What are the other Names for this Condition? (Also known as/Synonyms)

• Generalised Eruptive Keratoacanthomas
• Generalized Eruptive Keratoacanthomas of Grzybowski
• Grzybowski Syndrome

What is Generalized Eruptive Keratoacanthomas? (Definition/Background Information)

• Generalized Eruptive Keratoacanthomas describes a very uncommon and itchy disorder that is characterized by the presence of numerous firm bumps on several parts of the body such as the arms and legs, chest and back, and face. This disorder is also known as Grzybowski Syndrome
• In general, keratoacanthoma is a tumor of the hair follicles of low-grade malignancy that typically affects elderly men and women. Some consider it to be a variant of squamous cell carcinoma (SCC) of skin
• However, Generalized Eruptive Keratoacanthoma is a non-malignant condition that does not present any systemic signs and symptoms. Nevertheless, the condition responds poorly to treatment and the lesions may persist for a long period

Who gets Generalized Eruptive Keratoacanthomas? (Age and Sex Distribution)

• Generalized Eruptive Keratoacanthomas are generally observed in middle-aged adults
• Both males and females are equally affected
• There is no racial or ethnic preference observed. However, the disorder is extremely rare and only about 30 cases have been recorded worldwide

What are the Risk Factors for Generalized Eruptive Keratoacanthomas? (Predisposing Factors)

• Currently, the risk factors for Generalized Eruptive Keratoacanthomas are not identified

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Generalized Eruptive Keratoacanthomas? (Etiology)

• The cause of development of Generalized Eruptive Keratoacanthomas is not well-understood
• Even though the lesions present similarities to viral warts, Generalized Eruptive Keratoacanthomas are not associated with any viral infection

What are the Signs and Symptoms of Generalized Eruptive Keratoacanthomas?

The signs and symptoms of Generalized Eruptive Keratoacanthomas may vary from one individual to another. The onset of these skin lesions is sudden; a gradual increase in numbers over several months is noted. These lesions are described as being keratoacanthoma-like.

The signs and symptoms may include:

• Presence of numerous benign lesions that are in the form of papules and nodules that cause severe itchiness
• These lesions/tumors are found on the trunk, limbs (arms, legs, fingers, etc.), and face
• Several lesions on the face may cause skin thickening, giving it a mask-like appearance
• In some, the nodules may grow to larger sizes with pointed tips. These may be present for a few weeks and are then known to recede and disappear
• Sites of minor trauma may present new nodules (Koebner phenomena)
• Inside the mouth, these may appear as tiny white bumps on the tongue, cheek insides, and lips
• Nodules on the larynx (voice box) may cause hoarseness; on the eyelids, it may cause droopy eyelids

How is Generalized Eruptive Keratoacanthomas Diagnosed?

A diagnosis of Generalized Eruptive Keratoacanthomas may involve the following exams and procedures:

• Physical examination of the individual and medical history evaluation
• Dermoscopy: Dermoscopy is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Skin biopsy: A skin tissue biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

A differential diagnosis is important to exclude skin disorders that present similar signs and symptoms. These may include:

• Ferguson-Smith disease - an important differential diagnosis that presents multiple self-healing squamous epitheliomas in children and young adults
• Squamous cell carcinoma of skin

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Generalized Eruptive Keratoacanthomas?

The possible complications due to Generalized Eruptive Keratoacanthomas include:

• Emotional stress due to cosmetic issues due to persistence of the nodules
• Anxiety and depression
• Rarely, some tumors may affect the internal organs
• Recurrence of the condition following treatment
• Poor response to treatment in many individuals
• Treatment complications

How is Generalized Eruptive Keratoacanthomas Treated?

The treatment measures available for Generalized Eruptive Keratoacanthomas include:

• Symptomatic therapy such as anti-itch medications
• Use of moisturizing creams for dry skin
• Use of topical and oral retinoids
• Administration of medications such as methotrexate and 5-fluorouracil

How can Generalized Eruptive Keratoacanthomas be Prevented?

Presently, it is not possible to prevent Generalized Eruptive Keratoacanthomas.

What is the Prognosis of Generalized Eruptive Keratoacanthomas? (Outcomes/Resolutions)

The prognosis of Generalized Eruptive Keratoacanthomas may vary from one individual to another:

• In many cases, the condition does not respond well to treatment
• In some individuals, the condition has resolved following adequate treatment
• Rarely, a spontaneous resolution of the condition is seen
• Without treatment, the condition is longstanding, although it does not affect the overall health of the affected individuals

Additional and Relevant Useful Information for Generalized Eruptive Keratoacanthomas:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/healthy-living/skin-disorders/