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Cutaneous Mastocytoma

Solitary Mastocytoma


Presentation

  • A 50-day-old female child presented with a skin colored raised lesion on the dorsal aspect of the left wrist [Figure 1] since the age of 10 days, which gradually increased in size to attain the present size.[e-ijd.org]
  • Microscopically, an accumulation of mast cells, microgranulomas, eosinophils and 'flame figures' was present. A pathogenesis involving mast cell degranulation, eosinophil chemotactic factors and eosinophil major basic protein is discussed.[ncbi.nlm.nih.gov]
  • They usually present within 2 years of age, mostly within first 3 months 1.[f1000research.com]
  • Clinical description Patients generally present during infancy, with most presenting in the first three months of life. However, rare cases of onset in adulthood have been reported.[orpha.net]
Myxedema
  • Polymorphic light eruption 643 197 Porokeratoses 646 198 Porphyria cutanea tarda 650 199 Port wine stain nevus flammeus 653 200 Postinflammatory hyperpigmentation and other disorders of hyperpigmentation 658 201 Pregnancy dermatoses 662 202 Pretibial myxedema[books.google.com]
Relapsing Polychondritis
  • polychondritis 733 219 Rhinophyma 737 220 Rocky Mountain spotted fever and other rickettsial infections 739 221 Rosacea 744 222 Sarcoidosis 752 223 Scabies 757 224 Scleredema 761 225 Scleroderma 764 226 Sebaceous hyperplasia 769 227 Seborrheic eczema[books.google.com]
Yellow Nails
Necrolytic Migratory Erythema
  • migratory erythema 554 168 Nephrogenic systemic fibrosis 557 169 Neurofibromatosis type 1 559 170 Nevoid basal cell carcinoma syndrome 562 171 Nevus sebaceus 567 172 Notalgia paresthetica 569 173 Onchocerciasis 571 174 Oral lichen planus 574 175 Orf[books.google.com]
Trichotillomania
  • […] capitis 816 242 Tinea pedis and skin dermatophytosis 820 243 Tinea unguium 823 244 Tinea versicolor pityriasis versicolor 827 245 Toxic epidermal necrolysis and StevensJohnson syndrome 830 246 Transient acantholytic dermatosis Grover disease 834 247 Trichotillomania[books.google.com]

Workup

  • .: Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options. J Hematology & Oncology March 2011. [ ] 5.[mastocytosis.ca]
  • Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options. J Hematol Oncol, 4(10), 10.1186/1756-8722-4-10 [ii] Hartmann K, et al. (2016).[mastattack.org]
  • A bone scan, GI workup, and identification of the D816V c-kit mutation can also be helpful in cases where the diagnosis requires confirmation.[merckmanuals.com]
  • The pattern of aeroallergen and food sensitization and asthma in atopic patients with mastocytosis did not differ from that found in patients without mastocytosis but a comprehensive allergic workup (including skin prick testing, total and specific IgE[worldallergy.org]
Staphylococcus Aureus
  • aureus and PantonValentine leukocidin Staphylococcus aureus infections 499 154 Miliaria 505 155 Molluscum contagiosum 508 156 Morphea 512 157 Mucoceles 515 158 Mucous membrane pemphigoid 517 eumycetoma and actinomycetoma 522 160 Mycobacterial atypical[books.google.com]

Treatment

  • Conventional treatment with antihistamines was not completely effective and caused sedation.[ncbi.nlm.nih.gov]
  • Management and treatment Treatment is symptomatic and includes oral administration of antihistamines, topical steroids and the use of hydrocolloid dressings to cover the lesions.[orpha.net]
  • When it did not resolve, I sought treatment. I was initially given Triamcinilone cream, which I used for about 6 months, to no great avail.[medhelp.org]
  • Ameet Dandale were involved in clinical diagnosis, work-up, treatment and writing up of this case report.[f1000research.com]
  • […] of advanced mastocytosis; Treatment strategies of mediator related symptoms in mastocytosis; and Neuro and psychological involvement in Mastocytosis.[books.google.com]

Prognosis

  • Prognosis The prognosis for patients with onset during infancy is good with regression of most lesions during childhood and complete resolution by adolescence. Rare cases of persisting mastocytoma in adulthood have been reported.[orpha.net]
  • Articles in this issue include Human Mast Cell Signal Transduction; Mast cell tryptase role in homeostasis and coagulation; Mastocytosis: Current Classification and Diagnostic criteria; Epidemiology, risk factors and prognosis of mastocytosis; Mast cell[books.google.com]
  • Most mastocytosis cases have a good overall prognosis and are characterized by low mast cell burden, particularly at the early stages of the disease with limited or no systemic involvement.[consultant360.com]
  • Fortunately, the vast majority of children with solitary mastocytomas have a good prognosis with reduction or complete resolution of symptoms by puberty. [10] Due to spontaneous involution, solitary mastocytomas rarely remain symptomatic in older children[e-ijd.org]
  • In SM-AHNMD, the prognosis depends on the course of the associated haematological disorder. Agressive systemic mastocytosis and mast cell leukaemia have a poorer prognosis.[patient.info]

