Patients affected by FUMHD present with necrotic papules that progress to plaques and large coalescent ulcers. [statpearls.com]

Minor constitutional symptoms may be present. A patient with febrile ulceronecrotic PLEVA presents with acute constitutional symptoms such as high fever, malaise, and myalgias. Lesions of PLEVA may be associated with burning and pruritus. [emedicine.medscape.com]

Case Report A 49-year-old male, milk vendor by occupation, presented with erythematous papules and crusted plaques all over the body and ulcerations in the oro-genital tract of 15 days duration. [ijdvl.com]

[…] criteria of PLEVA: Somewhat larger papules of around 5-15mm Present on torso and proximal extremetires Itching may be present Ulceration may be present Both types of lesions can exist on the same patient at the same time; a biopsy is required to conclusively [pityriasislichenoides.com]

As PLC presents as small papules with scaling, pityriasis rosea and psoriasis need to be ruled out. [indianpediatrics.net]

• Cervical Lymphadenopathy

  Patients typically present with cervical lymphadenopathy, local tenderness, fever, and sweats, and cutaneous involvement has been described (as reviewed by Bosch et al 72 ). [academic.oup.com]

• Fever

  Here, we report a 31-year-old patient with neurofibromatosis who had generalised papulosquamous lesions especially on flexural areas, accompanying fever, malaise and muscle pain. [bibliomed.org]

  At the next clinic visit, the patient reported a fever of 39.4 °C. After reviewing the patient’s histopathology and clinical picture, along with the presence of fever, a final diagnosis of FUMHD was made. [mdedge.com]

  Pityriasis lichenoides with ulceronecrosis and hyperthermia (PLUH) is a severe variant of pityriasis lichenoides et varioliformis acuta that is characterized by high fever and papulo-necrotic skin lesions. [ncbi.nlm.nih.gov]

• Pain

  Here, we report a 31-year-old patient with neurofibromatosis who had generalised papulosquamous lesions especially on flexural areas, accompanying fever, malaise and muscle pain. [bibliomed.org]

  Skin ulceration can be extensive and painful, including oral and genital mucosal.[16] Common systemic symptoms include high fever, abdominal pain, diarrhea, central nervous system manifestations, joint pain, respiratory complications, and death.[16] Evaluation [statpearls.com]

  There is usually an accompanying high fever with painful skin tissue loss, and many signs including sore throat, abdominal pain, splenomegaly (enlarged spleen), conjunctival ulcers, and other nonspecific symptoms. [news-medical.net]

  In this condition, painful red papules with fluid accumulation form on the skin. Some ulcerate and bleed and form scars on healing. [dovemed.com]

  However, some people experience fever, headaches, joint pain and/or swelling of nearby lymph nodes. [2] There is a rare subtype of PLEVA called febrile ulceronecrotic Mucha-Haberman disease (FUMHD) in which papules rapidly develop into painful, large, [rarediseases.info.nih.gov]

• Constitutional Symptom

  After a period of a mild eruption, extensive polymorphous, papular and ulcerohemorrhagic skin lesions developed, associated with intermittent high temperature and constitutional symptoms. [ncbi.nlm.nih.gov]

  Minor constitutional symptoms may be present. A patient with febrile ulceronecrotic PLEVA presents with acute constitutional symptoms such as high fever, malaise, and myalgias. Lesions of PLEVA may be associated with burning and pruritus. [emedicine.medscape.com]

  Constitutional symptoms are usually mild (see complications below). [dermnetnz.org]

  There was no fever or constitutional symptoms. He had received measles rubella vaccine 7 days prior to the onset of lesions. He was not on any medications, and his previous vaccinations were uneventful. [ijdvl.com]

• Swelling

  Syphilis: Syphilides are usually accompanied by LK swelling, appearance rather monomorphic, frequent involvement of palms and face. Serology is conclusive! Histology is conclusive (plasma cell-rich dermatitis). [altmeyers.org]

  […] microbes out, preventing infections Helps you feel things like heat, cold, and pain Keeps your body temperature even Makes vitamin D when the sun shines on it Anything that irritates, clogs, or inflames your skin can cause symptoms such as redness, swelling [icdlist.com]

