A common differen­ tial diagnosis was obtained in 24%, for a 95.3% rate of synonymous, similar, or common differential diagnoses. Diagnosis was different in 3.9% and description was inadequate for diagnosis in 0.8%. The variation in diagnosis showed that some descriptive terms are prejudicial of certain diagnoses; that diagnostic terms are not all completely standardized; that some diagnoses are variants of another disease; and that drug-induced eruptions simulate many other diseases. A skin disease can usually be diagnosed by specific description. Most lesions that are not diagnostic from inspection are nodular. A computer can be programmed to list diagnoses according to morphologic description J Fam Pract 1990; 30:201-210. functional, transferable computer software system examination may, however, be excessively complex. Ob­ Afor the differential diagnosis of diseases of the skin, jectivity is improved by recording specific features ac­ called CLINDERM,* has been produced for use by phy­ cording to sets of standardized criteria.
    [Show full text]
  • Feline Urticaria Pigmentosa in Three Related Sphinx Cats
    Veterinary Dermatology 1996, 7, 227-233 Case Report Feline urticaria pigmentosa in three related Sphinx cats CARL0 B. VITALE,* PETER J. IHRKE,? THIERRY OLIVRYtS and ANTHONY A. STANNARDt Veterinary Medical Teaching Hospital, ?Department of Medicine and Epidemiology, School of Veterinary Medicine, University of California, Davis, California 9561 6, USA (Received 17 October 1995; accepted 14 March 1996) Abstract A pruritic maculopapular eruption with clinical and histological features similar to urticaria pigmentosa of humans is reported in three related Sphinx cats. All cats shared the same grandsire, had a juvenile onset of disease, and demonstrated similar historical, clinical and histological findings. Physical examination revealed widespread bilaterally symmetrical, erythematous, partially coalescing, crusted macules and papules on the trunk, limbs, neck and head. A few macules exhibited a dark brown pigmentation. Dermatographism could not be elicited in any of the cats. Histological examination of papules revealed the presence of a perivascular to diffuse dermal and subcutaneous infiltrate of well-differentiated mast cells. In one cat where systemic involvement was pursued, evidence of internal disease was not found. Keywords: cat, mastocytosis, urticaria pigmentosa. INTRODUCTION CASE REPORTS Mastocytosis is a heterogeneous group of diseases Cats I and 2 defined as an abnormal proliferation of mast cells in A 1.5-year-old intact grey and white female Sphinx various organs including skin, liver, spleen, kidney or cat (cat 1) was presented to the University of gastrointestinal tract. 1-5 The infiltrating mast cells California Veterinary Medical Teaching Hospital may exhibit a benign or malignant behaviour. The (VMTH) with an approximately 1-year history of symptoms of mastocytosis are related to the release of pruritic papular lesions.
    [Show full text]
  • Congenital: Growths-Conditions-Vascular Lesions-Hair-Nails Epidermolysis-Ichthyosis-Mastocytosis-Neurofibromatosis-Tuberous Sclerosis-Xeroderma
    Congenital: Growths-Conditions-Vascular Lesions-Hair-Nails Epidermolysis-Ichthyosis-Mastocytosis-Neurofibromatosis-Tuberous sclerosis-Xeroderma Other congenital malformations (Q80-Q85) Q80 Congenital ichthyosis Excludes1: Refsum's disease (G60.1) Q80.0 Ichthyosis vulgaris Q80.1 X-linked ichthyosis Q80.2 Lamellar ichthyosis Collodion baby Q80.3 Congenital bullous ichthyosiform erythroderma Q80.4 Harlequin fetus Q80.8 Other congenital ichthyosis Q80.9 Congenital ichthyosis, unspecified Q81 Epidermolysis bullosa Q81.0 Epidermolysis bullosa simplex Excludes1: Cockayne's syndrome (Q87.1) Q81.1 Epidermolysis bullosa letalis Herlitz' syndrome Q81.2 Epidermolysis bullosa dystrophica Q81.8 Other epidermolysis bullosa Q81.9 Epidermolysis bullosa, unspecified Q82 Other congenital malformations of skin Excludes1: acrodermatitis enteropathica (E83.2) congenital erythropoietic porphyria (E80.0) pilonidal cyst or sinus (L05.