Dermatopathia pigmentosa reticularis


Dermatopathia pigmentosa reticularis

Description, Causes and Risk Factors:

ICD-10-DC: Q82.4, R23/R23.1

A persistent purplish network-patterned discoloration of the skin caused by dilation of capillaries and venules due to stasis or changes in underlying blood vessels including hyalinization; rarely appears as a developmental defect.

The term dermatopathia pigmentosa reticularis (DPR) refers to a reddish-violet reticular discoloration of the skin that mainly affects the limbs. It is caused by an interruption of blood flow in the dermal arteries, either due to spasm, inflammation, or vascular obstruction, and is associated with diseases of varying etiology and severity. To establish the cause of r dermatopathia pigmentosa reticularis, it is essential to determine its course (chronic, acute, or fulminant), the presence of other cutaneous signs such as nodules, retiform purpura or necrosis, and the possible association of general symptoms or laboratory findings that suggest a particular systemic process.

Dermatopathia pigmentosa reticularis

The term dermatopathia pigmentosa reticularis is used to describe a reticular red-violaceous discoloration of the skin that typically affects the limbs, although it can also be generalized. It is secondary to organic or functional disorders of the dermal arteries or arterioles. Because arterioles can be affected by numerous conditions, dermatopathia pigmentosa reticularis has many possible causes. The condition has its origins in the reduction or interruption of blood flow at certain points in the path of the blood vessels due to spasm, inflammation of the arteriolar wall, or vascular obstruction.

Vascular obstruction can, in turn, be caused by thrombosis, embolic events, or vessel wall abnormalities.

To understand the events associated with dermatopathia pigmentosa reticularis, it is necessary to be familiar with the Anatomy & Physiology of the cutaneous blood vessels. The skin, like any organ, receives its blood supply via the arteries, which branch into arterioles at the junction between the dermis and the subcutaneous cellular tissue to form the deep vascular plexus, which runs parallel to the epidermis. This plexus is formed by the feeding artery and a network of arterioles measuring 100 µm in diameter; the walls of these vessels consist of 2 outer layers of muscle, an elastic lamina, and internally, the vascular endothelium. Smaller, communicating arterioles arise from the deep plexus and run perpendicularly to the epidermis to reach the upper dermis, where they form the superficial vascular plexus, which also runs parallel to the epidermis. The arterioles here are very small (diameter, 10 µm); their walls only have a single muscle layer and the internal elastic lamina is absent. The superficial plexus gives rise to capillaries that supply blood to the papillae; the blood then drains through the post-capillary venules into larger venules, which follow a path that is parallel but inverse to the afferent vessels. This entire structure forms a 3-dimensional network that acts as a single unit. Nonetheless, the clinical repercussions of vascular lesions vary according to the characteristics of the affected vessels and their location within the layers of the skin.

While many people who temporarily have this condition recover fully without another bout, or only have expression of the condition when exposed to cold, others may have a much harder time due to underlying causal factors. Unfortunately, treating these factors doesn't necessarily get rid of mottled skin appearance. Nevertheless, presence of this skin condition is well worth exploring because the conditions that can cause it may very well need treatment and should not be ignored. If people note anything but the most transient appearance of dermatopathia pigmentosa reticularis, and particularly if they notice it recurring, they need to discuss this with their PCP.

Symptoms:

Symptoms of dermatopathia pigmentosa reticularis include a mottled, or lace-like, appearance of reddish blue areas on the skin, numbness and tingling. The mottling is more apparent on the thighs and forearms, and sometimes the lower abdomen. It is more pronounced in cold weather.

Diagnosis:

Laboratory tests should include a complete blood count,coagulation studies, evaluation of kidney function, urinarysediment, proteinuria, antinuclear antibodies (ANA), complementlevels, antineutrophil cytoplasmic antibodies (ANCAs),cryoglobulin and cryofibrinogen levels, antiphospholipidantibodies, and hepatitis B &C serology. It is sometimesnecessary to conduct a more comprehensive study ofcoagulation factors such as protein C and S levels, factorV Leiden mutations, prothrombin G20210A genemutations, homocysteine levels, and the C677T mutationof the methylenetetrahydrofolate reductase gene.

Treatment:

There is no effective treatment for generalized dermatopathia pigmentosa reticularis in antiphospholipid syndrome. Skin lesions due to thrombosis can be treated with low doses of aspirin or anti-platelet therapy.

Systemic vasculitis should be treated with corticosteroids and immunosuppressant; doses and combinations will vary according to the clinical condition of the patient and the extent of organ involvement. Serious organ dysfunction requires the use of corticosteroids and cyclophosphamide pulse therapy. Combining low doses of corticosteroids with methotrexate or azathioprine is a good option for maintenance treatment, and Bactrim DS™ is recommended for maintenance in Wegener's granulomatosis to treat lung dysfunction.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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