What to do if you have a red, itchy rash all over the body

Pityriasis Lichenoides Chronica is a puzzling skin condition characterized by itchy rashes all over the body. It is rarely asymptomatic so many patients tend to ignore it.

A week after I arrived from Japan, a patient from four months ago visited me at St. Luke’s Medical Center in QC. His problem started four months ago with a widespread, slightly itchy rash scattered all over his torso and arms and legs that was bloody red and elevated (three- to four-mm bumps). Some were flaky but the majority was just a smooth red.

My first impression was a hypersensitivity reaction, for which I gave him the usual anti-itch therapy, together with an oral anti-inflammatory.

The rashes disappeared rapidly only to come back after three weeks with a vengeance, meaning more rashes that were red, elevated and itchy. So I decided to repeat the course of his previous medicines but this time at a slightly higher dose and slightly longer duration. Again, it improved by 100 percent but unfortunately resurfaced again after several days. So I decided to get a small piece of skin for investigation.

At this time, the majority of the rashes appeared to be tiny dots with a yellowish substance in it, so I thought it was probably folliculitis. I then prescribed him with antibiotics, which improved the rash again by around 80 percent. 

The tests revealed that the patient DS had Pityriasis Lichenoides Chronica (PLC). PLC is a skin condition of unknown cause. It usually manifests with papules (bumps) that develop scales, and then the skin is rendered flaky. It may be acute or chronic. The acute form can be itchy and painful, while the chronic form is usually without such symptoms. It can affect any part of the body, but chiefly affects the chest, back, and in rare cases, the mouth and genitalia.

PLC is rarely asymptomatic, as it is alarming. It is characterized by eruptions of multiple lesions followed by the gradual involution and fading of the crop. And, given the lack of symptoms, many patients tend to ignore it and, given the disease’s natural history of spontaneous involution, their wishes tend to be fulfilled: it goes away. Thus, a history of rhythmic eruptions that flake is a key finding. In the acute form, it may remain for a period of few months and then spontaneously disappear. In the chronic state, it may return after a symptom-free period of many years.

The acute form can develop to become chronic and longstanding. The incidence and prevalence of PLC are still unclear. An estimate incidence was around one in 2,000 in the United States. It can occur in an individual of any age, race and geographic area. However, it does maintain a slight male predisposition and tends to present in late childhood and early adulthood. The peak prevalence has shown to be around the third decade.

One of the leading etiological hypotheses is that Pityriasis Lichenoides is a form of atypical immune response in individuals genetically susceptible to a foreign agent/agents. A variety of infectious pathogens have been linked to this disease, including HIV, varicella-zoster virus, EBV, CMV, parvovirus B19, adenovirus, staphylococcus, mycoplasma, toxoplasma, and hepatitis C. The presence of these infections can make one vulnerable to PLC; however, PLC by itself is non-infectious and therefore does not spread by direct physical contact from one individual to another.

The use of medications such as Tegafur, estrogen-progesterone, Astemizole, Kampo (Japanese herbal medicine), radio-contrast iodine, HMG-CoA reductase inhibitors, and measles vaccine have also been associated with Pityriasis Lichenoides.

The pathophysiology of PLC is unknown.  It may represent a benign and abnormal immune response to an infection or medication or development toward a cutaneous T-cell neoplastic process. One study demonstrated the production of nitric oxide in the skin lesions of Pityriasis Lichenoides, which may play a role in pathogenesis.

Furthermore, it may be regarded as a para-neoplastic sign due to its occasional association with indolent lymphoma-like conditions such as lymphomatoid papulosis or parakeratosis variegate, as well as cutaneous lymphomas. It has likewise been associated with multiple autoimmune conditions, including rheumatoid arthritis, hypothyroidism, idiopathic thrombocytopenic purpura, and pernicious anemia.

Several reports described Pityriasis Lichenoides in pregnant woman is likely due to the immune modification that occurs during pregnancy. Koebner’s phenomenon has also been noted in Pityriasis Lichenoides, therefore, minimizing trauma and scratching of the skin is important with therapy.

Pityriasis Lichenoides has also been associated with periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome.

In difficult cases of Pityriasis Lichenoides, there is no treatment assurance. In some individuals, the use of oral antimicrobials and phototherapy may be beneficial. But wait; do not lose hope. Recent developments listed new oral medications as useful for the suppression of these rashes, some leading to long-term remission, and that is the best news ever. The prognosis of mild conditions is typically excellent.  When the skin lesions heal, it may result in scar formation causing cosmetic issues.  The longstanding lesions can cause self-image issues, emotional stress, and psychological trauma in some individuals leading to depression.

Because of the fatal or malignant features of some forms of Pityriasis Lichenoides (FUMHD or Febrile Ulceronecrotic Mucha Habermann disease and atypical transitions into skin lymphoma, a cancer that begins with blood cells called lymphocytes), early recognition and timely management are essential.  Likewise, lengthy periodic observation and repeated skin biopsies, particularly in patients in patients with long-term intermittent disease and marked alterations of disease, are advised to detect any impending malignant conversions.

In the meantime, we hope that future research will bring more understanding to this puzzling skin condition, and that insight into the origins of the disorder will provide explanations, designations, and appropriate management strategies.

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