  Marked upper and deep dermal perivascular lyphocytic inflammation (predominantly CD4+ T-cells) Frequently marked exocytosis of lymphocytes into the epidermis Fully developed lesions may show dermal and epidermal hemorrhage along with endothelial cell swelling [dermpedia.org]

  He had soreness and swelling of the arms and legs. A prior 12-day course of prednisone prescribed by a community dermatologist failed to improve the rash. A biopsy performed by a community dermatologist was nondiagnostic. [mdedge.com]

• Malaise

  Here, we report a 31-year-old patient with neurofibromatosis who had generalised papulosquamous lesions especially on flexural areas, accompanying fever, malaise and muscle pain. [bibliomed.org]

  Here, we report a 31-year-old patient with neurofibromatosis who had generalised papulosquamous lesions especially on flexural areas, accompanying fever, malaise and muscle pain. Key words: Pityriasis lichenoides et varioliformis acuta [scopemed.org]

  A rare severe form of PLEVA (Febrile Ulceronecrotic Mucha–Habermann Disease) has systemic features of malaise, fever, lymphadenopathy, arthritis, and/or bacteraemia. There may be mucosal, gastrointestinal, and pulmonary involvement. [dermnetnz.org]

  Patients sometimes have malaise and low grade fever before the eruption. Lesions may occur anywhere except for the mouth. A skin biopsy may help reveal the diagnosis. [randyjacobsmd.com]

• Sore Throat

  There is usually an accompanying high fever with painful skin tissue loss, and many signs including sore throat, abdominal pain, splenomegaly (enlarged spleen), conjunctival ulcers, and other nonspecific symptoms. [news-medical.net]

  Systemic symptoms may include high fever, sore throat, diarrhoea, abdominal pain, central nervous system symptoms, lung disease, enlarged spleen, arthritis, sepsis, anaemia, and conjunctival ulcers. [dermnetnz.org]

  There was no history of drug intake or sore throat prior to the onset of illness. There were no associated complaints of any joint pains, dysuria, respiratory or abdominal problem. There was no history of any extramarital contact. [ijdvl.com]

  People with FUMHD may also have symptoms such as high fever, sore throat, diarrhea, abdominal pain, neurologic abnormalities, lung disease, and other symptoms. [rarediseases.info.nih.gov]

• Pharyngitis

  We encountered a boy with periodic fever, aphthous-stomatitis, pharyngitis, adenitis syndrome, complicated by a papular rash representing pityriasis lichenoides et varioliformis acuta. [ncbi.nlm.nih.gov]

  + In 1989, Edwards et al described a child with a 3-week history of migratory arthralgias, monoarticular arthritis, acute pharyngitis, otitis media, and fevers to 104ºF.8 The girl developed a vesicular eruption localized primarily to the extremities [checkorphan.org]

  In 1989, Edwards et al described a child with a 3-week history of migratory arthralgias, monoarticular arthritis, acute pharyngitis, otitis media, and fevers to 104ºF. [7] The girl developed a vesicular eruption localized primarily to the extremities, [emedicine.com]

  Suspected infectious triggers include varicella zoster virus (VZV) [ 6 ], Human herpesvirus (HHV)-8 [ 7 ], streptococcal pharyngitis, Toxoplasma gondii, parvovirus B19, Epstein-Barr virus (EBV), and human immunodeficiency virus (HIV) [ 8 ]. [link.springer.com]

• Diarrhea

  Skin ulceration can be extensive and painful, including oral and genital mucosal.[16] Common systemic symptoms include high fever, abdominal pain, diarrhea, central nervous system manifestations, joint pain, respiratory complications, and death.[16] Evaluation [statpearls.com]

  People with FUMHD may also have symptoms such as high fever, sore throat, diarrhea, abdominal pain, neurologic abnormalities, lung disease, and other symptoms. [rarediseases.info.nih.gov]

• Arthritis

  Etanercept-induced pityriasis lichenoides chronica in a patient with rheumatoid arthritis. Case Rep Dermatol Med. 2015. 2015:168063. [Medline]. Said BB, Kanitakis J, Graber I, Nicolas JF, Saurin JC, Berard F. [emedicine.medscape.com]

  NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases Acrodermatitis (Medical Encyclopedia) Cryotherapy (Medical Encyclopedia) Cutaneous skin tags (Medical Encyclopedia) Dry skin -- self-care (Medical Encyclopedia) Erythema multiforme [icdlist.com]

  A rare severe form of PLEVA (Febrile Ulceronecrotic Mucha–Habermann Disease) has systemic features of malaise, fever, lymphadenopathy, arthritis, and/or bacteraemia. There may be mucosal, gastrointestinal, and pulmonary involvement. [dermnetnz.org]

  Code L21.0 Seborrhea capitis 2016 2017 2018 2019 Billable/Specific Code lichenoides L41.0 et varioliformis L41.0 (acuta) ICD-10-CM Codes Adjacent To L41.0 L40.5 Arthropathic psoriasis L40.51 Distal interphalangeal psoriatic arthropathy L40.52 Psoriatic arthritis [icd10data.com]

• Papule

  a disease of unknown cause that is characterized by the sudden appearance of polymorphous lesions (as papules, purpuric vesicles, crusts, or ulcerations) resembling chicken pox but tending to persist from a month to as long as years, occurs especially [merriam-webster.com]

  Multiple red–to–reddish brown or purpuric round papules that are 5-15 mm in diameter. [dermpathdiagnostics.com]

  Mucha-Habermann disease corresponds to the acute form and is characterised by recurrent erythematous, round papules, hemorrhage, blisters and varioliform vesiculopustules. [dermis.net]

  Patients affected by FUMHD present with necrotic papules that progress to plaques and large coalescent ulcers. [statpearls.com]

• Eruptions

  Patients present with a spontaneous eruption of erythematous macules that evolve into papules, rapidly undergoing hemorrhagic necrosis and ulceration. The disease tends to wax and wane with complete resolution, varying in weeks to years. [statpearls.com]

  An immune disorder of acute onset, which is more common in young men and characterised by successive waves of maculopapular, erythematous eruptions on the anterior trunk and flexor surfaces of the proximal parts of the extremities. [medical-dictionary.thefreedictionary.com]

  PLEVA most commonly affects the areas of the trunk, buttocks, and proximal extremities with eruptions occurring in successive crops. The lesions resolve with transient hyperpigmentation, hypopigmentation, and/or varioliform [like chickenpox] scars. [dermpathdiagnostics.com]

  No first-line treatment scheme succeeded in controlling the eruption of new lesions. The only therapeutic approach that eventually managed to cease the disease evolution was the combination of prednisolone and methotrexate. [ncbi.nlm.nih.gov]

• Ulcer

  Patients affected by FUMHD present with necrotic papules that progress to plaques and large coalescent ulcers. [statpearls.com]

  a disease of unknown cause that is characterized by the sudden appearance of polymorphous lesions (as papules, purpuric vesicles, crusts, or ulcerations) resembling chicken pox but tending to persist from a month to as long as years, occurs especially [merriam-webster.com]

  Ulcerations with hemorrhagic crusting were present at the margins of the nose as well as on the upper and lower lids. Oral mucosa showed multiple superficial and deep ulcers at angles of the lips, tongue and buccal mucosa. [ijdvl.com]

  These rapidly disintegrate, with lesional formation of erosions, ulcers, and hemorrhagic crusts. Hemorrhagic vesicles are less common. [altmeyers.org]

  Abstract: G.c. an 8 year old boy from Senglea, presented in July 1983 with crops of erythematous papules rapidly evolving into vesicles and ulcers with haemorrhagic crusts. [um.edu.mt]

• Skin Disease

  Abstract Pityriasis lichenoides et varioliformis acuta (PLEVA), is a sudden onset skin disease characterized by diffuse papulosquamous lesions with unknown etiology. [bibliomed.org]

  Lymphomatoid papulosis, for example, is a skin disease that mimics PLEVA, but the separation of the two must be emphasized because of differences in prognosis. [statpearls.com]

  Diseases Case method Issue Date: 1985 Publisher: University of Malta Medical School Citation: Vella Briffa, D. (1985). [um.edu.mt]