Etiology

  • Etiology Mutations in the KIT gene (4q11-q12) have been identified in some patients with CM, however, this mutation is rare in the pediatric population and the etiology and pathogenesis of cutaneous mastocytoma remains to be determined.[orpha.net]
  • Etiology in many patients involves an activating mutation (D816V) in the gene coding for the stem cell factor receptor c-kit, which is present on mast cells.[merckmanuals.com]
  • Therefore, the etiological diagnosis remains presumptive until skin testing is performed.[anesthesiology.pubs.asahq.org]
  • Etiology The etiology of mastocytosis remains unknown. Children who develop this disease usually have a self-limited disorder, whih often resolves before adulthood.[sdominko.tripod.com]
  • Etiology Mastocytosis probably is a hyperplastic response to an abnormal stimulus.[emedicine.medscape.com]

Epidemiology

  • Articles in this issue include Human Mast Cell Signal Transduction; Mast cell tryptase role in homeostasis and coagulation; Mastocytosis: Current Classification and Diagnostic criteria; Epidemiology, risk factors and prognosis of mastocytosis; Mast cell[books.google.com]
  • Summary Epidemiology Prevalence is unknown but mastocytomas are the second most frequent form of CM in children, accounting for 10-15% of cases.[orpha.net]
  • Mastocytosis (cutaneous and systemic): Epidemiology, pathogenesis, and clinical manifestations. . Accessed Feb. 19, 2017. AskMayoExpert. Systemic mastocytosis. Rochester, Minn.: Mayo Foundation for Medical Education and Research; 2016. Brown AY.[mayoclinic.org]
  • Mastocytosis (cutaneous and systemic): Epidemiology, pathogenesis, and clinical manifestations. UpToDate. December 5, 2016; . Habashy J and Robles DT. Mastocytosis. Medscape Reference. May 15, 2017; . Fett NM, Teng J, and Longley BJ.[rarediseases.info.nih.gov]
  • A mutation of the oncogene C-KIT D816V is found in many (but not all) cases. [ 9 ] Epidemiology [ 10 ] This is a rare and heterogeneous group of disorders, of which urticaria pigmentosa is the most common manifestation.[patient.info]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology Mastocytosis is now classified with the myeloproliferative neoplasms. [5] Increased local concentrations of soluble mast cell growth factor in lesions of cutaneous mastocytosis are believed to stimulate mast cell proliferation, melanocyte[emedicine.medscape.com]
  • Pathophysiology of mastocytosis. IL-1 interleukin-1; IL-6 interleukin-6; LTC4 leukotriene C4; PGD2 prostaglandin D2; TNF-α tumor necrosis factor-α. Fig. 1. Pathophysiology of mastocytosis.[anesthesiology.pubs.asahq.org]
  • Increased stomach acid production causing peptic ulcers (increased stimulation of enterochromaffin cell and direct histamine stimulation on parietal cell ) Malabsorption (due to inactivation of pancreatic enzymes by increased acid) [5] Hepatosplenomegaly Pathophysiology[en.wikipedia.org]
  • In addition to the age of onset, adult and pediatric forms of mastocytosis differ in their clinical course and pathophysiology.[sdominko.tripod.com]
  • Pathophysiology Mast cells originate from bone marrow progenitor cells and are distributed throughout the connective tissues. They are concentrated in the skin around the peripheral nerves and adjacent to blood and lymphatic vessels.[aafp.org]

Prevention

  • In most cases, only yearly checkups are necessary, and no treatment is required; preventive recommendations are warranted in individuals with systemic disease and constitutional symptoms. 6 There is no cure for more severe forms of mastocytosis, but a[consultant360.com]
  • The goal of management of a symptomatic solitary mastocytoma is to prevent the release of mast cell mediators and alleviate symptoms associated with mediator release, particularly pruritus.[e-ijd.org]
  • Treatment was continued with only a once a day application of topical tacrolimus for a month after clinical resolution to prevent further recurrence ( Figure 4 ).[f1000research.com]
  • Can mastocytosis be prevented or avoided? There is no way to completely avoid mastocytosis. But you may be able to help prevent symptoms by learning what triggers mastocytosis for you.[familydoctor.org]
  • Follow-up physical examinations and skin evaluations are important in the early diagnosis and prevention of this condition.[mastocytosisaustralasia.com]

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