67: Scleredema and Scleromyxedema Flashcards
What are the key epidemiological differences between Scleredema and Scleromyxedema?
Both conditions are rare and affect males and females equally. Scleredema typically has an onset in childhood or adolescence, while Scleromyxedema usually presents in mid to late adulthood.
What are the associated conditions for Scleredema?
Associated conditions for Scleredema include:
- Postinfectious (URTI - streptococcal) in children
- Adult onset diabetes mellitus
- Paraproteinemia, multiple myeloma
- Hyperthyroidism, connective tissue diseases, HIV
What are the clinical findings of Scleromyxedema?
Clinical findings of Scleromyxedema include:
- Generalized lichenoid eruption of minute (1-3mm) papules on extremities and trunk
- Localized confluent lichenoid plaques with possible erythema or hyperpigmentation
- ‘Bovine facies’
- Muscle weakness, contractures, and various systemic involvements such as pulmonary hypertension and neurologic disorders.
What is the pathogenesis of Scleredema?
The pathogenesis of Scleredema involves the deposition of Type 1 collagen and hyaluronate, leading to excessive mucin and collagen deposition.
What are the treatment options for Scleromyxedema?
Treatment options for Scleromyxedema include:
- Melphalan
- Glucocorticoids
- IVIG
- Thalidomide
- Extracorporeal photopheresis
- Interferon-α
- Combination chemotherapy
- PUVA therapy
- Autologous peripheral blood stem cell transplantation for severe cases.
What histopathological features are observed in Scleredema?
Histopathological features of Scleredema include:
- Nontapered/square appearance
- Decreased number or higher placement of eccrine units
- Normal fibroblast number and morphology
- Slightly thickened collagen bundles separated by mucin deposits
- Positive for Alcian blue and colloidal iron.
What is the likely diagnosis for a patient with nonpitting induration of the neck and shoulders, and what associated conditions should be investigated?
The likely diagnosis is scleredema. Associated conditions to investigate include postinfectious causes (e.g., streptococcal URTI in children), adult-onset diabetes mellitus, paraproteinemia, multiple myeloma, hyperthyroidism, connective tissue diseases, and HIV.
What is the expected course of scleredema associated with adult-onset diabetes mellitus, and how might it improve?
The course of scleredema associated with adult-onset diabetes mellitus is typically protracted but may improve with glucose control.
What systemic complications should be monitored in a patient with scleromyxedema presenting with restrictive lung disease and esophageal dysmotility?
Other systemic complications to monitor include muscle weakness, contractures, upper airway involvement, pulmonary hypertension, neurologic disorders (e.g., seizures, motor impairment, carpal tunnel syndrome, depression, memory loss, aphasia, peripheral neuropathy, psychosis).
What histopathological features differentiate scleromyxedema from nephrogenic systemic fibrosis?
Scleromyxedema is characterized by mucin deposition restricted to the upper half of the dermis and does not involve the pannicular septae. In contrast, nephrogenic systemic fibrosis shows more prominent involvement in the lower dermis and includes the pannicular septae.
What is the likely diagnosis for a child with acute onset of nonpitting induration following a streptococcal upper respiratory tract infection, and what is the prognosis?
The likely diagnosis is postinfectious scleredema. The prognosis is generally good, with the condition abating in 1-2 years.
What treatment options are available for severe scleromyxedema with a monoclonal paraproteinemia of the IgG-λ type?
Treatment options for severe scleromyxedema include glucocorticoids, IVIG, thalidomide, extracorporeal photopheresis, interferon-α, combination chemotherapy, PUVA, and autologous peripheral blood stem cell transplantation.
What advanced treatment modalities can be considered for a patient with scleredema associated with gammopathy?
Advanced treatment modalities for gammopathy-associated scleredema include extracorporeal photopheresis, IVIG, and melphalan.
What is the likely diagnosis for a patient with a lichenoid eruption of minute papules scattered on the extremities and trunk, and what are the histopathological findings?
The likely diagnosis is generalized scleromyxedema. Histopathological findings include superficial to mid-dermal mucin deposition, fibroblast proliferation, mild perivascular and interstitial T-cell infiltrate, and multinucleated histiocytes with or without elastophagocytosis.
What is the prognosis for a patient with a localized variant of scleredema, and what precautions should be taken?
The localized variant of scleredema is self-limiting, but follow-up is advised as it can be associated with internal organ involvement or progression to more generalized disease (atypical papular mucinosis).
What systemic diseases should be ruled out for a patient with scleromyxedema and normal thyroid function?
Systemic diseases to rule out include monoclonal paraproteinemia (IgG-λ type), multiple myeloma, and connective tissue diseases.
What is the underlying pathogenesis for a patient with scleredema presenting with prominent follicular ostia and a peau d’orange appearance?
The underlying pathogenesis involves excessive deposition of type 1 collagen and hyaluronate.
What treatments could be considered for a patient with scleromyxedema undergoing PUVA phototherapy if the condition is refractory?
If refractory, treatments such as glucocorticoids, IVIG, thalidomide, extracorporeal photopheresis, interferon-α, combination chemotherapy, and autologous peripheral blood stem cell transplantation could be considered.
What organ systems should be evaluated for involvement in a patient with scleredema and a history of restrictive lung disease?
Other organ systems to evaluate include the upper airway, pulmonary system (for hypertension), esophagus (for dysmotility), and the nervous system (for seizures, motor impairment, carpal tunnel syndrome, depression, memory loss, aphasia, peripheral neuropathy, psychosis).
What histopathological staining techniques can confirm the diagnosis of scleromyxedema in a patient with a lichenoid plaque and marked erythema?
Histopathological staining techniques such as Alcian blue and colloidal iron can confirm the diagnosis by highlighting mucin deposition.
What is the expected prognosis for a patient with chronic, treatment-resistant scleredema?
The prognosis for chronic, treatment-resistant scleredema is poor, especially if associated with respiratory failure, cerebral disease, or infection.
What are the characteristic clinical findings of scleromyxedema for a patient with bovine facies?
Characteristic clinical findings include smooth, waxy skin with tense dermal induration, prominent follicular ostia, and generalized or localized lichenoid eruptions.
What treatment options should be prioritized for a patient with scleredema and a history of multiple myeloma?
Treatment options should prioritize addressing the associated disease, such as using melphalan for multiple myeloma.
What other histological features are expected in a patient with scleromyxedema who has a mild perivascular and interstitial T-cell infiltrate?
Other histological features include superficial to mid-dermal mucin deposition, fibroblast proliferation, and multinucleated histiocytes with or without elastophagocytosis.