• Skin Lesion

  After a period of a mild eruption, extensive polymorphous, papular and ulcerohemorrhagic skin lesions developed, associated with intermittent high temperature and constitutional symptoms. [ncbi.nlm.nih.gov]

  The skin lesions completely resolved with 8 months of PUVA therapy. [tandfonline.com]

  lesion removal (Medical Encyclopedia) Skin lesion removal-aftercare (Medical Encyclopedia) Stasis dermatitis and ulcers (Medical Encyclopedia) [ Read More ] [icdlist.com]

• Burning Sensation

  sensation Some seasonal variation, worsening in winter and some improvement in sunlight Clinical images Images hosted on PathOut servers: Arms, courtesy of Mark R. [pathologyoutlines.com]

  sensation Most cutaneous lesions heal with transient or persistent hyper- or hypo-pigmentation. [dermnetnz.org]

  The papules may progress to form vesicles, pustules, and ulcers, and these lesions can be associated with pruritus or a burning sensation. PLEVA favors the trunk and proximal extremities, especially in the flexural regions. [statpearls.com]

  No symptoms are often experienced in PLC, but in PLEVA, the spots may itch, irritate, or cause a burning sensation as they appear. How will pityriasis lichenoides be diagnosed? The appearance of the rash will suggest the diagnosis. [skinhealthinfo.org.uk]

  As opposed to PLC, which is symptom less, PLEVA causes itching and burning sensation in the lesions. [epainassist.com]

Progress logically from the histologic pattern, through the appropriate workup, around the pitfalls, to the best diagnosis. [books.google.it]

The laboratory workup found a slightly elevated C-reactive protein (13mg/l; normal<3) with normal blood cell counts, liver and renal chemistries and negative antinuclear antibodies. [actasdermo.org]

As a result, thoughtful, individualized workup, clinicopathologic correlation, counseling and therapeutic management is essential, as one approach does not fit all patients. [link.springer.com]

Diagnostics Laboratory workup largely is a function of the acuity of the disease. [emedicine.medscape.com]

• Neutrophilia

  This severe variant of PLEVA can cause fatalities. [7] None of the patients in this study had any systemic abnormalities other than raised ESR and neutrophilia seen in five cases of PLEVA. [ijdvl.com]

• Lymphocytic Infiltrate

  Fixed drug reaction: apoptotic keratinocytes, vacuolated junctional zone, satellite necrosis, perivascular lymphocytic infiltrate. [altmeyers.org]

  Immunoperoxidase studies have shown the lymphocytic infiltrate consists of CD8/ cytotoxic T cells. Differential diagnosis of PLEVA Cutaneous T-cell lymphoma Lymphocytic vasculitis See smartphone apps to check your skin. [dermnetnz.org]

  infiltrate. 3- Leukocytoclastic vasculitis. the presence of vasculitic changes is consistent with the febrile variant of Mucha Habermann disease. [interactivederma.com]

  An amorphous brownish structure corresponds to a central crust consisting of basophilic material in the epidermis and wedge-shaped lymphocytic infiltrate in the dermis. [ncbi.nlm.nih.gov]

  Common Microscopic Features: - Lymphocytic infiltrate with mixed neutrophils and histiocytes, - Exocytosis, - Parakeratosis, - Red blood cell extravasation and - Necrosis of keratinocytes with intraepidermal vesicles. [histopathology-india.net]

Additionally, extensive studies may be warranted if the course of PLEVA has progressed to PLC and is unresponsive to treatment. [statpearls.com]

There is no single treatment for PLEVA, though phototherapy has been reported as useful; multiple oral medications including tetracycline, dapsone and pentoxifylline have been helpful and potent topicals can be used on lesions. [dermpathdiagnostics.com]

The papules typically heal without treatment within several weeks. But they may recur. [web.archive.org]

Doxycycline treatment and continuing treatment with bethametasone and gentamicin were recommended to the patient. [hrcak.srce.hr]

Lymphomatoid papulosis, for example, is a skin disease that mimics PLEVA, but the separation of the two must be emphasized because of differences in prognosis. [statpearls.com]