-) Sturge-Weber (-Dimitri) syndrome (Q85.8) Q82.0 Hereditary lymphedema Q82.1 Xeroderma pigmentosum Q82.2 Mastocytosis Urticaria pigmentosa Excludes1: malignant mastocytosis (C96.2) Q82.3 Incontinentia pigmenti Q82.4 Ectodermal dysplasia (anhidrotic) Excludes1: Ellis-van Creveld syndrome (Q77.6) Q82.5 Congenital non-neoplastic nevus Birthmark NOS Flammeus Nevus Portwine Nevus Sanguineous Nevus Strawberry Angioma Strawberry Nevus Vascular Nevus NOS Verrucous Nevus Excludes2: Café au lait spots (L81.3) lentigo (L81.4) nevus NOS (D22.-) araneus nevus (I78.1) melanocytic nevus (D22.-) pigmented nevus (D22.-) spider nevus (I78.1) stellar
    [Show full text]
  • MASTER DERMATOLOGISTS* BASED on DISEASES NAMED AFTER THEM by HERMAN GOODMAN, M.D
    MASTER DERMATOLOGISTS* BASED ON DISEASES NAMED AFTER THEM By HERMAN GOODMAN, M.D. NEW YORK CITY OR little more than a year, I have lyric and dramatic poetry, sculpture and been collecting material for a architecture. Hippocrates lived in this paper on “Eponyms of Derma- period. The “Father of Recent Medicine” tology,”1 and have especially has icterus gravis named after him as arranged some of the data in concise formHippocratic morbus niger. Calvities Hippo- Fand in chronological order for the Anna ls cratica is the designation of a type of total of Medic al His tory . The names of the baldness and Hippocratic nails are those men cited have come down to us by reason resulting from chronic pulmonary and of the fact that one or more dermatologic cardiac disease. Hippocrates was born on entities or diseases have been named after the Island of Cos about 460 b .c . After them; literally, they are godfathers of the death of his father, he went to Athens dermatology. The names of many master for the study of surgery. The profession of dermatologists who did not have diseases medicine owes to him the art of clinical named after them have been omitted in inspection and observation. The physician’s this article, but I hope at some future date oath has been named the Hippocratic oath, to enlarge on the present study by present- but it is far-fetched to consider him its ing others on the eponyms of dermatologic author. Hippocrates was the founder of anatomy, pathology and therapeutics. Grecian dermatology. His influence per- sisted for over two thousand years.
    [Show full text]
  • Pediatric Blistering Diseases MSU/Beaumont Health Dermatology Residency Program
    Pediatric Blistering Diseases MSU/Beaumont Health Dermatology Residency Program Matthew Laffer, DO & Dustin Portela, DO PGY-4 Chelsea Duggan, DO & Peter Jajou, DO PGY-3 Sonam Rama, DO & Rachel Cetta, DO PGY-2 Steven Grekin, DO Program Director Disclosures • No conflicts of interest to disclose Overview • Infectious Disease • Drug Related Disorders • Hereditary Disorders • Autoimmune Conditions • Miscellaneous Disorders INFECTIOUS Staphylococcus Scalded Skin Syndrome • Clinical Presentation • Fever, skin tenderness and erythema in groin folds, axillary regions, nose, that leads to a generalized superficial exfoliative dermatitis and bullae • +Nikolsky sign. Perioral crusting and fissuring http://medlibes.com/entry/staphylococcal-scalded-skin-syndrome Staphylococcus Scalded Skin Syndrome • Pathogenesis • Staphylococcus aureus group 2 phage (71) infects child at a distant site • Exfoliative toxins/Epidermolytic toxins A and B (ET-A, ET-B) bind to desmoglein-1 superficial bullae within the granular layer (subcorneal) • ET is a unique serine protease that shows lock and key specificity to desmoglein -11-2 • Diagnosis • Mainly clinical • Culture of the bullae will be NEGATIVE. Must culture site of initial infection • Treatment • Supportive care: IVF!! • Dicloxacillin Bullous Impetigo • Clinical Presentation • Flaccid bullae which rupture and leave honey colored crust behind • No surrounding erythema • Can have systemic manifestations such as malaise, diarrhea http://dermatologyoasis.net/bullous-impetigo-2/ http://www.adhb.govt.nz/newborn/TeachingResources/Dermatology/InfectiveLesions.htm
    [Show full text]