These cases highlight the importance of early recognition and treatment of this disease to prevent further morbidity and a potentially fatal prognosis. [ncbi.nlm.nih.gov]

The prognosis of mild condition is typically excellent. [dovemed.com]

Abstract Pityriasis lichenoides et varioliformis acuta (PLEVA), is a sudden onset skin disease characterized by diffuse papulosquamous lesions with unknown etiology. [bibliomed.org]

This rash tends to relapse and remit with variable duration, sometimes lasting up to years.[1] Etiology The exact etiology of PLEVA is not known, although there have been various hypotheses noted in the literature, including an atypical immune response [statpearls.com]

Epidemiology Most often affects adolescents and young adults, appearing before the age of 30 Etiology Unknown; Linked to viruses, vaccines and other illness Pathogenesis Appears as multiple papules that evolve into vesicles and hemorrhagic crusts Clinical [dermpathdiagnostics.com]

Till date only 31 cases of PLUH have been reported in English literature. [2] Although the exact etiology for PLUH is unknown, several reports have suggested the possibility of hypersensitivity to an infectious agent. [ijdvl.com]

Epidemiology Most often affects adolescents and young adults, appearing before the age of 30 Etiology Unknown; Linked to viruses, vaccines and other illness Pathogenesis Appears as multiple papules that evolve into vesicles and hemorrhagic crusts Clinical [dermpathdiagnostics.com]

You might also be interested in Dermatology Occurrence/Epidemiology This section has been translated automatically. Incidence is 1:6000/1:12,000 Etiopathogenesis This section has been translated automatically. Unknown (immune complex vasculitis?). [altmeyers.org]

Neurology and Neurosurgery Czech and Slovak Ophthalmology Czech and Slovak Psychiatry Czech Geriatric Review Czech Gynaecology Czech Rheumatology Czech Dental Journal Czech Urology Czech-Slovak Dermatology Czecho-Slovak Pathology Czech-Slovak Pediatrics Epidemiology [prolekare.cz]

(See "Epidemiology, clinical manifestations, and diagnosis of herpes simplex virus type 1 infection", section on 'Eczema herpeticum' and "Epidemiology, clinical manifestations, and diagnosis of herpes simplex virus type 1 infection".) [medilib.ir]

The disease does not discriminate by race or gender and mainly affects children and young adults, with a slight male predominance.[1] Pathophysiology Due to the ambiguity of the etiology of PLEVA, there are various theories regarding the pathophysiology [statpearls.com]

Pathophysiology, classification, and treatment. Am J Clin Dermatol. 2007;8:29-36. [scielo.br]

Pityriasis lichenoides: pathophysiology, classification, and treatment. Am J Clin Dermatol. 2007;8:29–36. CrossRef PubMed Google Scholar López-Villaescusa MT, Hernández-Martín A, Colmenero I, Torrelo A. Pityriasis lichenoides in a 9-month-old boy. [link.springer.com]

Historical Perspective Classification Pathophysiology Causes Differentiating Pityriasis Lichenoides et Varoliformis Acuta from other Diseases It is commonly misdiagnosed as chickenpox or rosacea. [wikidoc.org]

These cases highlight the importance of early recognition and treatment of this disease to prevent further morbidity and a potentially fatal prognosis. [ncbi.nlm.nih.gov]

Your skin Holds body fluids in, preventing dehydration Keeps harmful microbes out, preventing infections Helps you feel things like heat, cold, and pain Keeps your body temperature even Makes vitamin D when the sun shines on it Anything that irritates [icdlist.com]

[…] a chronic condition that needs sometimes referred to as parapsoriasis variegata or parapsoriasis lichenoides.Pityriasis lichenoides chronica information including symptoms, diagnosis, misdiagnosis, treatment, causes, patient stories, videos, forums, prevention [copacopyqupi.j.pl]

However, chronic and severe cases are difficult to treat Please find comprehensive information on Acute Pityriasis Lichenoides regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention, [dovemed.com]

Although there’s no known cure, a diet that supports your immune system may be able to reduce symptoms and help prevent potentially serious complications. [livestrong.com]